An inbred Arab family with three neonates affected by microlissencepha
ly syndrome is reported. Brain magnetic resonance imaging in the index
case revealed very thin brain mantle with agyria-pachygyria, agenesis
of the corpus callosum, and hypoplasia of the brainstem and cerebellu
m. All three neonates had microcephaly, arthrogryposis multiplex conge
nita, and micropenis. The presence of three affected newborn infants i
n a consanguineous family suggests an autosomal-recessive mode of inhe
ritance of this syndrome. The spectrum of microlissencephaly syndrome
is reviewed. (C) 1998 by Elsevier Science Inc. All rights reserved.