TY - CHAP M1 - Book, Section TI - Chapter 43. Stiff-Person Syndrome A1 - Gershanik, Oscar S. A2 - Watts, Ray L. A2 - Standaert, David G. A2 - Obeso, Jose A. PY - 2012 T2 - Movement Disorders, 3e AB - The stiff-person syndrome (SPS) is a rare neurologic disorder of uncertain cause, characterized by severe and incapacitating axial and proximal limb rigidity due to continuous motor unit activity. Rigidity is often enhanced by anxiety, sudden movements, or external stimuli causing intermittent painful muscle spasms, often leading to skeletal deformity. Variants of this disorder include focal involvement of one limb (“stiff-limb syndrome”), or additional neurological symptoms suggestive of involvement of subcortical gray matter (“progressive encephalomyelitis with rigidity and myoclonus”) (PEWR) and occasionally secondary to malignant disease (paraneoplastic SPS). Antineuronal antibodies often associated with other autoimmune diseases are characteristic features of this disorder. The disease follows a progressive, unremitting course, resulting in pronounced disability, if left untreated. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/28 UR - neurology.mhmedical.com/content.aspx?aid=55803743 ER -