Probable New Daily Persistent Headache After COVID-19 in Children and Adolescents.

INTRODUCTION: Headache has been cited as both a primary symptom and a sequela of infection with the novel coronavirus. Cases of long coronavirus disease (COVID) headache have already been documented in adults, but literature on similar cases in children and adolescents is scant. CASE REPORT: We present three cases of persistent headache after infection with COVID-19 in pediatric patients presenting to a tertiary headache center. CONCLUSION: Infection has been suggested as a trigger for chronic headaches, specifically those of the new daily persistent headache type. Although the association between new daily persistent headache and COVID-19 remains unclear, these cases highlight the importance of awareness of the neurological sequelae of novel coronavirus infection in children and adolescents.

A Single Institution's Experience of Primary Headache in Children With Celiac Disease.

BACKGROUND: Few studies exist examining the frequency of primary headache in children with celiac disease and the impact of a gluten-free diet on primary headache symptomology. This study explores characteristics and frequency of headaches in children with celiac disease and response to gluten-free diet at a single institution. METHODS: Medical records were reviewed for children with celiac disease confirmed by the presence of elevated tissue transglutaminase IgA levels and histologic changes consistent with the diagnosis of celiac disease on small bowel biopsy. Eligible participants were contacted via letter for participation in a phone survey regarding headaches. Phone interviews were conducted 2 weeks after notification and lasted approximately 10 minutes. Headaches were classified according to ICHD-3 criteria. RESULTS: 247 eligible patients or their families were contacted. A total of 132 (53.44%) agreed to participate. One participant was excluded due to insufficient information provided. Overall, 51 of 131 participants had recurrent headache defined as at least 1 episode per month (39%, 95% confidence interval [CI]: 31%-47%) and 33 had migraine with or without aura (25%, 95% CI: 18%-33%). Twenty-eight had frequent tension-type headache (22%, 95% CI: 15%-29%). Thirty-two participants noted headaches before a confirmed diagnosis of celiac disease. Twenty-two of 32 participants (68.75%) noticed decreased headache frequency or intensity, or both, after starting the gluten-free diet. CONCLUSION: This study suggests that at least one-third of children and adolescents with celiac disease have recurrent headaches at the time of diagnosis. A gluten-free diet led to improved headache symptomology in a significant number of these patients.

The Clinical Spectrum of Mosaic Neurofibromatosis in Children and Adolescents.

BACKGROUND: Segmental neurofibromatosis was initially described by Miller and Sparks (1977) as manifestations of neurofibromatosis limited to a dermatomal, localized distribution. Now termed mosaic neurofibromatosis, previous literature described this disease in children and adolescents with individual case reports and small-numbered case series. This study presents a large series of children and adolescents with mosaic neurofibromatosis. METHODS: A retrospective chart review of a single institution medical record database was performed on all cases of mosaic neurofibromatosis diagnosed between the years 1998 and 2017. Eligible subjects were determined by 2 criteria: (1) segmental or unilateral expression of one of more signs of NF I according to those outlined in the NIH criteria and (2) were under 18 years of age at the time of diagnosis. Select information extracted include location of clinical features, NF manifestations (neurofibromas, plexiform neurofibromas, café-au-lait spots, freckling, Lisch nodules), presence of a diffuse area of cutaneous hyperpigmentation, and other significant medical conditions. RESULTS: Sixty-eight cases met established criteria. Average age at diagnosis was 8.28 ± 4.47 years. Thirty-seven (54%) were male and 31 (46%) were female. Localization of the dermatologic manifestations is as follows: left side in 28 (41%) cases, right side in 32 (47%) cases, and bilateral in 8 (11%) cases. Café-au-lait lesions appeared in 64 (94%) of cases and 14 (21%) had axillary and inguinal freckling. CONCLUSIONS: This study expands our understanding of the disease characteristics seen in children and adolescents with mosaic neurofibromatosis and confirms the need to focus on pigmentary changes in children with mosaic neurofibromatosis.

Migraine Variants in Children.

Migraine in children can manifest in ways that are markedly different from adult migraines. In children, migraine variants are often unaccompanied by headache and include conditions such as cyclic vomiting and abdominal migraine. Children who experience these conditions are often thought to have a disorder of the gastrointestinal tract, and when evaluation is unremarkable they may be diagnosed as having a conversion reaction. Complicated migraines, on the other hand, are often accompanied by focal neurological symptoms such as ataxia, hemiparesis, or altered level of consciousness that evoke great consternation in the examining clinician. Certain episodic syndromes that may hold interest to pediatricians are also discussed in this article, mostly to emphasize the ambiguity that still surrounds these disorders, such as migraine triggered by trauma. The cardinal rule that most of these disorders are diagnoses of exclusion and can only be confirmed after extensive evaluation, either by the pediatrician or pediatric neurologist, is emphasized. [Pediatr Ann. 2018;47(2):e50-e54.].

Migraine With Neurological Features in a Scuba Diver With a Patent Foramen Ovale.

Neurologic complications of decompression sickness have been observed for over half of a century. Little is known, however, about the risk of diving in patients that suffer from migraine with aura (MWA). We report the case of a pediatric patient with a history of migraine with aura, who was later found to have a PFO, who developed headache with neurological symptoms during a scuba diving lesson.

A Series of 211 Children with Probable Essential Tremor.

BACKGROUND: The objective of this study was to characterize the clinical features, course, and treatment of essential tremor (ET) in children. METHODS: A retrospective chart review was conducted over 25 years (1984-2011). Inclusion criteria were age <21 years and satisfying the core diagnostic criteria for ET. Primary exclusion criteria included other neurological findings, tremorogenic medications, sudden onset/stepwise progression, primary orthostatic tremor, and isolated task specificity; and secondary exclusion criteria were abnormal neuroimaging or metabolic/thyroid studies. Cases were reviewed for age, sex, family history, tremor characteristics, functional impairment, treatment, and follow-up. RESULTS: In total, 211 children had ET, including 130 males and 81 females. The mean ± standard deviation age was at diagnosis was 14.09 ± 5.0 years, the age of onset was 9.71 ± 5.62 years, and the age of onset was birth in 7 children. One hundred ninety-nine children had bilateral hand tremor, 34 had asymmetry, 9 had unilateral onset but later became bilateral. Twelve children had voice tremors, 13 had leg tremors, 5 had head tremors, and 7 had trunk tremors. Tremor at rest was present in 20 children. Thirty-five percent of the children had a family history of ET, including in a father (n = 21), mother (n = 13), brother (n = 6), sister (n = 3), and other family member (n = 28). Fifty-five percent of patients had functional disabilities, including writing (n = 66), eating (n = 28) drinking from a cup (n = 13), typing (n = 4), playing instruments (n = 6), buttoning (n = 6), and playing (n = 3). For treatment, 33 patients received propranolol, 1 received atenolol, 13 received primidone, 3 received metoprolol, and 1 received nadolol. In total, 99 patients were followed for a mean ± standard deviation of 1.82 ± 2.21 years.If left untreated, tremor remained unchanged in 33 patients, and 7 had an apparent short-term improvement. On propranolol, 15 of 20 patients significantly improved. CONCLUSIONS: This is the largest series to date of ET in children. The current findings indicate that onset at birth is possible, family history is less common in children than in adults, and tremor at rest is possible. Functional disability was noted in 55% of children, and 29.4% required medication.

Migraine Variants or Episodic Syndromes That May Be Associated With Migraine and Other Unusual Pediatric Headache Syndromes.

PURPOSE: To provide an overview of the clinical course for children and adolescents with migraine variants (M.V.), childhood periodic syndromes or the episodic syndromes that may be associated with migraine using the International Classification of Headache Disorders, 3rd Edition Beta version [ICHD-3] International Headache Society criteria for the diagnosis of each disorder. METHOD: Migraine is a complex set of neurological symptoms. This review encompasses the subtypes of M.V. or episodic syndromes that may be associated with migraine within the children and adolescent population. CONCLUSION: The episodic syndromes that may be associated with migraine or migraine variant is multilayered neurological disorder in young children and adolescents. Within the these generally pediatric syndromes there are associated disorders described in this review, to provide a clinical overview and including the less common forms of migraine, such as acute confusional migraine, trauma-triggered migraine, and transient global amnesia.

Massive Soft Tissue Neurofibroma (Elephantiasis Neuromatosa): Case Report and Review of Literature.

The authors review the literature on massive soft tissue neurofibroma. The methods included a review of 71 reports (PubMed search 1929-2012) with a total of 91 massive soft tissue neurofibroma patients and illustration of clinical and radiological progression of massive soft tissue neurofibroma on a patient with neurofibromatosis type 1. The mean age at initial examination was 21 years. Tumor onset was mostly in childhood years. The commonest affected body segment was the lower extremity (46%), followed by head/neck (30%). Surgical management was pursued in the majority of cases (79%). Bleeding was a common complication (25%). Recurrence was described in 12%; multiple resections cases were described. Malignant transformation occurred in 5%. Although massive soft tissue neurofibroma may be present early in life, massive tumor overgrowth may take years. Predicting disease progression and/or benefit of surgical intervention early in the disease course is challenging. Recurrence and malignant transformation are possible. Massive soft tissue neurofibroma does not respond to chemotherapy or radiotherapy and is associated with life-threatening surgical complications.

Sleep disorders in children with Tourette syndrome.

BACKGROUND: The objective of this study was to determine the frequency, nature, and impact of sleep disorders in children and adolescents with Tourette syndrome and to raise awareness about their possible inclusion as a Tourette syndrome comorbidity. METHODS: Using a prospective questionnaire, we interviewed 123 patients of age ≤21 years with a confirmed diagnosis of Tourette syndrome. Each completed questionnaire was then reviewed in accordance with Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, criteria for categorization to a form of sleep disorder. RESULTS: Of the 123 patients with Tourette syndrome, 75 (61%) had comorbid attention deficit hyperactivity disorder and 48 (39%) had Tourette without attention deficit hyperactivity disorder. The sleep problems observed included problems in the nature of sleep, abnormal behaviors during sleep, and impact of sleep disturbances on quality of life. Within these cohorts, 31 (65%) of the 48 Tourette-only patients and 48 (64%) of the 75 Tourette + attention deficit hyperactivity disorder patients could fit into some form of Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, coded sleep disorders. Of the 48 Tourette + attention deficit hyperactivity disorder patients with sleep disorders, 36 (75%) had insomnia signs, which could be explained by the co-occurrence of attention deficit hyperactivity disorder and high stimulant use. However, 10 (32%) of the 31 Tourette-only patients with sleep disorders had insomnia irrespective of attention deficit hyperactivity disorder or medication use. CONCLUSIONS: Sleep problems are common in children with Tourette syndrome irrespective of comorbid attention deficit hyperactivity disorder, justifying their inclusion as a comorbidity of Tourette syndrome.

Approach to the diagnosis and treatment of cyclic vomiting syndrome: a large single-center experience with 106 patients.

BACKGROUND: Cyclic vomiting syndrome is characterized by repeated, stereotypical vomiting episodes. The diagnosis is made by exclusion of other organic diseases, which can lead to extensive testing. It has been suggested that these patients can have mitochondrial dysfunction. The aim of the study was to examine the evaluation of our cyclic vomiting patients and to determine whether they had associated, undiagnosed metabolic abnormalities. METHODS: This retrospective study included 106 patients aged <21 years at diagnosis. Information regarding medical history, laboratory, and imaging studies were collected. Metabolic studies in plasma and urine were obtained when patients were well and when patients were in a vomiting cycle, including plasma amino acids, acylcarnitines, and urine organic acids. RESULTS: The mean age at diagnosis was 8.9 ± 5.0 years. Neuroimaging revealed previously unknown intracranial abnormalities in <10% of patients, none of whom explained the vomiting signs. Abdominal ultrasounds revealed abnormalities in 15% of patients during an acute episode and 7% of patients when well. Sixty-one patients had an upper gastrointestinal series, all of which were normal. A total of 92% of patients had laboratory testing with 38% indicating abnormalities possibly suggesting mitochondrial dysfunction. CONCLUSIONS: This large, single-center study further evaluated the need for more focused evaluation in patients with suspected cyclic vomiting syndrome. Thirty-eight percent of our patients had abnormalities in blood and/or urine suggesting mitochondrial dysfunction, which requires more detailed investigation in the future.

Diagnosis and treatment of idiopathic intracranial hypertension (IIH) in children and adolescents.

Idiopathic intracranial hypertension (IIH) is characterized by symptoms and signs of elevated intracranial pressure, elevated cerebrospinal fluid (CSF) pressure, normal CSF content, and normal brain with normal or small ventricles on neuroimaging studies. IIH in children has a wide spectrum of clinical presentation. Diagnostic criteria with modifications to adapt to the variations in children are discussed. Diagnostic and therapeutic options are reviewed.

Cerebral vasculopathy in children with neurofibromatosis type 1.

Cerebral vasculopathy is an important but underrecognized complication of neurofibromatosis type 1. Over a 10-year period, we retrospectively assessed the prevalence, clinical manifestations, management, and outcome of cerebral vasculopathy in children with neurofibromatosis type 1. Magnetic resonance imaging (MRI) of the brain was performed on 78% of the patients (312/398) of which 46% (143/312) had magnetic resonance angiography of the intracranial arteries; 4.8% (15/312) had cerebral vasculopathy. Approximately half were asymptomatic at presentation; none had neurologic deficits. Cerebral vasculopathy included moyamoya changes (7) and stenosis/occlusion of major intracranial arteries (8). On follow-up (mean 4 years), 2 patients developed radiologic progression; 1 was treated with aspirin alone, whereas another underwent revascularization surgery. Although cerebral vasculopathy in neurofibromatosis type 1 may be asymptomatic at presentation, there may be radiologic and clinical progression leading to morbidity and mortality. Magnetic resonance angiography should be considered with brain MRI for early detection and timely intervention of cerebral vasculopathy.

Headache in children with Tourette syndrome.

OBJECTIVE: The authors analyzed the frequency of occurrence of headaches in children and adolescents with Tourette syndrome (TS) to address their possible inclusion as a comorbidity. STUDY DESIGN: Using a prospective questionnaire, administered directly, we interviewed a total sample size of 109 patients with TS ≤ 21 years of age. The questionnaires were then analyzed according to the International Headache Society's diagnostic criteria. RESULTS: We found headaches to be present in 55% of the patients, with the 2 most common headache types being migraine headaches and tension-type headaches. The rate of migraine headache within the TS group was found to be 4 times greater than that of the general pediatric population, as reported in the literature. In addition, the rate of tension-type headache was found to be more than 5 times greater than that of the general pediatric population. CONCLUSIONS: Overall, the high rates of migraine and tension-type headache within this population support the proposition that headaches are a comorbidity of TS.

Hirayama disease in children from North America.

Hirayama disease has been mainly reported from Asia; only a few cases are from the Western hemisphere, particularly North America. This is a retrospective chart review of patients < 18 years, diagnosed with Hirayama disease from a single center over 10 years. We diagnosed 6 children (4 boys), 15.1 ± 1.2 years of age. Symptom onset was 3 months to 3 years before presentation. All had unilateral or bilateral asymmetric distal upper extremity weakness without objective sensory loss. Oblique amyotrophy and cold paresis were noted in 5. On electromyography, acute-on-chronic denervation was most frequently noted in cervical-8 (C8) and thoracic-1 (T1) myotomes followed by cervical-7 (C7) myotome in both upper limbs, sparing C5-C6 myotomes. Cervical magnetic resonance imaging (MRI) was abnormal in 3. Symptoms progressed over a mean of 16.5 months. Treatment consisted of placement of cervical collar. Heightened awareness of this entity among pediatric neurologists in North America will lead to early diagnosis and intervention, avoiding unnecessary investigations.

Reversible cerebral vasoconstriction syndrome: a rare entity in children presenting with thunderclap headache.

Reversible cerebral vasoconstriction syndrome is characterized by a reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurologic deficits or seizures. A 15-year-old boy presented with thunderclap headache. He had severe hypertension, although his neurologic examination was normal. Initial workup for thunderclap headache to exclude subarachnoid or intracranial hemorrhage, meningitis, pituitary apoplexy, or venous sinus thrombosis was negative. Brain magnetic resonance angiography and cerebral angiography demonstrated bilateral anterior and posterior circulation diffuse, multifocal, vascular irregularities (beading and stenosis) suggestive of underlying vasculopathy or vasculitis. He was started on verapamil. There was complete reversal of the vascular abnormalities in 6 weeks evident by magnetic resonance angiography, with resolution of headache and normalization of blood pressure. Reversible cerebral vasoconstriction syndrome has been rarely reported in children. This case report highlights the diagnostic dilemma and management of the rare childhood presentation of this condition.

Xerophthalmia and intracranial hypertension in an autistic child with vitamin A deficiency.

The authors present a 10-year-old boy with autism and idiopathic intracranial hypertension referred for evaluation of dry eyes. When questioned, the patient's parents reported that he had a restricted diet. Laboratory testing revealed hypovitaminosis A. The symptoms and signs of xerophthalmia rapidly resolved with oral and topical vitamin A supplementation.

Genetically confirmed CADASIL in a pediatric patient.

A 17-year-old girl presented with migraine with prolonged aura and aura without headache. Neurologic examination was normal. Her mother, who did not have a history of migraine, developed right-face and -arm numbness at the age of 45. Evaluation revealed white matter changes consistent with CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), and genetic testing showed a Notch3 gene mutation consistent with CADASIL. Our patient's MRI revealed white matter changes and the same Notch3 gene mutation. Low-dose aspirin was started in an attempt to prevent stroke. CADASIL is considered a degenerative disease of adult onset that leads to progressive neurologic deterioration. Onset of symptoms is in the third decade. Migraine, one of its most common manifestations, can develop in childhood. Evaluation for secondary causes is warranted in select pediatric patients who present with atypical migraine, when there is a family history of CADASIL or atypical patterns such as aura without headache, or in the presence of white matter abnormalities. The pathophysiology of CADASIL is poorly understood, and there is no proven effective therapy. Patients require genetic counseling and close follow-up. It is not known if interventions such as antiplatelet therapy are beneficial if instituted early in the course of the disease. Screening of family members at risk for CADASIL, even in the pediatric population, should be considered and offered to patients with CADASIL and their families. CADASIL has rarely been described in the pediatric population. This case report expands our current understanding of the disorder in children.