STIFF-MAN-SYNDROME - REPORT ON 4 CASES

Four patients are presented who exhibited progressive muscular rigidit y in both legs, the thoracolumbar, paraspinal and the abdominal muscle s. In only one patient, there was an initial involvement of the should er girdle muscles. Electromyography in all four patients at rest recor ded continuous electric activity resembling an interference pattern. F ollowing sudden exteroceptive stimuli the activity increased in amplit ude and density this corresponded clinically to painful spasms. All pa tients had oligoclonal banding in CSF In the serum and CSF of three pa tients IgG subtype autoantibodies were detected. These have been found to be directed against GABAergic nerve terminals in the rat and human cerebellum and, more specifically, to glutamic acid decarboxylase. Al l patients improved on clonazepam. Trials of intermittent high-dose me thylprednisolone administration gave relief from rigidity in one patie nt and permitted reduction of clonazepam in another. Intravenous immun oglobulins however had no effect in one patient.