Four children who exhibited akinetic mutism during the course of their neurologic diseases were treated with bromocriptine. Reversal of the akinetic mute states was evident in all patients. Pathways of the dopaminergic neurons are reviewed and a clinically useful mechanism which causes akinetic mutism is postulated.
Four children with varying clinical manifestations, but with the unifying feature of severe developmental delay, had bilateral enlargement of the sylvian fissure confirmed by magnetic resonance imaging (MRI). Subsequently, we examined 125 consecutive MRI scans of the heads of pediatric patients, looking for this insular exposure, and did not find it.… (More)
Burst suppression was recorded on electroencephalograms of 15 of 274 term infants (5.4%) in our hospital within a 5 year period. These 15 infants were examined, their perinatal histories reviewed, and detailed neurodevelopmental testing performed to examine the prognostic significance of burst suppression. Fourteen children (93%) had poor outcomes. Four… (More)
We studied five children with classic Friedreich's ataxia, using an audiologic test battery to determine the primary site of auditory dysfunction. None of the children had any hearing complaints, and all were tested soon after onset of symptoms. The audiologic test battery consisted of brainstem auditory evoked potential test, tympanometry, and acoustic… (More)
Paroxysmal and rhythmic lingual movements were observed in three children during a study designed to investigate epileptiform movements of oropharyngeal muscles in patients with chronic epilepsy. The movements were confined to the tongue, occurred mainly during sleep, and were observed again in two children 7 and 18 months later. These movements… (More)
The etiology of Rett syndrome is unknown. Structural mitochondrial abnormalities have been described in muscle in patients with Rett syndrome. We report three children with Rett syndrome and normal muscle mitochondrial structure on light and electron microscopy. However, all had abnormalities in mitochondrial respiratory chain enzymes.
Radionuclide cerebral imaging revealed no cortical flow, but recorded persistent dural sinus activity in 14 of 55 clinically brain dead children. Of these 14 children, 13 had isoelectric electroencephalograms. Postmortem liquefactive necrosis was present in 7 cases. Although studies that demonstrated the absence of flow were confirmatory of brain death,… (More)
A patient with the CHARGE association (Coloboma of the eye, Heart defect, Atresia of the choana, Retarded growth and development, Genital hypoplasia, and Ear anomalies or deafness) had intermittent hyperpnea and cerebellar hypoplasia; therefore, he had both the CHARGE association and Joubert syndrome. The 2 syndromes have not been previously linked. We… (More)