TY - JOUR
T1 - Acute Basal Ganglia Infarction in Propionic Acidemia
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
PY - 1995/1
Y1 - 1995/1
N2 - 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).
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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U2 - 10.1177/088307389501000104
DO - 10.1177/088307389501000104
M3 - Article
C2 - 7769171
AN - SCOPUS:0028870138
SN - 0883-0738
VL - 10
SP - 18
EP - 22
JO - Journal of child neurology
JF - Journal of child neurology
IS - 1
ER -