A new family is reported of a Bickers-Adams-Edwards syndrome. This family has been studied up to three generations. Two female carriers are known. Among the six male @entity1 who are affected, four are severely mentally retarded, have @entity508 of the legs, and survived with a mild @entity4651 , and two show a more severe and rapid progression of @entity135 . A @entity1276 , with remarkable @entity2554 , has been demonstrated by pneumoence-phalography in three @entity1 . A deformity of the thumbs links these six @entity1 together. One of them has been treated by a ventriculoperitoneal shunt, when 18 months old, without any improvement in the @entity16 . The @entity1032 is much more severe than could be expected from the degree of @entity1276 , at least as estimated clinically by the @entity4651 . @entity1276 is precocious, and the @entity2554 very advanced when seen by @entity970 studies. Recognition of the female carriers is not possible.

X-linked hydrocephalus, with @entity1276 , XXXX , and adduction-flexion deformity of the thumbs. Report of a family.

@entity1 :: ('9606', 'Species') :: ['children', 'boys'] @entity135 :: ('MESH:D006258', 'Disease') :: ['head enlargement'] @entity1032 :: ('MESH:D008607', 'Disease') :: ['mental deficiency'] @entity1276 :: ('MESH:D006849', 'Disease') :: ['hydrocephalus', 'Hydrocephalus', 'partial aqueductal stenosis'] @entity508 :: ('MESH:D009128', 'Disease') :: ['spasticity'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological condition'] @entity2554 :: ('MESH:C563906', 'Disease') :: ['ventricular dilatation'] @entity4651 :: ('MESH:D058627', 'Disease') :: ['macrocephaly'] @entity970 :: ('CHEBI:46793', 'Chemical') :: ['PEG']
@entity1032 :: (MESH:D008607,Disease) :: ['mental retardation']
