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February 5, 2014: Stiff Person Syndrome

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February 5, 2014

The presentation of insidious and progessive muscular rigidity, stiffness, and trigger-induced muscle spasms of the axial and proximal muscles should raise concern for the Stiff Person Syndrome (SPS). This rare movement disorder was detailed in 1956 by Drs. Frederick Moersch and Dr. Henry Woltman, who described the presentations and clinical courses of 14 patients they observed over a 32-year period [1]. Symptoms often present in adulthood with a female predominance. EMG will often demonstrate continuous motor unit potential firing [1]. SPS is associated with antibodies to an isoform of the glutamic acid decarboxylase enzyme, GAD65, present in approximately 60% of patients [2]. A paraneoplastic form of SPS is associated with antibodies to ampiphysin, a protein located on synaptic vessels. Symptomatic treatment with benzodiazepines, baclofen, or dantrolene can provide significant relief to patients. Given the association with autoantibodies, immunosuppressive regiments may be of benefit [2].

References

  1. Cicotto, Giuseppe; Blaya, Maike; Kelley, Roger. Stiff Person Syndrome. Neur Clinics 2013; 31: 319-328.
  2. Panzer, Jessica; Dalmau, Josep. Movement disorders in paraneoplastic and autoimmune disease. Cur Opin Neurol 2011; 24: 346-353.

Submitted by: Adam Numis, MD

Dr. Numis is a member of the Resident and Fellow Section of Neurology.

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