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         Charcot-marie-tooth Disease:     more books (25)
  1. 21st Century Complete Medical Guide to Charcot-Marie-Tooth Disease (CMT), Hereditary Motor and Sensory Neuropathy (HMSN), Peroneal Muscular Atrophy, Authoritative ... for Patients and Physicians (CD-ROM) by PM Medical Health News, 2004-03
  2. Coping with Charcot Marie Tooth Disease (Volume 1) by Diane M Gracely, 2010-06-16
  3. Charcot-Marie-Tooth Disease - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References by ICON Health Publications, 2004-09-16
  4. Charcot-Marie-Tooth disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Karen, MS, CGC Krajewski, 2005
  5. Charcot-Marie-Tooth Disease: A Practical Guide. Also Known as Hereditary Motor and Sensory Neuropathy and Peroneal Muscular Atrophy. by (No Author), 2000
  6. Charcot-Marie-Tooth Disease - A Bibliography and Dictionary for Physicians, Patients, and Genome Researchers by Philip M. Parker, 2007-07-20
  7. Charcot-Marie-Tooth disease: An entry from Thomson Gale's <i>Gale Encyclopedia of Children's Health: Infancy through Adolescence</i> by Christine Kelly, 2006
  8. Gale Encyclopedia of Medicine: Charcot Marie Tooth disease by CGC Karen M. Krajewski MS, 2002-01-01
  9. CHARCOT-MARIE-TOOTH DISEASE: A PROCTICAL GUIDE.
  10. Charcot-Marie-Tooth disease and multiple malignant melanomas: a case report.(Case study): An article from: Journal of Drugs in Dermatology by Ritu Saini, Stephanie Lehrhoff, et all 2010-02-01
  11. Charcot Marie Tooth Disease: An entry from Gale's <i>Gale Encyclopedia of Medicine, 3rd ed.</i> by Karen, MS, CGC Krajewski, 2006
  12. Charcot-Marie-Tooth Disorders (Annals of the New York Academy of Sciences)
  13. 2009 Conquering Charcot-Marie-Tooth (CMT) Disease - The Empowered Patient's Complete Reference - Diagnosis, Treatment Options, Prognosis (Two CD-ROM Set) by PM Medical Health News, 2009-04-04
  14. Charcot-Marie-Tooth (CMT) Disease Toolkit - Comprehensive Medical Encyclopedia with Treatment Options, Clinical Data, and Practical Information (Two CD-ROM Set) by U.S. Government, 2009-04-04

81. SupportPath.com: Charcot-Marie-Tooth Disease
SupportPath.com leads you to Internet resources for supportrelated information on hundreds of health, personal, and relationship topics.
http://www.supportpath.com/sl_c/charcot_marie_tooth_disease.htm
Charcot-Marie-Tooth Disease
Also called: CMT, Charcot-Marie Disease Other topics of interest on SupportPath.com:
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82. Charcot-Marie-Tooth Disease
charcotmarie-tooth disease by Ron Kennedy, MD, Santa Rosa, California. Charcot-Marie-Tooth (CMT) disease is the most common inherited neurological disorder
http://www.medical-library.net/sites/_charcot_marie_tooth_disease.html
Charcot-Marie-Tooth Disease
by Ron Kennedy, M.D., Santa Rosa, California
Charcot-Marie-Tooth (CMT) disease is the most common inherited neurological disorder, affecting approximately 150,000 Americans. CMT is found world-wide in all peoples. It was first described in 1886 by Jean-Marie-Charcot, Pierre Marie, and Howard Henry Tooth. CMT patients slowly lose normal use of their feet and legs, hands and arms as nerves to the extremities degenerate. The muscles in the extremities become weakened because of the loss of stimulation by the affected nerves. Also, there is a loss of sensory nerve function. Unlike muscular dystrophy in which the defect is in the muscles, CMT is a disorder in which the defect is in the nerves that control the muscles. A high arched foot is one of the first signs of this disorder. As the disease progresses, progressive deformation of the foot occurs. The patient develops a pes cavus foot with hammer toes. Foot drop and ankle sprains are frequent complications. The progressive muscle wasting leads to problems with walking, running, and balancing. To avoid tripping, patients with foot drop raise their knees unusually high resulting in high step gait. In some patients, muscle weakness may also occur in the thighs. Flat foot is also seen in patients with CMT. Hand function becomes affected because of progressive muscle atrophy, making fine manipulatory acts, like writing, difficult. Loss of nerve function in the extremities also leads to sensory loss. The ability to distinguish hot and cold is diminished as is the sense of touch.

83. CHARCOT-MARIE-TOOTH DISEASE
charcotmarie-tooth disease is an inherited degenerative disorder of the central charcot-marie-tooth disease presents in a variety of clinical forms and
http://www.curtin.edu.au/curtin/dept/physio/podiatry/encyclopedia/ciecle/
CHARCOT-MARIE-TOOTH DISEASE
Cie Cie Glencross
Figure 1: Charcot-Marie-Tooth typical cavus foot type and inverted champagne bottle leg
Back to Encyclopedia Index
INTRODUCTION
Scope of the Report
Aims of the Report
The report will cover the following areas:
  • Mode of Inheritance
  • Types of Charcot-Marie-Tooth disease
  • Pathogenesis of Charcot-Marie-Tooth disease
  • Pathology of Charcot-Marie-Tooth disease
  • Clinical Features
  • Diagnosis of Charcot-Marie-Tooth disease
  • Conservative and Surgical Treatment of Charcot-Marie-Tooth disease.

Back to Contents
OVERVIEW:
Prevalence
Mode of Inheritance
Types
Back to Contents
SPECIFICS:
PATHOLOGENESIS
Neural Pathogenesis
Muscular Pathogenesis
1. The muscles supplied by the longest axon of the Sciatic nerve are affected first.
2. The muscles showing the least bulk are foremost in showing wasting.
3. Muscles above the knee are not affected.
Downey et al (1992) and Mann and Missirian (1983; 1986) support this theory in explaining the pattern of muscle wasting and dysfunction. The intrinsic muscles of the foot are the first to atrophy as a result of their size and innervation by the medial and lateral plantar nerves. The anterior muscles of the leg are next to atrophy presumably as a result of their innervation be the smaller branch of the Sciatic nerve. As a result of the lack of muscle bulk, Peroneus Brevis and Peroneus Tertius atrophy before their counterpart Peroneus Longus. As Charcot-Marie-Tooth disease is progressive, the posterior leg muscle group and the triceps surae may be involved. However, because of the greatest muscle bulk, they are typically the last to atrophy (Downey, 1992).

84. New Gene For Charcot-Marie-Tooth Disease Involved In Cell Transport
By American Academy of Neurology , charcotmarie-tooth disease (CMT), which affects the peripheral nerves, comes in several forms and is due to mutation in
http://www.rxpgnews.com/research/genetics/article_1098.shtml
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85. Charcot Marie (Tooth) Disease
charcotmarie-tooth disease, GUADALAJARA NEURONAL TYPE. Type A. charcot-marie-tooth disease, NEURONAL TYPE, A; CMT2A. Type B. charcot-marie-tooth disease,
http://www.bdid.com/cmt.htm

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Charcot Marie (Tooth) Disease (CMT, Hereditary Motor and Sensory Neuropathy, Peroneal Muscular Atrophy)

86. Charcot-Marie-Tooth Disease
Find information about charcotmarie-tooth disease and links.
http://www.doctorpage.com/findit/Diseases_and_Conditions/Charcot_Marie_Tooth_Dis

doctorpage
Diseases and Conditions Charcot Marie Tooth Disease Displaying listings 1-4
  • CMTUS email support + discussion group for people with CMT A global, private, CMT email discussion group for sharing positive living ideas, practical solutions and new research modalities. 56 Links, polls, open 7 days, 24 hours per day. All with CMT are wellcome! http://www.egroups.com/group/CMTUS CMTnet: Charcot-Marie-Tooth Disease Information CMTnet is a repository of information for research and treatment of Charcot-Marie-Tooth (CMT). CMT is a hereditary progressive neuromuscular disorder that primarily effects the extremities. http://www.ultranet.com/~smith/CMTnet.html Charcot-Marie-Tooth Disease National Institutes of Health, Bethesda, Maryland 20892 Written August 1996 Index: : Charcot-Marie-Tooth disorder is an inherited neurological disease characterized by a slowly progressive ... http://www.ninds.nih.gov/healinfo/DISORDER/charcot/charcot.htm Charcot-Marie-Tooth disease (hereditary) Disease progressive neuropathic muscular atrophy; hereditary peroneal nerve dysfunction; neuropathy - peroneal A slowly progressive, inherited disorder characterized by of tissue in the feet and ... http://www.healthanswers.com/database/ami/converted/000727.html

87. Charcot-Marie-Tooth Disease
The calves are thin charcotmarie-tooth disease Archives of Rheumatology Archives of Rheumatology Blackburn Orthopaedic Foot and Ankle Service
http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=842

88. Charcot-Marie-Tooth Disease
charcotmarie-tooth disease. This public-orientated leaflet provides an overview of Charcot-Marie-Tooth (CMT) disease, along with access to information
http://omni.ac.uk/browse/mesh/D002607.html
low graphics
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth Disease Charcot-Marie-Tooth Disease / genetics
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth disease Patient Education Handout [Publication Type] Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth Disease / genetics
GeneReviews : Charcot Marie Tooth hereditary neuropathies Notes for physicians on Charcot Marie Tooth hereditary neuropathies (synonyms: CMT, hereditary motor and sensory neuropathies (HMSN), Dejerine-Sottas disease and peroneal muscular atrophy). This document includes diagnosis, a clinical description, incidence, molecular genetics and genetic counselling and testing. This resource forms part of GeneReviews (formerly GeneClinics profile), a peer-reviewed clinical genetic information resource that is funded by the US National Institutes of Health (NIH) and produced by the University of Washington, Seattle. This resource contains a summary and bibliographical references of the review. Last updated April 2005. Charcot-Marie-Tooth Disease / genetics
Last modified: 02 Sep 2005

89. PD & Charcot-Marie-Tooth Disease
PD charcotmarie-tooth disease. This article submitted by Rick Buchanan on 11/1/95. Does anyone know of a correlation between PD and CMT (also
http://neuro-www.mgh.harvard.edu/neurowebforum/ParkinsonsDiseaseArticles/PDCharc
This article submitted by Rick Buchanan on 11/1/95.
Does anyone know of a correlation between PD and CMT (also
known as Hereditary Sensory-Motor Neuropathy)? I was
diagnosed with CMT at 12 and Parkinson's at age 32 (I am
now 37 a bit young for PD...) My rather primitive attempts at a Medline search found enough
abstracts that I'm beginning to question whether this is
just an unfortunate coincidence. Among the refs were several
cases of full familial lines with both conditions. Since
the gene for CMT (type 1A) has been located (the chromosome
17 "stutter"), I wonder if this might not be a clue to some
(possible) hereditary facet of PD. Consider that Duvoison's update on his original twins study does point to a genetic connection of some kind. Strangely enough, I have I'd be interested in any opinions/comments anyone might have, especially from any of you research/physician types. I'm not a doctor (nor do I play one on TV) but I did my undergrad at Johns Hopkins (biochem) and have two siblings who are MDs, so I can follow most of the jargon.

90. Genes And You - Genetic Disorders - Charcot-Marie-Tooth Disease (CMT)
charcotmarie-tooth disease (CMT). WHAT IS CMT? CMT is an inherited condition in which there is a defect in the peripheral nerves (to the arms and legs).
http://www.gig.org.uk/genesandyou_CMT.htm
Genetic Interest Group - Working to benefit all people affected by genetic disorders
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    Who are you? Choose.. Student Teacher Medical Scientist/Research Member of GIG Patient Family Member Carer Press Other You are here: Education Genes and You Contents Charcot-Marie-Tooth Disease (CMT)
    Charcot-Marie-Tooth Disease (CMT)
    WHAT IS CMT? CMT is an inherited condition in which there is a defect in the peripheral nerves (to the arms and legs). CMT affects the transmission of messages along the nerves, either through deterioration of the Myelin (the insulating substance that wraps round the nerves) or of the axons, (depending on the type of CMT, though the axons are also affected in de-myelinating CMT later on). Myelin helps the electric signals (which tell the muscles to contract or relax) to travel along the nerves. When the myelin does not work well, as in CMT, these electric signals move very slowly. When the axons do not work properly, the electrical signals travel at near normal speed but are weaker. In both cases movement of the legs and arms is affected.
    HOW IS CMT PASSED ON?

91. Closing In On Treatment For Charcot-Marie-Tooth Disease?
CharcotMarie-Tooth (CMT) is a hereditary disorder of the peripheral nervous system that strikes 1 person in every 2500. Now, researchers from VIB have
http://www.eurekalert.org/pub_releases/2004-05/vfii-cio050704.php
Public release date: 7-May-2004
E-mail Article

Contact: Ann Van Gysel
ann.vangysel@vib.be

VIB, Flanders Interuniversity Institute of Biotechnology
Closing in on treatment for Charcot-Marie-Tooth disease?
Charcot-Marie-Tooth (CMT) is a hereditary disorder of the peripheral nervous system that strikes 1 person in every 2500. Increasing debilitation of the muscles ultimately leads to their disappearance in the lower legs, feet and hands. Now, researchers from VIB (the Flanders Interuniversity Institute for Biotechnology) have discovered a piece of the molecular puzzle of this disease. Two small 'heat shock' proteins turn out to be crucial - faults in the coding genes lead to the disease. Additional research has shown that these proteins play an important role in many other neurodegenerative disorders as well. This research moves us a step closer to the development of new therapies. CMT, a serious disorder without a cure CMT, the most common hereditary disorder of the peripheral nervous system, leads to debilitation of the muscles in the lower legs, feet and hands. The clinical picture is extremely variable, but older patients sometimes require a wheelchair. Today, only supportive treatment is available; there are still no effective therapies to retard or stop the progress of the disease. More insight into the molecular processes of this disease is essential for diagnosis and potential treatment. And exploration of the genes involved is a crucial step in this. VIB research findings

92. Muscular Dystrophy Campaign
causes and how it is passed on. charcotmarie-tooth disease does not describe a single disorder, but a group of conditions that are superficially similar.
http://www.muscular-dystrophy.org/information_resources/factsheets/medical_condi


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Charcot-Marie-Tooth disease
(Sometimes known as peroneal muscular atrophy)
From an original written Dr.Hilton-Jones MD, FRCP, FRCPE Clinical Director, Oxford MDC Muscle and Nerve Centre, and revised by Dr. Mary Reilly MD, FRCP, FRCPI Consultant Neurologist, National Hospital for Neurology and Neurosurgery, London, UK.
Download this factsheet as a Word document:
Charcot-Marie-Tooth muscular dystrophy factsheet
Contents:
What is Charcot-Marie-Tooth disease?
Charcot-Marie-Tooth disease is named after the three neurologists who first described the condition in 1886. Many different names have been used to describe Charcot-Marie-Tooth disease but the other commonly used name is hereditary motor and sensory neuropathy . This is an accurate term as it refers to the two primary features of this condition i.e. the condition is hereditary and it affects the motor and sensory peripheral nerves.
Charcot-Marie-Tooth disease, or hereditary motor and sensory neuropathy, is therefore is used to describe a group of conditions that give rise to weakness and wasting of the muscles below the knees and often those of the hands. Many affected people also have loss of feeling in the hands and feet, and this is the ‘sensory’ component. The term 'neuropathy' refers to the fact that it is the peripheral nerves (which connect the spinal cord to the muscles, joints and skin, carrying messages in both directions), which do not function normally. As the name implies, these are inherited disorders.

93. Muscular Dystrophy Campaign
This is the charcotmarie-tooth disease/HMSN homepage which links to more information about the condition.
http://www.muscular-dystrophy.org/about_your_condition/charcotmarietooth_disease


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Charcot-Marie-Tooth disease/HMSN home page
An introduction to Charcot-Marie-Tooth disease
Charcot-Marie-Tooth disease is known by several other names:
  • hereditary motor and sensory neuropathies hereditary hypertrophic neuropath peroneal muscular atrophy Dejerine-Sottas disease

Read the introduction in full
Key facts
  • What are the different types of Charcot-Marie-Tooth disease? How does Charcot-Marie-Tooth affect people?

Read the factsheet
Further support
Find out about our advice and information service, plus a wealth of information about adaptations, equipment and inclusive education in the Care and Support section.
Care and support
Charcot-Marie-Tooth disease/HMSN
Dr. Simon Hughes Year 3 - £49,308 Dr Hughes is looking into the changes seen in cells which participate in muscle repair. Using mouse models of Duchenne muscular dystrophy and hereditary motor and sensory neuropathy (also known as Charcot-Marie-Tooth disease), he is looking at the effect these changes have on disease progression.
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Find out about HNPP, symptoms of which are similar to Charcot-Marie-Tooth disease

94. Charcot-Marie-Tooth Disease Type I Diagnosed In A 5-year-old Boy After Vincristi
charcotmarie-tooth disease type 1 was diagnosed in the patient and, charcot-marie-tooth disease and Vincristine J Am Podiatr Med Assoc, May 1, 2003;
http://www.jaoa.org/cgi/content/abstract/99/3/165
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Case Reports
Charcot-Marie-Tooth disease type I diagnosed in a 5-year-old boy after vincristine neurotoxicity, resulting in maternal diagnosis
MJ Olek, B Bordeaux, and RT Leshner Charcot-Marie-Tooth disease type 1, also known as hereditary motor sensory neuropathy type 1, is an uncommon autosomal dominant disease that causes destruction of peripheral nerves with a varied clinical course, but often leads to muscle weakness. If the peroneal muscle is involved, the patient may develop a characteristic slapping gait. The dose-limiting side effect of the chemotherapeutic agent vincristine is usually its neurotoxicity. We report the case of a 5-year-old patient with leukemia who developed an acute polyneuropathy after treatment with vincristine. Charcot-Marie-Tooth disease type 1 was diagnosed in the patient and, subsequently, in his mother only after vincristine toxicity was observed.
This article has been cited by other articles:

95. Charcot-Marie-Tooth Disease
charcotmarie-tooth disease (CMT) is a progressive motor and sensory neuropathy (nerve disorder) There is no known cure for charcot-marie-tooth disease.
http://www.wcax.com/global/story.asp?s=2314422&ClientType=Printable

96. Hospital Practice: Charcot-Marie-Tooth Disease
Lupski JR DNA diagnostics for charcotmarie-tooth disease and related inherited Roa BB et al charcot-marie-tooth disease type 1A Association with a
http://www.hosppract.com/genetics/9705gen.htm
Molecular Genetics in Clinical Practice
Charcot-Marie-Tooth Disease:
A Gene-Dosage Effect
JAMES R. LUPSKI
Baylor College of Medicine
A broad spectrum of inherited neuropathy has been traced to three myelin genes, yet in the two most common disorders, there is no mutated gene. Instead, a gene has an extra or missing copy, as part of a 1.5-megabase DNA duplication or deletion. Eventually, duplications may emerge as a large fraction of all mutations. The discoveries have implications even for acquired disordersincluding carpal tunnel syndrome.
Dr. Lupski is Cullen Professor of Molecular and Human Genetics, Professor of Pediatrics, and Director, Medical Scientist Training Program, Baylor College of Medicine, Houston. In 1886, the French physicians Jean Martin Charcot and Pierre Marie, and independently the English physician Howard Henry Tooth, described an insidious, slowly progressive atrophy of distal limb muscles that would eventually be recognized as the most common inherited disorder of the peripheral nervous system. It is also among the most common of all genetic disorders, affecting one person in 2,500. Characteristically, the illness becomes evident during the first two decades of life. Often, patients are liable to trip and sprain their ankles, and footdrop forces them to lift the knee, resulting in an equine gait. Eventually, pes cavus appears, and distal muscle atrophy may cause a stork-leg appearance. Weakness of hand muscles usually begins later; in severe cases, claw-like deformity develops.

97. Charcot-Marie-Tooth Disease
charcotmarie-tooth disease refers to a hereditary neurological disorder affecting primarily the legs, feet and hands.
http://www.tylermedicalclinic.com/charcot-mari-tooth disease.htm
The Tyler Medical Clinic Assisted Conception - Affordable Infertility Treatments - List of Diseases - Charcot-Marie-Tooth Disease Charcot-Marie-Tooth Disease refers to a hereditary neurological disorder affecting primarily the legs, feet and hands. It is also known as Hereditary Motor-Sensory Neuropathy (HMSN) and Peroneal Muscle Atrophy. Charcot-Marie-Tooth (CMT) Disease is one of the more common hereditary diseases. One in 2,500 people is afflicted with some form of CMT. At least six genetic defects have been traced to CMT. Generally, CMT is inherited in an autosomal dominant fashion but some forms of CMT have been found to be X-linked. Symptoms of X-linked forms of CMT are generally more pronounced in males than females. Once a patient is diagnosed with CMT, it is crucial to determine the form of CMT the patient has. A thorough genetic and pedigree analysis should be conducted to determine mode of inheritance of that particular form of CMT. A new test is now available to help couples with a family history of X-linked CMT. Pre-Implantation Genetic Diagnosis (PGD) examines an embryo prior to implantation and determines the sex of the baby. The transmission of diseases that are more prevalent in one gender can be greatly reduced through PGD. A thorough genetic analysis by a genetic counselor is needed to determine if PGD will be of benefit to couples. Thus far, the following forms of CMT have been found to be X-linked

98. Charcot-Marie-Tooth-Syndrom - CMT
Information on this disease peroneal muscular atrophy or CMT.
http://www.kaikracht.de/cmt/
Charcot-Marie-Tooth-Syndrom

99. Charcot Marie Tooth Disease Related Books
Charcot Marie Tooth disease Related Books Molecular Genetics, and Therapy Clinical and electrodiagnostic features of charcotmarie-tooth syndrome
http://www.bdid.com/cmtbooks.htm

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100. CMTnet: Charcot-Marie-Tooth Disorders Information Exchange
CMTnet is a repository of information for research and treatment of charcotmarie-tooth (CMT). CMT is a hereditary progressive neuromuscular disorder that
http://users.rcn.com/smith.ma.ultranet/CMTnet.html
The purpose
CMTnet is a repository of information for research and treatment of Charcot-Marie-Tooth (CMT). CMT is a hereditary progressive neuromuscular disorder that primarily effects the feet, legs and hands. CMTnet is intended to provide information for both the medical and non-medical communities. Last updated: 1/12/2000. Enter CMTnet

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