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INTRODUCTION
Stiff-man Syndrome
is a rare disease of the nervous system. Progressively severe muscle stiffness
typically develops in the spine and lower extremities; often beginning
very subtly during a period of emotional stress. Most patients experience
painful episodic muscle spasms that are triggered by sudden stimuli. An
auto-immune component is typical and patients often have other auto-immune
disorders. Symptoms usually begin in the mid-forties. The severe muscle
stiff-ness of SPS responds to benzodiazepines. Testing with an EMG (electro-myogram)
needle will assist the diagnosis and testing the blood for anti-GAD (glutamic
acid decarboxylase) antibodies (if present at very high levels) will confirm
the diagnosis in the majority of people with SPS.
SPS is extremely rare. Although it is not possible to determine the exact
prevalence, it may occur in fewer that 1 per million. The disease is more
common in women (the ratio is 2 women for every man effected). There is
no predilection for any race or ethnic group. There is an association
with diabetes and perhaps over half of patients with SPS have or will
develop diabetes. Other autoimmune diseases have been found in association
with SPS, for example: thyroid disease and vitiligo. There is an increased
incidence of epilepsy. An important but especially rare variant of SPS
is associated with breast or lung cancer. Characterized by the production
of antibodies to amphiphysin, this variant of SPS has certain features
that distinguish it from the more typical SPS with GAD antibodies.
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