Biomedical subjects
Publications and source records attributed to S B Coker.
CT findings justified surgical intervention in four young patients who had suffered from intractable epilepsy for many years. Previous non-invasive and invasive neurodiagnostic procedures with these patients had failed to show focal cerebral lesions amenable to surgical treatment. All four patients achieved significant relief from seizures after the operations.
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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.
Phenacemide monotherapy controlled seizures in four children with intractable partial complex epilepsy. No toxicity occurred. Previous fatalities caused abandonment of this antiepileptic drug, although it is not established that the drug was at fault.
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.
Recurrent bouts of vomiting with headache and lethargy occurred in 6 children with ventriculoperitoneal shunts who had small ventricles rather than the expected dilated ones. Slit ventricle syndrome is not uncommon in children with shunts and its early recognition allows for appropriate surgical treatment.
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.
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.
Two patients with Candida meningitis are presented to emphasize cranial polyneuropathy and ventricular isolation with septation. These clinical and radiographic criteria can be used for early diagnosis and treatment.
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.
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.
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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.
Familial spastic paraparesis is characterized by progressive gait disturbance without associated sensory, cerebellar, or cranial nerve deficits. Mitochondrial disorders are associated with heterogenic clinical presentations, though not with spastic paraparesis. A patient with familial spastic paraparesis had deficiencies of respiratory chain enzyme complex I, III, and IV. Progressive spasticity was arrested after treatment with coenzyme Q, carnitine, vitamin C and K. Familial spastic paraparesis may represent a mitochondrial disorder.