Neurological involvement in the nevoid basal cell carcinoma syndrome

Abstract

✓ Four patients who had medulloblastomas in association with the nevoid basal cell carcinoma syndrome are reported, for a total of six known cases. Involvement of the central nervous system appears to be an integral component of this syndrome. The medulloblastoma appear earlier in life (50% of the patients are less than 2 years old), and the prognosis appears to be better (five of the six are still living). Possible reasons for these features are considered, and other associated abnormalities are discussed.