The neuropathy of Dejerine and Sottas: report of an Indian family.
We report here a family in which 4 out of 11 sibs of unrelated parents showed the typical clinical features of Dejerine-Sottas disease. Sensory disturbance was present in only one case and age at onset was delayed to 4 years in another. The others all conformed to the infantile form of the disease. This is the first time in which mental retardation has been reported in this disorder.
Bharucha
E P
EP
Children's Orthopedic Hospital, Bombay, India.
Sulaiman
R
R
Bharucha
N E
NE
D000293:Adolescent; D001284:Atrophy; D002648:Child; D004576:Electromyography; D005260:Female; D005385:Fingers / Q000002:abnormalities; D015417:Hereditary Sensory and Motor Neuropathy / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008607:Intellectual Disability / Q000150:complications*; D008297:Male; D009132:Muscles / Q000473:pathology; D010375:Pedigree; D014034:Toes / Q000002:abnormalities