Late-onset neurologic disease in glutaryl-CoA dehydrogenase deficiency.

Külkens, S; Harting, I; Sauer, S; Zschocke, J; Hoffmann, G F; Gruber, S; Bodamer, O A; Kölker, S · Neurology · 2005

case_series · Level IV

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Abstract

Neurologic disease in glutaryl-CoA dehydrogenase (GCDH) deficiency usually presents with acute encephalopathic crises before 2 years of age. The authors report two previously asymptomatic patients with macrocephaly presenting with progressive neurologic deterioration and a severe leukoencephalopathy during adolescence or adulthood.

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