Stiff-Person Syndrome

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= Overview =
 * Autoimmune disorders with antibodies directed at neurologic targets:
 * SPS: Glutamic Acid Decarboxylase (GAD)
 * Isaac’s syndrome: peripheral nerve Voltage-Gated potassium Channels (VGKC)
 * causes Acquired Neuromyotonia

History and Symptoms

 * Marked rigidity and painful spasms, particularly in the thoracic and lumbosacral paraspinal, abdominal, and proximal lower extremity muscles
 * Onset usually insidious
 * Persistent contraction leads to a “board-like” quality
 * Fixed thoracolumbar lordosis of spine develops in nearly all cases from co-contraction of abd/paraspinal muscles
 * Intermittent painful spasms which may lead to falls due to instability
 * 10% have generalized seizures or myoclonus
 * Spasms are NOT present during sleep

Laboratory Findings

 * Paraneoplastic cases often are GAD negative, have anti-Amphyphysin

CSF abnormal with increased IgG and oligoclonal bands
 * 65% have anti-GAD antibodies

MRI and CT
Conventional MRI and CT commonly normal

Other Diagnostic Studies
Serum CK level often elevated due to constant state of contraction of muscles

Acute Pharmacotherapies

 * Benzodiazepines (enhance the affinity of GABA receptors)
 * Oral or intrathecal baclofen
 * Vigabatrin (irreversible inhibitor of GABA transaminase)
 * Valproic Acid, Neurontin
 * Injected botulinum toxin benefits some
 * Prednisone, plasmapheresis, IVIG all used with some success, but no randomized trials

Acknowledgements
The content on this page was first contributed by: Stephen Wiviott, M.D.

List of contributors:

For Patients
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