The spectrum of indomethacin-responsive headaches in children and adolescents.

BACKGROUND: Headaches with marked, specific response to indomethacin occur in children, but the phenotypic spectrum of this phenomenon has not been well-studied. METHODS: We reviewed pediatric patients with headache showing ≥80% improvement with indomethacin, from seven academic medical centers. RESULTS: We included 32 pediatric patients (16 females). Mean headache onset age was 10.9 y (range 2-16 y). Headache syndromes included hemicrania continua (n = 13), paroxysmal hemicrania (n = 10), primary stabbing headache (n = 2), short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (n = 1), primary exercise headache (n = 1) and primary cough headache (n = 1). Adverse events were reported in 13, most commonly gastrointestinal symptoms, which often improved with co-administration of gastro-protective agents. CONCLUSION: Indomethacin-responsive headaches occur in children and adolescents, and include headache syndromes, such as primary cough headache, previously thought to present only in adulthood. The incidence of adverse events is high, and patients must be co-treated with a gastroprotective agent.

Approach to Pediatric Intractable Migraine.

PURPOSE OF REVIEW: Intractable migraine in children and adolescents is a significant cause of disability and decreased quality of life (QoL) in this population. Challenges include lack of unifying definition for intractable migraine, and limited data on best-practice management in this age group, with most current treatment pathways extrapolated from adult studies or expert consensus. RECENT FINDINGS: A comprehensive approach in the evaluation and management of intractable migraine in this age group encompasses excluding secondary causes of headache; making an accurate diagnosis; identifying and appropriately managing modifiable risk factors; and initiating appropriate pharmacologic therapy to reduce disability, improve health-related quality of life, reduce risk of progression, and develop adaptive pain coping strategies. Several strategies for management of pediatric intractable migraine including use of acute medications, bridge therapy in outpatient setting, emerging therapies for preventive therapy, and a stepwise combination therapy for management of pediatric intractable migraine in emergency and inpatient setting are presented based on available clinical data, safety/tolerability, availability, cost-effectiveness, and expert consensus. This descriptive review of the available literature focuses on approach to therapy for acute intractable migraine in a pediatric population including outpatient, emergency department (ED), and inpatient management.

Nutraceuticals and Behavioral Therapy for Headache.

PURPOSE OF REVIEW: Headache affects and disables at least 1 billion people worldwide. Patients and providers seek new therapies to relieve headache without the side effects and financial burden of current treatments. This narrative review highlights recent treatment advances in integrative headache medicine: nutraceuticals and behavioral therapies. RECENT FINDINGS: Growing use of complementary and alternative medicine (CAM) therapies for headache (riboflavin, coenzyme Q10, magnesium, vitamin D, melatonin) alongside mainstream treatments is increasing with improving evidence of quality, safety, and tolerability. Increasing interest in medical cannabis is tempered by lack of evidence regarding safety and efficacy. Behavioral therapies including cognitive behavioral therapy (CBT), biofeedback, mindfulness-based stress reduction (MBSR), and acceptance and commitment therapy (ACT) improve patient resiliency and self-efficacy outcomes and reduce disability. The body of evidence for nutraceutical and behavioral CAM interventions for headache continues to grow and improve in quality. Providers and patients should educate themselves regarding CAM therapies as part of integrative headache management. Future studies should examine combinatorial trials of CAM therapies against current standards of headache care.

The intersection of COVID-19, school, and headaches: Problems and solutions.

OBJECTIVE: To equip clinicians with recommendations specific to concerns related to the novel coronavirus disease 2019 (COVID-19), which impact the physical, emotional, and social health of youth with headache disorders. BACKGROUND: COVID-19 has affected societies on a global scale including children and youth with chronic headache disorders. Many concerns are predicted to arise in the 2020-2021 school year, whether classes are conducted in-person or virtually. METHODS: Clinical impressions were combined with a review of the literature, although limited due to the recent nature of this issue. RESULTS: We describe recommendations to support caregivers and youth as they face changes expected with the return to school in the fall of 2020. CONCLUSION: Although there are significant concerns for caregivers and youth with migraine given the context of changes related to the pandemic, there are many recommendations that can help minimize exacerbations of the physical, emotional, and social health of youth with chronic migraine.

Migraine Care in the Era of COVID-19: Clinical Pearls and Plea to Insurers.

OBJECTIVE: To outline strategies for the treatment of migraine which do not require in-person visits to clinic or the emergency department, and to describe ways that health insurance companies can remove barriers to quality care for migraine. BACKGROUND: COVID-19 is a global pandemic causing widespread infections and death. To control the spread of infection we are called to observe "social distancing" and we have been asked to postpone any procedures which are not essential. Since procedural therapies are a mainstay of headache care, the inability to do procedures could negatively affect our patients with migraine. In this manuscript we review alternative therapies, with particular attention to those which may be contra-indicated in the setting of COVID-19 infection. DESIGN/RESULTS: The manuscript reviews the use of telemedicine visits and acute, bridge, and preventive therapies for migraine. We focus on evidence-based treatment where possible, but also describe "real world" strategies which may be tried. In each section we call out areas where changes to rules from commercial health insurance companies would facilitate better migraine care. CONCLUSIONS: Our common goal as health care providers is to maximize the health and safety of our patients. Successful management of migraine with avoidance of in-person clinic and emergency department visits further benefits the current urgent societal goal of maintaining social distance to contain the COVID-19 pandemic.

Headache Hygiene in Pediatrics: Brushing Up on the Basics.

Headache hygiene refers to self-management behaviors and practices aimed at reducing headache-related disability and improving self-efficacy. Although self-management interventions have an established place in the management of a wide range of chronic conditions, there is still not a standardized approach to this in pediatric headache. In this article, we focus on headache hygiene approaches including education, lifestyle interventions, and psychologic interventions. We also present our center's resource compilation, made available to patients by quick response code technology, as an example of a structured approach to headache hygiene. Further work should explore a standardized approach to headache hygiene and strategies to support adherence, including the use of technology as an innovative health care delivery pathway.

Pseudotumor Cerebri Syndrome in Children.

PURPOSE OF REVIEW: Pseudotumor cerebri syndrome (PTCS) may affect both children and adults; however, the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS. RECENT FINDINGS: Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cut-off of 25 cm H20 (or 28 cm H2O in a sedated or obese child). Morbidity related to visual loss, pain and reduced quality of life lends urgency towards accurately identifying, evaluating and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.

Occipital Neuralgia and Cervicogenic Headache: Diagnosis and Management.

PURPOSE OF REVIEW: Occipital neuralgia (ON) and cervicogenic headache (CGH) are secondary headache disorders with occipital pain as a key feature. Due to significant phenotypic overlap, differentiating ON and CGH from primary headache disorders such as migraine or tension-type headache, or other secondary headache disorders, can be clinically challenging. This article reviews the anatomy, clinical features, unique diagnostic considerations, and management approaches relating to ON and CGH. RECENT FINDINGS: Conservative therapeutic approaches are considered first-line. Anesthetic nerve blocks may have a dual role in both supporting diagnosis and providing pain relief. Newer minimally invasive procedures, such as pulsed radiofrequency (PRF) and occipital nerve stimulation (ONS), represent an exciting therapeutic avenue for severe/refractory cases. Surgical interventions should be reserved for select patient populations who have failed all other conservative and minimally invasive options, to be weighed against potential risk. ON and CGH represent an ongoing diagnostic challenge. Further studies are required to consolidate efficacy regarding the comprehensive management of ON and CGH.

A New Patient With Intermediate Severe Salla Disease With Hypomyelination: A Literature Review for Salla Disease.

BACKGROUND: Likely pathogenic variants in SLC17A5 results in allelic disorders of free sialic acid metabolism including (1) infantile free sialic acid storage disease with severe global developmental delay, coarse facial features, hepatosplenomegaly, and cardiomegaly; (2) intermediate severe Salla disease with moderate to severe global developmental delay, hypotonia, and hypomyelination with or without coarse facial features, and (3) Salla disease with normal appearance, mild cognitive dysfunction, and spasticity. PATIENT DESCRIPTION: This five-year-old girl presented with infantile-onset severe global developmental delay, truncal hypotonia, and generalized dystonia following normal development during her first six months of life. Brain magnetic resonance imaging showed marked hypomyelination and a thin corpus callosum at age 19 months, both unchanged on follow-up at age 28 months. Urine free sialic acid was moderately elevated. Cerebrospinal fluid free sialic acid was marginally elevated. Sequencing of SLC17A5 revealed compound heterozygous likely pathogenic variants, namely, a known missense (c.291G>A) variant and a novel truncating (c.819+1G>A) variant, confirming the diagnosis of Salla disease at age 3.5 years. CONCLUSION: We report a new patient with intermediate severe Salla disease. Normal or marginally elevated urine or cerebrospinal fluid free sialic acid levels cannot exclude Salla disease. In patients with progressive global developmental delay and hypomyelination on brain magnetic resonance imaging, Salla disease should be included into the differential diagnosis.

Recurrent Gastrointestinal Disturbance: Abdominal Migraine and Cyclic Vomiting Syndrome.

Primary headache disorders, including migraine, are some of the most common neurological disorders presenting to hospital. Episodic syndromes that may be associated with migraine, including recurrent gastrointestinal disturbances such as abdominal migraine and cyclic vomiting, often pre-date or co-occur with the onset of migraine in a child who is at risk of developing the headache condition. The purpose of this review is to evaluate the two most common episodic syndromes, abdominal migraine and cyclic vomiting syndrome, including their pathophysiology, common presentations, and diagnostic criteria. Differential diagnosis and "red flag" features are outlined, and an approach to diagnostic work-up is offered. Finally, we provide an evidence-based review of management options and long-term prognosis. Future research should include randomized trials for the acute and preventive treatment of these disorders, as well as research as to whether early intervention can prevent progression to migraine and/or mitigate migraine severity.

A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice.

BACKGROUND: Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. METHODS: A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. RESULTS: There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. CONCLUSION: We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.