Document Detail

Megalencephalic leukodystrophy in an Asian Indian ethnic group.
MedLine Citation:
PMID:  8805171     Owner:  NLM     Status:  MEDLINE    
Over a 10-year period, we reviewed 30 patients with leukodystrophy, megalencephaly, and a relatively benign course. Most of these patients (26) belonged to a distinctive ethnic group called the Agrawals. Head circumference exceeded the 95th percentile in 28 patients; 22 patients had seizures; 22 had pyramidal signs (16 more pronounced and 6 mild); and 16 had cerebellar ataxia. The median age of onset of symptoms was 1.8 years. Interictal electroencephalogram (EEG) was abnormal in 21 of 23 patients. The inheritance is possibly autosomal recessive.
B S Singhal; R D Gursahani; V P Udani; A A Biniwale
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  14     ISSN:  0887-8994     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  1996 May 
Date Detail:
Created Date:  1996-12-03     Completed Date:  1996-12-03     Revised Date:  2006-05-23    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  291-6     Citation Subset:  IM    
Department of Neurology, Bombay Hospital Institute of Medical Sciences, India.
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MeSH Terms
Brain / abnormalities*
Child, Preschool
Cognition Disorders / diagnosis,  genetics*
Diffuse Cerebral Sclerosis of Schilder / diagnosis,  genetics*
Ethnic Groups / genetics*
Magnetic Resonance Imaging
Middle Aged
Retrospective Studies
Tomography, X-Ray Computed

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