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         Isaacs Syndrome:     more detail
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1. Baylor Neurology Case Of The Month
A summary and general discussion about Isaacs' syndrome.Category Health Conditions and Diseases isaacs syndrome...... Diagnosis 1) Isaacs' Syndrome 2) Syncope The patient was then dischargedand was to be followed in clinic. A Review of Isaacs' Syndrome.
http://www.bcm.tmc.edu/neurol/challeng/pat2/summary.html
Summary and Discussion Amit Verma, M.B.B.S.
Chief Resident, Department of Neurology Diagnosis:
The patient was admitted to the hospital and received 6 courses of plasma exchange. He was subsequently started on oral prednisone and cyclophosphamide and during his hospital stay noticed a marked improvement in the myokymia and muscle strength. The myotonia improved as well. The patient was then discharged and was to be followed in clinic.
A Review of Isaacs' Syndrome
Epidemiology
The condition is extremely rare and has therefore never been studied in a controlled fashion. A recent review of all reported cases by Jamieson and Katiriji in 1994 suggests an equal incidence in both sexes. The disease appears to present earlier in life with most patients being less than 40 years of age at the time of symptom onset. A study of 3 families by Ashizawa et al in 1983 suggested a dominant mode of inheritance in the familial form of the disease.
Etiology and Pathogenesis
The etiology of the disease remains unclear. Isaacs in 1961 was the first person to localize the disorder to the peripheral nerves. Pharmacological localization of the generator of the myokymia seems to confirm the initial observations of Isaacs. More exact localization has been less fruitful. The electrical activity appears to originate from multiple sites in the axon. There is some evidence to suggest the role of autoimmunity and, specifically, antibodies against the potassium channel in motor nerve terminals. The potential importance of circulating antibodies is supported by the fact that plasma exchange is effective in treatment of some patients with this disease. A recent case has been reported in which a patient met criteria for both Isaacs' syndrome and CIDP, another potential autoimmune disease.

2. HONselect - Isaacs Syndrome
Translate this page English isaacs syndrome, - Myokymia, Continuous - Neuromyotonia - Pseudomyotonia- Acquired Neuromyotonia - Continuous Muscle Activity Syndrome - Gamstorp
http://www.hon.ch/HONselect/RareDiseases/C05.651.392.html
List of rare diseases: English Deutsch
Language:
MeSH term:
Accepted terms:
English: Isaacs Syndrome - Myokymia, Continuous
- Neuromyotonia
- Pseudomyotonia
- Acquired Neuromyotonia
- Continuous Muscle Activity Syndrome
- Gamstorp-Wohlfart Syndrome
Français: ISAACS, SYNDROME Deutsch: Isaacs-Syndrom - Myokomie, anhaltende - Neuromyotonie - Pseudomyotonie - Isaacs-Mertens-Syndrom Español: SINDROME DE ISAACS - MIOCIMIA CONTINUA - NEUROMIOTONIA - SEUDOMIOTONIA - PSEUDOMIOTONIA Português: SINDROME DE ISAACS - MIOQUIMIA CONTINUA - NEUROMIOTONIA - PSEUDOMIOTONIA HONselect ressources Definition: Yes Articles: Yes Images: No News: No Conferences: No Clinical trials: Yes Web sites: English Yes Français No Deutsch No Español No Português No Home About us Site map Feedback ... HONewsletter http://www.hon.ch/HONselect/RareDiseases/C05.651.392.html Last modified: Thu Jul 25 2002

3. Isaacs Syndrome - Benign Fasiculations
ranked one of the best hospitals in America. Subject isaacs syndrome benign fasiculations. Topic Area Neuromuscular
http://www.medhelp.org/forums/neuro/archive/4267.html
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A not-for-profit organization Questions in The Neurology Forum are being answered by doctors from
The Cleveland Clinic , consistently ranked one of the best hospitals in America. Subject: isaacs syndrome - benign fasiculations
Topic Area: Neuromuscular
Forum: The Neurology and Neurosurgery Forum
Question Posted By: Rita on Sunday, March 29, 1998
My husband was diagnosed with benign fasiculation then later Isaac's Syndrome. So far he has tried Klonopin, Quinine, Tegretol, Dilantin, etc. None seem to help except the Klonopin which takes the edge off of the nervousness and tingling he has. It does not help the muscle jerks at all. He has had this for 11 years and is in otherwise good health. These muscle jerks started in the legs and have now progressed to all parts of the body. There is no wasting of the muscles. Is there any help out there other than what we've found. We are desperate for an answer. After a while it tends to weigh on you physically and especially emotionally. Thanks for any help.
The standard treatment of Isaac's syndrome is phenytoin and carbamazepine and
valproate are used as a second line agents, it would appear however that your

4. ?ISAACS SYNDROME?
I have a dear friend who has been diagnosed. with isaacs syndrome. He has been suffering for 3 years now
http://neuro-www.mgh.harvard.edu/neurowebforum/NeuromuscularArticles/ISAACSSYNDR
?ISAACS SYNDROME?
This article submitted by artemis on 8/24/96.
Author's Email: artemis005@aol.com
Please help. I have a dear friend who has been diagnosed
with Isaacs Syndrome. He has been suffering for 3 years now
and has tried every conceivable treatment available with no
relief. He is asking me to reach out to others in this situation.
I have read many of the postings here at the Webforum; the
people here are certainly very knowledgable concerning
this and other ailments.
Specifically, it would be interesting to hear about any special diets
etc. which people have tried, and also, how do you think you contracted and/or developed this disorder? My friend's neurologist believes it was initiated by a chemical used in dental work he had previous to the onset of Isaacs. Please also advise as to pertinent journal articles and the like. There seems to be very little out there. And, finally, are there any support groups for this. If not, we would like to start onemaybe via the internet. If there are any folks with isaacs in the New England area please email me . Thanks for your help! Good luck to everyone.

5. Information About Isaacs' Syndrome
isaacs syndrome is excessive and spontaneous activity of a nerve fiber.
http://www.medhelp.org/perl6/neuro/archive/6335.html
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A not-for-profit organization Questions in The Neurology Forum are being answered by doctors from
The Cleveland Clinic , consistently ranked one of the best hospitals in America. Subject: Information about Isaacs' Syndrome
Topic Area: Neurology - General
Forum: The Neurology and Neurosurgery Forum
Question Posted By: Ellie on Saturday, July 04, 1998
I would like to know what the early symptoms of Isaacs' are. Are bulbar symptoms a common complaint? How does this disease progress and what are the common treatmeants and drugs? What tests are used to diagnose? Thank you in advance. Isaacs Syndrome is excessive and spontaneous activity of a nerve fiber. Twiching, spasms, and rippling of muscles (Myokyemia) are evident, the latter being the main clinical sign. There is also delayed relaxation after muscle contraction. Generalized muscle stiffness is also a symptom. Complaints of muscle aches are common, but severe muscle pains is not common. Any muscle group may be affected. Stiffness and slowness of movement may eventually make walking difficult, in extreme cases all voluntary activity is blocked. EMG may help in the diagnosis (show myokymia). The syndrome arises in childhood or adult life either in association with an peripheral neuropathy or an inherited coordination problem that may respond to acetazolamide, or remit spontaneously. Treatment with dilantin or tegretol may abolisth continuous muscle activity. Plasma exchange may help if intractible. If you are interested in getting an evaluation at CCF, call 1-800-CCF-CARE. Good Luck.

6. Isaacs Syndrome Articles, Support Groups, And Resources
isaacs syndrome articles, support groups, and resources for patientsfrom Med Help International (www.medhelp.org). isaacs syndrome.
http://www.medhelp.org/HealthTopics/Isaacs_Syndrome.html
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7. A Case Of Isaacs' Syndrome
Medicine A case of isaacs syndrome with Trousseau s phenomenon wacstudied electrophysiologically and histologically. The needle
http://www.amc.seoul.kr/~salee/a_isaacs.htm
A Case of Isaacs' Syndrome Joong Koo Kang, MD., Sang-Ahm Lee, MD., Kwang Kuk Kim, MD., Myoung Chong Lee, MD. Department of Neurology, University of Ulsan College of Medicine Gheeyoung Choe Department of Pathology, University of Ulsan College of Medicine
A case of Isaacs syndrome with Trousseau s phenomenon wac studied electrophysiologically and histologically. The needle electromyography showed typical neuromyotonic discharges of spontaneous recurrent high-frequency(150-300Hz) bursts of motor unit activity with 0.5-3 second of duration and typical waning pattern This discharge was not suppressed by brachial plexus block Motor and sensory nerve conduction velocities were either s!ightly below or in the lower range of normal. In repetitive nerve stimulation test a decremental response to low rate stimulation was noted in abductor digiti quinti muscle. On sural nerve biopsy, there was a decrease in the number of myelinated nerve fibers showing both axonal and myelin degeneration The clinical and electrophysiologic abnormalities improved considerably after treatment with phenytoin. J Korean Neurol Assoc 1993;11:592-598.

8. Searchalot Directory For Isaacs Syndrome
Related Web Sites. Baylor Neurology Case of the Month A summary andgeneral discussion about Isaacs' syndrome. Readers Digest Health
http://www.searchalot.com/Top/Health/ConditionsandDiseases/NeurologicalDisorders
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9. Searchalot Directory For Musculoskeletal Disorders
Feet (24); Fractures (23); isaacs syndrome (3); Melorheostosis (3).Nail Patella Syndrome (9); Neuromuscular Diseases (128); Ollier Disease
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11. GoGuides.Org Directory | Isaacs Syndrome
Home Health Fitness Disabilities Disorders Conditions Diseases Nervous System Disorders Neuromuscular Diseases isaacs syndrome
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Isaacs Syndrome:
    Number of Matches: 3
    Baylor College of Medicine: Patient # 2
    - Find a case study and report on a 39 year old male diagnosed with isaacs syndrome.
    URL: http://www.bcm.tmc.edu/neurol/challeng/pat2/summary.html eMedicine: Myokymia - Presented is a clinical report authored by Suying Song, MD.
    URL: http://www.emedicine.com/neuro/topic235.htm NORD: Isaacs' Syndrome - Find the synonyms, a general discussion and further resources.
    URL: http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Isaacs'%20Syndrome
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12. GoGuides.Org Directory | Isaacs Syndrome
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13. Directory :: Look.com
isaacs syndrome (3) See Also. Sites. Baylor Neurology Case of the MonthA summary and general discussion about Isaacs' syndrome. NORD
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14. Directory :: Look.com
Cleidocranial Dysplasia (6) Congenital Anomalies (162) Connective Tissue (296) Costochondritis(4) Feet (24) Fractures (23) isaacs syndrome (3) Melorheostosis
http://www.look.com/searchroute/directorysearch.asp?p=339150

15. Mioti: Medical Condition
not available. NORD Isaacs' Syndrome....... Condition isaacs syndrome. isaacs syndrome. Washington UniversitySchool of Medicine.
http://www.mioti.com/cat/condition/condition.asp?Cat=IsaacsSyndrome

16. Mioti: Medical Condition
Irritable Bowel Syndrome, • Irritable Colon. • Isaacs Merten Syndrome,• isaacs syndrome. • Islet Cell Cancer, • Ivemark Syndrome. General Topics.
http://www.mioti.com/cat/condition/results.asp?Alpha=I

17. WebGuest - Open Directory : Health : Conditions And Diseases : I
Intrauterine Dwarfism@ (5); Iritis@ (27); Irritable Bowel Syndrome@ (70);isaacs syndrome@ (3). Last update 1102 PT, Monday, July 8, 2002
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  • 18. Isaacs Syndrome Website Results :: Linkspider UK
    isaacs syndrome Websites from the Linkspider UK. isaacs syndrome Directory.Complete Results for isaacs syndrome Related Topics.
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    19. Musculoskeletal Disorders Website Results :: Linkspider UK
    Costochondritis (4); Feet (24); Fractures@ (23); isaacs syndrome@ (3); Melorheostosis@(3). Nail Patella Syndrome@ (9); Neuromuscular Diseases@ (128
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  • 20. Continuous Muscle Fiber Activity ISAACS, Journal Of Neurology, Neurosurgery And
    DL, SatyaMurti, S., Davis, L. and Drachman, DB, isaacs syndrome with laryngeal involvementan unusual presentation of myokymia, Neurology, 29 1612-5, 1979.
    http://www.remarkablemedicine.com/Clinical/clinicaluses/neuromuscular/musclefibe
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    Continuous Muscle Fiber Activity Isaacs Journal of Neurology, Neurosurgery and Psychiatry describes the syndrome of continuous muscle fiber activity in two patients, marked by progressive muscle stiffness increased by voluntary muscle contractions and accompanied by fasciculation and weakness. Electromyography recorded a state of constant rapid dysrhythmic discharge of independent muscle fibers. The author defined the state as myotonic afterdischarge and likened it to post-tetanic afterdischarge. Neither patient showed response to numerous medications including quinidine, procainamide, cortisone, and atropine. Treatment with PHT (100 mg q.i.d.) resulted in dramatic improvement in symptoms and electromyographic changes both at rest and on voluntary effort. In a five-year follow-up, the author reported that both patients were still well and had lost their abnormal stiffness. He noted that on the occasions when PHT had been stopped, the symptoms returned. 176. Isaacs, H., A syndrome of continuous muscle-fibre activity.

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