Steven B. Coker

Neonatal burst suppression: Its developmental significance

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 (26%) died in infancy. Ten survivors were followed for a mean of 30 months (range: 18-61 months). Nine have severe motor deficits, 6 require total custodial care, 5 have intractable epilepsy, and 3 are blind. Their mean Gesell Developmental Quotient was 28; a score of less than 68 suggests severe retardation. A single patient, the only 1 with a perinatal history complicated by an easily-treated bacterial meningitis, achieved normal growth and development. This study documents the dire prognosis of neonatal burst suppression. The appearance of burst suppression, though transient, portended death or severe neurodevelopmental disability in 93% of our patients.

Rett syndrome and mitochondrial enzyme deficiencies.

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. (J Child Neurol 1991;6:164-166).

Neurologic anomalies of Perrault syndrome

We report on an 18-year-old man with neurosensory hearing loss and his sister with neurosensory hearing loss, ovarian dysgenesis, mental retardation, generalized ataxia of the trunk and limbs, and saccadic dysmetria. A CT scan showed cerebellar hypoplasia. The cardinal manifestations of Perrault syndrome in females are neurosensory hearing loss and ovarian dysgenesis. Other anomalies, including neurologic and skeletal, have been reported in other individuals with Perrault syndrome. We review the neurologic anomalies in previous patients with Perrault syndrome. Neurologic data are available on 14 of 21 girls; 7 of 14 had neurologic abnormalities. The high incidence of neurologic anomalies suggest that ataxia or mental retardation may not be just coincidental findings, but pleiotropic manifestations of Perrault syndrome.

Neonatal stroke: Description of patients and investigation into pathogenesis

We report 15 patients with neonatal stroke reviewed retrospectively. Twelve patients had left middle cerebral artery involvement--the same ratio observed in 36 previously reported patients with strokes. We studied 20 normal neonates prospectively to determine whether a difference in flow velocity could be detected between the left and right middle cerebral arteries; pulsed Doppler ultrasound, which compared both velocities, demonstrated no significant difference.

Akinetic mutism: Pharmacologic probe of the dopaminergic mesencephalofrontal activating system

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.

Leigh disease presenting as Guillain-Barré syndrome

A 4-year-old male evidenced the criteria for the diagnosis of Guillain-Barré syndrome. Eventually a diagnosis of Leigh disease was made based on magnetic resonance imaging and mitochondrial enzyme deficiencies. Although chronic neuropathy has been reported with Leigh disease, this is the first reported patient with acute symmetric motor polyneuropathy.

CHARGE and Joubert Syndromes: Are They a Single Disorder?

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 discuss their similarities and review the literature.

Radionuclide cerebral imaging for confirmation of brain death in children: The significance of dural sinus activity

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, dural sinus radioactivity in the delayed static images persisted in the presence of brain death.

Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct

The bobble-head doll syndrome has been associated with third ventriculomegaly due to cysts or aqueductal stenosis. A case is described in which the aqueduct and fourth ventricle are enlarged, but the third ventricle is not. The unifying pathogenesis for this and previous cases is pressure on the dorsomedial red nucleus.

Myelopathy secondary to neonatal bacterial meningitis

Myelopathy is an infrequently reported complication of bacterial meningitis. Four patients with neonatal meningitis and cervical myelopathy are reported. This complication may be more frequent than presumed and should be closely assessed during evaluation. The conditions of most previously reported survivors improved or resolved and the majority involved the cervical spinal cord.