Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2015 Dec 22;7(12):e427.
doi: 10.7759/cureus.427.

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

Adnan Bashir Bhatti  1 , Zarine Anwar Gazali  2
Affiliations
Review

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

Adnan Bashir Bhatti et al. Cureus. .

Abstract

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.

Keywords: autoimmune diseases; glutamic acid decarboxylase antibody; neurological disorders; stiff man syndrome; stiff person syndrome.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Major achievements that contributed to Stiff Person Syndrome (SPS) treatment and research.
Figure 2
Figure 2. Clinical features of Stiff Person Syndrome (SPS).
See this image and copyright information in PMC

References

    1. Progressive fluctuating myscular rigidity”stiff man syndrome”; report on case and some observation in 13 other cases. Moersch FP, Woltman HW. Proc Staff Meet Mayo Clin. 1956;31:421–427. - PubMed
    1. A new and effective drug in the treatment of Stiff man syndrome: preliminary report. Howard FM. Proc Staff meet Mayo Clin. 1963;38:203–212. - PubMed
    1. Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. Solimena M, Folli F, Denis-Donini S, Comi GC, Pozza G, De Camilli P, Vicari AM. N England J Med. 1988;318:1012–1020. - PubMed
    1. Steroid- responsive and dependant stiff-man syndrome: a clinical and electrophysiological study of two cases. Piccolo G, Cosi V, Zandrini C, Moglia A. Ital J Neurol Sci. 1988;9:559–566. - PubMed
    1. Autoimmune Stiff Person Syndrome and related myelopathies: Understanding of electrophysiological and immunological processes. Rakocevic G, Floeter MK. Muscle Nerve. 2012;45:623–634. - PMC - PubMed

LinkOut - more resources