We have located links that may give you full text access.
English Abstract
Journal Article
Review
[Stiff-person syndrome and other myelopathies constitute paraneoplastic neurological syndromes].
Brain and Nerve = Shinkei Kenkyū No Shinpo 2010 April
Stiff-person syndrome (SPS) is an autoimmune neurological disorder characterized by rigidity of the trunk and proximal limb muscles, intermittent superimposed spasms, and increased sensitivity to external stimuli. It has been more than 50 years since Moerch and Woltman reported the first 14 cases with this syndrome. During the last half century, many autoantibodies discovered, such as anti-glutamic acid decarboxylase (GAD), anti-amphiphysin, anti-gephyrin, and anti-gamma-aminobutyric acid A receptor-associated protein (GABARAP) antibodies. There is strong evidence that in SPS, GABAergic neurotransmission is impaired by these pathogenic autoantibodies; however, the exact antigenic target remains unknown. This chapter focuses on the recent advances in the diagnosis, immunopathogenesis, and treatment of paraneoplastic SPS. Paraneoplastic SPS accounts for approximately 5% of all cases of SPS, and is associated with anti-amphiphysin, anti-gephyrin, and anti-Ri antibodies. In addition, author has reported cases of patients with SPS who were positive for anti-GAD antibodies and subsequently developed cancer. Because SPS often develops before the diagnosis of cancer, patients diagnosed with SPS should be monitored for the development of cancer. The treatment of SPS includes the administration of GABA enhancing and antispasmogenic drugs and immunomodulating therapies such as the administration of intravenous immunoglobulin (IVIG). Treatments for cancer occasionally produce symptomatic improvement in patients with paraneoplastic SPS. Although the understanding and treatment of SPS have evolved, the disease remains underdiagnosed. In the past, some patients with SPS have been diagnosed with psychiatric disorders. Therefore, it is important to increase awareness of SPS among practicing physicians.
Full text links
Related Resources
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app