[Chronic hemorrhagic pancreatitis in gallbladder polyposis as an initial symptom of metachromatic leukodystrophy]

A 4 1/2 year old boy without previous neurologic disorders developed chronic hemorrhagic pancreatitis and was shown to have polyposis of the gallbladder. Neurologic symptoms emerged at the age of 5 years. The sonographic pattern of an echogenic gallbladder was suspect of metachromatic leukodystrophy. The definitive diagnosis was made by the findings of very low arylsulfatase A activity in the white blood cells and deposits of sulfatides in the stroma of the polyps of the gallbladder.