Stiff-leg syndrome: a focal form of stiff-man syndrome.
We report on 2 patients who presented stiffness and spasms similar to those of stiff-man syndrome (SMS) that were limited to one leg for up to 11 years. Patients had serum glutamic acid decarboxylase (GAD) autoantibodies in high titer, clinical evidence of organ-specific autoimmunity, and electromyographic pattern of continuous motor unit activity with abnormally enhanced exteroceptive reflexes. The clinical and immunological profile suggests that this disorder may be a focal form of SMS.
Saiz
A
A
Service of Neurology, Department of Medicine, Hospital Clínic i Provincial, University of Barcelona, Spain.
Graus
F
F
Valldeoriola
F
F
Valls-Solé
J
J
Tolosa
E
E
D001323:Autoantibodies / Q000032:analysis; D004576:Electromyography; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D007866:Leg / Q000503:physiopathology*; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology; D012018:Reflex / Q000502:physiology; D016750:Stiff-Person Syndrome / Q000145:classification / Q000276:immunology / Q000503:physiopathology*
D001323:Autoantibodies; D005968:Glutamate Decarboxylase