Pediatric neurology
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Two particularly common, and frequently frightening, forms of syncope and anoxic seizure in early childhood are pallid and cyanotic breath-holding spells. Pallid breath-holding spells result from exuberant vagally-mediated cardiac inhibition. Cyanotic breath-holding spells are of more complex pathogenesis, involving an interplay among hyperventilation, Valsalva maneuver, expiratory apnea, and intrinsic pulmonary mechanisms. ⋯ Spontaneous resolution of breath-holding spell, without sequelae, is anticipated. Reassurance is the mainstay of therapy. Occasionally, pharmacologic intervention may be of benefit.
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Pediatric neurology · Feb 1996
Case ReportsCerebellar dysplasia and unilateral cataract in Marinesco-Sjögren syndrome.
The classic features of Marinesco-Sjögren syndrome include bilateral cataracts, cerebellar ataxia, and mental deficiency with an autosomal recessive inheritance pattern. Weakness and a variety of other characteristics are present inconsistently. ⋯ We report a patient with near normal intelligence, unilateral cataract, and the previously unreported magnetic resonance imaging findings of cerebellar dysplasia, arachnoid cyst, and absent septum pellucidum. A review of the literature suggests significant heterogeneity in the Marinesco-Sjögren syndrome.
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Pediatric neurology · May 1995
Case ReportsTransient dystonia of infancy, a result of intrauterine cocaine exposure?
Intrauterine cocaine exposure has been associated with multiple transient and permanent neurologic sequelae. Although dystonic reactions have been reported in cocaine users, infantile dystonia following intrauterine exposure has not. We describe 4 infants testing positive for cocaine metabolite at birth with subsequent transient dystonic reactions, beginning at 3 hours to 3 months of age and persisting for several months.
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Pediatric neurology · Apr 1995
Comparative StudyEffect of prehospital treatment on the outcome of status epilepticus in children.
Diazepam is administered to children in status epilepticus by paramedics in many Emergency Medical Services systems throughout the United States despite the lack of clear evidence that this therapy is safe and effective when employed in the prehospital environment. We reviewed the clinical course of 45 episodes of generalized convulsive status epilepticus (SE) in 38 children to determine the effect of prehospital diazepam therapy (given rectally or intravenously) on the clinical course of SE and subsequent patient management. ⋯ There were no significant differences between rectal and intravenous diazepam therapy with regard to SE duration, intubation, or recurrent seizures in the emergency department. These data suggest that prehospital administration of diazepam may shorten the duration of SE in children and simplify the subsequent management of these patients in the emergency department.