Biomedical subjects
S B Coker
Publications and source records attributed to S B Coker.
Postneonatal vitamin B6-dependent epilepsy.
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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.
The diagnosis of childhood neurodegenerative disorders presenting as dementia in adults.
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The open opercular sign: diagnosis and significance.
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. Pathological correlation in 1 child revealed arhinencephaly and abnormal gyral formation; another is known to have migrational abnormalities. We suggest that the open operculum is a sign of arrested development and is associated with other anomalies and a poor prognosis.
Occluded fourth ventricle after multiple shunt revisions for hydrocephalus.
Trapped occluded fourth ventricle has been considered a rare occurrence. Intraventricular hemorrhage followed by repeated shunt revisions may increase the risk (8/47 cases). Because premature infants with intraventricular hemorrhage and shunted hydrocephalus often have preexisting neurologic abnormalities, dilation may produce clinically undetected further neurologic damage. Shunting improved function in both currently treated as well as 13 of 14 previously treated patients. In light of this observation, the importance of recognition is stressed.
MS or AIDS?
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Phenacemide therapy of complex partial epilepsy in children: determination of plasma drug concentrations.
We used monotherapy with phenacemide to treat complex partial seizures in 13 children who were refractory to conventional antiepileptic drug therapy. Twelve patients responded with a reduction in seizure frequency, and 5 have been totally seizure free since the start of therapy. Phenacemide therapy was well tolerated with a minimum of untoward side effects and no evidence of irreversible drug toxicity. We developed a rapid and sensitive assay for the determination of plasma phenacemide concentrations by high performance liquid chromatography to monitor drug levels during therapy. Seizure control was achieved at plasma drug levels that ranged from 16 to 75 micrograms/ml. The median effective dose in our series was 52 micrograms/ml. The recurrence of seizures in three patients was, in each case, associated with trough plasma phenacemide levels below 50 micrograms/dl.
Early abnormalities of brainstem auditory evoked potentials in Friedreich's ataxia: evidence of primary brainstem dysfunction.
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 reflex measurements. The results indicated that the brainstem was the primary site of auditory dysfunction.
Surgically correctable lesions detected by CT in 143 patients with chronic epilepsy.
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.
Letter: Safe alternative to tracheostomy in acute epiglottitis.
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Determination of B1c-B1a globulin in children.
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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.
The use of phenacemide for intractable partial complex epilepsy in children.
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.
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.
Cyclic vomiting and the slit ventricle syndrome.
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.
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.