Recent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients

Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gai...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2015-12, Vol.7 (12), p.e427-e427
Main Authors: Bhatti, Adnan Bashir, Gazali, Zarine Anwar
Format: Article
Language:English
Subjects:
Allergy/Immunology
Breast cancer
Cognitive behavioral therapy
Genetics
Monoclonal antibodies
Neurology
Pediatrics
Targeted cancer therapy
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Summary:Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic variant of SPS is more common in patients with breast cancer who harbour amphiphysin antibodies, followed by colon cancer, lung cancer, Hodgkin's disease, and malignant thymoma.  Currently, the treatment for SPS revolves around improving the quality of life by reducing the symptoms as far as possible with the use of GABAergic agonists, such as diazepam or other benzodiazepines, steroids, plasmapheresis, and intravenous immunoglobulin (IVIG). There have been random clinical trials with Rituximab, but nothing concrete has been suggested. A treatment approach with standard drugs and cognitive behavioral therapy (CBT) seems to be promising.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.427