Progressive neurological disorder associated with obstructive jaundice and vitamin E deficiency.
A ten-year-old girl had obstructive jaundice in the newborn period which persisted for 4 years despite choledochojejunostomy at 6 weeks. From the age of 6 years she developed a progressive neurological syndrome characterized mainly by dysarthria and ataxia. A causal relationship with her profound vitamin E deficiency seemed likely. Treatment with vitamin E over a 2 1/2 year period appeared to arrest the progression of the neurological deficit and subsequent increase in dosage produced some improvement in her ataxia.
D001259:Ataxia / Q000150:complications*; D002648:Child; D002779:Cholestasis / Q000150:complications*; D004401:Dysarthria / Q000150:complications*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000150:complications; D013064:Speech Disorders / Q000150:complications*; D013577:Syndrome; D014811:Vitamin E Deficiency / Q000150:complications*