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1 Child Neurology Quality Measurement Set Approved by the Child Neurology Quality Measurement Work Group on December 15, 2016. Approved by the AAN Quality and Safety Subcommittee on December 22, 2016. Approved by the AAN Practice Committee on December 28, 2016. Approved by th e American Academy of Neurology Institute Board of Directors on January 3, 2017. Appr oved by the Child Neurology Society Board of Directors on January 9, 2017. 1

2 Disclaimer and related data specifications developed by the American Performance Measures (Measures) are intended to facilitate quality improvement activities Academy of Neurology Institute (AANI) by providers. AANI Measures: 1) are not clini cal guidelines and do not establ ish a standard of medical care, and have not been tested for all ations; 2) are not continually updated and may potential applic not reflect the most recent information; and 3) are subject to review and may be revised or rescinded at any time by the AANI. The measures, while copyrigh ted, can be reproduced and distributed, without modificat ion, for noncommercial purposes ( e.g., use by health care providers in connection with their practices ); they must not be altered w ithout prior written approval from the AANI. Commercial use is defined as the sale, license, or di stribution of the measures for on of the measures into a produc t or service that is sold, licensed, commercial gain, or incorporati or distributed for com ures require a license agreement mercial gain. Commercial uses of the meas between the user and the AAN. Ne ither the AANI nor its members are responsible for any use of the measures. AANI Measures and related data spe cifications do not mandate an y particular cours e of medical care and are not intended to subs titute for the independent pro fessional judgment of the treating provider, as the information does not account for individual va riation among patients. In all cases, the selected course of r in the context action should be considered by th e treating provide of treating the individual patien t. Use of the information is v oluntary. AANI provide this , and make no warranty, expresse information on an “as is” basis d or implied, regarding the specifically disclaim any warrant ies of merchan tability or fitn ess for a information. AANI assumes no responsibility for a ny injury or damage to persons particular use or purpose. AANI or property arising out of or rel ated to any use of this inform ation or for any errors or omissions. ©2017 American Academy of Neurol ogy Institute. All rights reser ved. Limited proprietary coding is c ations for convenience. Users of ontained in the measure specific the proprietary coding sets shoul d obtain all necessary license s from the owners of these code sets. The AAN and its members disclaim all liability for use or accuracy of any Current Procedural Terminology (CPT®) or ot her coding contained in the specifications. ICD-10 copyright 2012 International Heal th Terminology Standards Devel opment Organization CPT ® is a registered trademar k of the American Medical Associa tion and is copyright 2017. CPT® codes contained in the Measur e specifications are copyrigh t 2004-2016 American Medical Association. 2

3 Table of Contents Work Group Participants ... ...4 ... ... Invited Organizations ... ...4 Improving Outcomes for Patients i n Pediatric Neurology Care ... ...5 ... Topic Importance ... ...6 Clinical Evidence Base ... ... ...7 Additional Child Neu rology Measures ... ...8 Work Group Recommendations ... ...10 Definitions and Abbreviations ... ...11 ... Desired Outcomes ... ... ...11 Intended Care Audience, Settings , and Patient Populations ... ...13 Other Potential Measures ... ... ...14 Measure Harmonization ... ... ...14 Technical Specifications Overview ... ... 15 ... Measure Exceptions ... ...15 Public Comment Feedback ... ...15 ... Testing and Implementation of the Measurement Set ... ...16 Child Neurology Measures ... ... ...17 First line treatment for Infantile Spasms ... ...17 hildren with epilepsy ... ...21 Rescue seizure therapy for c ...25 Time to third line therapy for RCSE ... Neuropsychological/neurode velopmental screening ... ...29 Screening for co-morbid conditions of TD or TS ... ...33 Management of co-morbid s ymptoms of TD or TS ... ...36 Behavioral therapy for chronic TD or TS ... ...39 Transition from pediatric ne urology to adult neurology ... ...42 Psychological interventions for chronic headache ... ...46 BoNT-A for spasticity and dystonia ... ...50 Genetic testing for GDD ... ...53 Contact Information ... ... ...57 References ... ... ...57 3

4 Work Group Participants Chairs Jeffrey Buchhalter, MD, FAA N (Child Neurology Society) n Academy of Neurology) Anup Patel, MD (America Work Group Members American Physical Therapy Association American Academy of Neurology Lynn Jeffries, PT, DPT, PhD, PCS Karen Ballaban-Gil, MD Anne Berg, PhD Child Neurology Society Daniel Fain, MD, FAAN Tim Feyma, MD Cynthia Keator, MD Kiran Maski, MD Sanjeev Kothare, MD Zachary Grinspan, MD, MS Gogi Kumar, MD Migvis Monduy, MD Epilepsy Foundation Diego Morita, MD Lisa Meunier American Academy of Pediatrics Independent Zachary Grinspan, MD, MS Lori Billinghurst, MD, MSc, FRCPC Ann Yeh, MD American Association of Neuroscience Nurses Erin Fecske, MSN, RN, CNRN, CPNP Facilitators David Michelson, MD (American Academy American Academy of Physical Medicine of Neurology) and Rehabilitation M. Cristina Victor io, MD (American Amy Houtrow, MD, PhD, MPH Academy of Neurology) American Epilepsy Society Staff Kevin Chapman, MD Amy Bennett, JD Gina Gjorvad American Headache Society Erin Lee Christina Szperka, MD Becky Schierman, MPH Invited Organizations The following organizations were clined: American College of invited to participate, but de Emergency Physicians, Tourette A ssociation of America, Muscular Dystrophy Association, American Psychological Associatio n, American Psychiatric Associ ation, American Academy for Cerebral Palsy and Developmental Medicine, American Occupationa l Therapy Association, American College of Physicians 4

5 Improving Outcomes for Patients Purpose of Measures ology Society (CNS) formed a In 2016, the American Academy o f Neurology (AAN) and Child Neur elines, current evi Child Neurology Work Group (Work Group) to review existing guid dence, and gaps in care in order to develop a measur ement set for pediatric neurol ogy that promotes quality improvement and drives better outcomes for neurologically-ill children. The AAN and CNS developed these elief that specialists should play a quality measures based on the b erformance improvement and could major role in selecting and creating measures that will drive p CNS formed the Work Group with possibly be used in future accountability programs. The AAN and representatives from professional associations and patient advo cacy organizations to ensure any measures developed include input from a ll members of the healthcare team . All members of the Work Group were required to disclose financial relationships with industry and other entities to avoid actual, potential, or perceived conflicts of interest. Quality Improvement a ness nd Caring for Children with Neurologic Ill Although the practice of Child N eurology has become significant ly more complicated in recent years due to the sheer amount of new information and the ever increasing amount of administrative responsibilities into the patient-family interac - Neurology’s commitment to provide the tion, one thing remains constant optimal care for each patient. Confronted with the reality of limited Class 1 evidence to guide the vast majority of our clinical decisions, it seems logical to fall ba ck onto individual knowledge and experience with only the best intent in mind. However, in doing so, we wi ll not achieve the advancement of care that we all so desire. An alternative approach is to engage in the process of Quality Improvement (QI) that has been so successful in reduci ng morbidity and mortality in t he domains of cardiology, hypertension and stroke. At the core of QI methodology is the process of rev iewing what is known about a es and then altering the plan to achieve problem/disorder, formulating a plan for care, measuring outcom nes are formulated based upon best improved outcomes in an iterative manner. Specifically, guideli evidence and consensus and the results determined by prospectiv ely identified, desired outcomes, i.e. quality metrics. The metrics can be categorized into those whi ch reflect processes (e. g. how often did a e clinical outcomes (e.g. reduction in patient receive a recommended treatment) and those that indicat seizures). m fee-for-service to ‘value-based ’ reimbursement, quality metrics have As medicine has moved away fro become the ‘currency’ by which incumbent upon the Child Neurology we will be judged. Thus, it is community to create metrics that have meaning for our patients and families to avoid measures being imposed by those external to car e delivery. The metrics establ ished by this work group are a first effort in that direction. The topics were chosen based upon clinical imp ortance as well as an existing evidence base that allowed recommendations. The quality metrics propose d are intended to be reasonable to achieve and have high face validit y (i.e. agreement that the me tric is worthwhile). Undoubtedly there will be changes in the metrics with u se, but these can only be disco vered with application in a clinical environment. The AAN and CNS st rongly encourage constructive f eedback that will not only lead to better care for our patients, but also reflect the value we pro vide to patients and families. AAN Measure Development Process Once a topic has been approved , AAN staff seek out a leadership team that consists of two co-chairs who are content experts and two facilitators from the Quality and S afety Subcommittee. Following approval of s through the AAN process. AAN then this topic, the AAN partnered with CNS to develop these measure 5

6 commissions a multidisciplinary work group to evaluate availabl e evidence and literature around the ship team and Work Group create draft topics defined by the leadership team and AAN staff. The leader measure concepts for discussion. An in-person meeting is held t o discuss and refine the measure concepts. asure. The Work Group votes to approve or not approve each proposed me Following the meeting, measures are further refined and posted for public comment. The leadership team reviews the public comments and refines the measures accordingl y. After the measures have been finalized the Work Group votes to approve or not approve the wh ole measure set. If approved by the Work Group, AAN staff facilitate ing group drafts a manuscript which is internal AAN approvals. A writ an executive summary of the measurement set that is submitted f Neurology . AAN or publication in measures undergo a maintenance review every three years. Topic Importance Child neurology does not focus on one disease alone, but encomp asses signs, symptoms and conditions spanning the neurological spectrum in the inpatient and outpati ent settings. After consideration of a variety of conditions and measure s, the leadership team determi ned this measurement set would focus on ilepsy, tic disorder and Toure tte syndrome, headache, cerebral palsy, infantile spasms, seizures and ep global developmental delay , and transitions in care. Headache and Migraine Headache, in particular migrain e, is a common pediatric problem worldwide. Approximately 8% of children and adolescents are pr one to develop it over at least a 3-month period and it can become a 1 chronic and disabling disorder. The prevalence of migraine for those under 20 years of age is 7.7% with 1 the prevalence in females reported at 9.7% and 6.0% in males. An analysis estimated that 60% of 1 children are at risk for headach e with females being affected a t a greater rate (67%) than males (58%). as “pain localized to the hea Chronic daily headache is defined d occurring 15 or more days per month for 2 more than 3 months”. graine and other While there are many pharmacol ogic treatments available for mi primary headache disorders, the zed controlled trials to provide guidance re are limited pediatric randomi to an effective and safe medica tion to use as a preventative tr eatment. Non-pharmacologic treatment of egral part of treatment plans. erventions have become an int headache such as psychological int Tourette syndrome and Tic disorder disorder characterized by repetitive, Tic disorder, including Tourette syndrome (TS), is a neurologic 3,4 stereotyped involuntary movements and vocalizations. The symptoms typically start in childhood between the ages of 3 and 9 year s of age. TS and other tic diso rders can last a lifetime or improve with 4 age. ed to affect between 0.3 and 1% of the population with males being The prevalence of TS is estimat 3,5 affected at a higher rate (1.06%) than females (0.25%). TS and tic disorders are known to have multiple sorder (ADHD), obsessive compulsive ntion deficit hyperactivity di co-morbid conditions such as atte disorder (OCD), oppositional defiance disorder (ODD), and mood disorders including depression and anxiety. Infantile Spasms psy condition that primarily a ffects those in infancy. The prevalence of IS Infantile Spasms (IS) is an epile 6 6 ranges from 2 to 3.5 per 10,000 live births. noted is hypsarrhythmia. A common EEG abnormality Neurodevelopmental regression of ten can accompany infantile spa sms. Poor developmental outcomes are 7 often associated with this condition when the spasms fail to re spond to treatment. First line treatments dnisolone, and vigabatrin. include ACTH, high dose pre Status Epilepticus 6

7 Status epilepticus (SE) refers to the condition of seizures whi ch exceed a specific time threshold and/or g to his or her baseline level of alertness and cognition. It is estimated recur without the person returnin 8 that 50,000 to 150,000 people in the United States will have an episode of SE each year. SE can be convulsive (i.e. seizures with e eatures) or non-convulsive (e.g. absence ither or both tonic or clonic f status). As convulsive SE is the type most associated with morb idity and mortality, it has been the focus ildren uses a duration >5 minutes to of treatment guidelines. The most recent guideline involving ch 8 therapeutic cascade. initiate the time-driven Cerebral Palsy Cerebral Palsy (CP) is a diagnos nd significant impairment of voluntary motor is that refers to a life-long a 9 activity due to a brain injury that occurs prior to early child hood. The CDC reports that 3.3 per 1,000 live 9 births are diagnosed with CP. CP can manifest with different d , egrees of spasticity, dystonia a, incoordination, and hypotonia and can affect the limbs in a diplegic, choreoathetoid movements, ataxi hemiplegic, or tetraplegic pattern. Medical treatments for spas ticity and dystonia include oral medications such as baclofen and oral tizan idine, benzodiazepines, dantrole ne, and trihexyphenidyl. Invasive treatments include surgical placement of an intrathecal baclofe n pump or deep brain stimulator, surgical selective dorsal rhizotomy, and intramuscular injections of bot ulinum toxin. Supportive measures include the use of orthoses and adaptive eurorehabilitative phys ical, occupational, equipment and involvement in n 10 and speech therapies. Global Developmental Delay Global Developmental Delay (GDD) is a diagnosis given to childr en showing significant delay in acquiring early childhood developm ental milestones in more than two of these domains: motor, speech aptive. It is not synonymous and language, cognitive, social ad with intellectual disability, which can be difficult to diagnose in children younger than age 5, but sugge sts a concern for long-term cognitive ability d 3% of children meet criteria for GDD is estimated that between 1% an and functional independence. It 11 with autism considered separately. It is estimated that 40% of otherwise unexplained GDD can best be 12, 13 explained by genetic and metabolic ironmental factors. disorders rather than by env Genetic testing ecific diagnosis has a number of benefits for patients and families and can result in that establishes a sp 14 specific changes in management. Transition to Adult Neurology s remains a challenge for a ll patients with chronic illness in need of The transition of adolescent patient ss the gaps that currently exist in this adult care. A formal transition process is recommended to addre 15 process. Neurology conditions often continue into adulthood and should follow a similar treatment format. Clinical Evidence Base The co-chairs and facilitators, guided by a medical librarian, conducted a comprehensive search to asures, and consensus recommen dations in the National Guidelines identify published guidelines, me Clearinghouse, the National Quality Measures Clearinghouse, Pub Med, MEDLINE, EMBASE, and the Cochrane Library. The Work Group reviewed existing literature a nd consulted the following clinical practice guidelines published, which included:  Evidence-based guideline update: Medical treatment of infantile spasms  Summary of recommendations for the management of infantile seiz ures: Task Force for the ILAE Commission of Pediatrics anagement using GRADE  An evidence-based guideline for pediatric prehospital seizure m methodology 7

8  Guidelines on the management of prolonged acute convulsive seiz ures in out-of-hospital settings: A gap to be filled Benzodiazepine use for emergency treatment of seizures: A revie  w. cus Guidelines for the evaluation and management of status epilepti   Evidence-based Guideline: Treatme nt of Convulsive Status Epilep ticus in Children and Adults  f the ILAE Task Force on A definition and classification of status epilepticus –Report o Classification of Status Epilepticus Summary of recommendation for the management of infantile seizu res: Task Force for the ILAE  Commission on Pediatrics European clinical guidelines for Tourette syndrome and other ti c disorders. Part I: assessment  Practice Parameter for the Assessment and Treatment of Children and Adolescents with Tic  Disorders  pulsive skin picking, chronic t ic disorders and trichotillomania Evidence-based assessment of com in children  European clinical guidelines for Tourette syndrome and other ti c disorders. Part III: behavioural interventions. Canadian guidelines for the evid ence-based treatment of tic dis orders: Behavioural therapy, deep  anial magnetic stimulation brain stimulation, and transcr Clinical report – Supporting the health care transition from ad olescence to adulthood in the  medical home Psychological therapies for the management of chronic and recur  rent pain in children and adolescents.  ogical interventions for migr aine effective in children and Clinical Answers: Are nonpharmacol adolescents?  Practice Parameter: Pharmacologic treatment of spasticity in ch ildren and adolescents with cerebral palsy (an evidence-based review) Spasticity in children and young people with non-progressive br ain disorders  Evidence report: Genetic and meta bolic testing on children with global developmental delay   microarray is the first-tier c linical diagnostic test for Consensus statement: Chromosomal individuals with developmental disabilities or congenital anoma lies  Unexplained developmental delay/learning disability: guidelines for best practice protocol for first line assessment and genetic/metabolic/radiological invest igations Additional Child Neurology Measures eatment needed for the clinically diverse No one measurement set is able to capture all the aspects of tr rology. This measurement set is focused on measuring the quality of care patient population of child neu itions or diseases, and does not address the whole scope of each condition provided for a variety of cond or disease, nor all of pediatric neurology. In addition to this measurement set, the AAN has additional mea sures that are applicable to the pediatric population: Epilepsy Measurement Set (2014)  o Percent of all visits for patients with a diagnosis of epilepsy where the seizure frequency of each seizure type was documented o Percent of patients with a dia gnosis of epilepsy with seizure f requency > 0 for whom an frequency was offered or discuss intervention to reduce seizure ed with the patient or caregiver. 8

9 o with seizure type and Percent of all visits for patients with a diagnosis of epilepsy umented OR testing ordered to epilepsy etiology or syndrome doc determine etiology of epilepsy, seizure type, or epilepsy syndrome o Percent of all patients with a d iagnosis of epilepsy with activ e anti-seizure therapy side effects for whom an intervention was discussed Percent of all patients with a d iagnosis of epilepsy, or their caregivers, who were o provided with personalized safety issue and epilepsy education at least once annually. where the patient was o Percent of all visits for patients with a diagnosis of epilepsy screened for psychiatric or behavioral disorders. o diagnosed with epilepsy All female patients of childbearing potential (12-44 years old) who were counseled or referred for counseling for how epilepsy and its treatment may affect contraception OR pregnancy at least once a year o Percent of all patients with a d iagnosis of treatment resistant (intractable) epilepsy who were referred for consultation to a comprehensive epilepsy cent er for additional management of epilepsy.  Headache Measurement Set (2014) Percentage of patients age 12 y o of migraine who were ears and older with a diagnosis prescribed a guideline recommended medication for acute migrain e attacks within the 12 month measurement period. o Percentage of patients aged 12 years and older diagnosed with p rimary headache disorder and taking opioid containing med pioid containing ication who were assessed for o medication overuse within the 12-month measurement period and t reated or referred for treatment if identified as overusing opioid containing medicati on. o Percentage of patients diagnosed with a primary headache disord er, who are actively taking an acute headache medica tion and experiencing headaches ≥15 days per month for 3 months, who were assessed for medication overuse headache (MO H). o he (MOH) within the past 3 Percentage of patients diagnosed with medication overuse headac ho had a medication overuse months or who screened positive for possible MOH (measure 6a) w plan of care created or who were referred for this purpose. Percentage of patients diagnosed with primary headache and who o have a normal neurological examination for whom advanced brain imaging (CTA, CT, MRA or MR I) was NOT ordered. Percentage of patients with a dia gnosis of primary headache dis order whose health related quality o sessed with a tool(s) during at least two of life (HRQoL) was as visits during the 12 month measurement period AND whose h re stayed the same or improved. ealth related quality of life sco gnosis of migrain e headache or o Percentage of patients age 6 years old and older who have a dia ed disability days during the cervicogenic headache and for whom the number of headache-relat past 3 months is documented in the medical record. o All patients diagnosed with migraine headache or cervicogenic h eadache who had a headache management plan of care developed or reviewed at least once dur ing the 12 month measurement period.  Multiple Sclerosis Measurement Set (2014) ved a new diagnosis of multiple sclerosis in the past 12 o Percentage of patients who recei months who fulfilled international criteria. o Percentage of patients with MS who had an MRI with and without gadolinium within 24 months of diagnosis compa red with a baseline MRI. o Percentage of patients with MS who have a MS disability scale s core documented in the medical record in the past 12 months. Percentage of patients with MS w ho were screened for fall risk in past 12 months. o o Percentage of patients with MS w ho have had a bladder infection in past 12 months. o Percentage of patients with MS w ho are counseled on the benefit s of exercise and appropriate physical activity for patients with MS in the past 12 months. 9

10 o s results are maintained Percentage of patients with MS whose most recent score indicate rating instrument for patien ts with MS in past 12 or improved on a validated fatigue months. Percentage of patients aged 12 y ears and older with MS who were o screened for clinical depression using an age appropr iate standardized depression scr eening tool at least once in past 12 months. Percentage of patients aged 12 y ears and older with MS whose mo o st recent score indicates results are maintained ression screening or improved on a validated dep instrument for patients with MS in past 12 months. o Percentage of patients with MS whose most recent score indicate s results are maintained or improved on an age appropria 12 months. te Quality of Life tool in past  Muscular Dystrophy Measurement Set (2013) o All patients diagnosed with Duche nne muscular dystrophy (DMD) p rescribed appropriate DMD disease modifying pharmaceutical therapy. o All patients diagnosed with a mu scular dystrophy (MD) for whom a MD multi- r the plan was updated at disciplinary care plan was developed, if not done previously, o least once annually. o All patients diagnosed with a mu scular dystrophy (MD) who had a pulmonary status evaluation ordered. o Patients diagnosed with a muscular dystrophy (MD) who had a car diac status evaluation ordered. All visits for patients with a diagnosis of a muscular dystroph y (MD) where the patient o had a scoliosis evaluation ordered. o All visits for patients diagnosed with a muscular dystrophy (MD ) where the patient was referred for physical, occupational, or speech/swallowing thera py. o All visits for patients diagnosed with muscular dystrophy (MD) where the patient’s nutritional status or growth t rajectories were monitored. o ) where the patient was All visits for patients diagnosed with a muscular dystrophy (MD validated and reliable rference with function using a queried about pain and pain inte instrument. All patients with a diagnosis of a muscular dystrophy(MD), or t o heir caregivers who were e care, or end-of-life counseled about advanced health care decision making, palliativ issues at least once annually. ommendations Work Group Rec 2016 Child Neurology Measurement Set Appropriate firs line treatmen t fo r Infantile Spasms t r children with epilepsy Rescue seizure therapy fo r r efractory convulsive status epilepticus (RCSE) Time to third line therapy fo Neurodevelopmental / neuropsychological screening in epilepsy Screening fo r r o r Tourette syndrome co-morbid conditions of tic disorde Managemen of co-morbid symptoms of tic disorde r o r Tourette syndrome t Behavioral therapy fo r tic disorde r o r Tourette syndrome Transition fro pediatric neurology to adul t neurology m Psychological interventions fo r chronic headache Botulinu m Toxin Serotype A (BoNT-A) fo r spasticity and dystonia global developmental delay (GDD) Genetic testing fo r 10

11 Definitions and Abbreviations The Work Group utilized the following definitions and abbreviat ions in the measurement set:  ACTH: Adrenocorticotropic hormone  ADHD: Attention Deficit Hyperactivity Disorder  BoNT-A: Botulinum Toxin Serotype A CBIT: Comprehensive Behavioral Treatment for Tics   CP: Cerebral Palsy CMA: Chromosomal Microarray   DSM: Diagnostic and Statistical Manual of Mental Disorders  ED: Emergency Department  EEG: Electroencephalogram  ERP: Exposure Response Prevention Therapy GDD: Global Developmental Delay   HCT: Health Care Transitions  HRT: Habit Reversal Training  IS: Infantile Spasms  LGS: Lennox-Gastaut Syndrome  OCD: Obsessive Compulsive Disorder  ODD: Oppositional Defiance Disorder  RCSE: Refractory Convulsive Status Epilepticus  SLD: Specific Learning Disability  TD: Tic Disorder  TS: Tourette Syndrome The AAN has a Quality Improvement Glossary, which provides more in depth explanations and is available at aan.com/practi ce/quality-measures/quality-resource s. Desired Outcomes This list represents the optimal outcomes for pediatric neurolo gy care facing diagnoses epilepsy, seizures, rs, headache, cerebral palsy, g lobal developmental delay, and care Tourette syndrome or tic disorde transitions. Additional informa eloped by the work group link to desired tion on how process measures dev outcomes is located below in the measure specifications. Treatment of infantile spasms  Rapid elimination of seizures  Elimination of hypsarrhythmia on EEG Decrease morbidities   Optimize developmental outcomes  Improve quality of life Pre-hospital rescue medication for seizures  Decrease morbidity and mortality  Decrease unnecessary h ealthcare utilization  Improve quality of life  Avoid unnecessary hospitalization Avoid emergency service utilization  11

12 Third line therapy for seizure cessation Increase the probability of  medication responsiveness  Decrease mortality  Decrease seizure  Decrease medication morbidities  Optimize neurological and cognitive outcomes  Improve quality of life Screening for neuropsychological testing  Increase utilization of testing  Increase developmental attainment Improve quality of life  Screening for co-morbid conditions of tics/Tourette’s Recognize and properly address common co-morbid conditions  Early diagnosis   Improve quality of life Maximize general function  Referral for co-morbid conditions Decrease tic burden   Improve co-morbid conditions Early diagnosis and treatment   Improve quality of life  Maximize general function Behavioral therapy for tics/Tourette’s Decrease tic burden  Non-pharmacologic treatment option   Increase utilization of behavioral therapy  Improve quality of life  Maximize general function Transition to adult neurology  Improve transitions to outpa tient and adult providers  Increase patient/caregiver satisfaction  Increase patient/caregiver knowledge of their own diagnosis  and follow-up Increase patient/caregiver understanding of the management plan Increase patient/caregiver engage  cess ment in treatment decision pro  Address all patient/caregiver needs and engage patients on a pe rsonal level Psychological intervention for chronic headache Reduction of pain and disability   Reduce the occurrence of adverse effects associated with medica tion overuse  Improve quality of life Maximize general function  Evaluation for BoNT-A for CP 12

13  Reduction of spasticity and dystonia Reduction of pain and disability  Reduce the need for surgical correction of contractures  Increase caregiver knowledge of treatment options  Maximize general function  Genetic testing for GDD  Provide patients with a definitive etiologic diagnosis  Reduce unnecessary and invasive testing nce for complications  Improve appropriate surveilla  s Increase patient access to treatments and experimental protocol  Improve patient access to support services and networks  Improve caregiver knowledge of prognosis  Improve family understanding of recurrence risk  Reduce caregiver uncertainty and anxiety Intended Care Audience, Settin gs, and Patient Populations The AAN encourages the use of th other health care professionals, ese measures by physicians and practices, and health care systems, where appropriate, to achie ve improved performance. These measures are intended as steps that providers, practices, and systems ca n take towards optimized clinical outcomes for children with n eurological illness. Applicable Care Settings 2016 Child Neurology Measurement Set Outpatient Inpatient R esidential Emergency Departmen t X X Appropriate first line treatment for infantile spasms X X X X py for children with Rescue seizure thera y epileps X X erapy for refractory Time to third line th convulsive status epilepticus (RCSE) X Neuropsychological/neurodevelopmental screenin y g in epileps X Screening for co-morbid conditions of tic y Tourette s r o r ndrome disorde X Management of co-morbid symptoms of tic Tourette s y ndrome r o r disorde X Behavioral therapy for tic disorder or ndrome y Tourette s X Transition from pediat ric neurology to adult gy neurolo X X Psychological interventions for chronic headache X X Botulinum Toxin Serotype A (BoNT-A) for spasticit a d y stoni an y d 13

14 X Genetic testing for global developmental dela (GDD) y Other Potential Measures The measures developed are a r esult of a consensus process. Wor k Group members are given an es in advance of the in-person meeting where all measures are reviewed opportunity to submit new measur and edited individually. After each measure has each individual on the work group votes been discussed, stain from voting on each measure to approve, not approve, or ab . The Work Group discussed potential measures for development, prior t o and during the meeting and t he Work Group voted to not approve:  Education of patient and family o n the diagnosis of Tic Disorde r or Tourette Syndrome,  ffects among patients with Tic D isorder or Tourette Syndrome Assessment of medication side e Treated with Anti-Psychotic Drugs, Patients with epilepsy receivi ng baseline neuropsychological te sting,   Developmental and behavioral screening for pre-school-aged chil dren and infants with epilepsy,  Follow up visit for headache prophylactic,  Migraine prophylactic refills and follow-up,  Follow-up visit for patients taking ADHD medication,  ADHD medication adherence and follow-up visits  s Disease modifying treatment for children with multiple sclerosi Trial dose of pyridoxine for ne  onates with ongoing seizures Appropriate outcome for infantile spasms  The Work Group felt these concepts were not ready for developme nt at this time due to lack of evidence. oncepts be revisited when this The Work Group recommends these c measurement set is updated in 3 years. Measure Harmonization Many existing AAN quality measure d by others, apply to the child s, as well as measure develope neurology patient population. T he Work Group reviewed existing measures on the topics included in this measurement set. Efforts were mad e to reduce duplicative measur es when possible. Time to third line therapy for seizure cessati on for Refractory Convulsive Status Epilepticus asure set on patients with generalized One measure exists in the AAN’s Inpatient and Emergency Care me convulsive status epilepticus w ho are treated with a non-benzod iazepine antiepileptic/anti-seizure medication following the administration of a benzodiazepine. Th e measure applies to patients aged 16 years of age and older. This ex isting measure harmonizes with t he child neurology measure which captures patients younger than 16 years of age. uropsychological or neurodevelopmental deficits Patients with epilepsy receiving screening for ne This measure does harmonize with an existing adult measure on s creening for psychological co- morbidities as they would be ide ntified on this testing. Howev er, it expands the screening to including other disorders that are commonly seen in children that do not necessarily apply to the adult epilepsy population. Proper transition of pediatric neurology patients to adult neurology care 14

15 This measure harmonizes across all neurology disease states and covers both child and adult neurology ioning care, nothing specific exists on the providers. Although several existing measures deal with transit p and importance in this area, the work transfer of care from child to adult neurology. Based on the ga group felt a separate measure was warranted for neurological pa tients. Technical Specifications Overview cal specifications for measures that include data from: The Work Group developed techni Electronic Health Record (EHR) Data   Electronic Administrative Data (Claims)  Registry Administrative claims specifica tions are not provided for measu res given the AMA’s decision to discontinue the maintenance of C PT II codes. The AAN is in the process of creating code value sets and es with EHRs, when possible. A listing of the logic required for electronic capture of the quality measur the quality data model elements, c ode value sets, and measure l ogic (through the CMS Measure Authoring Tool) for each of the measures will be made available at a later date. These technical specifications will be updated as warranted. The measurement set includes me asures that require the use of v alidated screening tools. The Work Group discussed and determined tha t multiple tools should be of fered to allow providers to determine which tool best meets their i ndividual practice needs. Tools ma y be subject to copyright and require licensing fees. Measure Exceptions A denominator exclusion is a f actor supported by the clinical e vidence that removes a patient from inclusion in the measure popula tion. For example, if the denomi nator indicates the measure is for all ge, a patient who is 19 years o patients aged 0 to 18 years of a f age is excluded. A denominator exception is a c ondition that should remove the p atient, procedure or unit of measurement from the denominator only if the numerator criteria are not met . The AAN includes three possible types of should not be included in a measure denominator: medical (e.g., exceptions for reasons why a patient ., declination or religious beli ef), or system (e.g., resource limitation) contraindication), patient (e.g reasons. For each measure, there must be a clear rationale to p ermit an exception for a medical, patient, or system reason. The Work Group provided explicit exceptions when applicable for ease of use in eMeasure development. Public Comment Feedback The draft measurement set was put on the AAN website for public comment from August 30 through September 30. Specific segments of the AAN membership w ere notified of the zations were contacted opportunity to review and comment. Additionally, over 20 organi regarding the opportunity to provi de comment. Based on these co mments the following major changes were made:  General: o Greater consistency of wording and formatting across the measur e set o Grammatical errors were identified and fixed o Exceptions incorporated into denom inator statements across the measure set Infantile Spasms:  15

16 o Additional exceptions added  Abortive Seizure Therapy: o Added language regarding non-FDA approved treatments Therapy for Refractory Convul sive Status Epilepticus  Screening for neurodevelopmental or neuropsychological deficits in epilepsy  o Changed verbiage of the type of testing to more accurately refl ect DSM V language o Added list of questionnair e-based screening tools  Screening for co-morbid conditi ons of Tic Disorder and Tourette Syndrome o Changed language from “screened” to “queried”  Management of Co-Morbid Symptoms of Tic Disorder or Tourette Sy ndrome o Added exception  Counseling or Referral for Beha vioral Therapy for Tic Disorder or Tourette Syndrome o Added exception  Transitions of care: o itions of Care measure to prov ide more guidance on Significant revision to the Trans how to conduct a transition  Psychological Interventi ons for Chronic Headache None o  Treatment for Spasticity and Dystonia o Took out references to CP  Genetic Testing for Global Developmental Delay o Refined denominator verbiage of the Measurement Set Testing and Implementation The measures in this set are b eing made available without any p rior testing. The AAN encourages testing of this measurement set for feasi bility and reliability by orga nizations or individuals positioned to do so. Select measures will be beta test ed once the set has been relea sed, prior to submission to the National Quality Forum for possible endorsement. 16

17 First line treatment for infantile spasms Measure Description t t fo r infantile spasms (IS) line treatmen Percentage of patients receiving appropriate firs Measure Components Patients who received any guideline recommended first line ther Numerator apy* as initial Statement after initial, treatment for IS as soon as diagnosed, but no later than 1 week confirmed diagnosis** *Guideline Recommended Treatments:  Adrenocorticotropic hormone (ACTH)  High dose prednisolone  vigabatrin (VGB) **Diagnosis is usually defined as lexion or seizure marked by momentary f with onset occurring extension of the neck, trunk, extremities, or any combination, in first year of life with or w thmia. ithout the presence of hypsarrhy Recommended treatments subject to change if approved treatments added after measure approval. Denominator All patients aged 2 weeks to 24 months diagnosed with IS Statement Denominator  Medical provider identified all 3 treatments are contraindicate d Exceptions  Caregiver refuses all 3 treatments  these Patient participating in a research trial that precludes use of medications as first line therapy.  Presence of an inborn error of met abolism disorder (may include , but not limited to: (1) disorders of am ino acid metabolism (phenylketon uria, yrodoxal-5- dihydropteridine reductase deficiency, pyridoxine deficiency, p phosphatase deficiency, folinic acid deficiency), (2) organic a cidurias (D- c aciduria, propionic acidemia, maple syrup glyceric aciduria, methylmaloni urine disease), (3) disorders of fatty acid oxidation (short-ch ain acyl- coenzyme A dehydrogenase enzyme deficiency), where alternative therapy 1 is recommended and/or more appropriate.  t line treatment. Resective epilepsy surgery is recommended as firs Exception reatment for their Patients that are surgical candidates may not need medication t Justification infantile spasms. Parent/caregivers may refuse first line treat ments. Provider may have good evidence that all three treatments are contraindicate d. There may be times when the medical provider deems the risks of these three treatments to outweigh the benefits as first line therapy. Should the opportu nity arise in the future for a trial, patients may need to be excluded from these treatm ents. Patients with inborn errors of metabolism can h ave a treatment to correct the error of metabolism and reverse symptomology includi ng the infantile spasms. Theref ore, first line infantile spasms treatments may no e necessary. b t 17

18 Supporting d supporting ed verbatim from the reference The following statements are quot Guideline & articles: Other  “The evidence is insufficient to recommend the use of prednisol one, References ACTH for dexamethasone, and methylprednisolone as being as effective as 2 short-term treatment of infantile spasms”  short-term treatment of infant ile “ACTH or VGB may be offered for 2 spasms. Evidence suggests that ACTH may be offered over VGB”  “Hormonal therapy (ACTH or predni solone) may be considered for use in preference to VGB in infants with to possibly cryptogenic infantile spasms, 2 improve developmental outcomes”  “A shorter lag time to treatment of infantile spasms with eithe r hormonal therapy or VGB may be consider ed to improve long-term cognitive 2 outcomes”  “VGB is most effective in the first line treatment of infantile spasms when 3 is” used in children with normal development at the time of diagnos  “Children with infantile spasm w ho respond to VGB first are mor e likely to 3 undergo seizure resolution over time than those who failed VGB”  “The results show that high dose ACTH appears to be more effect ive than 4 prednisolone”  “...vigabatrin is most likely to be effective in the first line t reatment of infantile spasms, not related to tuberous sclerosis complex in children with 5 normal development at the time of diagnosis” “Lead time to treatment was 7 days or less in 11, 8-14 days in  16, 15 days to 1 month in 8, 1-2 months in 15, >2 months in 21 and not know n in 6. d with a Each month of reduction in age at onset of spasms was associate 3.1 [95% confidence interval (CI) 0.64-5.5, p = 0.03] decrease, and each increase in category of lead time duration associated with a 3.9 (95% 6 CI 7.3-0.4, p = 0.014) decrease in VABS, respectively “ 7 hort-term control of spasms”  “ACTH is preferable in the s 7 “Oral steroids are probably eff ective in the short-term control of spasms”   “Data are insufficient to comme nt on the optimal preparation, d osage, and 7 duration of treatment of steroids” “Vigabatrin is possible effec tive in the short-term control of spasms,  7 especially in the case of tuberous sclerosis complex”  “Treatment with ACTH/oral stero ids may result in better long-te rm neurodevelopmental outcome than treatment with vigabatrin in ch ildren 7 with epileptic spasms due to unknown etiologies” “A shorter interval from the onset of spasms to treatment initi ation may  opmental outcome, especially in cases improve the long-term neurodevel 7 where there is no identified etiology”  “The shorter the “lag time” (time from spasms onset to commence ment of 7 therapy) the better the developmental outcome”  “Hormone treatment controls spasm s better than does vigabatrin initially, but not at 12-14 months of age. B etter initial control of spasm s by hormone treatment in those with no identif ied underlying aetiology may lead to 8 improved developmental outcome” d fewer  “In particular, the poor respon se to nonstandard medications an 10 relapses with ACTH over oral steroids were noted” 18

19 Measure Importance chance for improved Relationship to Patients that receive first line therapy for IS have a greater Desired Outcome ased risk for developmental dela y and potentially clinical outcomes such as decre less chance of developing epilepsy such as Lennox-Gastau Syndrome (LGS). t Use of non-standard or evidence based treatment or treatment th at has been shown Opportunity for 9 Improvement to be ineffective for IS still occurs significantly. National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains ☐ Care Coordination ☐ Population/Public Health Efficient Use of Healthcare Resources ☐ ☒ Clinical Process/Effectiveness Harmonization N/A with Existing Measures Measure Designation Measure ☒ Quality improvement (Check Purpose ☒ Accountability all tha t apply) Type of Measure Process ☒ (Check all that Outcome ☐ apply) ☐ Structure Level of Individual Provider ☒ Measurement ☒ Practice (Check all that ☒ System apply) Care Setting ☒ Outpatient (Check all that Inpatient ☒ apply) Emergency Departments and Urgent Care ☐ Residential (i.e., nursing f acility, domiciliary, home care) ☐ Data Source ☒ Electronic health record (EHR) data (Check all that ☐ Administrative Data/Claims apply) ☒ Patient Medical Record ☒ Registry References 1. Gkampeta A, Pavlous E. Infantile Spasms (West Syndrome) in Chil dren With Inborn Errors y 2012; 27:1295-1301. iterature. Journal of Child Neurolog of Metabolism: A Review of the L 19

20 2. : Medical treatment of Go C, Mackay M, Weiss S, et al. Evidence-based guideline update logy 2012; 78:1974-80. infantile spasms. Neuro Jones K, Boyd J, Go C, et al. Vigabatrin in the first line trea tment of infantile spasms. 3. Epilepsy Currents 2015; 15:533-534. ile spasms post vigabatrin 4. Jones K, Go C. ACTH vs. prednisolone in the treatment of infant failure. Epilepsy Currents 2014; 14:447-448. t for infantile spasms not related 5. Jones K, Go C, Boyd J, et al. Vigabatrin as first-line treatmen to tuberous sclerosis complex. Pediatric Neurology 2015; 53:141 -145. O'Callaghan FJ, Lux AL, Darke K, E dwards SW, Hancock E, Johnson AL, Kennedy CR, 6. eatment and of age of onset Newton RW, Verity CM, Osborne JP. The effect of lead time to tr ears in infantile spasms: eviden on developmental outcome at 4 y ce from the United Kingdom Infantile Spasms Study. Epile psia. 2011 Jul; 52(7):1359-64 7. Wilmshurst J, Gaillard W, Vinayan KP, et al. Summary of recomme ndations for the management of infantile seizur E Commission of Pediatrics. es: Task Force Report for the ILA Epilepsia 2015; 56:1185-1197. 8. Lux AL, Edwards SW, Hancock E, et al. The United Kingdom Infant ile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developm ental and epilepsy outcomes to age 14 months: a multicenter randomized trial. Lanc et Neurol 2005; 4:712-7. 9. f infantile spasms: A Widjaja E, Go C, McCoy B, Snead O. Neurodevelopmental outcome o psy Research 2015; 109:155-162. review and meta-analysis. Epile Knupp K, Coryell J, Nickels KC, e t al. Response to treatment in a prospective national 10. nn Neurol 2016; 79:475-84. infantile spasms cohort. A Denominator ICD-10 Code (Eligible G40.82 Infantile spasms Population) AND CPT E/M Service Code 99221, 99222, 99223 Initial hospital care 30, 50, or 70 minutes, per day, for the evaluation and managem ent of a patient; 99231, 99232, 99233 Subsequent hospital care 15, 25, or 35 minutes, per day, for the evaluation and management of a patient 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, t; or 60 minutes for the evaluation and management of a new patien 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o of an established patien r r the evaluation and managemen t 40 minutes fo t 20

21 Rescue seizure therapy f or children with epilepsy Measure Description ved appropriate and correctly d osed rescue seizure therapy for children Percentage of patients who recei with epilepsy Measure Components Patients who receive or have received a prescription for an app Numerator ropriately dosed* Statement ospital^ setting. rescue seizure therapy (i.e., midazolam, diazepam) in the pre-h 1,2,3,4,5,6,7,8,9 *Appropriate dose recommendations:  Intranasal, buccal, or IM midazolam: all ages: 0.1 to 0.4 mg/kg/dose (maximum 10 mg)  Rectal Diazepam: 6 months – 5 years: 0.5 mg/kg/dose; 6-11 years: 0.3 mg/kg/dose; > 12 years: 0.2 mg/kg/dose (maximum 20 mg) de of the emergency department (ED) and hospital ^Pre-hospital setting means outsi (i.e. ambulance, home, school, etc.). Please note, currently n o FDA approved treatment for prolonged seizur es in the prehospital setting exi st and recommendations are fo r clinical standard and accepted practice use. Denominator Patients aged 6 months and older with documented prolonged conv ulsive^ seizure Statement > 5 minutes ^Convulsive is defined as: Tonic, clonic, tonic-clonic, myoclonic Denominator  s d for all abortive medication Patient contraindication documente Exceptions Patient/caregiver refuse  IV access established   Undocumented seizure duration recorded  Documentation that supports patients have self-resolving seizur es that last more than five minutes Exception hey should be excluded If a patient has a contraindication, such as an allergy, then t Justification r caregiver should be allowed t o refuse a treatment. due to risk of harm. A patient o oute, it can be utilized and the measure as written As IV access is an acceptable r would not apply. For patients where the seizure duration is un known, it would be difficult to assess when the abortive medication should be give n. Certain patients will have prolonged seizures self-abort. The intent of an abor tive medication is to y not be needed for stop a seizure that otherwise would not stop. Therefore, it ma all seizures greater than five minutes if it is documented that the patient has seizures tha self-abor t afte r five minutes. t d supporting ed verbatim from the reference The following statements are quot Supporting Guideline & articles: Other  [In the EMS setting] “We recommend that prehospital protocols f or seizure References managemen in children utilize alternative (non-IV) routes of drug t 21

22 administration as first-line therapy for treating children with status 10 epilepticus” (PR)  [In the EMS setting] “We recommend buccal midazolam over rectal 10 diazepam for prehospital seizure cessation and control”  [In the EMS setting] “We recommend IM midazolam over PR diazepa m 10 for prehospital seizure cessation and control” [In the EMS setting] “We suggest intranasal (IN) midazolam over PR  10 diazepam for prehospital seizure cessation and control”  [In the EMS setting] “We suggest t hat in children with convulsi ve status epilepticus requiring medication management in the prehospital setting, edication trained prehospital personnel s hould be allowed to administer m 10 without online medical direction”  gency seizure rescue plan in p lace, “While most families have an emer f of responders provided could knowledge gaps exist. Nearly hal not correctly verbalize how to administer rescue medication and nea rly half were not aware of respiratory depression as a side effect. A st andardized regular reviews at clinic vis its is needed training program by nursing, with in the home management of acute to improve parental proficiency 11 seizures” “Of the 32 children who presented  d in the community, 19 (59%) ha evidence that they had been giv en rescue medication prior to ar rival at hospital. This confirms previous reports that appropriate and t imely 12 treatment is not being administer ed in many cases of prolonged seizure” ons to  “Most existing guidelines do not provide practical recommendati rescue caregivers in out-of-hospital settings on the administration of medication. Filling this gap is critical to ensure that childre n at risk of tion prolonged acute convulsive seizure s receive their rescue medica ereby quickly and safely regardless of where their seizure occurs, th avoiding unnecessary treatment delays, clinical sequelae and co stly 13 admission to hospital” “Published data support the effi cacy and safety of nonintraveno us routes of  administration for midazolam, when compared to diazepam adminis tered ents with status epilepticus, in the doses via any route in treating pati mal choice studied. Midazolam has characteristics that may make it an opti 14 for the treatment of seizing patients”  ds that offer “There is a perceived need for alternative administration metho r different fast onset of effect and rapid and convenient administration fo populations with varying needs/preferences. Mounting evidence s upports multiple safe and effective alternative routes of BDZ administr ation for 15 rapid treatment of seizures in children with adults”  “Based on our results, many of th e visits of patients to the ED or hospital could have been possibly preven ted with appropriate doses of an 16 e m ergency seizure medication” Measure Importance Relationship to It is anticipated that by increasing the number of patients wh o have abortive ization and Desired Outcome medications available when need ed will decrease healthcare util 10,16 decrease episodes of treatmen t resistan t seizures (status epilepticus). 22

23 Opportunity for A study of high utilizers of ED care for seizures had not been prescribed an 17 Improvement abortive medication fo prolonged convulsive seizures. r National Quality ☐ Patient and Family Engagement Strategy Patient Safety ☐ Domains Care Coordination ☐ ☐ Population/Public Health ☒ Efficient Use of Healthcare Resources ☐ Clinical Process/Effectiveness Harmonization N/A with Existing Measures Measure Designation Measure ☒ Quality improvement (Check Purpose ☒ Accountability all tha t apply) Type of Measure Process ☒ (Check all that ☐ Outcome apply) ☐ Structure Level of ☒ Individual Provider Measurement ☒ Practice (Check all that ☐ System apply) Care Setting Outpatient ☒ (Check all that Inpatient ☒ apply) ☒ Emergency Departments and Urgent Care ☒ Residential (i.e., nursing f acility, domiciliary, home care) Data Source ☒ Electronic health record (EHR) data (Check all that ☐ Administrative Data/Claims apply) Patient Medical Record ☒ ☒ Registry References 1. tal diazepam in acute Bhattacharyya M, Kalra V, Gulati S. Intranasal midazolam vs rec childhood seizures. Pediatr Neurol 2006; 36:355-9. 2. ntranasal midazolam Harbord MG, Kyrkou NE, Kyrkou MR, Kay D, Coulthard KP. Use of i to treat acute seizures in pae diatric community settings. J Pae diatr Child Health 2004; 40:556-8. 3. Holsti M, Sill BL, Firth SD, Filloux FM, Joyce SM, et al. Preho spital intranasal midazolam for the treatment of pediatric seizures. Pediatr Emerg Care 200 7; 23(3):148-53 4. Wermeling DP. Intranasal delivery of antiepileptic medications for the treatment of seizures. r ; 6(2):352-8. Neurotherapeutics. 2009 Ap 23

24 5. study and Wermeling DP, Record KA, Archer SM, Rudy AC. A pharmacokinetic rbed intranasal midazolam pharmacodynamic study, in healthy volunteers, of a rapidly abso formulation. Epilepsy Res 2009; 83:124-32. Wilson MT, Macleod S, O ’ Regan ME. Nasal/buccal midazola m use in the community. Arch 6. Dis Child 2004; 89:50-1. Wolfe TR, Macfarlane TC. Intranasal midazolam therapy for pedia tric status epilepticus. Am 7. J Emerg Med 2006; 24:343-6. 8. tion and management of Brophy GM, Bell R, Claassen J, et al. Guidelines for the evalua it Care. 2012; 17(1):3-23. status epilepticus. Neurocr 9. Treatment of convulsive Glauser T, Shinnar S, Gloss D, et al. Evidence-Based Guideline: status epilepticus in children a nd adults: Report of the Guidel ine Committee of the American Epilepsy Society. Epilepsy Curr. 2016 Jan-Feb; 16(1):48-61. 10. or pediatric prehospital Shah M, Macias C, Dayan P, et al . An evidence-based guideline f cy Care 2014; 18 ethodology. Prehospital Emergen seizure management using GRADE m (Suppl 1):15-24. Cain L, Nickels KC, Wirrell EC , et al. Parent knowledge on home management of acute 11. seizures. Epilepsy Currents 2013; 13:22. Hunter L, Sidebotham P, Appleton R, Dunkley C. A review of the quality of care following 12. lds with epilepsies. Seizure 2 015; 24:88-92. prolonged seizures in 1-18 year o 13. Lagae L, Arzimanoglou A, Beghi E . Guidelines on the management of prolonged acute convulsive seizures in out-of-hospital settings: A gap to be fi lled. European Journal of Paediatric Neurology 2013; 17:S74-75. 14. us diazepam for the McMullan J, Sasson C, Pancioli A, Silbergleit R. Midazolam vers in children and young adults: A treatment of status epilepticus meta-analysis. Progressive Clinical Practice 2010; 17:575-582. 15. Pellock J, Haut S, Seinfeld S. B enzodiazepine use for emergency treatment of seizures: A review. Epilepsy Currently 2014; 14:179. 16. ilepsy Patients Using Cohen D, Patel A, Wood E. Emergency Department Diversion for Ep Quality Improvement Methodology. AAP National Conference & Exhi bition October 2015. 17. Patel A. Variables associated with emergency department and/or unplanned hospital utilization fo r children with epilepsy. Epilepsy & Behavio r 2014; 31:172-175. Denominator ICD-10 Code (Eligible R56.8 Seizures (otherwise unspecified) G40.xx Epilepsy (otherwise unspecified) Population) AND CPT E/M Service Code 99221, 99222, 99223 Initial hospital care 30, 50, or 70 minutes, per day, for the evaluation and management of a patient; 99231, 99232, 99233 Subsequent hospital care 15, 25, or 35 minutes, per day, for the evaluation and management of a patient 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o t r 40 minutes fo r the evaluation and managemen t of an established patien 24

25 efractory convulsive status epi Time to third line therapy for r lepticus (RCSE) Measure Description herapy for seizure cessation for refractory Percentage of patients who receive d the start of a third line t convulsive status epilepticus (RCSE) Measure Components Numerator Patients who were started on a third line therapy* within 60 mi nutes of seizure onset (inpatient setting) or after arrival to the emergency dep artment (ED) Statement (outpatient setting) Definitions: on-benzodiazepine *For use in this measure, “third line therapy” means a second n anti-epileptic drug (AED)/anti-seizure drug or a continuous IV infusion ine) of a medication for seiz (benzodiazepine or non-benzodiazep ures. Third line therapies include but are not limited to:  Continuous IV infusion: midazolam, pentobarbital, propofol, thi opental  Different non-benzodiazepine AED medications: fosphenytoin, levetiracetam, valproic acid, phenobarbital e is acceptable (ex. NOTE: A medicine that is the same but given by a different rout Oral then IV) Patients > 1 month old with refractory convulsive status epilep Denominator ticus (RCSE)^ Statement ^RCSE means ongoing clinical or electrographic seizures despite 2 appropriate 1 medications, one of which is typically no enzodiazepine. a b t Denominator  Patient/caregiver refuse Exceptions eatment are not seizure control  Care team documents goals of tr  Patient in palliative care setting Patient is participating in a clinical trial for the treatment  of status epilepticus  Intervention is delayed by clinical status such as hypotension precluding intravenous access Exception caregivers and care The parent or caregiver may ref use the treatment. The patient’s Justification o longer impactful; further treatment is futile and n team may have determined that therefore, the goal may not be seizure control or the patient h as entered into a palliative care setting. Patients participating in clinical tr ials should not be included fo this measure as the trial protocol will dictate the treatmen t plan. r d supporting ed verbatim from the reference The following statements are quot Supporting articles: Guideline & Other  “There is international consen sus that convulsive seizures last ing more than References injury, 30 minutes may cause long-term consequences, including neuronal 2 neuronal death, alteration of neuronal networks, and functional defic its” 25

26  timeline or optimal time window exists “Although no evidence-based AED rrent SE treatment protocols reco mmend for this AED sequence, most cu onset. If that the first AED be administered within 5 minutes of seizure seizures persist, moving to the next AED class in the sequence should be done by 10 minutes and, if repeat ed AED doses do not control SE , the initiation of anesthetic dosing via continuous infusions should be started by 3 30-70 minutes of seizure onset.“ “Timely AED administration and rapidly moving along the sequenc e of  3 e.” AED classes are intended to st op seizures as quickly as possibl “The algorithm starts with a stabilization phase (0-5 minutes),  which includes standard initial first aid for seizures. The initial t herapy phase duration reaches 5 minutes and sh should begin when the seizure ould conclude by the 20-minute mark when response (or lack of respon se) to initial therapy should be apparen t. A benzodiazepine (specifica lly IM midazolam, IV lorazepam, or IV diazepam) is recommended as the initial demonstrated efficacy, safety, a nd tolerability therapy of choice, given their egin when (level A, four class I RCTs). The second-therapy phase should b the 40- the seizure duration reaches 20 minutes and should conclude by minute mark when response (or lack of response) to the second t herapy tions include fosphenytoin (le vel U), should be apparent. Reasonable op valproic acid (level B, one class II study) and levetiracetam ( level U). There is no clear evidence that any one of these options is bet ter than the others. The third therapy phase should begin when the seizure d uration reaches 40 minutes. There is no clear evidence to guide therapy in this 4 phase (level U).” “Definitive control of SE should be established within 60 min o f onset. All  patients presenting with SE will apy (i.e., need emergent initial AED ther 1st line) and urgent control AED therapy (i.e., 2nd line) in ad dition to AED 1 maintenance therapy, even if SE is immediately controlled.” Measure Importance with cessation of Relationship to Patients with status epilepticus have better long term outcomes Desired Outcome seizures as quickly as possible. P rolonged uncontrolled status epilepticus carries risk of increase morbidity and mortality. 3 Opportunity for A significant gap exists in time to treatment for RCSE. Refractory status Improvement epilepticus patients “are not being treated with a third-line a nti-epileptic drug until 3 after 2 hours”. Although only limited data exist s, this is a significant opport unity to improve the care of children with RCSE. National Quality ☐ Patient and Family Engagement Strategy Patient Safety ☒ Domains ☐ Care Coordination ☐ Population/Public Health ☐ Efficient Use of Healthcare Resources Clinical Process/Effectiveness ☐ 26

27 Harmonization Harmonizes with AAN’s Inpatient a nd Emergent QM developed for p atients >16 with Existing years of age. One measure exists addressing patients with gener alized convulsive status epilepticus who are treated with a non-benzodiazepine an tiepileptic/anti- Measures seizure medication following the administration of a benzodiaze pine. The measure applies to patients aged 16 year s of age and older. This measur e is needed to capture performance fo patients younge r r than 16 years of age. Measure Designation Measure Quality improvement ☒ (Check Purpose ☐ Accountability all tha t apply) Type of Measure ☒ Process (Check all that ☐ Outcome apply) Structure ☐ Level of ☐ Individual Provider Measurement Practice ☒ (Check all that ☒ System apply) Care Setting Outpatient ☐ (Check all that ☒ Inpatient apply) Emergency Departments and Urgent Care ☒ acility, domiciliary, home care) ☐ Residential (i.e., nursing f Data Source Electronic health record (EHR) data ☒ (Check all that ☐ Administrative Data/Claims apply) ☒ Patient Medical Record ☒ Registry References t al. Guidelines for the Evaluat ion and Management of Status 1. Brophy G, Bell R, Claassen J, e Epilepticus. Neurocr it Care 2012; 17:3-23. 2. Trinka E, Cock H, Hesdorffer D, Rossetti AO, Scheffer IE, Shinn ar S, Shorvon S, Lowenstein DH. A definition and classification of status epilepticus--Report o f the ILAE Task Force on Classification of Status Epilepticus. Epilepsia. 2015 Oct; 56(10):1515-23. , Agadi S, et al. Time from convul sive status epilepticus 3. Sanchez Fernandez I, Abend NS onset to anticonvulsant administration in children. Neurology 2 015; 84:2304-2311. 4. Glauser T, Shinnar S, Gloss D, et al. Evidence-Based Guideline: Treatment of Convulsive Status Epilepticus in Children a nd Adults: Report of the Guidel ine Committee of the American Epilepsy Society. Epilepsy Currents 2016; 16:48-61. Denominator ICD-10 Code cal) (partial) idiopathic epil G40.001 Localization-related (fo epsy and epileptic (Eligible Population) syndromes with seizures of loca lized onset, not intractable, wi th status epilepticus G40.011 Localization-related (focal) (partial) idiopathic and epileptic syndromes s with seiures of localized onset, intractable, with status epilepticu 27

28 G40.201 Localization-related (fo cal) (partial) symptomatic epi lepsy and epileptic status epilepticus. syndromes with complex partial seizures, not intractable, with cal) (partial) symptomatic epi lepsy and epileptic G40.211 Localization-related (fo syndromes with complex partial seizures, intractable, with stat us epilepticus. G40.301 Generalized idiopathic e pilepsy and epileptic syndrome s, not intractable, with status epilepticus. G40.311 Generalized idiopathic e pilepsy and epileptic syndrome s, intractable, with status epilepticus. psy and epileptic syndromes, no t intractable, with G40.401 Other generalized epile status epilepticus. G40.411 Other generalized epile psy and epileptic syndromes, in tractable, with status epilepticus. G40.501 Epileptic seizures rela ted to external causes, not int ractable, with status epilepticus G40.901 Epilepsy, unspecified, not intractable, with status ep ilepticus ticus G40.911 Epilepsy, unspecified, intractable, with status epilep AND CPT E/M Service Code 99221, 99222, 99223 Initial hospital care 30, 50, or 70 minutes, per day, for the evaluation and management of a patient; 99231, 99232, 99233 Subsequent hospital care 15, 25, or 35 minutes, per day, for the evaluation and managemen t t of a patien 28

29 Neuropsychological/neurod evelopmental screening Measure Description neurodevelopmental o r neuropsychological deficits Percentage of patients with epilepsy screened fo r Measure Components Patients who were screened* or r eferred for screening for neuro developmental Numerator Statement its within 1 year of initial epi and/or neuropsychological defic lepsy diagnosis g the patient or *Screened is defined as using a validated instrument or queryin caregiver to determine the presence or absence of symptoms. Denominator Patients aged 1 month and older diagnosed with epilepsy within the past 12 months Statement without severe or profound intellectual disability who are not currently under the care of a psychiatrist/psychologis t Denominator Patient/caregiver refuse  Exceptions Exception Some children have significant intellectual inability that prec lude them for being Justification able to participate adequately i n testing. A patient or caregiv er have the right to refuse testing. If a patient already has neuropsychological or neurodevelopmental services, they would no t need additional screening o r referrals. Supporting ed verbatim from the reference d supporting The following statements are quot articles: Guideline & Other  “Establishing the presence of a deficit in a particular area of cognition that References ent (e.g., is hypothesized to be related to a deficit in academic achievem working memory in reading) should be part of the process of det ermining the presence of an SLD [specific learning disability] according to some 1 definitions of SLD.” vioral comorbidity in childhoo  “Given the high rate of neurobeha d epilepsy itive and eening of all children for cogn and noted under recognition, scr behavioral difficulties would seem warranted, as has been previ ously 2 recommended.” “Given general brain developmen t and changes/vulnerabilities to  seizures and comorbid behavioral health s ymptoms in puberty, screening s hould throughout the developmental co urse of the take place at seizure onset and 3 youth’s epilepsy to achieve optimal quality of life.”  “Neuropsychological assessment s hould be considered in children , young people and adults in whom it is important to evaluate learning disabilities and cognitive dysfunction, partic ularly in regard to language a nd 4 memory.”  “Referral for a neuropsychological assessment is indicated: - When a child, young person or a dult with epilepsy is having educational or occupational difficulties - When an MRI has identified abnormalities in cognitively importa nt brain regions r - When a child, young person or adul t complains of memory or othe 4 cognitive defici t s and/o r cognitive decline” 29

30  hildren and “Screening for developmental de lay is important for all young c 5 especially those with epilepsy.”  “Formal screening, as recommended by the American Academy of pediatrics, with a well-validated measure such as the ASQ-3 is ideal and 5 should r feasible.” b e done wheneve Measure Importance Increase the percent of patients who get neuropsychological tes ting in order to Relationship to Desired Outcome address issues and obtain proper treatment Literature suggests that neurops ychological deficits are presen t in many children Opportunity for 6,7,8,9,10 with epilepsy independent from control of seizures. Improvement delines into clinical Eom et al., discuss the lack of implementation of screening gui 5 gap in high quality care. practice which results in a When interventions are hological or neurodevelopmental initiated earlier for neuropsyc issues there is an 11 increase in developmental attainme nt that benefits the patient long term. The Work Group identified the fo llowing questionnaire-based too ls to assist in screening: Child Behavior Checklist (CBCL)  rd Behavior Assessment System for Children, 3  edition (BASC-3) nd  Behavior Rating Inventory of Executive Function, 2 edition (BRIEF-2)  Conners’ third edition (Conne rs-3) (ADHD rating scale)  NICHQ Vanderbilt Assessment Scale Ages & Stages Questionnaires, Third Edition (ASQ-3)  Strengths & Difficulties Questionnaires (SDQ)   Pediatric Quality of Life Inventory (PedsQL) Neuro QoL/PROMIS measures  nd Child Depression Inventory, 2  edition (CDI-2) nd  Multidimensional Anxiety Scale for Children, 2 edition (MASC-2) Psychosocial Assessmen t Tool (PAT)  National Quality Patient and Family Engagement ☐ Strategy Patient Safety ☐ Domains Care Coordination ☐ ☐ Population/Public Health ☐ Efficient Use of Healthcare Resources ☒ Clinical Process/Effectiveness Harmonization creening for This measure does harmonize with an existing adult measure on s with Existing psychological co-morbidities as they would be identified on thi s testing. This Measures measure is needed as it expands t he screening to include other disorders that are commonly seen in children that do not necessarily apply to the adult epilepsy population. Measure Designation 30

31 Measure Quality improvement ☒ Purpose (Check ☐ Accountability all tha t apply) Type of Measure ☒ Process (Check all that ☐ Outcome apply) Structure ☐ Level of Individual Provider ☒ Measurement Practice ☒ (Check all that ☐ System apply) Care Setting ☒ Outpatient (Check all that ☐ Inpatient apply) ☐ Emergency Departments and Urgent Care ☐ Residential (i.e., nursing f acility, domiciliary, home care) Data Source ☒ Electronic health record (EHR) data (Check all that Administrative Data/Claims ☐ apply) ☒ Patient Medical Record ☒ Registry References 1. epsy: a review. Epilepsy Reilly C, Neville B. Academic achie vement in children with epil Research 2011; 97:112-123. 2. . Neurobehavioral comorbiditi es in children with active Reilly C, Atkinson P, Das K, et al 586-1593. epilepsy: a population-based study. Pediatrics 2014; 133(6): e1 Wagner J, Guilfoyle S, Rausch J , Modi A. Psychometric validatio n of the Pediatric Symptom 3. study. Epilepsy & Behavior Checklist-17 in a pediatric population with epilepsy: A methods 2015; 51:112-116. 4. National Institute for Health and Care Excellence. Epilepsies: diagnosis and management. es-diagnosis-and- 2012. https://www.nice.org.uk/guidance/cg137/resources/epilepsi . Accessed on May 2, 2016. management-35109515407813 F, Berg A. A simple behavioral Eom S, Dezort C, Fisher B, Zelko -developmental checklist 5. versus formal screening for children in an epilepsy center. Epi lepsy & Behavior 2015; 46:84- 87. 6. Verrotti A, Matricardi S, Rinaldi VE, Prezioso G, Coppola G. Ne uropsychological impairment in childhood absence ep ilepsy: Review of the literat ure. Journal of the Neurological Sciences 2015; 359:59-66. 7. Hermann B, Jones J, Sheth R, et al. Children with new-onset epi lepsy: neuropsychological status and brain structure. Brain 2006; 129:2609-19. Berg A, Langfitt J, Testa F, et al. Residual cognitive effects of uncomplicated idiopathic and 8. cryptogenic epilepsy. Epilepsy & Behavior 2008; 13:614-619. 9. Berg A, Hesdorffer D, Zelko F. Special education participation in children with epilepsy: What does it reflect? Epilepsy & Behavior 2011; 22:336-341. tus at seizure onset in 10. Fastenau P, Johnson C, Perkins S, et al. Neuropsychological sta children. Neurology 2009; 73:526-34. 31

32 11. A. Routine developmental, autis m, behavioral, and Eom S, Fisher B, Dezort C, Berg l Medicine & Child psychological screening in epilepsy care settings. Developmenta Neurology 2014; 56:1100-5. Denominator ICD-10 Code (Eligible R56.8 Seizures (otherwise unspecified) Population) G40.xx Epilepsy (otherwise unspecified) AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, or 40 minutes fo r the evaluation and managemen t of an established patien t 32

33 Querying for co-morbid condition s of tic disorder (TD) and Tour ette syndrome (TS) Measure Description r behavioral co-morbid conditions of Percentage of patients who were queried for psychological and/o tic disorde (TD) o r Tourette syndrome (TS) r Measure Components Numerator Patients who were queried^ for symptoms of psychological and/or behavioral co- Statement morbid conditions* at least once per year. Definitions: *Co-morbid conditions (to meet measure requirements must query for all conditions in the list below):  Mood disorders, including depression and anxiety,  Obsessive compulsive disorder (OCD),  Attention Deficit Hyperactivity Disorder (ADHD), AND  Oppositional Defiant Disorder (ODD) absence of ^Queried is defined as asking or inquring about the presence of symptoms Denominator All patients aged < 18 years with the diagnosis of TD* or TS wh o do not have an Statement existing diagnosis of a comorbid condition 1 *Tic disorders include:  Chronic or transient (DSM IV)  Persistent or provisional (DSM V)  Motor and vocal  Other tic disorder no t Tic disorde r  specified Denominator Patient/caregiver refuse  Exceptions Exception ent and caregivers Exception for patient and caregiver declinations needed as pati aluation for results to be mean ingful. need to be willing to undergo ev Justification d supporting The following statements are quot ed verbatim from the reference Supporting Guideline & articles: Other  “Recommendations are given to assess the most prevalent comorbi d 2 References conditions, i.e. ADHD and OCD.”  “Lastly, due to high rates of comorbidity in children with CTD, global assessment measures like the TODS-CR and TODS-PR may be useful as they assess the severity of tics in addition to common comorbid 3 symptoms.” ination for  “The assessment for tic disorde rs should involve a careful exam 4 comorbid psychiatric conditions.” 33

34 Measure Importance Tic disorder is frequently associated with psychiatric conditio ns and presence of Relationship to worse than the tics itself, c an significantly Desired Outcome these co-morbid conditions can be 3 impair function and can affective cognitive performance. Screening for these conditions will lead to early diagnosis and treatment. Opportunity for It is estimated that between 80% to 90% of patients with Touret te syndrome have 5 both tics and psychiatric manifestations. Improvement Their quality of life is impacted by these 5 accompanying psychiatric conditions. National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains ☐ Care Coordination Population/Public Health ☐ Efficient Use of Healthcare Resources ☐ ☒ Clinical Process/Effectiveness Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ (Check Purpose ☒ Accountability all tha apply) t Type of Measure ☒ Process (Check all that ☐ Outcome apply) ☐ Structure Level of ☒ Individual Provider Measurement Practice ☒ (Check all that System ☒ apply) Care Setting ☒ Outpatient (Check all that ☐ Inpatient apply) ☐ Emergency Departments and Urgent Care Residential (i.e., nursing f ☐ acility, domiciliary, home care) Data Source ☒ Electronic health record (EHR) data (Check all that ☐ Administrative Data/Claims apply) ☒ Patient Medical Record ☒ Registry References 1. American Psychiatric Associati on. (2013). Diagnostic and statis tical manual of mental disorders: DSM-5. Washington, D.C: American Psychiatric Association . 34

35 2. Cath DC, Hedderly T, Ludolph AG, et al. European clinical guide lines for Tourette syndrome dolescent Psychiatry 2011; assessment. European Child & A and other tic disorders. Part I: 20:155-71. McGuire JF, Kugler BB, Park JM, et al. Evidence-based assessmen t of compulsive skin 3. . Child Psychiatry & Human and trichotillomania in children picking, chronic tic disorders Development 2012; 43:855-83. Murphy T, Lewin A, Starch E, et al. Practice Parameter for the Assessment and Treatment of 4. Disorders. Journal of the Ame rican Academy of Child & Children and Adolescents with Tic Adolescent Psychiatry 2013; 52:1341-59. 5. Rizzo R, Gulisano M, Pellico A, Valeria Cali P, Curatolo P. Tou rette Syndrome and of Different Severities and Com plexities. Journal of Child Comorbid Conditions: A Spectrum Neurology 2014; 29:1382-1389. Denominator ICD-10 Code F95.1 tic chronic (Eligible Population) F95.2 Tourette syndrome AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o r 40 minutes fo r the evaluation and managemen t of an established patien t 35

36 Management of co-morbid symptoms of tic disorder (TD) or Touret te syndrome (TS) Measure Description nt for co-morbid symptoms of tic Percentage of patients who were treated or referred for treatme disorde (TD) o r Tourette syndrome (TS) r Measure Components Numerator Patients who were treated* or ref erred for treatment for comorb id condition(s)** Statement annually. *Treated is an intervention and/or medication implemented for c o-morbid conditions **Co-morbid conditions:  Mood disorders, including depression and anxiety,  Obsessive compulsive disorder (OCD),  Attention Deficit Hyperactivity Disorder (ADHD), AND Oppositional Defian t Disorde r (ODD)  Denominator All patients aged 18 years of a ge and below with the diagnosis of TD* or TS and Statement co-morbid mood disorder, OCD , ADHD, or ODD diagnosis who are no t currently under the care of a psychiatrist/psychologist 1 *Tic disorders include:  Persistent, provisional (DSM V)  Chronic, Transient (DSM IV)  Motor and vocal Other tic disorder  Tic disorde r no t specified  Denominator  Patient/caregiver refuse Exceptions Exception Some patients may not be receptiv e to treatment/intervention. I f patient is already under psychiatric or psychological care they would not need a r eferral for further Justification management. d supporting ed verbatim from the reference The following statements are quot Supporting articles: Guideline & Other  “80-90% of patients with Tourette syndrome have comorbid disord ers such References as attention deficit hyper-activity disorder, depression, anxie ty, and obsessive-compulsive disorder, which often impair the quality o f life more 2 than the tics themselves and ar e accordingly the main target of treatment.”  “It is recommended that psychiatric disorders accompanying Tour ette syndrome should be treated in th e same way as when they occur i n the 2 absence of Touret t e syndrome.” Measure Importance 36

37 Relationship to e mild but have significant sym ptoms from the co- There are patients whose tics ar 2 Desired Outcome morbid disorders. gement of co-morbid conditions Treatment or referral for mana e and improve tic frequency as wel l. will lead to improved quality of lif 2 Psychiatric manifestations appear in 80-90% of patients. Patient quality of life is Opportunity for Improvement ying psychiatric conditions of severely impaired by the accompan tic disorders and 2,3 Tourette’s syndrome. Patients and their caregivers often find the co-morbid 4 conditions to e more challenging than the tic itself. b National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains ☐ Care Coordination ☐ Population/Public Health Efficient Use of Healthcare Resources ☐ Clinical Process/Effectiveness ☒ Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ (Check Purpose ☐ Accountability all tha apply) t Type of Measure Process ☒ (Check all that Outcome ☐ apply) ☐ Structure Level of Individual Provider ☒ Measurement ☒ Practice (Check all that ☒ System apply) Care Setting Outpatient ☒ (Check all that ☐ Inpatient apply) Emergency Departments and Urgent Care ☐ Residential (i.e., nursing f acility, domiciliary, home care) ☐ Data Source Electronic health record (EHR) data ☒ (Check all that ☐ Administrative Data/Claims apply) ☒ Patient Medical Record ☒ Registry References 1. American Psychiatric Associati on. (2013). Diagnostic and statis tical manual of mental . disorders: DSM-5. Washington, D.C: American Psychiatric Association 37

38 2. A, Muller-Vahl K. Review articl e: Tourette syndrome Ludolph AG, Roessner V, Munchau ildhood, adolescence and adulthood and other tic disorders in ch . Deutsches Arzteblatt International 2012; 48:821-828. Eapen V, Snedden C, Crncec R, P 3. ome, co-morbidities and ick A, Sachdev P. Tourette syndr quality of life. Australian & New Zealand Journal of Psychiatry 2016; 50:82-93. 4. Murphy T, Lewin A, Storch E, et al. Practice Parameter for the Assessment and Treatment of Children and Adolescents with Tic D ican Academy of Child & isorder. Journal of the Amer Adolescen t Psychiatry 2013; 52:1341-1359. Denominator ICD-10 Code (Eligible F95.1 tic chronic Population) F95.2 Tourette syndrome AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o r 40 minutes fo r the evaluation and managemen t of an es t ablished patien t 38

39 Behavioral therapy for tic disord er (TD) or Tourette syndrome ( TS) Measure Description counseled or referred for behav Percentage of patients who were ioral therapy for management of chronic tic disorde r (TD) o r Tourette syndrome (TS) Measure Components Numerator Patients who were counseled or re ferred for behavioral therapy* Statement from the below list at least one of the therapies *Behavioral therapy (must provide to meet measure): Comprehensive behavioral tr  eatment for tics (CBIT), OR Habit reversal training (HRT), OR  Relaxation training, OR   Exposure and Response Prevention Therapy (ERP) Denominator Patients aged 8 years of age and above diagnosed with chronic^ TD or TS without Statement severe or profound intellectual disability who are currently no t receiving behavioral therapy ^Chronic is defined as greate than one yea r r Denominator  Patient/caregiver refuse Exceptions  t has already received a referral in the 12-month measuremen t yea r Patien Exception py and may not be Active patient participation is a necessity in behavioral thera Justification suitable for extremely young and cognitively disabled patients; and if patient and ven in the measurement a referral has already been gi family refuse to participate. If period, additional referrals should no t b e required. ed verbatim from the reference The following statements are quot d supporting Supporting Guideline & articles: Other  “Intervention built on HRT appear s to be effective for decreasi ng tic 1 References severity in children and adolescents.”  “Based on the current available evidence, we have made strong recommendations for HRT and ERP, p referably embedded within a supportive, psycho-educational pr ogram, and with the option of combining 2 either of these approaches with drug treatment.”  “In summary, studies indicate that HR is effective for both voc al and motor medications as tics, for children as well as adults, for patients receiving TS tic severity as well as tic fre quency, and with well as those not doing so, for 3 no evidence of symptom substitution.”  “Preliminary results indicate that ERP is effective for vocal a nd motor tics, for children as well as adults, for tic severity as well as tic frequency, with no indications of a rebound effect. Since younger children are less aware of premonitory sensory motor phenomena it has to be clarified if t here is an 3 age effect respective an age limit for ER but also for HR.” ons have no  “Preliminary results seem to indicate that cognitive interventi 3 specific additive value in t he treatmen t of tics.” 39

40  “This meta-analysis found a medium to large treatment effect fo r BT across 4 RCTs for TS (SMD=0.67).” managed with pharmacotherapy, this  “While TS has traditionally been quantitative synthesis suggests BT presents an alternative trea tment option s to psychotropic medications – with comparable treatment effect supporting current treatment reco mmendations by some profession al 4 organizations that BT serve as a first-line treatment for TS.”  “When examining treatment response on the CGI-Improvement, participants receiving BT were five times more likely to respon d to 4 treatment compared to individuals receiving comparison interven tions.”  TD should be considered when tic s cause “Behavioral interventions for C ity, or if behavioral-responsi ve impairment, are moderate in sever 5 psychiatric comorbidities are present.” Measure Importance There is no cure for tics and wh duce tic frequency, Relationship to ile pharmacologic agents can re Desired Outcome Behavioral treatment there are potential significant side effects from medications. better long term does not have adverse effects and its possible advantage is its 6,7,8 effects beyond the duration of therapy. The use of behavioral treatment will improve frequency of tics and increase functioning, adaptation and coping skills to the fluctuating nature of the disorder. Opportunity for The typical treatment for severe tics is antipsychotics, alpha agonists, and 1,2 anticonvulsants. Improvement These medications are effective but often have many side effec ts 1,2 and rarely eradicate the tics completely. Behavioral therapies have been around e last decade with for a long time, but have recently had a growing interest in th 2 many RCTs b eing completed. National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains ☐ Care Coordination ☐ Population/Public Health ☐ Efficient Use of Healthcare Resources ☒ Clinical Process/Effectiveness Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ (Check Purpose ☐ Accountability all tha t apply) Type of Measure ☒ Process (Check all that ☐ Outcome apply) ☐ Structure 40

41 Level of ☒ Individual Provider Measurement ☒ Practice (Check all that ☒ System apply) Care Setting Outpatient ☒ (Check all that Inpatient ☐ apply) ☐ Emergency Departments and Urgent Care Residential (i.e., nursing f ☐ acility, domiciliary, home care) Data Source ☒ Electronic health record (EHR) data (Check all that Administrative Data/Claims ☐ apply) Patient Medical Record ☒ Registry ☒ References 1. Hwang GC, Tillberg CS, Scahill L. Habit reversal training for c hildren with Tourette nal of Child & Adolescent Psyc hiatric Nursing 2012; syndrome: update and review. Jour 25:178-83. 2. Steeves T, McKinlay BD, Gorman D, et al. Canadian guidelines fo r the evidence-based treatment of tic disorders: Beha mulation, and transcranial vioural therapy, deep brain sti magnetic stimulation. Canadian J ournal of Psychiatry 2012; 57:1 44-151. 3. Verdellen C, van De Griendt J, H artmann A, Murphy T. European c linical guidelines for Tourette syndrome and other tic d isorders. Part III: behavioura l and psychosocial interventions. European Child & Adolescent Psychiatry 2011; 20: 197-207. 4. behavior therapy for McGuire JF, Piacentini J, Brennan EA, et al. A meta-analysis of sychiatric Research 2014; 50:106 -12. Tourette Syndrome. Journal of P Murphy T, Lewin A, Starch E, et al. Practice Parameter for the 5. Assessment and Treatment of Children and Adolescents with Tic Disorders. Journal of the Ame rican Academy of Child & Adolescent Psychiatry 2013; 52:1341-59. 6. cotherapy for pediatric DeNadai AS, Storch EA, McGuire JF, et al. Evidence-based pharma chronic tic disorders. J Cent Nerv Syst Dis 2011; 3:125- obsessive-compulsive disorder and 142. 7. Pringsheim T, Doja D, Gorman D , et al. Candaian guidelines for the evidence-based treatment of tic disorders: pharm acotherapy. Can J Psychiatry 2 012; 57:133-43. 8. t al. Behavior therapy for children with Tourette Piacentini J, Woods DW, Scahill L, e disorder: a randomized controlled trial. JAMA 2010; 303:1929-37. Denominator ICD-10 Code (Eligible F95.1 tic chronic Population) F95.2 TS F72 Severe intellectual disability F73 Profound intellectual disability AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o t of an established patien r 40 minutes fo r the evaluation and managemen t 41

42 Transition to adult neurology care Measure Description a neurological transition plan o Percentage of patients who had f care Measure Components Numerator Pediatric neurology patients with chronic ongoing neurological condition > 13 Statement plan of care* years of age that have had a doc umented neurological transition n to patient and/o initiated and updated a nnually with copy give r caregiver *Neurological transition plan of care must include ALL of the f  ollowing, 1 but not limited to: nd surgical history related to o Medical plan (pertinent medical a neurological condition, current and past neurological medicatio ns and future needed testing) with adverse effects, previous Discussion of existing office transition policy with expected y ear o of transition given to patient and caregiver 2,3,4 o Patient self-management skills assessment 2,3 Transition readiness assessment o o Patient current and expected legal competency o Patient plan for employment, school, vocation, placement (for profound intellectual disability patients), etc. o Emergency plans (medical power of attorney, living will, DNR, plans for guardianship for patients with profound intellectual disability) o r accepting care of neurological Name of provider providing o condition (a t time of transition only) Pediatric neurology patients conditions > 13 Denominator with chronic ongoing neurological Statement years of age Denominator  A patient does not need continued care Exceptions Patient/caregiver refuses to see the adult provider or particip ate in the  transition planning Exception If a patient does not need continued care, there is no need for transition planning to Justification adult care. If a patient or caregiver refuses to see the adult provider or participate ountable. in the transition planning, then the provider should not be acc d supporting ed verbatim from the reference The following statements are quot Supporting articles: Guideline & Other  “Transition planning should be a standard part of providing car e for all References youth and young adults, and every patient should have a transit ion plan 5 regardless of his or her sp ecific health care needs.”  “A key component of supporting th e transition process is the pr imary care medical home having an explicit office policy that describes th e practice’s ss at which approach to health care transition, including the age and proce 5 youth shif t to an adul t model of care.” 42

43  must understand and address pati ents’ “The medical home team members eeds during transition and recog and parents’ perspectives and n nize that this process is complex and potentia lly emotional for parents a nd other 5 caregivers/guardians.” “For transition planning to su cceed, providers, and parents/car egivers must  view the youth as the driver in the process and encourage the y outh to to the fullest assume increasing responsibility for his or her own health care 5 extent possible.”  iseases originating in childhoo d who are “The population of adults with d hospitalized at children’s hosp itals is increasing, with varyin g disease- specific changes over time. Our findings underscore the need fo r proactive identification of strategies to care for adult survivors of ped iatric 6 diseases.” “Quality of life is an important construct relevant to HCT [Hea  lth Care Transitions]. Future research should identify valid measures as sociated with each outcome and further e xplore the role that quality of life plays in the HCT process. Achieving consen sus is a critical step toward the development of reliable and obj es ective comparisons of HCT outcom 7 across clinical conditions a nd care delivery locations.”  “Both disease complexity and failure of transition planning app ear to have contributed to the increased admission of young adults to the R CH [Royal Children’s Hospital]. While greater support of transition plann ing is needed, there are also concerns about the lack of appropriate s ervices within the adult sector for young adults with complex, multidis ciplinary 8 healthcare needs.” “Strengthening the capacity for transitioning from a service th at is family  focused to one with an individual orientation requires a paradi gmatic shift and clear identification of roles and responsibilities in the h ealth care 9 system.” “The child neurologist has a critical role in planning and coor dinating the  ith neurologic conditions from the pediatric successful transition of youth w 1 to adul t health care system.” Measure Importance Relationship to Adolescent and young adult neurol ogy patients will be properly transitioned to Desired Outcome adult neurology care. No gap will occur. Rate of proper comple tion will occur with tisfaction with transition, st ability or improved patient and caregiver sa improvement of neurological condition, decrease emergency utili zation and 1 improved quality of life. Opportunity for transition process Currently only a minority of practices participate in a formal which has led to poor quality o f care, decreased patient satisf action, and increased Improvement 10 healthcare utilization and costs. Tools available:  Got Transition™ a program of T he National Alliance to Advance Adolescent Health: www.gottransition.org Data Resource Center for Child and Adolescent Health:  www.childhealthdata.org 43

44  Institute for Healthcare Improvement: http://www.ihi.org/Topics/TripleAim/Pages/default.aspx The Transition Readiness Assessme actor  nt Questionnaire (TRAQ): Its F Structure, Reliability, and Validity  National Consensus Document: ons.org/content/128/1/182 http://pediatrics.aappublicati The National Alliance to A dvance Adolescent Health  AES Transition Tools:  esources/practice_tools/transi tion_tools_a https://www.aesnet.org/clinical_r dolescents CNF Transition Tools: http://www.childneurologyfoundation.org/p atients-  or-caregivers/transition-into-adulthood/  AHRQ: children- https://www.effectivehealthcare.ahrq.gov/ehc/products/546/1920/ special-needs-transition-report-140617.pdf n-adolescen -medicine https://psnet.ahrq.gov/webmm/case/348/transitions-i t National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains Care Coordination ☒ Population/Public Health ☐ ☐ Efficient Use of Healthcare Resources ☐ Clinical Process/Effectiveness Harmonization This measure harmonizes across all neurology disease states and covers both child with Existing and adult neurology providers . Although several existing measur es deal with Measures transitioning care, nothing specific exists on the transfer of care from child to adult neurology. Based on the gap and importance in this area, the wo rk group felt a separate measure was warranted fo r neurological patients. Measure Designation Measure Quality improvement ☒ Purpose (Check ☐ Accountability all tha t apply) Type of Measure ☒ Process (Check all that Outcome ☐ apply) ☐ Structure Level of Individual Provider ☒ Measurement Practice ☒ (Check all that ☒ System apply) Care Setting ☒ Outpatient (Check all that ☐ Inpatient apply) ☐ Emergency Departments and Urgent Care 44

45 ☒ Residential (i.e., nursing f acility, domiciliary, home care) Data Source ☒ Electronic health record (EHR) data (Check all that ☐ Administrative Data/Claims apply) Patient Medical Record ☒ ☒ Registry References 1. Brown L, Camfield P, Capers M, et al. The neurologist’s role in supporting transition to adult health care. Neurology 2016; 87:1-6. Sawicki GS, Lukens-Bull K, Yin X, et al. Measure the transition readiness of youth with 2. special healthcare needs: Validation of the TRAQ – Transition R eadiness Assessment Questionnaire. J Pediatr Psychol 2011; 36:160-171. 3. assessment questionnaire Wood DL, Sawicki GS, Miller MD, et al. The transition readiness ediatr 2014; 14:415-422. (TRAQ): its factor structure, reliability, and validity. Acad P 4. Jurasek L, Ray L, Quigley D. Development and implementation of an adolescent epilepsy transition clinic. J Neuroci Nurs 2010; 42:181-189. 5. American Academy of Pediatrics. Clinical Report – Supporting th e Health Care Transition from Adolescence to Adulthood in th e Medical Home. Pediatrics 2 011; 128: 182-200. 6. Goodman D, Hall M, Levin A, et al. Adults with chronic health c onditions originating in childhood: inpatient experience in children’s hospitals. Pediat rics 2011; 128: 5-13. 7. ., et al. International and in terdisciplinary identification of Fair, C., Cuttance, J., Sharma, M . JAMA Pediatrics 2016; 170:205- healthcare transition outcomes 211. 8. Lam PY, Fitzgerald B, Sawyer S. Y oung adults in children’s hosp itals: why are they there? Med J Australia 2005; 182:381-384. 9. Rapley P, Davidson P. M. Enough of the problem: a review of ti me for health care transition solutions for young adults with a chronic illness, Journal of C linical Nursing 2010 ; 19:313- 323. 10. Camfield P, Camfield C. Transition to adult care for children w ith chronic neurological disorders. Annals of Neurology 2011; 69:437-444. Denominator ICD-10 Code (Eligible G00-G99 Diseases of the nervous system Population) AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 visit 5, 10, 15, 25, Office or other outpatient o r 40 minutes fo r the evaluation and managemen t of an established patien t 45

46 Psychological intervention s for chronic headache Measure Description Percentage of patients who have gical or bio-behavioral interventions been counseled to seek psycholo fo r t of chronic headache managemen Measure Components Patients > 8 years of age who have been counseled to seek a beh avioral health Numerator Statement evaluation or are referred for psychological or bio-behavioral interventions* to manage chronic headache^. *Interventions include any of the following: Cognitive behavioral therapy, OR   Relaxation, OR  Biofeedback ^“Chronic headache” is defined as a headache occurring more tha n 15 days per month fo more than 3 months r Patients > 8 years of age diagno sed with chronic headache witho ut severe or Denominator Statement profound intellectual disability who is not currently under the care of a psychologist Denominator Patient/caregiver refuse  Exceptions Exception cannot be enforced to Active participation is essential in psychological therapy and Justification patients and families who do refuse such treatment. Lack of sup port for such me patients and therapy from insurance companies will also be a barrier, and so family may refuse as a result. d supporting ed verbatim from the reference The following statements are quot Supporting Guideline & articles: Other “Psychological treatments are effective in reducing pain intens  ity for References children and adolescents (<18 year s) with headache and benefits from 1 therapy appear to be maintained.”  ogical treatments are effective in reducing “There is evidence that psychol hat therapies pain intensity in children and adolescents with headache, and t have such as relaxation and cognitive behavioural therapy (CBT) may lasting effect for improving m ood and reducing pain for chronic headache; however, it is not possible to d istinguish effectiveness for mi graine versus 2 other types of chronic headache.”  “Children and adolescents with CDH pose a significant problem b ecause of their impairment and of the possible social costs of their head ache. Drugs, fo r b oth used as preventive medications o r as pain-killers, are insufficien t 46

47 the management of these patients; a more global approach should be 3 warranted, involving also a psychological support.” “Providers treating pediatric migraine should be routinely reco mmending  CBT as an evidence-based treatme nt strategy for decreasing pain 4 experience and improving quality of life.”  proved their “It can be safety concluded that psychological treatments have efficacy on the top level of evidence, which means that methodo logical fficacy, and well-designed randomized controlled studies exist, supporting e 5 meta-analyses confirm these results on a hierarchically higher level.”  “CBT for children with headache is effective both in the short and long term. Especially when standardized treatment programs are used, group sessions are highly effective in t erms of headache frequency, h eadache 6 duration, or headache intensity.” “Biobehavioral management is a n essential pillar of pediatric h eadache  which can be integrated into clinical management, several principles of 7 practice.”  “There is strong evidence for the efficacy of cognitive behavio ral therapy, 7 relaxation treatment, and biofee .” dback in reducing headache pain  “As in adults, psychological t herapies should be discussed with families of all children with headache as an option or complementary to pharmacological management, especi ally in the following situati ons: patients with frequent headache; c hronic daily headache with hi gh risk cant stressors; associated psyc hiatric factors for persistence; signifi disorders; overuse of medication , and intolerance to or lack of benefit from 7 appropriate drugs.” Measure Importance and adolescents is common, can b e disabling and Recurrent headache in children Relationship to Desired Outcome evidence that associated with co-morbid psychiatric conditions. There is good multidisciplinary psychological and bio-behavioral therapies are essential in the 1,2 management of recurrent headache. There are also studies showing that isolated 4,8,9 ain. medical intervention do not lead to sufficient alleviation of p Hence recommending psychological therapy for headache can lead to red uction of headache frequency, prevent futu re headache attacks, modify cog nitive and behavioral mechanisms aggravati ng pain which lead to improve fu nction and less disability. Opportunity for apies for children Several reviews have showed eff ectiveness of psychological ther 4 Improvement ce, few patients receive this intervention. with headache. Despite this eviden National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains ☐ Care Coordination ☐ Population/Public Health ☐ Efficient Use of Healthcare Resources Clinical Process/Effectiveness ☒ 47

48 Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ Purpose (Check Accountability ☒ all tha t apply) Type of Measure ☒ Process (Check all that ☐ Outcome apply) ☐ Structure Level of ☒ Individual Provider Measurement ☒ Practice (Check all that ☐ System apply) Care Setting Outpatient ☒ (Check all that ☒ Inpatient apply) ☐ Emergency Departments and Urgent Care Residential (i.e., nursing f acility, domiciliary, home care) ☐ Data Source ☒ Electronic health record (EHR) data (Check all that Administrative Data/Claims ☐ apply) ☒ Patient Medical Record Registry ☒ References 1. Eccleston C, Palermo TM, de C Williams AC, et al. Psychological therapies for the management of chronic and recurrent pain in children and adoles cents. Cochrane Database Syst Rev. 2012; 12:CD003968. 2. Clinical Answers: Are nonpharmacol ogical interventions for migr aine effective in children and adolescents? Evidence-Based Child Health a Cochrane Review Journal. 2013; 8:754-8. 3. Chiappedi M, Mensi MM, Termine C, Balottin U. Psychological the rapy in adolescents with chronic daily headache. Curr Pain Headache Rep 2016; 20:3. 4. Ernst M, O ’ Brien H, Powers S. Cognitive-Beha vioral Therapy: How medical pr oviders can increase patient and family openness and access to evidence-bas ed multimodal therapy for pediatric migraine. Headache 2015; 55:1382-96. 5. Kroner-Herwig B. Psychological treatments for pediatric headach e. Expert Review of Neurotherapeutics 2011; 11:403-410. Kropp P, Meyer B, Landgraf M, et al. Headache in children: Upda te on biobehavioral 6. treatments. Neuropediatrics 2013; 44:20-24. 7. Sieberg C, Juguet A, von Baeyer C, Seshia S. Psychological Inte rventions for Headache in Children and Adolescents. Can J Neurol Sci 2012; 39:26-34. 8. Kroner JW, Hershey AD, Kashikar-Auck SM, et al. Cognitive behav ioral therapy plus amitriptyline for children and adolescents with chronic migrain e reduces headache days to < r month. Headache 2016; 56:711-6. 4 pe 48

49 9. . Psychotherapy versus usual care in pediatric migraine Balottin U, Ferri M, Racca M, et al b t study. Ital J Pediat r 2014; 40:6. lind controlled pilo and tension-type headache: a single- ICD-10 Code Denominator (Eligible G43.7X migraine Population) R51.X headache F72 severe intellectual disability F73 profound intellectual disability AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o r 40 minutes fo r t of an established patien t the evaluation and managemen 49

50 Botulinum Toxin Serotype A (BoNT -A) for spasticity or dystonia Measure Description Percentage of patients with sp asticity or dystonia who were eva luated or referred or treated with BoNT- A Measure Components Numerator njection Patients who were evaluated OR treated OR referred for BoNT-A i Statement Denominator ith moderate to severe localize d/segmental All patients < 18 years of age w Statement spasticity or dystonia in the upper and/or lower extremities Denominator Patient/caregiver refuse  Exceptions BoNT-A is contraindicated   Patient has established care with another neurology or non-neur ology provide r tha t can evaluate the need fo r and/o r provide BoNT-A injections Exception Not all patients and parents may agree to the procedure. If a p atient has a Justification contraindication to BoNT-A, suc h as prior adverse reaction, the n they should be excluded due to risk of harm. The patient may be seeing a diffe rent practitioner for their BoNT-A injection needs making additional evaluation redun dant and urdensome. b Supporting d supporting The following statements are quot ed verbatim from the reference articles: Guideline & Other “For localized/segmental spasticity that warrants treatment, bo tulinum  References fe as an effective and generally sa toxin type A should be offered 1 treatment.”  “Consider botulinum toxin type A treatment in children and youn g people 2 in whom focal spasticity of the upper limb is: o Impeding motor function o Compromising care and hygiene o Causing pain o Impeding tolerance of other treatments, such as orthoses o Causing cosmetic concerns to the child or young person”  “Consider botulinum toxin type A t reatment where focal spastici ty of the 2 lower limb is: o Impeding gross motor function o Compromising care and hygiene o Causing pain Disturbing sleep o 50

51 o Impeding tolerance of other treatments, such as orthoses and us e of equipment to support posture Causing cosmetic concerns to o the child or young person” ople with spasticity should have access t “Children and young pe  o a network of care that uses agreed care pathways supported by eff ective to communication and integrated tea m working, and provides access . The healthcare professionals experienced in the care of such people sing, network team should provide local expertise in paediatrics, nur ise, therapy. Access to other expert physiotherapy, and occupational and including orthotics, orthopaedic surgery (and/or neurosurgery), 3 paediatric neurology, may be provided locally or regionally.” “After diagnosis, ensure that all children and young people wit h spasticity  3 are referred withou r m .” delay to an appropriate membe of the network tea t Measure Importance BoNT-A is established as an eff ective treatment for localized/s Relationship to egmental spasticity 1 and dystonia. Desired Outcome ve While there is conflicting evidence regarding its use to impro etter delivery of motor function, improving spasticity and dystonia can provide b care and hygiene, improve tolerance to other treatments (such a s orthoses and equipment to support posture), reduce pain from spasticity, red uce disturbance of sleep from pain and spasticity. 3 Opportunity for Early referral to services will allow for stimulation of motor development. Improvement National Quality Patient and Family Engagement ☐ Strategy ☐ Patient Safety Domains ☒ Care Coordination Population/Public Health ☐ ☐ Efficient Use of Healthcare Resources Clinical Process/Effectiveness ☐ Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ (Check Purpose ☒ Accountability all tha t apply) Type of Measure Process ☒ (Check all that ☐ Outcome apply) ☐ Structure Level of ☒ Individual Provider Measurement ☒ Practice System ☐ 51

52 (Check all that apply) Care Setting ☒ Outpatient (Check all that ☐ Inpatient apply) ☐ Emergency Departments and Urgent Care ☒ acility, domiciliary, home care) Residential (i.e., nursing f Data Source ☒ Electronic health record (EHR) data (Check all that ☐ Administrative Data/Claims apply) Patient Medical Record ☒ Registry ☒ References 1. Delgado MR, Hirtz D, Aisen M, et al. Practice Parameter: Pharma cologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review). Neurology 2010; 74:336-343. 2. National Institute for Health and Care Excellence. Spasticity i n children and young people with non-progressive brain disorders. Accessed on 6/22/16. 3. Mugglestone M, Eunson P, Murphy MS. Spasticity in children and young people with non- b rain disorders: summary of NICE guidance. BMJ 2012; 345:e4845. progressive Denominator ICD-10 Code (Eligible R25.2 Spasticity Population) G24.9 Dystonia AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 visit 5, 10, 15, 25, Office or other outpatient o r 40 minutes fo r the evaluation and managemen t of an established patien t 52

53 Genetic testing for global developmental delay Measure Description had genetic testing ordered fo Percentage of patients who global developmental delay (GDD) r Measure Components Numerator Patients for whom chromosomal m icroarray (CMA) was ordered. Statement Denominator logy f age with GDD* of unknown etio All children less than 6 years o Statement ills of more than 2 Standard De viations below *GDD defined as developmental sk age-matched peers in 2 or more lopment (motor, aspects of the 5 domains of deve 1 speech and language, cognitive, social, adaptive). Denominator Patient/caregiver refuse  Exceptions  Referred to o r unde r the care of a geneticist Exception Parental/caregiver approval is necessary in proceeding with gen etic testing. If a Justification patient has an established gene ional evaluation is ticist providing services, addit redundant and burdensome. d supporting ed verbatim from the reference The following statements are quot Supporting articles: Guideline & Other  “Microarray testing is abnormal on average in 7.8% of subjects with 1 References ).” GDD/ID and in 10.6% of those w ith syndromic features (Class III  “Karyotype studies are abnormal in at least 4% of subjects with GDD/ID 1 and in 18.6% of those with syndromic features (Class II and III ).”  DD/ID, in “StFISH testing is abnormal in at least 3.5% of subjects with G 5% of those at least 4.2% of those with syndromic features, in as few as 0. te/severe with mild impairment, and in at least 7.4% of those with modera 2 impairment (Class I, II, and III).”  “Mutation in X-linked genes ma y explain up to 10% of all cases of GDD/ID. Testing of XLID genes has a yield of 42% in males from definitely X-linked families and of 17% in males from possibly X-linked 2 families (Class III).” e  “MeCP2 mutations are found in 1.5 % of girls with moderate/sever 1 GDD/ID and in less than 0.5% of males with GDD/ID (Class III).” 53

54  “Screening for IEMs in children een 0.2% with GDD/ID has a yield of betw and 4.6%, depending on the presence of clinical indicators and the range of 2 testing performed (Class III).” “Testing for CDGs has a yield of up to 1.4%, and testing for cr eatine  1 synthesis and transport disorder ass III).” s has a yield of up to 2.8% (Cl  with the appropriate genetic t esting, as “...Confirm the clinical diagnosis 3 warranted by clinical circumstances.”  “If a specific diagnosis is suspected, arrange for the appropri ate diagnostic studies to confirm including si ngle-gene tests or chromosomal m icroarray 3 test.” “If diagnosis is unknown and no suspected,  clinical diagnosis is strongly begin the stepwise evaluation process: Chromosomal microarray should be performed in all o 3 Fragile X genetic testing s hould be performed in all” o  “If no diagnosis established: o Male gender and family history suggestive X-linkage, complete XLID panel that contains genes causal of nonsyndromic XLID and complete high-density X-CMA. Consider X-inactivation skewing in the mother of the proband. Female gender: complete MECP 2 deletion, duplication, and o 3 sequencing study”  “It is important to emphasize the new role of the genomic micro array as a the child with an first-line test, as well as the renewal of efforts to identify 3 inborn error of metabolism.”  gher diagnostic yield (15%-20%) for geneti c testing “CMA offers a much hi ded DD/ID, ASD, or MCA than a G-ban of individuals with unexplained syndrome and other recognizable karyotype (~3%, excluding Down chromosomal syndromes), primar ily because of its higher sensiti vity for 4 submicroscopic deleti ons and duplications.”  “Available evidence strongly supports the use of CMA in place o f G- tier cytogenetic diagnostic tes banded karyotyping as the first- t for patients with DD/ID, ASD, or MCA. G-bande d karyotype analysis should be reserved for patients with obvious chromosomal syndromes (e.g., Down syndrome), a family history of chromosomal rearrangement, or a history of 4 multiple miscarriages.” Measure Importance Relationship to ads to specific While identifying the genetic cause of GDD only occasionally le Desired Outcome formation regarding therapy, establishing an etiologic diagnosis will a) provide in symptomatic management and/or sur veillance for known complicati ons; b) provide validation of the medical probl em and empower families to advoc ate for their child; c) assist family in obtaining services particularly in schools and condition specific family support; d) provide information on the genetic mechanism and recurrence risks; e) prevent unnecessary or redundant diagnostic tests; f) provide access to possible research treatmen t protocols. Opportunity for initial clinical At least 30-50% of cases of GDD/ID remain unexplained after an 4 Improvement evaluation b y a neurologis t o r geneticist. 54

55 National Quality ☐ Patient and Family Engagement Strategy ☐ Patient Safety Domains Care Coordination ☐ Population/Public Health ☐ ☐ Efficient Use of Healthcare Resources Clinical Process/Effectiveness ☒ Harmonization N/A with Existing Measures Measure Designation Measure Quality improvement ☒ Purpose (Check ☐ Accountability all tha apply) t Type of Measure ☒ Process (Check all that Outcome ☐ apply) ☐ Structure Level of ☒ Individual Provider Measurement ☒ Practice (Check all that System ☒ apply) Care Setting ☒ Outpatient (Check all that ☐ Inpatient apply) ☐ Emergency Departments and Urgent Care Residential (i.e., nursing f acility, domiciliary, home care) ☐ Data Source Electronic health record (EHR) data ☒ (Check all that ☐ Administrative Data/Claims apply) ☒ Patient Medical Record ☒ Registry References 1. tic yield of the neurologic asse ssment of the Majnemer A, Shevell MI. Diagnos developmentally delayed child. J Pediatr 1995; 127:193-9. 2. EH, et al. Evidence report: Gen etic and metabolic testing on Michelson DJ, Shevell MI, Sherr children with global developmen tal delay. Neurology 2011; 77:16 29-1635. 3. Moeschler JB, Shevell M. Comprehen sive Evaluation of Child with intellectual disability or Global developmental delays. P ediatrics 2014; 134:e903-918. 4. Miller DT, Adam MP, Aradhya S, et al. Consensus Statement: Chro mosomal Microarray is the first-tier clinical diagnos tic test for individuals with De velopmental Disabilities or 2010; 86:749-764. t congenital anomalies. Am J Hu m Gene 55

56 5. , et al. Evaluation of mental retardation: Curry CJ, Stevenson RE, Aughton D onference: American College of Medical Genetics. Am. J. recommendations of a Consensus C Med. Genet. 1997;72:468–477. Denominator ICD-10 Code F88.X Global developmental delay (Eligible Population) AND CPT E/M Service Code 99201, 99202, 99203, 99204, 99205 Office or other outpatient visit 10, 20, 30, 45, or 60 minutes for the evaluation and management of a new patien t; 99211, 99212, 99213, 99214, 99215 Office or other outpatient visit 5, 10, 15, 25, o r r the evaluation and managemen t 40 minutes fo t of an established patien 56

57 Contact Information For more information about quality measures please contact: American Academy of Neurology 201 Chicago Ave Minneapolis, MN 55415 Phone: (612) 928-6100 Fax: 612-454-2744 [email protected] Abu-Arafeh I, Razak S, Sivaraman B, Graham C. Prevalence of hea dache and migraine in 1. children and adolescents: a system atic review of population-bas ed studies. Developmental Medicine & Child Neurology 2010; 52:1088-97. 2. Chiappedi M, Mensi MM, Termine C, Balottin U. Psychological The rapy in Adolescents with Chronic Daily Headache. Curr Pain Headache Rep 2016; 20:3. 3. Cath D, Hedderly T, Ludolph A, et al. European clinical guideli nes for Tourette Syndrome and other tic disorders. Part I: asse ssment. Eur Child Adolesc Psyc hiatry 2011; 20:155-171. 4. Tourette Syndrome Fact Sheet, NIH /tourette/detail_tourette.htm http://www.ninds.nih.gov/disorders Accessed on 4/20/16 5. Knight T, Steeves T, Day L, et al. Prevalence of Tic Disorders: A Systematic Review and Meta- Analysis. Pediatric Neurology 2012; 47:77-90. Widjaja E, Go C, McCoy B, Snead O. Neurodevelopmental outcome o f infantile spasms: A 6. systematic review and meta-ana lysis. Epilepsy Research 2015; 10 9:155-162. 7. Ryan N, Coryell J, Mytinger J, e t al. The National Infantile Sp asms Consortium (NISC): Moving Towards Standardization of Car omes in Infantile Spasms. e and Improved Treatment and Outc Epilepsy Currents 2015; 15:490. 8. Glauser T, Shinnar S, Gloss D, et al. Evidence-based Guideline: Treatment of Convulsive Status Epilepticus in Children and Adults : Report of the Guideline Com mittee of the American Epilepsy Society. Epilepsy Currents 2016; 16:48-61. 9. Cerebral Palsy fact Sheet, NIH. /cerebral_palsy/detail_cerebr al_palsy.htm#3104_2 http://www.ninds.nih.gov/disorders 57

58 10. Shamsoddini A, Amirsalari S, Hollisaz M-T, Rahimnia A, Khatibi- Aghda A. Management of Spasticity in Children with Cerebral Palsy. Iranian Journal of Pediatrics . 2014; 24(4):345-351. 11. Michelson D, Shevell M, Sheer E, et al. Evidence Report: Geneti c and metabolic testing on children with global developmen tal delay. Neurology 2011; 77:16 29-1635. 12. Kaufman L, Ayub M, Vincent JB. The genetic basis of non-syndrom ic intellectual disability: a review. J Neurodev Disord. 2010 Dec; 2(4):182–209. 13. Rauch A, Hoyer J, Guth S, et al . Diagnostic yield of various ge netic approaches in patients with unexplained developmental delay or Genet A. 2006 Oct 1; mental retardation. Am J Med 140(19):2063–74. 14. Riggs E, Wain K, Riethmaier D, e t al. Chromosomal microarray im pacts clinical management. Clin Genet. 2013 Feb;85(2):147–53. 15. American Academy of Pediatrics. Clinical Report – Supporting th e Health Care Transition from 128: 182-200. Medical Home. Pediatrics 2011; Adolescence to Adulthood in the 58

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