Anti-glycin-receptor antibody related stiff-person syndrome under treatment with an immune checkpoint inhibitor

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Treatment of stiff person syndrome with rituximab.

This case report is about the novel use of the anti-CD20 antibody, rituximab, in the treatment of a 41 year old woman with stiff person syndrome. She was admitted to hospital as an emergency with prolonged and painful extensor spasms affecting the neck and back, arms, and legs. The disease had progressed despite a favourable initial response to conventional treatment with intravenous immunoglob...

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The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome.

OBJECTIVE To evaluate the clinical spectrum of anti-GAD-positive patients with stiff-person syndrome (SPS) and provide reproducible means of assessing stiffness. BACKGROUND SPS can be difficult to diagnose. Delineation of the clinical spectrum in a well defined population will increase diagnostic sensitivity. METHODS In 20 anti-GAD-positive patients with SPS (six men, 14 women), screened am...

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Stiff Person Syndrome.

Stiff-person syndrome or Moersch-Woltmann is a very rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms involving axial and limb musculature. It is an autoimmune disorder resulting in a malfunction of aminobutyric acid mediated inhibitory networks in the central nervous system. We describe a patient of stiff person syndrome.

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Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture. Most patients demonstrate exaggerated lum...

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ژورنال

عنوان ژورنال: Journal of Neurology

سال: 2020

ISSN: 0340-5354,1432-1459

DOI: 10.1007/s00415-020-10351-2