SEVERE MID-CERVICAL KYPHOSIS WITH CORD COMPRESSION IN LARSENS-SYNDROME AND DIASTROPHIC DYSPLASIA - UNRELATED SYNDROMES WITH SIMILAR RADIOLOGIC FINDINGS AND NEUROSURGICAL IMPLICATIONS

Mid-cervical kyphosis in diastrophic dysplasia (DD) with cord compress ion and weakness has been recognized for the last three decades. A str ikingly similar situation exists in Larsen's syndrome (LS), an otherwi se unrelated problem of weakness with lay joints and dislocations with typical facies. Forty patients with DD were studied of whom seven had cervical spine kyphosis: one patient had spontaneous correction over the ensuing two decades. Seven patients with LS were studied of whom f our had cervical spine changes with cord compression. There was one su dden death among the patients with LS and no cases of spontaneous corr ection. Muscle weakness in DD and LS should lead to evaluation of cerv ical spine kyphosis with cord compression. Plain films, CT and recentl y MRI are useful.