['Stiff-person'-syndrome].
In two patients, a man aged 54 years and a woman aged 49 years, stiff-person syndrome was diagnosed. This is a rare disorder of the central nervous system, with signs of an autoimmune pathogenesis. Patients present with pain and stiffness of the lower back, a complaint that is regularly seen in general practice. Moreover, the disease causes hypertonia and very painful cramps of the lower back and legs. Electromyographic examination in the resting condition reveals continuous muscle activity in the long back muscles, which decreases following the administration of diazepam. In 60% of patients, antibodies to glutamic acid decarboxylase may be found in the serum or cerebrospinal fluid; this enzyme is involved in the production of the inhibiting neurotransmitter gamma-aminobutyric acid. Both patients were treated with diazepam, baclofen and corticosteroids. Stiff-person syndrome is a rare but treatable disorder that should be considered when patients present with stiffness and pain in the lower back and upper legs.
Vogels
R L
RL
St. Lucas-Andreas Ziekenhuis, afd. Neurologie, Postbus 9243, 1006 AE Amsterdam. r.vogels@slaz.nl
van Orshoven
N P
NP
de Koning-Tijssen
M A
MA
Wouda
E J
EJ
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid; D001418:Baclofen / Q000627:therapeutic use; D003975:Diazepam / Q000627:therapeutic use; D004576:Electromyography; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D017116:Low Back Pain / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009125:Muscle Relaxants, Central / Q000627:therapeutic use*; D016750:Stiff-Person Syndrome / Q000503:physiopathology / Q000628:therapy*; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D009125:Muscle Relaxants, Central; D005968:Glutamate Decarboxylase; D001418:Baclofen; D003975:Diazepam