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Acute Basal Ganglia Infarction in Propionic Acidemia

Department of Neurosciences, University of California San Diego, San Diego, CA

Deborah L. Marsden, MD

Department of Pediatrics, University of California San Diego, San Diego, CA

Sylvia Capistrano-Estrada, MD

Department of Pediatrics, University of California San Diego, San Diego, CA

Ronald Hamilton, MD

Department of Pathology University of California San Diego, San Diego, CA

Marjorie R. Grafe, MD

Department of Pathology University of California San Diego, San Diego, CA

Wade Wong, MD

Department of Radiology, University of California San Diego, San Diego, CA

William L. Nyhan, MD, PhD

Department of Pediatrics, University of California San Diego, San Diego, CA

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. (J Child Neurol 1995;10:18-22).

Journal of Child Neurology, Vol. 10, No. 1, 18-22 (1995)
DOI: 10.1177/088307389501000104


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