21. Information About Stiff-person Syndrome stiffperson syndrome is a rare progressive neurological disorder Stiff-personsyndrome may begin as recurring episodes of stiffness and spasms, http://www.mamashealth.com/syndrome/stiff.asp

Mamashealth.com Home Syndromes Pages Neuroleptic Malignant Nighttime Eating Patellofemoral Pain Piriformis ... Zollinger-Ellison Links More Syndromes Email Mama What is Stiff-Person Syndrome? Stiff-person syndrome is a rare progressive neurological disorder characterized by constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs. It usually begins in young adults, first involving muscles of the trunk and progressing to affect muscles of the legs. Stiff-person syndrome may begin as recurring episodes of stiffness and spasms, often precipitated by surprise or minor physical contact. Autoimmune disorders such as diabetes pernicious anemia , and thyroiditis may occur more frequently in patients with stiff-person syndrome. What Causes Stiff-Person Syndrome? The exact cause of stiff person syndrome is not known. Symptoms of Stiff-Person Syndrome? Symptoms of stiff-person syndrome may occur gradually. The most common symptoms are: Constant painful contractions Muscle spasms - esp. back and upper legs but sometimes the arms and neck.

22. Information About Stiff-person Syndrome SturgeWeber syndrome is a neurological disorder indicated at birth by One of the main complications of Sturge-Weber syndrome is the loss of nerve cells http://www.mamashealth.com/syndrome/sturge.asp

Mamashealth.com Home Syndromes Pages Neuroleptic Malignant Nighttime Eating Patellofemoral Pain Piriformis ... Zollinger-Ellison Links More Syndromes Email Mama What is Sturge-Weber Syndrome? Sturge-Weber syndrome is a neurological disorder indicated at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face. The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the trigeminal nerve just beneath the surface of the face. Symptoms of Sturge-Weber Syndrome? Some common symptoms of Sturge-Weber Syndrome are: Seizures Seizures begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark. White part of eye may be a reddish color. What Causes Sturge-Weber Syndrome? The exact cause of Sturge-Weber Syndrome is unknown. Types of Sturge-Weber Syndrome? There are 4 main types of Sturge-Weber Syndrome Type 1 Type 1 is the most common type. Characterized by facial and brain angiomas and may involve glaucoma.

23. Diagnose-Me: Condition: Stiff Man Syndrome Stiff person syndrome is rather unique among neurologic diagnoses because of Individuals with stiffperson syndrome may have difficulty making sudden http://www.diagnose-me.com/cond/C550180.html

Home FAQ Start The Analyst Stiff Man Syndrome Contributing risk factors Other conditions that may be present Stiff Man Syndrome , also known as Moersch-Woltmann Syndrome or Stiff Person Syndrome or SPS, is a neuromuscular condition in which a hyperactive startle reflex results in the contraction of the muscles, thus causing violent spasms. These spasms are capable of slamming the victim into walls and furniture. The disorder's cause is unknown, although evidence is pointing increasingly to the fact that it is an autoimmune disorder. Stiff person syndrome is rather unique among neurologic diagnoses because of its lack of significant similarity to any other neurologic disease. Although rare, once observed it is quite unforgettable. Possibly the closest related disease is tetanus because both conditions affect peripheral inhibition via central mechanisms and both conditions inhibit central gamma-aminobutyric acid GABA ) systems. Although they appear completely normal, those afflicted with Stiff Man Syndrome must avoid any situation where they might be exposed to sudden sounds or stimulation. Often, they remain isolated in their home and excluded from normal living - orphaned from society by this rare and ruthless disorder. SPS is a rare progressive neurological disorder characterized by constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs.

24. Stiff Person Syndrome stiffperson syndrome is an extremely rare neurological disorder characterizedby progressive muscle stiffness (rigidity) and spasms. http://www.bchealthguide.org/kbase/nord/nord326.htm

var hwPrint=1;var hwDocHWID="nord326";var hwDocTitle="Stiff Person Syndrome";var hwRank="1";var hwSectionHWID="nord326-Header";var hwSource="en-caQ2_05";var hwDocType="Nord"; National Organization for Rare Disorders, Inc. Stiff Person Syndrome Important It is possible that the main title of the report Stiff Person Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Moersch-Woltmann Syndrome Muscular Rigidity - Progressive Spasm SMS Stiff Man Syndrome Disorder Subdivisions None General Discussion Stiff-person syndrome is an extremely rare neurological disorder characterized by progressive muscle stiffness (rigidity) and spasms. It usually begins in young adults, first involving muscles of the trunk and progressing to affect muscles of the legs. This syndrome may begin as recurring (intermittent) episodes of stiffness and spasms, often precipitated by surprise or minor physical contact. Those affected have a characteristic stiff-legged way of walking (gait) and increase in the curvature of the spine (lordotic posture). The exact cause of stiff person syndrome is not known. Resources American Autoimmune Related Diseases Association, Inc.

25. Stiff-Person Syndrome Topic - Unified Search Environment stiffperson syndrome Topic Tree stiff-person syndrome MSH/MH/D016750 MSH/PM/D016750 MSH/PM/D016750 Moersch-Woltmann Syndrome MSH/EP/D016750 http://www.use.hcn.com.au/portals/shared/subject.`Stiff-Person Syndrome`/home.ht

Stiff-Person Syndrome Topic Tree Definition: 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 advanced cases. (Adams et al., Principles of Neurology, 6th ed, p1492; Neurology 1998 Jul;51(1):85-93) Synonyms and Source Vocabularies: Stiff-Person Syndrome Moersch-Woltmann Syndrome Stiff-Man Syndrome MUSCULAR RIGIDITY AND SPASM, PROGRESSIVE Stiffman Syndrome Nervous System Diseases Central Nervous System Diseases Cranial Nerve Diseases Demyelinating Diseases ... Neurologic Manifestations Neuromuscular Diseases Chronic Fatigue Syndrome Muscular Atrophy, Spinal Myopathy Peripheral Nervous System Diseases ... Autonomic Nervous System Diseases

26. IngentaConnect Lupus Revealing Itself After A Stiff-person Syndrome Lupus revealing itself after a stiffperson syndrome. Authors Goëb, V.; Dubreuil,F.; Cabre, P.; Jean-Baptiste, G.; Arfi, S. Source Lupus, Volume 13, http://www.ingentaconnect.com/content/arn/lu/2004/00000013/00000003/art00013

Home About Ingenta Ingenta Labs Help For Publishers Why go online? Why choose IngentaConnect? Why choose CustomConnect? Access and authentication ... Contact us For Researchers About IngentaConnect Search and browse Publications available Accessing articles ... Register For Librarians Why choose IngentaConnect? Why choose IngentaConnect Premium? Activating Subscriptions Purchasing articles ... Browse Publications Lupus revealing itself after a stiff-person syndrome Authors: Source: Lupus , Volume 13, Number 3, 1 March 2004, pp. 215-215(1) Publisher: Hodder Arnold Journals View Table of Contents full text options Document Type: Letter section The full text article is available for purchase $32.08 plus tax The exact price (including tax) will be displayed in your shopping cart before you check out. You will be able to remove this item from your shopping cart at any time before you have completed check-out. Back to top Terms and Conditions Page Help Quick Search Electronic content Fax/Ariel content Journal or book title Sign in: User name Password Remember me forgotten your password?

27. Stiff-person Syndrome Although the name stiffperson syndrome is more accurate in both the epidemiologicand Neurology Movement disorders stiff-person syndrome http://www.medlink.com/PublicCIP.ASP?access=public&UID=MLT000DA&code=

28. The Health Library — Nervous System And Brain Stiff Person Syndrome. stiffperson syndromeNINDS Stiff Person SyndromeeMedicine Immunotherapy Treatment Shows Dramatic Results for . http://healthlibrary.stanford.edu/resources/internet/bodysystems/neuro_spinaldis

Diseases and Disorders Use these links to jump directly to your topic of interest in Nervous System and Brain: Signs and Symptoms: Pain Neurologic Manifestations Language Disorders Perceptual Disorders Central Nervous System: Brain Diseases Brain Injury Infections Malformations ... Tumors Other Diseases and Disorders: Autoimmune Nervous System Diseases Autonomic Nervous System Diseases Cranial Nerve Disorders Degenerative Nervous System Diseases ... Sleep Disorders Other Topics: General Nervous System and Brain Anatomy Diagnostic Procedures Neural Transplantation Spinal Cord Diseases (Jump to: Overviews Cauda Equina Syndrome Cervical Spondylotic Myelopathy Hydromyelia ... Tranverse Myelitis Overviews Spinal Cord Disorders:Merck Manual Spinal Cord Diseases: MedlinePlus Cauda Equina Syndrome Cauda Equina Syndrome:AANS Cauda Equina Syndrome FAQ:Cauda Equina Syndrome Support Group Cauda Equina Syndrome:eMedicine Cervical Spondylotic Myelopathy Cervical Spondylotic Myelopathy (CSM):American Academy of Family Physicians Cervical Spondylotic Myelopathy: A Common Cause of Spinal Cord Dysfunction in Older Persons:American Academy of Family Physicians Understanding Cervical Spondylotic Myelopathy:Spine-health.com Hydromyelia Hydromyelia:NINDS Neural Tube Defects See Genetics and Birth Defects, Neural Tube Defects

29. Arch Neurol -- Abstract: Brain {gamma}-Aminobutyric Acid Changes In Stiff-Person Brain {gamma} Aminobutyric Acid Changes in stiff-person syndrome BackgroundPatients with stiff-person syndrome (SPS) have circulating antibodies http://archneur.ama-assn.org/cgi/content/abstract/62/6/970

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 62 No. 6, June 2005 Featured Link E-mail Alerts Original Contribution Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Related articles in this issue Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Levy LM Dalakas MC Contact me when this article is cited Topic Collections Musculoskeletal Syndromes (Chronic Fatigue, Gulf War) Neurology, Other Topic Collection Alerts Brain -Aminobutyric Acid Changes in Stiff-Person Syndrome Lucien M. Levy, MD, PhD Igor Levy-Reis, MD Mavis Fujii, MD Marinos C. Dalakas, MD Arch Neurol. Patients with stiff-person syndrome (SPS) have circulating antibodies against glutamic acid decarboxylase, the rate-limiting enzyme responsible for the synthesis of -aminobutyric and unexpected spasms can be explained on the basis of reduced or impaired inhibitory neurotransmitters, such as GABA, it is

30. Arch Neurol -- Abstract: Stiff-Person Syndrome Following West Nile Fever, June 2 Background stiffperson syndrome is a rare autoimmune disorder associated withantibodies against glutamic acid decarboxylase (GAD), the key enzyme in http://archneur.ama-assn.org/cgi/content/abstract/61/6/938

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 61 No. 6, June 2004 Featured Link E-mail Alerts Observation Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Hassin-Baer S Mendelson E Contact me when this article is cited Topic Collections Viral Infections Neuropathology Topic Collection Alerts Stiff-Person Syndrome Following West Nile Fever Sharon Hassin-Baer, MD Eilon D. Kirson, MD, PhD Lester Shulman, PhD Aron S. Buchman, MD Hanna Bin, PhD Musa Hindiyeh, PhD Lea Markevich Ella Mendelson, PhD Arch Neurol. Stiff-person syndrome is a rare autoimmune disorder associated with antibodies against glutamic acid decarboxylase (GAD), the key enzyme in -aminobutyric acid synthesis. In most

31. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Neurological_Disor stiffperson syndrome - Information sheet compiled by National Institute of NORD Stiff Person Syndrome - Offers synonyms, a general discussion and http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Neurologica

@import "/styles/navbar.css"; @import "/styles/tabStyles.css"; Set home page Bookmark site Add search Latest News ... Email to friend Text Size A A A Front Page ... Muscle Diseases : Stiff-Person Syndrome Subtopics See Also: Health: Conditions and Diseases: Neurological Disorders: Spinal Cord Health: Conditions and Diseases: Rare Disorders Search Google: Stiff-Person Syndrome News Books Links ... UF Scientist Finds Unexpected Link Between Cat And Human AIDS Viruses (September 9, 2005) full story Mayo Clinic Develops New Coma Measurement System (September 8, 2005) full story Nitric Oxide Could Extend Fertility (September 8, 2005) Biochemistry , an American Chemical Society journal. full story Scientists Discover Scaramanga Gene's Bond With Breast Cancer (September 7, 2005) full story Flipped, Expelled, Copied, And Shrunk: Researchers Document Dramatic Genome Alterations During Primate Evolution (September 6, 2005) Genome Research presents a series of studies that provide insight into the evolution and variation of primate genomes. The issue will appear online and in print on September 1, concomitant with the publication of the chimpanzee genome sequence in the journal Nature full story K-State Researchers Study Insects' Immune System (September 5, 2005)

32. Baylor Neurology Case Of The Month The stiffperson syndrome, although described as a rare disorder, In thisregard, three forms of the stiff-person syndrome are generally recognized 1) http://www.bcm.edu/neurology/challeng/pat40/summary.html

Patient #40 Summary and Discussion Madhavi Thomas, M.D. Diagnosis: Stiff-person Syndrome Summary and Differential Diagnosis The clinical presentation of this patient is a relatively typical case of stiff-person syndrome . The patient had stiffness and rigidity of muscles, predominantly in the lower limbs, which resulted in slowly progressive impairment of her ability to ambulate. She had frequent superimposed episodes of spasms, precipitated by emotional stress and by sudden movements. Her intellectual functions were normal. She had increased muscle tone and hyperreflexia on examination, consistent with her stated complaints, and occurring in the absence of sensory findings. The electromyographic study showed continuous muscle activity despite relaxation, suggesting excessive activity of otherwise normal motor units. A test for serum antibodies glutamic acid decarboxylase (GAD) was positive, providing additional support for the diagnosis of stiff-person syndrome. Several of the respondents suggested the possibility of a paraneoplastic association for this patient's diagnosis of stiff-person syndrome, which is occasionally reported (e.g., Folli et al., 1993). Although the patient did have a small (

33. Patient 40 Selftest stiffperson syndrome. 1. Which of the following is a diagnostic feature of theclassical stiff-person syndrome? A. rigidity with superimposed spasms http://www.bcm.edu/neurology/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?

34. Journal Of Clinical Neuromuscular Disease - UserLogin A Patient With Atypical stiffperson syndrome An Electrophysiological Study We conclude that the patient has atypical stiff-person syndrome. http://www.jcnmd.com/pt/re/jclnnmd/fulltext.00131402-200109000-00005.htm

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35. Journal Of Clinical Neuromuscular Disease - UserLogin Stiffness, Spasticity, or Both A Case Report of stiffperson syndrome stiff-person syndrome (SPS) was considered when axial stiffness became evident http://www.jcnmd.com/pt/re/jclnnmd/fulltext.00131402-200206000-00003.htm

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36. Paraneoplastic Neurological Syndromes: Professional Area Stiff-Person Syndrome stiffperson syndrome (SPS) is a rare neurological disorder first described byMoersch Dalakas MC, Li M, Fujii M, Jacobowitz D. Stiff person syndrome. http://www.pnseuronet.org/professionals/stiffman.htm

You are in Professional area Stiff-Person Syndrome Clinical Features Associated antibodies Anti-GAD antibodies (figure1) are present in 60% of patients and stain the axon hillocks of Purkinje cells and diffuse nerve terminals in the molecular and granular layers of cerebellum. In addition, antibodies to pancreatic islet cells, gastric parietal cells, thyroid microsomes, and thyroglobulin are frequently found in patients positive for anti-GAD antibodies. The relationship of autoantibodies to the pathogenesis of the disease remains to be determined. Anti-GAD antibodies are also present in patients with insulin dependent diabetes mellitus and autoimmune polyendocrine syndrome but the anti-GAD level in these syndromes is usually much lower than in SPS. Figure 1. Immunohistochemistry on frozen sections of rat cerebellum. Anti-GAD antibodies positive sera recognise the axon hillocks of Purkinje cells and diffuse nerve terminals in the molecular and granular layer of cerebellum. Anti-amphiphysin antibodies (figure 2) are directed to a 128 kd neuronal protein concentrated in nerve terminals. Rare paraneoplastic SPS present anti-GAD antibodies alone or associated with anti-amphiphysin antibodies.

37. Log In Problems Oct. 7, 2004 (Toronto) — Patients with stiffperson syndrome (SPS) have reducedlevels of peptides related to gamma-aminobutyric acid (GABA), according to http://www.medscape.com/viewarticle/490843

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38. References The stiff man and stiff man plus syndrome. J Neurol. 1999;246 648652. Levy LM, Dalakas MC, Floeter M. The stiff-person syndrome an autoimmune 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.

39. BioMed Central | Full Text | Stiff-person Syndrome To obtain access to Current Treatment Options in Neurology through your institutionuse the options below. If you would like information about a personal http://www.biomedcentral.com/1092-8480/5/79

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40. BioMed Central | Abstract | Stiff-person Syndrome stiffperson syndrome (SPS) is a progressive neurologic disorder characterizedby 1) stiffness that is prominent in axial muscles, with co-contraction of http://www.biomedcentral.com/1092-8480/5/79/abstract

home journals A-Z subject areas advanced search ... Issue 1 Viewing options Abstract Full text PDF Associated material: PubMed record Related literature: Articles citing this article on Google Scholar Other articles by authors Vasconcelos OM ... Similar articles (PubMed) Tools: E-mail to a friend Download references Opinion Stiff-person Syndrome Olavo M Vasconcelos MD and Marinos C Dalakas MD Neuromuscular Diseases Section National Institute of Neurological Disorders and Stroke, 10 Center Drive MSC 1382 Building 10 Room 4N248, National Institutes of Health, Bethesda, MD, 20892, USA Current Treatment Options in Neurology Published Abstract Terms and Conditions Privacy statement Information for advertisers Contact us

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