Acute permethrin neurotoxicity: Variable presentations, high index of suspicion.

Permethrin is a synthetic Type I pyrethroidal neurotoxic pesticide that has been responsible for accidental animal deaths. Despite its widespread use, there are no published case reports on pediatric intensive care unit admissions due to permethrin exposure. We report the unusual and varied presentations of permethrin toxicity in three siblings presenting to a tertiary care pediatric intensive care unit (PICU). While there is no standard clinical diagnostic test for permethrin, accurate diagnosis was obtained by rapidly analyzing the offending agent. In the absence of a known antidote for permethrin, supportive management was initiated and resulted in a favorable outcome for all three siblings.

Postictal psychosis in a child.

Postictal psychosis is a state of psychosis following repeated or prolonged complex partial seizures with or without secondary generalization and is well described in adult epilepsy literature. It is sparsely reported in the pediatric literature. This report describes a 12-year-old male presenting with status epilepticus who developed psychotic symptoms. Diagnosis of postictal psychosis was made after correlating clinical symptoms with video-electroencephalographic monitoring. The clinical course of this illness is profiled, and the literature reviewed.

Vigabatrin for infantile spasms.

We reviewed 20 infants receiving vigabatrin for infantile spasms. Patients were not enrolled in a formal study. All families obtained the medication abroad. Age at initiation of vigabatrin ranged from 1 to 48 months; nine infants had received prior treatment with various antiepileptic medications. Patients were begun on the lowest practical dose of 125-250 mg/day, with gradual daily increments to a target of 100 mg/kg/day, but maintained at the lowest effective dosage. Video electroencephalogram was obtained to document resolution of spasms and hypsarrhythmia. Of 20 infants, 12 responded with cessation of spasms and resolution of hypsarrhythmia, at doses of 25-135 mg/kg/day (median = 58 mg/kg/day). Partial responses were observed in six patients, whereas two had no response at 111 and 125 mg/kg/day. Additional new seizure types developed in three infants after initial response to vigabatrin. Increasing the vigabatrin did not have any clinical benefit. Vigabatrin is an effective, well-tolerated treatment for infantile spasms. The response is dose-independent, suggesting that starting at a low dose and gradually increasing, rather than beginning with an arbitrary 100 mg/kg/day dose is advantageous.

Mitochondrial disorders: a potentially under-recognized etiology of infantile spasms.

Infantile spasms represent an age-dependent response of the immature brain to a wide variety of insults. An unselected group of children with infantile spasms were reviewed to determine etiology; a metabolic work-up was undertaken if the etiology was unclear from history and examination (cryptogenic). Of the 56 infants, 34 had a recognizable etiology (symptomatic), 1 had normal development (idiopathic), and 21 had cryptogenic infantile spasms. Among the latter, results of plasma lactate and pyruvate or urine organic acids were available in 17. In 2 infants (monozygotic twins), mitochondrial DNA testing revealed the relatively common A3243G mitochondrial mutation. In these twins and 11 of the remaining 15, body fluid metabolite testing suggested possible defective energy metabolism. Our twins and previous reports suggest that mitochondrial disorders should be considered in the differential diagnosis of infantile spasms. Among our cases remaining cryptogenic, signs of abnormal energy metabolism were prevalent, suggesting that metabolic derangements may be common causes or secondary consequences of infantile spasms.