XXXX : clinical features and response to @entity1885 treatment.

@entity1 @entity1885 @entity5219 @entity194 @entity8684 @entity1167 @entity14799 @entity1282 @entity346 @entity21 @entity528
BACKGROUND: @entity14799 ( @entity14799 ) encompasses several syndromes of bilateral symmetric, spongy degeneration of the caudate nucleus, putamen, and globus pallidus. The familial form of @entity14799 is rare, and inheritance is either autosomal recessive or maternal. METHOD: The authors describe an Israeli Bedouin kindred in which 15 @entity1 born to consanguineous parents were affected with @entity14799 . They evaluated the clinical and radiologic evolution of the disease in 11 @entity1 and the cerebral pathologic findings in one @entity1 . Three of the @entity1 were treated with oral @entity1885 100 mg/day. RESULTS: Inheritance was apparently autosomal recessive. The untreated @entity1 had a similar clinical picture including @entity1167 beginning at the age of 7 to 15 months, @entity8684 , and @entity1282 . @entity346 appeared at a late stage. MRI, performed at various stages of the disease, showed severe @entity21 . Postmortem study in one @entity1 showed severe @entity194 of the lenticular nuclei with @entity5219 and @entity528 . @entity1885 , 100 mg/day, administered to the proband over a period of 15 months, may have slowed progression. In two other @entity1 treatment was initiated earlier and appeared to arrest or improve disease. CONCLUSIONS: @entity14799 was inherited as an autosomal recessive trait. Clinical features included @entity1167 , @entity1282 , and @entity8684 . Imaging and pathology showed @entity194 and @entity21 . Oral @entity1885 may have benefited three @entity1 .
