Stiff-Person Syndrome: A Treatment Update and New Directions

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A critical update on the immunopathogenesis of Stiff Person Syndrome.

BACKGROUND Stiff Person Syndrome (SPS) is a relatively rare but often overlooked autoimmune neurological disorder that targets antigens within the brain's inhibitory pathways resulting in incapacitating stiffness and spasms that impact on the patients' quality of life. Although a number of immunomodulating therapies significantly improve the patients' symptoms, the exact pathogenic mechanisms r...

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Treatment of stiff person syndrome with rituximab.

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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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Stiff person syndrome.

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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Stiff-person syndrome.

The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism ...

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ژورنال

عنوان ژورنال: Cureus

سال: 2020

ISSN: 2168-8184

DOI: 10.7759/cureus.11995