[Marked clinical improvement by plasmapheresis in a patient with stiff-man syndrome: a case with a negative anti-GAD antibody].
We described a 56-year-old man with stiff-man syndrome, who was markedly improved after plasmapheresis therapy. He had a 12-year history of progressive painful stiffness of his back and limbs, muscle cramps and difficulty in walking. He had been taking oral diazepam and prednisolone. On examination the abdominal and paraspinal muscles and limbs were continuously contracting, confirmed by surface and needle electromyography. Antibodies against glutamic acid decarboxylase (GAD) and pancreatic islet cells in the serum were negative, but antinuclear antibody and anti-smooth-muscle antibody were present. The patient underwent a course of 4 double filtration plasma exchanges of 3,000 ml each in an 8-day period. Plasmapheresis resulted in marked clinical improvement. The disappearance of muscular cramps and a reduction of stiffness occurred within 24 hours after the first plasmapheresis, and he was able to walk unassisted. The patient's subjective improvement continued over 4 months after the plasma exchange. This case provides additional evidence of the autoimmune mechanism of stiff-man syndrome. Plasmapheresis is one choice in the management for stiff-man syndrome.
Nakamagoe
K
K
Department of Neurology, University of Tsukuba.
Ohkoshi
N
N
Hayashi
A
A
Hisahara
S
S
Shoji
S
S
D000974:Antibodies, Antinuclear / Q000302:isolation & purification; D001323:Autoantibodies / Q000032:analysis* / Q000302:isolation & purification; D015551:Autoimmunity; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009130:Muscle, Smooth / Q000276:immunology; D010956:Plasmapheresis; D016750:Stiff-Person Syndrome / Q000209:etiology / Q000628:therapy*
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D005968:Glutamate Decarboxylase