21. Patient 40 Selftest Patient 40 stiffperson syndrome 1. Which of the following is a diagnosticfeature of the classical stiff-person syndrome? A. rigidity http://www.bcm.tmc.edu/neurol/challeng/pat40/selftest.html

Patient #40 Stiff-person Syndrome 1. Which of the following is a diagnostic feature of the classical stiff-person syndrome? A. rigidity with superimposed spasms B. rigidity with myoclonus C. dystonia and intermittent spasms D. depression and spasms 2. Which of the following endocrine abnormalities have been described in patients with stiff-person syndrome? A. insulin-dependent diabetes mellitus B. hypothyroidism C. premature ovarian dysfunction D. all of the above 3. Which of the following electromyographic findings stongly suggests a diagnosis of stiff-person syndrome? A. increased motor unit activity on exercise B. decreased muscle activity or electromyographic silence C. signficant decrement or repetitive nerve stimulation D. increased spontaneous motor unit activity which cannot be abolished on relaxation 4. Which of the following autoantibodies have been associated with the stiff-person syndrome? A. anti-glutamic acid decarboxylase (GAD) B. anti-amphiphysin C. anti-thyroglobluin D. a and b E. all of the above 5. Which of the following treatments would be LEAST useful for patients with the stiff-person syndrome?

22. Patient 40 Test Answer 4e Patient 40 Question 4 stiffperson syndrome You are correct. All of the above autoantibodieshave been reported in patients with the stiff-person syndrome. http://www.bcm.tmc.edu/neurol/challeng/pat40/answer4e.html

Patient #40 Question 4 Stiff-person Syndrome You are correct. All of the above autoantibodies have been reported in patients with the stiff-person syndrome. The best evidence for a potential pathogenic role for autoantibodies in the stiff-person syndrome is found in the association with anti-GAD antibodies. Research is ongoing as to whether anti-amphiphysin antibodies may also play a pathogenic role. Antibodies against endocrine glands may coexist with the stiff-person syndrome, and may potentially play a pathogenic role in autoimmune disorders associated with this syndrome. Department of Neurology, Baylor College of Medicine

23. Stiff-Person Syndrome The Official Patient's Sourcebook on stiffperson syndrome (Moersch-WoltmannSyndrome; Muscular Rigidity - Progressive Spasm; Stiff Man Syndrome). http://www.icongrouponline.com/health/Stiff_Person_Syndrome.html

ICON Health Publications Official Health Sourcebooks The Official Patient's Sourcebook on STIFF-PERSON SYNDROME (Moersch-Woltmann Syndrome; Muscular Rigidity - Progressive Spasm; Stiff Man Syndrome) Revised and Updated for the Internet Age Paperback Book Order by phone: 800-843-2665 (within USA) 1-201-272-3651 (from outside USA) Electronic File * E-Book version sent via e-mail in 2 business days Electronic File * E-Book version sent via e-mail in 2 business days Pages Price $28.95(USD) ISBN Published Synopsis A comprehensive manual for anyone interested in self-directed research on Stiff Person Syndrome. Fully referenced with ample Internet listings and glossary. Related Conditions/Synonyms Moersch-Woltmann Syndrome; Muscular Rigidity - Progressive Spasm; Stiff Man Syndrome Description Table of Contents Introduction Overview Organization Scope Moving Forward PART I: THE ESSENTIALS Chapter 1. The Essentials on Stiff-Person Syndrome: Guidelines Overview What Is Stiff-Person Syndrome? Is There Any Treatment? What Is the Prognosis? What Research Is Being Done?

24. The Health Library — Nervous System And Brain Stiff Person Syndrome. stiffperson syndromeNINDS. Stiff-Man SyndromeAmericanAutoimmune Related Diseases Association. Stiff Person SyndromeNINDS. http://healthlibrary.stanford.edu/resources/internet/bodysystems/nervoussystem2.

Diseases and Disorders Use these links to jump directly to your topic of interest: Anatomy Autoimmune Nervous System Diseases Brain Diseases Brain Injury ... Transplantation Nervous System and Brain: Page 1 Page 3 Page 4 Page 5 ... Page 9 Dementias General Information Dementia:MEDLINEplus Forgetfulness:It's Not Always What You Think: National Institute on Aging (NIA) Dementia:Family Caregiver Alliance Mild Cognitive Impairment (NIA Memory Impairment Study):NIA ... Tauopathies: New Discoveries, New Knowledge:NIA Alzheimer's Disease Alzheimer's Disease:MEDLINEplus Alzheimer's Disease:National Institute of Mental Health (NIMH) About Alzheimer's Disease:NIA Alzheimer's Disease,Unraveling the Mystery: NIA ... Let's Talk About When Someone You Love Has Alzheimer's Disease:netLibrary [for Kids] Speaking Our Minds : Personal Reflections From Individuals With Alzheimer's:netLibrary Early-Stage Alzheimer's Disease:Family Caregiver Alliance Alzheimer's Disease and Plaques:Society for Neuroscience Interpreting Alzheimer's Abnormalities:Society for Neuroscience ... Early-Onset Familial Alzheimer Disease:GeneReviews, University of Washington See also Senior Health, Alzheimer's Care

25. The Health Library — Immune System Scleroderma. Fibromyalgia. Sjogren's Syndrome. Grave's Disease. stiffperson syndrome.Guillain-Barre Syndrome. Transverse Myelitis. Goodpasture Syndrome. Uveitis. http://healthlibrary.stanford.edu/resources/internet/bodysystems/immune3.html

Diseases and Disorders Use these links to jump directly to your topic of interest: Acquired Immune Deficiency Syndrome Allergies Autoimmune Diseases General Immune System ... Transplantation Immunology Immune System: Page 1 Page 2 Autoimmune Diseases Autoimmune Diseases (General):MEDLINEplus Understanding Autoimmune Diseases:National Institute of Allergies and Infectious Diseases Frequently Asked Questions About Autoimmunity:American Autoimmune Related Diseases Association Coping With Autoimmunity:American Autoimmune Related Diseases Association ... Autoimmune Disease in Women:American Autoimmune Related Diseases Association Index to Specific Autoimmune Diseases Addison's Disease Immune Thrombocytopenic Purpura Alopecia Areata IgA Nephropathy ... Wegener's Granulomatosis Antiphospholipid Syndrome Antiphospholipid Antibody Syndrome:Aetna InteliHealth Antiphospholipid Syndrome:Women's Health, UK Antiphospholipid Syndrome:American Autoimmune Related Diseases Association Chronic Fatigue Syndrome Chronic Fatigue Syndrome:MEDLINEplus Chronic Fatigue Syndrome:NIAID About Chronic Fatigue and Immune Dysfunction Syndrome:CFIDS Association of America Chronic Fatigue Syndrome:Centers for Disease Control and Prevention ... Chronic Fatigue Syndrome State of the Science Conference (2000):U.S. Dept of Health and Human Services [PDF] Chronic Fatigue Immune Dysfunction Syndrome:American Autoimmune Related Diseases Association Essential Mixed Cryoglobulinemia Essential Mixed Cryoglobulinemia:MedicineNet Cryoglobulinemia:Johns Hopkins Vasculitis Center Essential Mixed Cryoglobulinemia:American Autoimmune related Diseases Association Fibromyalgia

26. Untitled Document Unwanted motor unit activation is known to occur in the stiffperson syndrome and,consequently, this syndrome will be the core dysfunction of the course. http://www.akm.ch/ens2003/programme/teachingCourse_sa.htm

Teaching Courses (half day) Saturday, June 14, 2003 Dizziness and vertigo Chairpersons: M. Dieterich, Mainz; T. Yousry, London The visual system and vertigo A. Bronstein, London Assessing vestibular function: which tests, when? M. Fetter, Karlsbad-Langensteinbach The vestibular system and vertigo M. Dieterich, Mainz Imaging vestibular disorders T. Yousry, London Clinical neurophysiology Chairpersons: J. Valls-Solé, Barcelona; H.M. Meinck, Heidelberg The role of EMG evaluation in the diagnosis of muscle hyperactivity syndromes J. Valls-Solé, Barcelona Myokymia and neuromyotonia L. Gutmann, Morgantown Stiff-person syndrome H.M. Meinck, Heidelberg Encephalomyelitis with rigidity P. Brown, London Morvan’s syndrome R. Liguori, Bologna Clinical neuropathology Chairpersons: K.V. Toyka, Würzburg; A. Probst, Basel The experts in the audience are encouraged to contribute in their views and present interesting cases of their own but, please, contact a faculty member at least 2 weeks ahead of the course to fit it in appropriately. Brain pathology in dementia/neurodegeneration A. Probst, Basel

27. References Levy LM, Dalakas MC, Floeter M. The stiffperson syndrome an autoimmune disorderaffecting neurotransmission of gamma-aminobutyric acid. Ann Intern Med. http://www.medscape.com/content/2001/00/41/50/415033/415033_sug.html

Suggested Reading for: Case 9: A Woman with Spasms and Stiff Legs Antoine JC, Absi L, Honnorat J, et al. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors, Arch Neurol. 1999;56:172-177. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. Neurol Neurosurg Psychiatry. 1998;65:633-640. Brown P, Marsden CD. The stiff man and stiff man plus syndrome. J Neurol. 1999;246: 648-652. Fathallah-Shaykh HM. Paraneoplastic neurological syndromes: paraneoplastic or neurological? Arch Neurol. 1999;56:151-152. Floyd S, Butler MH, Cremona O, et al. Expression of amphiphysin I, an autoantigen of paraneoplastic neurological syndromes, in breast cancer. Mol Med. 1998;4:29-39. Levy LM, Dalakas MC, Floeter M. The stiff-person syndrome: an autoimmune disorder affecting neurotransmission of gamma-aminobutyric acid. Ann Intern Med. 1999;131:522-530. Murinson BB, Vincent A. Stiff-person syndrome: autoimmunity and the central nervous system. CNS Spectrums. 2001;6:427-433.

28. References 2001 Medscape Portals, Inc. Table 1. Differential Diagnosis of StiffPersonSyndrome. Peripheral Central stiff-person syndrome. Focal lesions of spinal cord http://www.medscape.com/content/2001/00/41/50/415033/415033_tab.html

Tables for: Case 9: A Woman with Spasms and Stiff Legs Table 1. Differential Diagnosis of Stiff-Person Syndrome Peripheral Isaac's syndrome Central Stiff-person syndrome Focal lesions of spinal cord Intrinsic neoplasm Syringomyelia Traumatic Vascular Paraneoplastic segmental myelitis Infectious/toxic causes Acute poliomyelitis Borreliosis Encephalomyelitis lethargica Acute or chronic tetanus Strychnine Tetanus Table 2. Clinical Syndromes Classic SPS Paraneoplastic SPS Patient population Sporadic Occult or diagnosed cancer Autoantibody GAD, Other* Amphiphysin, Hu , Gephyrin Disease association Type 1 diabetes mellitus, intractable epilepsy Breast cancer and SCLC Colon cancer, Hodgkin's disease Clinical presentation Increased lumbar lordosis, proximal limb stiffness Upper or lower limb stiffness EMG Continuous motor unit activity (CMUA) Continuous motor unit activity NCS Normal Normal Antimicrosomal, antithyroglobulin, anti-islet cell, and anti-parietal cell antibodies. Anti-Hu antibodies can be seen in a majority of patients with antiampiphysin antibodies and small cell lung cancer (SCLC).

29. Directory :: Look.com stiffperson syndrome (4) See Also. Sites. MCW HealthLink An article aboutstiff-person syndrome, a rare progressive neurological disorder. http://www.look.com/searchroute/directorysearch.asp?p=594583

30. WebGuest - Open Directory : Health : Conditions And Diseases : Neurological Diso Sites MCW HealthLink An article about stiff-person syndrome, a rareprogressive neurological disorder. NORD - Stiff Person Syndrome http://directory.webguest.com/index.cgi/Health/Conditions_and_Diseases/Neurologi

Browse thru 1000's of books about health, mind and body: About Us Privacy Statement Acceptable Use Policy Legal Notices ... Contact Us the entire directory only in Muscle_Diseases/Stiff-Person_Syndrome Top Health Conditions and Diseases Neurological Disorders ... Muscle Diseases : Stiff-Person Syndrome See also: Health: Conditions and Diseases: Neurological Disorders: Spinal Cord Health: Conditions and Diseases: Rare Disorders Sites: MCW HealthLink - An article about Stiff-person syndrome, a rare progressive neurological disorder. NORD - Stiff Person Syndrome - Offers synonyms, a general discussion and further resources. Stiff-Person Syndrome - Information sheet compiled by National Institute of Neurological Disorders and Stroke. Last update: 3:19 PT, Tuesday, March 11, 2003 Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor

31. The Infography About Stiff-Man Syndrome Floeter MK, VallsSole J, Toro C, Jacobowitz D, Hallett M. Physiologic studiesof spinal inhibitory circuits in patients with stiff-person syndrome. http://www.infography.com/content/865274012491.html

Search The Infography: Stiff-Man Syndrome A professor whose research specialty is Stiff-Man Syndrome recommends these sources. Six Superlative Sources Lorish TR, Thorsteinsson G, Howard FM Jr. Stiff-man syndrome updated. Mayo Clin Proc. 1989 Jun;64(6):629-36. Review. Solimena M, Folli F, Aparisi R, Pozza G, De Camilli P. Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med. 1990 May 31;322(22):1555-60. Solimena M, De Camilli P. Autoimmunity to glutamic acid decarboxylase (GAD) in Stiff-Man syndrome and insulin-dependent diabetes mellitus. Trends Neurosci. 1991 Oct;14(10):452-7. Review. Stayer C, Meinck HM. Stiff-man syndrome: an overview. Neurologia. 1998 Feb;13(2):83-8. Review. Folli F, Solimena M, Cofiell R, Austoni M, Tallini G, Fassetta G, Bates D, Cartlidge N, Bottazzo GF, Piccolo G, et al. Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer. N Engl J Med. 1993 Feb 25;328(8):546-51. Butler MH, Hayashi A, Ohkoshi N, Villmann C, Becker CM, Feng G, De Camilli P, Solimena M. Autoimmunity to gephyrin in Stiff-Man syndrome. Neuron. 2000 May;26(2):307-12. Other Excellent Sources Gordon EE, Januszko DM, Kaufman L. A critical survey of stiff-man syndrome. Am J Med. 1967 Apr;42(4):582-99. Review.

32. Mary Kay Floeter, Electromyography Section, NINDS Sandbrink F, NA Syed, M Fujii, MC Dalakas, and MK Floeter (2000) Motorcortex excitability in stiffperson syndrome, Brain 123, 2231-2239 . http://intra.ninds.nih.gov/Lab.asp?Org_ID=97

33. Ivanhoe's Medical Breakthroughs - Discussion Groups wkhazen03@cox.net 03/17/03; stiffperson syndrome - judy nicol 03/06/03» RE stiff-person syndrome dschaerer@hotmail.com 03/07/03; http://www.ivanhoe.com/discussion/p_discussion.cfm?forumID=84&action=msglist

34. Stiff-man Syndrome NLM Medline definition of stiffperson syndrome A condition characterized by persistentspasms (SPASM) involving multiple muscles, primarily in the lower http://www.ion.ucl.ac.uk/library/patient/stiff.htm

Stiff-Man Syndrome NLM Medline definition of stiff-person syndrome: A condition characterized by persistent spasms (SPASM) involving multiple muscles, primarily in the lower limbs and trunk. The illness tends to occur in the fourth to sixth decade of life, presenting with intermittent spasms that become continuous. Minor sensory stimuli, such as noise and light touch, precipitate severe spasms. Spasms do not occur during sleep and only rarely involve cranial muscles. Respiration may become impaired in advance cases. Pubmed Medline search on stiff-man syndrome Pubmed Medline search on stiff-person syndrome Stiff-person Syndrome factsheet: National Institute for Neurological Disorders and Stroke Support Groups National Organization for Rare Disorders (NORD) P.O. Box 8923, New Fairfield, CT 06812-1783 Tel: (203) 746-6518 or (800) 999-6673

35. Cleveland Clinic Health System - Health Information Search Results The following information is available for the topic StiffPersonSyndrome. Please select one. , stiff-person syndrome. New Search. http://www.cchs.net/health/getcontents.asp?DocID=do-query&TopicId=1361

36. Cleveland Clinic Health System - Health Information , Stenosis, Spinal. , Steroids. -, Steroids, Anabolic. -, stiff-person syndrome.-, Stomach Cancer (Gastric Cancer). -, Strep Throat. -, Stress Management. -, StressTest. http://www.cchs.net/health/getcontents.asp?DocID=do-query&txtChar=S

37. Neuromuscular Diseases stiffperson syndrome About stiff-person syndrome - NINDS (US); StiffmanSyndrome Foundation Geilman family - (US); Stiff-man Syndrome http://www.mic.ki.se/Diseases/c10.668.html

search help staff Neuromuscular Diseases Patients and laypersons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider. Motor Cortex illustration Earles et al. ] - Furman Univ. (US) EMG and Nerve Conductions Homepage JF Jabre The Neuromuscular Disease Information Center - REHABinfo Network/UC-Davis (US) Neuromuscular Diseases - MDA (US) About some Neuromuscular Diseases - Wake Forest Univ. Neuromuscular Disorders Information - Musc Dystrophy Group of (UK)+ N. Ireland Neuromuscular Disease Center , and one-page summaries of Differential Diagnoses of Neuromuscular Syndromes - School of Medicine/Washington Univ., St Louis (US) Diagnostic Criteria for Neuromuscular Disorders - European Neuromuscular C. Motor Neuron Disease Lower and Upper Motor Neuron Disorders - Merck Manual Motor Neuron Awareness S Killeen ] - (IE) Clinical Features of Motor Neuron Disease - Bethlehem Health Care, Inc. (AU) Motor Neuron Disease and Animal Models - WUSTL (US) About Primary Lateral Sclerosis - NINDS/NIH (US) Primary Lateral Sclerosis F Reyerse Primary Lateral Sclerosis (PLS) C Armon ] - eMedicine A question + answer re Primary Lateral Sclerosis by the experts at MDA A two pages Open letter to Dr. Prahl about his anti-PLS drug C-3036

38. Zeal.com - United States - New - Personal - Health - Conditions & Illnesses - Br Add a Site Profile. 1. Cleveland Clinic stiff-person syndrome http//www.clevelandclinic.org/health/health-info/docs/1300/ http://www.zeal.com/category/preview.jhtml?cid=274722

39. IM Abstract 40-9 Case 20 stiffperson syndrome Associated with Cerebellar Ataxia and High GlutamicAcid Decarboxylase Antibody Titer Satoshi Kono, Hiroaki http://www.naika.or.jp/im/im40/ab40-9/c400920.html

Stiff-person Syndrome Associated with Cerebellar Ataxia and High Glutamic Acid Decarboxylase Antibody Titer Satoshi Kono, Hiroaki Miyajima, Masahiro Sugimoto, Yoji Suzuki, Yoshitomo Takahashi and Akira Hishida From the First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu Glutamic acid decarboxylase (GAD) is the main target of humoral autoimmunity in patients with insulin-dependent diabetes mellitus (IDDM) and stiff-person syndrome. We reviewed the case of a 46-year-old woman who had cerebellar ataxia before getting stiff-person syndrome and IDDM with high anti-GAD autoantibody titers. This was a rare case in which there were both the clinical symptoms of stiff-person syndrome and cerebellar ataxia. In western blot analysis her serum reacted with 65-kDa proteins from rat cerebellum, cerebral cortex, and spinal cord. Autoantibodies to GAD may cause functional impairment of ƒÁ-aminobutyric acid (GABA) neurons in the spinal cord as well as in the cerebellum. ÅIXV“ú2001/11/26@E-mail : naika@naika.or.jp

40. Stiff-Person Syndrome Website Results :: Linkspider UK stiffperson syndrome Websites from Linkspider UK. Keyword Stiff-PersonSyndrome. Linkspider UK Directory stiff-person syndrome Search for. http://www.linkspider.co.uk/Health/ConditionsandDiseases/NeurologicalDisorders/M

Stiff-Person Syndrome Websites from Linkspider UK Keyword: Stiff-Person Syndrome Linkspider UK Directory Stiff-Person Syndrome Search for Directory Tree: Top Health Conditions and Diseases Neurological Disorders ... Muscle Diseases : Stiff-Person Syndrome (4) Add URL Advertise Here! Personalize Amazon ... Weather See Also: Health: Conditions and Diseases: Neurological Disorders: Spinal Cord Health: Conditions and Diseases: Rare Disorders MCW HealthLink - An article about Stiff-person syndrome, a rare progressive neurological disorder. NORD - Stiff Person Syndrome - Offers synonyms, a general discussion and further resources. WorldMedicus - A description of Stiff-Person syndrome, the alternate names and a link to articles. Stiff-Person Syndrome - Information sheet compiled by National Institute of Neurological Disorders and Stroke.

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