The affected members of the family described in this article exhibit c
ongenital nonprogressive atrophy and weakness of lower limb muscles in
association with contractures. Clinical and laboratory findings suppo
rt a dominant lower motor neuron disorder. DNA analysis excluded linka
ge of the disease with SMA markers on the long arm of chromosome 5. Th
e condition must be differentiated from congenital and infantile SMA,
from ''arthrogryposis multiplex congenita, distal type,'' and from non
hereditary types of congenital arthrogryposis. (C) 1994 John Wiley &
Sons, Inc.