IV Immune Globulin Effective for Stiff-Person Syndrome, But...

Stiff-person syndrome (SPS) is a presumably autoimmune disorder of the CNS characterized by severe rigidity of truncal and extremity muscles along with intermittent muscle spasms. Antibodies directed against glutamic acid decarboxylase (GAD) are detected in approximately 60% of SPS patients. GAD is responsible for the conversion of glutamic acid to gamma aminobutyric acid (GABA), a primary inhibitory neurotransmitter in the CNS. Impaired GABA-ergic transmission could be responsible for the clinical manifestations seen in SPS, although the pathogenic role of anti-GAD antibodies has been questioned. Data from small series of patients treated with prednisone, plasma exchange, or intravenous immune globulin (IVIG) have been reported; findings h…