Acute Basal Ganglia Infarction in Propionic Acidemia

Richard H. Haas; Deborah L. Marsden; Sylvia Capistrano-Estrada; Ronald Hamilton; Marjorie R. Grafe; William L. Nyhan; Wade Wong

doi:10.1177/088307389501000104

Acute Basal Ganglia Infarction in Propionic Acidemia

Richard H. Haas, Deborah L. Marsden, Sylvia Capistrano-Estrada, Ronald Hamilton, Marjorie R. Grafe, William L. Nyhan, Wade Wong

Pathology

Research output: Contribution to journal › Article › peer-review

52 Scopus citations

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

Original language	English (US)
Pages (from-to)	18-22
Number of pages	5
Journal	Journal of child neurology
Volume	10
Issue number	1
DOIs	https://doi.org/10.1177/088307389501000104
State	Published - Jan 1995

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Clinical Neurology

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title = "Acute Basal Ganglia Infarction in Propionic Acidemia",

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

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AU - Haas, Richard H.

AU - Marsden, Deborah L.

AU - Capistrano-Estrada, Sylvia

AU - Hamilton, Ronald

AU - Grafe, Marjorie R.

AU - Nyhan, William L.

AU - Wong, Wade

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AB - 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).

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