Stiff person spectrum disorder (SPSD) phenotype

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Stiff person spectrum disorder (SPSD) phenotype. The sphincters are involved in half of cases. Fully referenced neurology checklist.

Stiff limb syndrome

  • The sphincters are involved in half of cases
  • The brainstem is involved in a third of cases
  • Most are anti-GAD antibody negative

Stiff person syndrome (SPS) plus: types

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Stiff person syndrome (SPS) with encephalomyelitis

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Other SPS variants

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Proposed diagnostic criteria

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References

  1. Lockman J, Burns TM. Stiff person syndrome. Pract Neurol 2007; 7:106-108.
  2. Chia NH, McKeon A, Dalakas MC, et al. Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria. Ann Clin Transl Neurol 2023 (Online ahead of print).
  3. Hadavi S, Noyce AJ, Leslie RD, Giovannoni G. Stiff person syndrome. Pract Neurol 2011; 11:272-282.
  4. Dalakas MC. Stiff person syndrome: advances in pathogenesis and therapeutic interventions. Curr Treat Options Neurol 2009; 11:102-110.

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