Acute basal ganglia infarction in propionic acidemia - PubMed (original) (raw)
Case Reports
Acute basal ganglia infarction in propionic acidemia
R H Haas et al. J Child Neurol. 1995 Jan.
Abstract
An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of "metabolic stroke" is more complicated than previously thought.
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