Occurrence of Dandy-Walker anomaly in a familial case of distal arthogryposis type IIB

We present a family affected with distal arthrogryposis type IIB (DA IIB) in three generations. The coincidental finding of Dandy-Walker anomaly in the proposita arouses the question whether Dandy-Walker anomaly is part of the DA IIB spectrum or whether the Aase-Smith syndrome belongs to the DA spectrum. Am. J. Med. Genet. 95:477–481, 2000. © 2000 Wiley-Liss, Inc.