Paraneoplastic Stiff Person Syndrome With Anti-amphiphysin Antibodies Presenting With Pruritus as the Initial Manifestation: An Unusual Case

Stiff person syndrome (SPS), also known as Stiff-man syndrome/Moersch-Woltman syndrome, is a rare disorder of the central nervous system, first described in 1956, characterized by rigidity and stimulus-triggered painful muscle spasms of predominantly axial and proximal limb muscles. There are many v...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2023-02, Vol.15 (2), p.e35249
Main Authors: Raj, Aiswarya, Alapatt, Paul, Johny, Paul, Vv, Ashraf
Format: Article
Language:English
Subjects:
Allergy/Immunology
Antibodies
Autoimmune diseases
Breast cancer
Cancer therapies
Case reports
Electromyography
Encephalomyelitis
Metastasis
Morphology
Neurological disorders
Neurology
Oncology
Pain
Proteins
Pruritus
Steroids
Tumors
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Summary:Stiff person syndrome (SPS), also known as Stiff-man syndrome/Moersch-Woltman syndrome, is a rare disorder of the central nervous system, first described in 1956, characterized by rigidity and stimulus-triggered painful muscle spasms of predominantly axial and proximal limb muscles. There are many variants of SPS; these include the classical SPS, stiff leg syndrome, and paraneoplastic variant. The paraneoplastic variant of SPS is more common in patients with breast cancer with anti-amphiphysin antibodies, followed by colon carcinoma, lung carcinomas, thymoma, and Hodgkin's lymphoma. A possible autoimmune origin for the disease has been proposed, including antibodies against glutamic acid decarboxylase and amphiphysin. We thus describe a case of anti-amphiphysin antibody-positive SPS, which initially manifested with generalized pruritus. After extensive investigations and removing her underlying tumor, she reported complete recovery of her symptoms.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.35249