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.