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[Stiff person syndrome associated with thymoma].

作者信息

Perri Marcella, Pellegrini Debora, Uribe Roca Claudia, Gonzalez Fabio, Buero Agustín, Chimondeguy Domingo, Bruetman Julio E

机构信息

Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina.

Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina. E-mail:

出版信息

Medicina (B Aires). 2023;83(4):626-630.

Abstract

Stiff-person syndrome is a rare neurological condition characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostration. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detection of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We report a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

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