Stiripentol for the treatment of seizures associated with Dravet syndrome in patients 6 months and older and taking clobazam.

INTRODUCTION: Stiripentol (STP) is a structurally unique molecule with anticonvulsant and neuroprotective properties in animal and human studies. STP enhances gamma-aminobutyric acid (GABA)ergic neurotransmission and inhibits multiple hepatic isoenzymes (i.e. cytochrome P450 system) involved in the metabolism of other antiseizure medications (ASMs) potentiating their anticonvulsant effects and has proven to be a promising therapy in Dravet Syndrome (DS). AREAS COVERED: The authors review randomized clinical trials and observational studies showing STP efficacy, safety, and tolerability when used as adjunctive therapy with VPA and clobazam in patients with DS. Moreover, they include recent evidence of its use in patients<2 years of age. EXPERT OPINION: Evidence on STP demonstrates clinically meaningful efficacy in both short and long term in patients with DS. In addition to reducing convulsive seizure frequency, STP also markedly reduces the number of status epilepticus episodes and associated medical complications which are more common in younger children. STP adverse effects are generally not severe and often resolve following STP dose reduction or adjustments of concomitant ASMs. STP is approved by the FDA for children aged 6 months and older with DS who are also taking clobazam, making it the only DS-specific ASM for children under age 1 year.

Cerebral sinovenous thrombosis in pediatric hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) may result in thrombotic central nervous system complications. We present a child with diarrhea-associated HUS who developed new-onset focal seizures secondary to cerebral sinovenous thrombosis (CSVT). Her CSVT was treated with low-molecular-weight heparin. The patient's seizures were controlled with levetiracetam, and her HUS was managed supportively with hemodialysis. Repeat imaging nearly 6 months following presentation and initiation of anticoagulation demonstrated cerebral sinus enlargement and persistent intraluminal webbing. Anticoagulation was discontinued after 6 months, and she did not experience long-term gross neurologic sequelae. CSVT is a complication of HUS that has not been previously described. In this report, we summarize the thrombotic central nervous system complications of pediatric HUS.

Episodic and chronic migraine in children.

Migraine is the most common acute and recurrent headache syndrome in children. This condition has unique clinical characteristics in the pediatric population, that can evolve with age, and significantly impact a child's quality of life, affecting their education, socialization, and family life. The purpose of this review is to describe the varied clinical features of migraine seen in children, and discuss potential treatment options for pediatric migraine, including chronic migraine. In many patients a multifaceted approach involving lifestyle changes, treatment of comorbid conditions, and pharmacological treatments are needed for optimal headache control. WHAT THIS PAPER ADDS: Migraine presents as various phenotypes in children, sometimes evolving as the child ages. An expanded range of treatment options exists for the challenging chronic migraine patient.

MIGRAÑA CRÓNICA Y EPISÓDICA EN NIÑOS: La migraña es la cefalea sindromática más común y recurrente en niños. Esta condición tiene características clínicas únicas en la población pediátrica, que puede evolucionar con la edad, e impacta significativamente la calidad de vida del niño, afecta su educación, socialización y su vida familiar. El objetivo de esta revisión es describir la variabilidad de las características clínicas de la migraña observadas en el niño y se discute las potenciales opciones terapéuticas para la migraña pediátrica, incluyendo la migraña crónica. En muchos pacientes, un abordaje multifacético que incluya cambios en el estilo de vida, tratamiento de condiciones comórbidas, y, tratamiento farmacológico, son necesarias para un óptimo control de las cefaleas.

ENXAQUECA EPISÓDICA E CRÔNICA EM CRIANÇAS: Enxaqueca é a síndrome de dores de cabeça aguda e recorrente mais comum em crianças. Esta condição tem características clínicas únicas na população pediátrica que podem evoluir com a idade, e impactar significativamente a qualidade de vida da criança, afetando sua educação, socialização e vida em família. O propósito desta revisão é descrever os variados aspectos clínicos da enxaqueca em crianças, e discutir potenciais opções de tratamento para a enxaqueca pediátrica, incluindo a enxaqueca crônica. Em muitos pacientes uma abordagem multifacetada envolvendo mudanças no estilo de vida, tratamento de condições comórbidas, e tratamentos farmacológicos é necessária para o controle adequado da dor de cabeça.

Jeavons Syndrome: Clinical Features and Response to Treatment.

BACKGROUND: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized. METHODS: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016. Criteria for Jeavons syndrome included all of the following: (1) eyelid myoclonia with or without absences, (2) eye-closure-induced seizures or electroencephalography paroxysms, and (3) seizure onset after 12 months of age. We reviewed and described the epilepsy history, antiepileptic drug trials, and response to treatments. RESULTS: Mean age at seizure onset was 7.3 years, and 80% were female. Absence seizures (63%) and generalized tonic-clonic seizures (23%) were most common at onset. Diagnosis was delayed by an average of 9.6 years. After a median follow-up of two years, 80% of patients had drug resistant epilepsy and 70% experienced generalized tonic-clonic seizures. Generalized tonic-clonic seizures and seizure types other than absence seizures increased the risk of drug-resistant epilepsy (P values 0.049 and 0.03, respectively). Valproic acid, lamotrigine, ethosuximide, and levetiracetam were the most effective in reducing seizures by more than 50%. CONCLUSIONS: The diagnosis of Jeavons syndrome is often delayed. Generalized tonic-clonic seizures and seizure types other than absence seizures may be predictors of drug-resistant epilepsy among patients with Jeavons syndrome.

The Comorbidity of Migraine and Epilepsy in Children and Adolescents.

Migraine and epilepsy share a number of clinical attributes, including pathophysiology and clinical expression. Both are paroxysmal in nature and thus constitute episodic disorders, yet either may be chronic and/or recurrent. Epileptic seizures and migraine headaches may be mistaken one for the other and may even overlap. In particular, occipital lobe seizures may be misdiagnosed as migraine auras. In this article, we review the relationship between migraine and epilepsy, including the known genetic contributions to both conditions, prodromal, ictal, and postictal headache and shared pathophysiology and treatment options. We describe clinical conditions in which both migraine and epilepsy are prominent features. Lastly, we discuss electronecephaographic abnormalities that have been known to occur in individuals with migraine.

Abnormal movements in children with migraine.

The cause and treatment of functional movement disorders and nonepileptic spells in children is poorly understood, and an association with migraine has not previously been reported. We retrospectively reviewed children diagnosed with chronic or episodic migraine at our institution from 2006 to 2013 to determine the proportion with nonorganic movement disorders, their phenomenology, provoking factors, and natural history. Thirty-two patients were identified, representing 4.3% of patients with chronic migraine and 0.9% of patients with episodic migraine. Twenty-four of the 32 (75%) had chronic migraine, whereas 8 (25%) had episodic migraine. Nonepileptic spells was the most common phenomenon in both cohorts, followed by tremor and functional gait disorders. Severe migraine attacks preceded these movements in the majority of patients. With appropriate migraine therapy, significant reduction or resolution of these movements was reported. We conclude that nonorganic movement disorders are observed in pediatric migraine, are more prevalent among chronic migraineurs, and can resolve with improved pain control.