Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149958 (complex partial seizures)
 2,563 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Blood mercury was raised to levels exceeding 1,000 ng/gm by weekly oral doses of methylmercury hydroxide to squirrel monkeys. Operant behavior tests were employed and neurological signs were recorded on film. Sudden visual disturbance occured with subacute exposure. Prolonged exposure resulted in impaired coordination with impairment of scotopic vision and possibly also sensory disturbances. Scotopic vision was tested by determining the critical fusion intensity (cfi) at 10 cps. An increase in cfi was the earliest neurological sign appearing in some monkeys months before other signs could be detected. Poisoned monkeys exhibited typical cerebral cortical lesions. The visual cortex was invariably involved. Extension to adjacent cortical areas increased with increasing duration of exposure and increasing brain mercury. The microgram per gram brain tissue was the lowest concentration of methylmercury seen wiht morphological lesions.
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PMID:Neurotoxicity of methylmercury in squirrel monkeys. Cerebral cortical pathology, interference with scotopic vision, and changes in operant behavior. 114 98

3-Hydroxy-3-methylglutaryl coenzyme A lyase deficiency, an inborn error of ketone body synthesis and leucine degradation, is a rare disorder. There are few reports demonstrating clinical and neuroradiologic findings of this condition. This report describes a 3.5-year-old previously healthy male who was admitted with complex partial seizures and was diagnosed as having 3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency. The patient's previous medical history was unremarkable. His development and neurologic examination were normal. Cranial magnetic resonance imaging indicated prominent corticospinal tract and pontine involvement with focal cerebral white matter changes which have not been described in the literature before.
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PMID:Corticospinal tract involvement in a patient with 3-HMG coenzyme A lyase deficiency. 1687 13