Stiff Person Syndrome
Occurs due to hyperexcitability of Anterior Horn Cells. Caused by interference of GABA mediated spinal cord inhibitory
mechanisms. Symptoms are progressive, painful rigidity punctuated by intense
muscle spasm. Affect the axial and paraspinal muscles. Symptoms limited to one limb.
The most common pathologic correlate is anti–glutamic acid decarboxylase (GAD) antibodies (It has been associated with a wide range of neurologic diseases).
Additional possible pathophysiologic etiologies in patients negative for GAD antibody include postsynaptic elements such as synaptophysin, amphiphysin, gephyrin, and GABA-transaminase.
It is also associated with a number of non-neurologic diseases, including diabetes mellitus and thyroiditis.
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