Stiff-man syndrome. Presentation of a new case

• Published in: Revista de neurologiá Vol. 25; no. 143; p. 1084
• Main Authors: Fernández-Barreiro, A, Villaverde, R, Salmerón, P, Morales, A, Martínez-García, F A, Meca, J E, Tortosa, D
• Format: Journal Article
• Language: Spanish
• Published: Spain 01.07.1997
• Subjects: Anticonvulsants - administration & dosage; Anticonvulsants - therapeutic use; Autoimmunity; Brain - pathology; Contracture - diagnosis; Contracture - physiopathology; Diabetes Mellitus, Type 1; Diazepam - administration & dosage; Diazepam - therapeutic use; Dose-Response Relationship, Drug; Electromyography; Female; Glutamate Decarboxylase - metabolism; Humans; Middle Aged; Muscle, Skeletal - physiopathology; Stiff-Person Syndrome - diagnosis; Stiff-Person Syndrome - drug therapy; Stiff-Person Syndrome - pathology; Vitiligo
• ISSN: 0210-0010

The stiff-man syndrome (SPR) is a rare neurological condition characterized by the presence of marked, involuntary rigidity of the axial muscles and limbs, together with intense painful muscle spasms which characteristically occur following external stimuli such as sudden noises, brusque movements or emotional stimuli. Symptoms are markedly improved by sleep and by diazepam. The aetiology is unknown, although it is associated with certain auto-immune disorders, particularly diabetes mellitus (DM) and others such as thyroid disease, vitiligo, pernicious anaemia, adrenal insufficiency, etc. The presence of high titres of glutamate-decarboxylase (GAD) antibodies seems to indicate an auto-immune aetiology. We describe a 59 year old woman who complained of an illness starting seven years previously with progressive pain and contractions of the dorso-lumbar muscles, which later spread to the cervical and abdominal muscles and to the upper limbs. From the time of onset, there were exacerbations of the condition following external stimuli. Also there was generalized vitiligo and whilst this progressed, DM developed (which finally required insulin to control it). On EMG there was continuous muscular activity and absence of muscle relaxation. The presence of high titres of anti-nuclear antibodies, gastric parietal cell antibodies and anti-GAD was very marked. There was a spectacular response to treatment with diazepam, with progressive improvement although ever increasing dosage was required. A new case of the 'stiff-man' syndrome is presented and aspects of aetiopathology and treatment are reviewed.