Letter to the Editor February 14, 2019

Progressive Encephalomyelitis With Rigidity: Stiff Person Syndrome Variant Associated With Multiple Sclerosis

Shivani Naik, MD; Murali K. Kolikonda, MD; Steven Lippmann, MD

Prim Care Companion CNS Disord 2019;21(1):18l02360

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To the Editor: Stiff person syndrome (SPS) is characterized by progressive muscle spasms, rigidity, and hyperlordosis. Progressive encephalomyelitis with rigidity (PER), a variant of stiff person syndrome, is characterized by limb and trunk rigidity, painful muscle spasms, ocular motor disturbances, dysphagia, dysarthria, and hyperekplexia. PER differs from SPS usually based on the presence of brainstem, autonomic, and long-tract signs and also a more aggressive course.

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