SPS: Understanding the complexity. Book Review

Full Text via DOI: 10.1016/j.jns.2019.06.021 PMID: 31377632

Cited authors

  • El-Abassi, Soliman, Villemarette-Pittman, England

Abstract

  • INTRODUCTION\nMETHOD\nRESULTS\nDISCUSSION\nStiff-person syndrome (SPS), first described in 1956 by Moersch and Woltman, is a progressive autoimmune disorder with core features of chronic fluctuating progressive truncal and limb rigidity and painful muscle spasms leading to gait difficulties, falls and an appearance that resembles tin soldiers. The syndrome is a rare, highly disabling disorder of the central nervous and frequently results in significant disability. Understanding of the etiology, clinical spectrum, diagnostic workup and therapeutic modalities for this painful and disabling disorder has vastly evolved over the past few years with more confidence in classifying and treating the patients. The purpose of this review is to increase the awareness, early detection, and treatment of this disabling disease.\nPubMed was searched, all date inclusive, using the following phrases: stiff person syndrome,anti-Glutamic acid decarboxylase (Anti-GAD) antibody syndrome, Progressive encephalomyelitis with rigidity and myoclonus (PERM), and Paraneoplastic Stiff Person syndrome. No filters or restrictions were used. A total of 888 articles were identified.\nThe results were narrowed to 190 citations after excluding non-English and duplicate reports. Clinical presentation, laboratory testing, treatment, and prognosis were categorized and summarized.\nIn this article we will discuss the epidemiology, presentation and classification. Explain the pathophysiology of SPS and the autoimmune mechanisms involved. Discuss the diagnostic approach and treatments available, as well as, the prognosis and outcome.

Authors

Publication date

  • 2019

Published in

International Standard Serial Number (ISSN)

  • 0022-510X

Start page

  • 137

End page

  • 149

Volume

  • 404