ISSN:
1432-1920
Keywords:
Brain, CT
;
Brain, diseases
;
Basal ganglia
;
Hydrocephalus
;
Enzymes
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Serial CT findings in an infant with glutaric aciduria type I (GA-I) are reported. The major CT features were dilatation of the insular cisterns, regression of the temporal lobes, with “bat wings” dilatation of the Sylvian fissures and hypodensity of the lenticular nuclei. CT changes preceded the onset of symptoms by 3 months. An improvement in the temporal lobe atrophy was seen after a period of treatment, coinciding with marked clinical improvement. A peculiar feature was the presence of external hydrocephalus, which diverted the attention from manifestations of the primary disease and thus consitituted a diagnostic pitfall. The delineation and recognition of the characteristic radiologic manifestations of GA-I are essential for allowing an adequate radiologist/clinican interaction in diagnosing this inborn error of metabolism.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00593342
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