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         Stiff-person Syndrome:     more detail
  1. The Official Patient's Sourcebook on Stiff-Person Syndrome: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2002-10-10
  2. Stiff Person Syndrome
  3. Stiff person syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Bruno Azevedo, Iuri, MD, PhD Louro, 2005
  4. Rare disease may offer clues to MS. (Moersch-Woltman syndrome): An article from: Inside MS by Phyllis Shaw, 1989-06-22

81. Stiff Person Syndrome And Myasthenia Gravis. Saravanan PK, Paul J, Sayeed Zaheer
Stiff person syndrome and myasthenia gravis. Saravanan PK, Paul J, Sayeed ZaheerAhmed. Stiff person syndrome and myasthenia gravis. Neurol India 2002;5098100.
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2002;volume=50;iss

82. Stiff Person Syndrome And Myasthenia Gravis.,Saravanan PK, Paul J, Sayeed Zaheer
Year 2002 Volume 50 Issue 1 Page 98100. Stiff person syndrome and myasthenia gravis. Saravanan PK, Paul J, Sayeed ZaheerAhmed.
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2002;volume=50;iss

83. Bioline International - Stiff Person Syndrome And Myasthenia Gra
Stiff Person syndrome and Myasthenia Gra. Saravanan, PK and Paul, J. and Sayeed, Zaheer Ahmed (2002) Stiff Person syndrome and Myasthenia Gra.
http://bioline.utsc.utoronto.ca/archive/00000356/
Bioline International Home About Browse Search ... Help
Stiff Person Syndrome and Myasthenia Gra
Saravanan, P.K. and Paul, J. and Sayeed, Zaheer Ahmed (2002) Stiff Person Syndrome and Myasthenia Gra. Neurology India Full text available as:
PDF
- Requires Adobe Acrobat Reader or other PDF viewer.
Abstract
Association of stiff person syndrome, an immune related disorder of anterior horn cells and myasthenia gravis an endplate disorder with similar pathogenesis, is rare. This communication documents this association in the Indian literature for the first time. EPrint Type: Journal (Paginated) Keywords: Stiff person syndrome, Myasthenia gravis ni02026 Subjects: Medicine Neurology ID Code: Deposited By: Sun, Yibing Deposited On: 12 June 2003 Alternative Locations: http://www.bioline.org.br/abstract?ni02026 http://www.neurologyindia.com/vol50-1/FullArticles/shortreport04.pdf
Site Administrator: chan@utsc.utoronto.ca

84. AAPM&R - Case No. 47, Cont
muscles twitching involving predominantly the abdominal muscles and her history of insulin dependent diabetes mellitus is suggestive of stiff person syndrome.
http://www.aapmr.org/education/archive/emg0010e.htm

What is a Physiatrist?
Legislative, Business and Clinical Practice Issues Annual Assembly Medical Education ... EMG
EMG Case No. 47, October 2000, continued
Diagnostic Impression The presence of continuous motor unit activity associated with this individual's clinical history of muscles twitching involving predominantly the abdominal muscles and her history of insulin dependent diabetes mellitus is suggestive of stiff person syndrome.
  • What other diagnostic procedures (laboratory tests, etc.), if any, are needed? What treatment would you recommend?
Commentary V Stiff person syndrome is characterized by slowly progressive increasing stiffness affecting the axial musculature, predominantly the paraspinal and abdominal muscles as well as the lower and upper limbs. On rare occasion symptoms may be confined to a limb or an upper or lower extremity. Over time there is an increasing frequency in the rate of muscle spasms. Some attacks of muscle spasms are so severe that individual may be thrown to the floor. Attacks may be triggered by alterations in emotion, startle, and loud noises. Severe cases may have marked contraction of the hip and knee extensors, ankle dorsiflexors, and abdominal and paraspinal muscles. In advanced cases contraction of the paraspinal, abdominal and intercostals muscles may be seen. Hyperlordosis of the lumbosacral spine is common secondary to lumbosacral paraspinal muscles contraction, as is hunching of the shoulders secondary to cervical paraspinal hypertrophy.

85. AAPM&R - Case No. 47, Cont
Stiff Person syndrome. Isaacs syndrome. Stiff person syndrome involves the abdominal muscles and is frequently associated with diabetes mellitus.
http://www.aapmr.org/education/archive/emg0010b.htm

What is a Physiatrist?
Legislative, Business and Clinical Practice Issues Annual Assembly Medical Education ... EMG
EMG Case No. 47, October 2000, continued
History A 22-year-old woman with a 4-year history of insulin dependent diabetes mellitus and hypothyroidism presents to the electrodiagnostic laboratory with a chief complaint of involuntary muscle tightening and spasms. She has not experienced pain in association with the spasms, and she has no muscle cramps. She denies weakness. Her symptoms began 8 years ago while she was in high school, and the muscle tightening and spasms have been slowly progressive in severity and duration. The episodic tightening or twitching of her muscles involves her abdominal, hand, and lower limb muscles bilaterally. These paroxysmal episodes last for approximately 5 seconds and occur many times during the day. On occasion, she notices that objects fall from her hands. She believes that cold may induce her symptoms. Initially, she was not concerned about the abdominal spasms since they helped maintain her muscle tone.
  • Prior to continuing, please develop a differential diagnosis and list each possible diagnosis in order of likelihood.

86. Abstract ID A25
Abstract ID A25. Abstract Title anesthetic management of a patient with a Stiff Person syndrome. Stiff person syndrome and Anesthesia Case report.
http://www.asra.com/Abstracts/Complete/Accepted/A25.final.html
Abstract ID: A25
Abstract Title: anesthetic management of a patient with a Stiff Person Syndrome
Poster Type: Either
Category:
ABSTRACT BODY
Background: 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, resulting in an unsteady gait. Symptoms may occur gradually, spreading from the back and legs to involve the arms and neck.
Stiff-person syndrome is an autoimmune disorder that may be associated with diabetes, pernicious anemia, and thyroiditis (1). We describe the successful anesthetic management of a patient with stiff person syndrome undergoing a right inguinal hernia repair, utilizing a somatic paravertebral block supplemented with conscious sedation.
Case report:
A 65 years old male was scheduled for elective repair of a large right indirect inguinal hernia. His past medical history included diabetes mellitus, hypertension, depression, and stiff person syndrome. His laboratory workup revealed the presence of antibodies to glutamic acid decarboxylase enzyme (GAD) and elevated levels of IgA and thyroglobulin antibodies. An electromyogram (EMG) showed failure of relaxation of the proximal muscle group of both lower extremities and the right upper extremity and suggested a possible central disorder of the motor unit. In addition to a baclofen intrathecal pump, the patient was receiving intravenous immunoglobulins (IVIG) every 2 weeks.

87. Stiff Person Syndrome In A Child
Neither is MGH or MGH Neurology responsible for the content of any articles or replies. No messages are screened for content. stiff person syndrome in a child.
http://neuro-www.mgh.harvard.edu/forum_2/FibromyalgiaF/12.2.992.20PMstiffpersons
This Web Forum is not moderated in any sense. Anyone on the Internet can post articles or reply to previously posted articles, and they may do so anonymously. Therefore, the opinions and statements made in all articles and replies do not represent the official opinions of MGH and MGH Neurology. Neither is MGH or MGH Neurology responsible for the content of any articles or replies. No messages are screened for content.
stiff person syndrome in a child
This article submitted by D.D. on 12/2/99.
Email Address: ian.donaldson @bmts.com
Our daughter who is know 13 years old has just been diagnosed with an auto-immune disorder named "Stiff Person Syndrome". It is very rare and it is quite hard to find out any information about this disorder, especially in a child. It took our neurologist and several other doctors over 2 years to come up with a diagnosis. My daughter typically had intense pain in her back and legs. She missed a year of school, and was in and out of a wheel chair for two years. She would get these attacks of spasms that would rack her body for 1-2 weeks. She would have to be hospitalized during this time because the spasms were so intense. Last March 1999, she suffered one of these attacks and the doctors tried immuno=-globulin and she has been improving ever since. She goes for monthly infusions for two days and this seems to be allowing her to have a normal active life.
My main question is ...Has anyone else have this diorder? How is it treated? Has it ever gone into remission? If Immuno-therapy was used do you get side-effects-headaches, nausea, etc..?

88. Immunotherapy Treatment Provides Relief From Symptoms In Stiff Person Syndrome -
Kerala. past interviews Disease Profile. Immunotherapy treatment provides relief from symptoms in stiff person syndrome. Thursday
http://www.pharmabiz.com/article/detnews.asp?articleid=3682§ionid=6

89. Immunotherapy Treatment Shows Dramatic Results For Rare
Immunotherapy Treatment Shows Dramatic Results For Rare Neurological Disorder Stiff Person syndrome BETHESDA, MD December 27, 2001 An immunologic therapy
http://www.pslgroup.com/dg/210dae.htm

90. RARE DISEASES LEGISLATION INTRODUCED
Dramatic Results Reported in Stiff Person syndrome Study. On Dec. 27, 2001, The New England Journal of Medicine reported that intravenous
http://www.malattiemetaboliche.it/articoli/rare_disease.htm
RARE DISEASES LEGISLATION INTRODUCED You may reprint NORD's News Briefs in your newsletter or other publication as long as credit is given to The National Organization for Rare Disorders (NORD), with reference to our web site: " http://www.rarediseases.org " .
One bill in the House of Representatives is named The Rare Diseases Act of 2002 (H.R. 4013), which establishes an official Office of Rare Diseases at the NIH. Congressmen John Shimkus (R-IL) and Henry Waxman (D-CA) introduced this bill. The Office will promote and coordinate research on rare disorders, and will create academic centers for research on these conditions. The Office of Rare Diseases would receive $24 million per year for this program.
The second bill in the House is named The Rare Diseases Orphan Product Development Act of 2002 (H.R. 4014). Congressmen Mark Foley (R-FL) and Henry Waxman (D-CA) introduced this bill. This law would provide $25 million per year for the FDA's Orphan Products Research Grant Program, which supports clinical trials of new orphan drugs, diagnostics, medical devices and medical foods. This program received only $12 million from Congress this year, which is less than the funds appropriated for this research in 1995. To date, 27 new products (24 orphan drugs and 3 medical devices) have been developed (and are currently on the American market), through these grants to academic scientists and small companies. Action Needed The two bills are critically important to people with orphan diseases who are waiting for new treatments and cures to be developed for their health conditions. In order to enact these important laws, interested people are urged to contact their congressional representatives and ASK THEM TO CO-SPONSOR H.R. 4013 AND 4014. More than half of the members of the House of Representatives MUST sign-on to the bills before the entire House can vote on them. If we want these laws to be enacted this year, this must occur before Congress recesses before the election season this autumn.

91. Anaesthsia Correspondence Web Site
Step Three View Archived Correspondence. 284, Anaesthesia for Stiff–Person syndrome, Link to abstract,
http://www.anaesthesiacorrespondence.com/Correspond3.asp?articleid=2520_24&archi

92. Powerlifting And Fitness
Information for beginner fitness enthusiasts, athletes, powerlifters, and those dealing with health difficulties.
http://www.dtl.org/health/subject/stiff_person.htm
This page has been incorporated in the new
Fitness for One and All
Web site.
You should be automatically re-directed to this new site in 5 seconds.
If not, the URL is www.FitnessforOneandAll.com

93. Deutsche Gesellschaft Für Neurologie: Stiff Man-Syndrom (SMS; Synonym: Stiff Pe

http://www.dgn.org/133.0.html
Definition
Das Stiff man-Syndrom ist klinisch charakterisiert durch massive rigide Steigerung des Muskeltonus mit schmerzhaft einschießenden Spasmen, episodische adrenerge autonome Dysregulation und agoraphobische Angststörung. Die Muskeldehnungsreflexe können gesteigert sein, weitere neurologische Symptome fehlen. Beschränkung der Hauptsymptome auf eine Gliedmaße (Stiff limb-Syndrom, SLS) kommt als "Minusvariante" vor. Bei der "Plusvariante" des SMS (progressive Enzephalomyelitis mit Rigidität und Myoklonien, PERM) treten u.U. nur flüchtige neurologische Symptome (z.B. Augenbewegungsstörungen, Pyramidenbahnzeichen, Ataxie, Paresen) hinzu. Der Nachweis von Autoantikörpern gegen Glutamat-Dekarboxylase (GAD) mit intrathekaler Antikörperproduktion bei ca 70 % der Patienten und assoziierte Autoimmunerkrankungen bei ca. 50 % (vor allem Typ 1 Diabetes mellitus, Autoimmun-Hyperthyreose) sind Argumente für eine immunologische Genese.
Klinik
Erkrankungsalter 13 - 72 Jahre (Mittel 46 Jahre) Spontanverlauf und –prognose schleichende Progression über Monate, nachfolgend Stabilität über Jahrzehnte, selten auch schubförmige Verschlechterung (vor allem bei der Plusvariante PERM). Im Krankheitsverlauf können neue neurologische Symptome hinzukommen (z.B. Augenbewegungsstörungen, Pyramidenbahnzeichen, Ataxie, Paresen). Spontanheilungen sind extrem selten.

94. AWMF Online - Leitlinie Neurologie: Stiff Man-Syndrom (SMS; Synonym: Stiff Perso

http://www.uni-duesseldorf.de/WWW/AWMF/ll/neur-080.htm
AWMF online
A rbeitsgemeinschaft der
W issenschaftlichen
M edizinischen
F achgesellschaften
AWMF-Leitlinien-Register Nr. 030/080 Entwicklungsstufe:
Stiff man-Syndrom (SMS; Synonym: Stiff person-Syndrom)
Definition
Klinik
    Erkrankungsalter
    13 - 72 Jahre (Mittel 46 Jahre)
    Spontanverlauf und -prognose
    Fixierte Hyperlordose, Ankylosen, Subluxationen, Spontanfrakturen
    Angstattacken
    Gesteigerte Schreckreaktionen
    Autonome Dysregulation profuses Schwitzen, Tachykardie, Mydriasis, arterielle Hypertension, Tachypnoe, oft von Spasmen begleitet
    Assoziation mit Autoimmun-Endokrinopathien
    Typ 1 Diabetes mellitus, Autoimmun-Thyreoiditis, Immunhyperthyreose, B12-Hypovitaminose
    Fehldiagnose Konversionsneurose
    Komplikationen
    Lebensbedrohliche autonome Entgleisungen, insbesondere bei Medikamentenentzug (vor allem Benzodiazepine)
Untersuchungen
    Notwendig
    SMS PERM). EMG Liquor
    Im Einzelfall erforderlich
Differentialdiagnosen
  • Tetanus, Strychnin-Intoxikation ( EMG: Verlust der reflektorischen Inhibition, z.B: Masseterreflex)
  • erworbene Hyperekplexie (obligater Kopfretraktionsreflex)
Intraspinale Prozesse
axiale Dystonie
klinisch und elektrophysiologisch fehlende Reflexanomalien, geringe emotionale Beeinflussbarkeit

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