Diagnostic Algorithm for Pediatric Headaches: A Clinical Improvement Initiative.

BACKGROUND: The widespread variation in diagnosing primary headache disorders in children and adolescents results in reduced quality and high costs. Defining an algorithm for primary headache diagnoses in children and adolescents is part of a larger initiative to standardize and improve care. The aim of this algorithm was to increase the accuracy of headache diagnosis by formal criteria to more than 80% of patient encounters. METHODS: A team of headache specialists, nurse practitioners, nurses, data analysts, and business specialists developed an algorithm based on available scientific evidence. This algorithm was vetted and adapted by the neurology faculty and headache specialists until final consensus was reached. Following three months of testing and validation, the algorithm was disseminated to general pediatric neurology clinics. The following information was gathered: percent of encounters utilizing the algorithm, percentage of encounters with appropriate diagnosis by formal criteria, percentage of encounters with appropriate testing ordered, and average cost per headache visit. RESULTS: Correct diagnosis of primary headache by International Classification of Headache Disorders-3 criteria improved from 72% to 90% and appropriate testing improved from 80% to 94%. By the end of analysis, 94% of encounters were correctly implementing the algorithm. A year-long tracking revealed decreased cost of headache evaluation by 6% compared with the year prior. CONCLUSIONS: A standardized algorithm improved the diagnostic accuracy in general child neurology clinics. Expanding the algorithm to primary care and pediatric emergency rooms could have a greater impact on headache evaluation and diagnosis; this should result in improved care and outcomes with reduced cost.

Clinical Reasoning: A Teenager With Right-Sided Headache and Periorbital Changes.

While migraine is the most common headache disorder in children and adolescents presenting to a neurologist, other primary headache disorders are important to recognize. Trigeminal autonomic cephalalgias represent a rare group of primary headache disorders with different characteristics, workup, and management. In this study, we present an adolescent with 1 common and 1 unique headache phenotype, followed by a guided discussion of the differential diagnoses, workup, treatments, and a brief summary of further management considerations.

Management of Chronic Migraine in Children and Adolescents: Where are We in 2022?

Migraine is a neurological disorder that affects millions of children and adolescents worldwide. Chronic migraine is a subtype of migraine in which patients experience headaches for more days than not each month, with accompanying symptoms of phonophobia, photophobia, nausea or vomiting for most of these headaches. The burden and impact of chronic migraine in the daily lives of children and adolescents is substantial, requiring a holistic, multidisciplinary, and biopsychosocial approach to conceptualization and treatment. The purpose of this review is to provide a comprehensive "2022" overview of acute and preventive treatments for the management of chronic migraine in youth. We first describe diagnostic criteria for chronic migraine and highlight the state of evidence for acute and preventive treatment in children and adolescents. We then discuss emerging treatments currently receiving rigorous clinical research effort, special considerations for the treatment of chronic migraine in children and adolescents, and avenues for improving existing treatments and expanding access to evidence-based care.

The diagnostic spectrum of ATP1A3-related disorders: 3 new patients.

BACKGROUND: ATP1A3-related disorders are rare but increasingly recognized syndromes with overlapping phenotypes. CLINICAL OBSERVATIONS: A male child and his mother with c.2452G>A (p.Glu818Lys) mutation and an unrelated child with c.2428A>T (p.Ile810Phe) mutation in the ATP1A3 gene are reported. RESULTS: The first child presented with fever-induced flaccid unresponsiveness and the diagnosis was made after extensive negative workup except for abnormal EMG showing low amplitude motor responses with acute denervation; his symptomatic mother went undiagnosed for thirty years until his diagnosis. An unrelated male child presented with symptoms most consistent with the rapid-onset dystonia-Parkinsonism (RDP) phenotype but with intermediate features of alternating dystonia with choreoathetoid movements two years after a c.2428A>T (p.Ile810Phe) mutation was found. CONCLUSION: ATP1A3-related disorders have variable manifestations and can remain undiagnosed for decades. Treatment remains mostly supportive. With the increasing use of genetic testing for broad indications, further research into effective therapies is necessary.

Risk factors for subsequent febrile seizures in the FEBSTAT study.

OBJECTIVES: To identify risk and risk factors for developing a subsequent febrile seizure (FS) in children with a first febrile status epilepticus (FSE) compared to a first simple febrile seizure (SFS). To identify home use of rescue medications for subsequent FS. METHODS: Cases included a first FS that was FSE drawn from FEBSTAT and Columbia cohorts. Controls were a first SFS. Cases and controls were classified according to established FEBSTAT protocols. Cumulative risk for subsequent FS over a 5-year period was compared in FSE versus SFS, and Cox proportional hazards regression was conducted. Separate analysis examined subsequent FS within FSE. The use of rescue medications at home was assessed for subsequent FS. RESULTS: Risk for a subsequent FSE was significantly increased in FSE versus SFS. Any magnetic resonance imaging (MRI) abnormality increased the risk 3.4-fold (p < 0.05), adjusting for age at first FS and FSE and in analyses restricted to children whose first FS was FSE (any MRI abnormality hazard ratio [HR] 2.9, p < 0.05). The risk for a second FS of any type or of subsequent FS lasting >10 min over the 5-year follow-up did not differ in FSE versus SFS. Rectal diazepam was administered at home to 5 (23.8%) of 21 children with subsequent FS lasting ≥10 min. SIGNIFICANCE: Compared to controls, FSE was associated with an increased risk for subsequent FSE, suggesting the propensity of children with an initial prolonged seizure to experience a prolonged recurrence. Any baseline MRI abnormality increased the recurrence risk when FSE was compared to SFS and when FSE was studied alone. A minority of children with a subsequent FS lasting 10 min or longer were treated with rectal diazepam at home, despite receiving prescriptions after the first FSE. This indicates the need to further improve the education of clinicians and parents in order to prevent subsequent FSE.