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Biomedical subjects

S B Coker

Publications and source records attributed to S B Coker.

At least 19 recordsLinked to original sources
PubMed1996

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.

Abnormalities, Multiple↗
PubMed1996

Ptosis associated with sinusitis.

An adolescent male developed eye pain and a drooping lid. Imaging revealed adjacent pansinusitis and a swollen levator palpebrae and superior rectus muscle. Compression of a branch of the oculomotor nerve is the postulated cause because vertical eye movements were normal.

Acute Disease↗
PubMed1995

Transient 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.

Cocaine↗
PubMed1995

Connatal Leigh disease.

Two children are described with pathology-proven Leigh disease. Rather than the typical degenerative course with loss of acquired development, they presented with a static encephalopathy manifested by seizures from birth and failure to acquire any milestones. A similar connatal presentation has been reported in other degenerative disorders, such as Pelizaeus-Merzbacher disease. Heredodegenerative disorders should be considered when no cause is discovered for a severe, congenital, static encephalopathy.

Brain↗
PubMed1994

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.

Atrophy↗
PubMed1991

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.

Adolescent↗
PubMed1989

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.

Cerebral Cortex↗
PubMed1989

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.

Cerebral Hemorrhage↗
PubMed1988

MS or AIDS?

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗
PubMed1987

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.

Adolescent↗
PubMed1983

Aspartylglucosaminuria in the United States.

Aspartylglucosaminuria (AGU) was diagnosed in two unrelated males with progressive mental retardation, coarse facies and skeletal abnormalities. Until now, this disorder has been described in predominantly Finnish populations with only one previous case reported in the U.S. We conclude that AGU may be more common in non-Finnish populations than the number of reported cases would indicate and should be included in the differential diagnosis in patients with suspected lysosomal storage disorders regardless of their geographical or ethnic backgrounds.

Adolescent↗
PubMed1983

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.

Brain↗
PubMed1981

Paroxysmal, rhythmic lingual movements and chronic epilepsy.

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 corresponded to episodic desynchronization of the electroencephalogram and were attributed to an unusual form of subcortical seizures.

Adolescent↗