EVIDENCE OF CENTRAL-NERVOUS-SYSTEM INVOLVEMENT IN WATSON SYNDROME

In 1967, Watson described 3 families with an autosomal dominant condit ion characterized by pulmonary valvular stenosis, cafe-au-lait spots, and short stature. Presumed hamartomatous lesions have been observed i n neurofibromatosis type I, but they were not reported to date in Wats on syndrome. We report another family with Watson syndrome, in which 1 patient manifested increased intensity T-2-weighted lesions on magnet ic resonance imaging similar to those occurring in neurofibromatosis t ype I and possibly hamartomas. This finding demonstrates the overlap b etween neurofibromatosis type I and Watson syndrome and supports the h ypothesis that those conditions are allelic or, less likely, that the gene that determines the Watson phenotype is very closely linked to th e neurofibromatosis type I locus.