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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 18-22 |
| Number of pages | 5 |
| Journal | Journal of Child Neurology |
| Volume | 10 |
| Issue number | 1 |
| State | Published - 1995 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Clinical Neurology
- Pediatrics, Perinatology, and Child Health
Cite this
Acute basal ganglia infarction in propionic acidemia. / Haas, R. H.; Marsden, D. L.; Capistrano-Estrada, S.; Hamilton, R.; Grafe, Marjorie; Wong, W.; Nyhan, W. L.
In: Journal of Child Neurology, Vol. 10, No. 1, 1995, p. 18-22.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Acute basal ganglia infarction in propionic acidemia
AU - Haas, R. H.
AU - Marsden, D. L.
AU - Capistrano-Estrada, S.
AU - Hamilton, R.
AU - Grafe, Marjorie
AU - Wong, W.
AU - Nyhan, W. L.
PY - 1995
Y1 - 1995
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.
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.
UR - http://www.scopus.com/inward/record.url?scp=0028870138&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028870138&partnerID=8YFLogxK
M3 - Article
VL - 10
SP - 18
EP - 22
JO - Journal of Child Neurology
JF - Journal of Child Neurology
SN - 0883-0738
IS - 1
ER -