PROGRESSIVE ENCEPHALOMYELITIS WITH RIGIDITY, DIABETES-MELLITUS AND RETINOPATHY - AN ANTI-GAD SYNDROME

A 59-year-old woman with a past history of pigmentary retinopathy deve loped progressive encephalomyelitis with rigidity (PEWR) characterized clinically by cerebellar ataxia, pyramidal signs and stiffness with p ainful muscle spasms. At the same period, retinopathy strikingly worse ned, Her serum contained antibodies against glutamic acid decarboxylas e (GAD). She developed diabetes mellitus under corticotherapy, which w as inefficient. Neurologic signs dramatically improved with diazepam a nd high dose of intravenous immunoglobulin (i.v. Ig). Anti-GAD antibod ies have been described in stiff man syndrome, but in only one previou s case of PEWR. Presence of such autoantibodies in serum and improveme nt following i,v. IgG infusion strongly suggest an autoimmune mechanis m involving, in our case central nervous system, pancreas and probably retina.