Stiff person syndrome (SPS), stiff limb syndrome, jerking SPS and progressive encephalomyelitis with rigidity and myoclonus (PERM) are a family of rare, insidiously progressive diseases of the central nervous system. They all share the core clinical features of appendicular and axial rigidity caused by continuous involuntary motor unit activity, and superimposed stimulus-sensitive spasms. There is good evidence for a primary auto-immune aetiology. Anti-glutamic acid decarboxylase (anti-GAD) antibodies, specifically to the GAD65 isoform, are present in serum or cerebrospinal fluid of 60–80% of patients with SPS and its variants. A paraneoplastic form of SPS is recognized in about 5%, associated with a different profile of auto-antibodies. Repeated intravenous immunoglobulin is the mainstay of disease-modifying therapy in SPS. Rigidity and spasms may be treated symptomatically with benzodiazepines, baclofen, tiagabine and levetiracetam. After an initial progressive phase, patients with SPS generally stabilize over a period of months to years. However, 10% will require prolongedadmission to intensive care at some stage during the disease. Sudden death has been reported in asmany as 10% of patients because of unexplained metabolic acidosis or autonomic crises. The prognosis in paraneoplastic SPS, jerking SPS and PERM, in terms of mortality, is generally worse than in primary SPS.
Dr. Mark R. Baker, Institute of Neuroscience, The Henry Wellcome Building, The Medical School, University of Newcastle upon Tyne, Newcastle upon Tyne, NE2 4HH (UK), Tel. +44 191 2226897, Fax +44 191 2225227, E-Mail email@example.com
Number of Print Pages : 19
Book Serie: Frontiers of Neurology and Neuroscience, Vol. 26, Year 2009
Editor(s): Bogousslavsky, J. (Montreux)
ISSN: 1660-4431 (Print), eISSN: 1662-2804 (Online)
For additional information:
Book Title: Immune-Mediated Neuromuscular Diseases
Editor(s): Pourmand R (ed)
For additional information:
Copyright / Drug Dosage
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.