ACUTE BASAL GANGLIA INFARCTION IN PROPIONIC ACIDEMIA

An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clin ical presentation was with acute aphasia, generalized hypotonia, and m uscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic c ontrol. Organic acids in the cerebrospinal fluid were unremarkable. Th ese observations indicate that the pathophysiology of ''metabolic stro ke'' is more complicated than previously thought.