81. Disorder Information - Hereditary Motor And Sensory Neuropathies (HMSN) hereditary motor And sensory Neuropathies (HMSN). CharcotMarie-Toothdisease (CMT) Peroneal muscular atrophy. http://www.mdac.ca/english/disorder-info/disorder-info-11.htm

Hereditary Motor And Sensory Neuropathies (HMSN) Charcot-Marie-Tooth disease (CMT) Peroneal muscular atrophy What is HMSN? What are the different types of HMSN? How disabling is the disorder? What are the early symptoms? ... print What is HMSN? HMSN, or Charcot-Marie-Tooth syndrome, as it is widely known, is a group of hereditary conditions marked by slowly progressive muscle weakness and atrophy (wasting) in the feet, lower legs, hands and forearms. Many people also experience a mild loss of sensation in the limbs, fingers and toes. The weakness results from the degeneration of peripheral nerves (which connect the spinal cord to the muscles, joints and skin, carrying messages in both directions) rather than from a degenerative process in the muscle tissue itself. Charcot-Marie-Tooth disease (CMT), named for three physicians who first identified it in 1886, is also known as peroneal muscular atrophy because it primarily affects the peroneal muscles on the shin that allow one to pull up the foot. Although initially felt to be one disorder, there are now thought to be numerous types of this disease that differ in clinical severity, genetic patterns of inheritance and changes noted in nerve biopsies. Collectively, this group of disorders is known as hereditary motor and sensory neuropathies (HMSN) and this is becoming the preferred term. However, the term Charcot-Marie-Tooth (CMT) disease is still very commonly used and generally refers to HMSN Type I. What are the different types of HMSN?

82. Muscular Dystrophy Campaign Hereditary Motor And Sensory Neuropathies (HMSN) hereditary motor and sensory Neuropathies (HMSN). (Sometimes known Campaign.What are hereditary motor and sensory neuropathies? The http://www.muscular-dystrophy.org/information/keyfacts/hmsn.html

@import "../../css/cssstyle.css"; Receive copies of Target MD Get your message to over 30,000 readers by advertising in Target MD magazine. Call for details: David N Russell Hereditary Motor and Sensory Neuropathies (HMSN) (Sometimes known as Charcot-Marie-Tooth Disease or Peroneal muscular atrophy) December 2000 From an original written by the late Prof Anita Harding MD, FRCP and revised by Dr D.Hilton-Jones MD, FRCP, for the Muscular Dystrophy Campaign. What are hereditary motor and sensory neuropathies? The term hereditary and sensory neuropathy (HMSN) 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 a 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. Many different names have been used to describe HMSN in the past, and this is rather confusing. They are often referred to as peroneal muscular atrophy, because the peroneal muscle on the outside of the calves are particularly affected. Another commonly used name is Charcot-Marie-Tooth disease, after the three neurologists who first described the condition in 1886. Other names include Dejerine-Sottas disease and hereditary hypertrophic neuropathy. HMSN is now the preferred term, largely because Charcot-Marie-Tooth disease and peroneal muscular atrophy are more general terms used to describe a wider group of conditions including one form of spinal muscular atrophy.

83. Muscular Dystrophy Campaign Hereditary Motor And Sensory Neuropathies (HMSN) hereditary motor and sensory Neuropathies (HMSN). (Sometimes known Campaign.What are hereditary motor and sensory neuropathies? The http://www.muscular-dystrophy.org/information/keyfacts/hmsn.html?&print=YES

84. Hereditary Motor And Sensory Neuropathies http//www.ncbi.nlm.nih.gov/disease/Metabolism.html. Obesity Paroxysmal nocturnalhemoglobinuria Phenylketonuria Refsum_disease Tangier_disease http://freesense.free.fr/Diseases/c/341.htm

http://www.ncbi.nlm.nih.gov/disease/Metabolism.html Obesity Paroxysmal nocturnal hemoglobinuria Phenylketonuria Tay-sachs_disease METABOLISM is the means by http://www.ncbi.nlm.nih.gov/disease/Brain.html The nervous_system Alzheimer_disease Charcot-marie-tooth_disease Epilepsy Friedreich's ataxia Niemann-pick_disease Tropes Web V1.0

85. Neurology -- Abstracts: Auer–Grumbach Et Al. 54 (1): 45 Ulceromutilating neuropathy in an Austrian kinship without linkage to hereditarymotor and sensory neuropathy IIB and hereditary sensory neuropathy I loci. http://www.neurology.org/cgi/content/abstract/54/1/45

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Figures Only for this Article Full Text of this Article PDF Version of this Article Data suppl ... Email this article to a friend Correspondence: Submit a response to this article Similar articles found in: Neurology Online PubMed PubMed Citation This Article has been cited by: other online articles Search PubMed for articles by: Hartung, H.-P. Alert me when: new articles cite this article Download to Citation Manager Neurology American Academy of Neurology Articles Ulcero-mutilating neuropathy in an Austrian kinship without linkage to hereditary motor and sensory neuropathy IIB and hereditary sensory neuropathy I loci K. Wagner, PhD V. Timmerman, PhD P. De Jonghe, MD, PhD and H.-P. Hartung, MD michaela.auergrumbach@kfunigraz.ac.at OBJECTIVE: To elucidate genetic heterogeneity in ulcero-mutilating neuropathy. BACKGROUND: Ulcero-mutilating features and sensory loss have been observed in hereditary sensory neuropathy (HSN) and hereditary motor and sensory neuropathy (HMSN). HSN is characterized by

86. Orthoguide.com Hereditary Motor And Sensory NeuropathiesNeuromuscular Home Page Search results for hereditary motor and sensory Neuropathies . SearchAltaVista for more on hereditary motor and sensory Neuropathies . http://www.mymedline.com/ortho/Hereditary_Motor_and_Sensory_Neuropathies.php3

Search results for "Hereditary Motor and Sensory Neuropathies" Orthoguide Matches 1 - 2 of 2 Disorders: Neuromuscular: Neuromuscular Home Page [http://www.neuro.wustl.edu/neuromuscular/] Disorders: Developmental: Genline - on Charcot Marie Tooth Hereditary Neuropathies. [http://www.hslib.washington.edu/genline/cmt.html] Search AltaVista for more on 'Hereditary Motor and Sensory Neuropathies' Global Search Add Url Free Medline ... Search Enter Keywords to Search and Your Choice of Search Arguments Or And Match entire phrase Orthoguide.com

87. Inherited Peripheral Neuropathies Several subtypes of inherited peripheral neuropathies were delineated and classifiedas hereditary motor and sensory neuropathies (HMSN), hereditary motor http://molgen-www.uia.ac.be/CMTMutations/CMT.cfm

Inherited Peripheral Neuropathies C harcot-Marie-Tooth disease (CMT) is the most common inherited disorder of the peripheral nervous system. Soon after the original description by J.M. Charcot, P. Marie and H.H. Tooth in 1886, it became apparent that this syndrome is clinically and genetically heterogeneous. Several subtypes of inherited peripheral neuropathies were delineated and classified as hereditary motor and sensory neuropathies (HMSN), hereditary motor neuropathies (HMN) and hereditary sensory (and autonomic) neuropathies (HSAN). All these types are further subdivided in several subtypes. The classification provides a valuable framework for both diagnostic and research purposes. In this classification, HMSN type I and HMSN type II correspond to CMT type 1 (CMT1, OMIM entries ) and CMT type 2 (CMT2, OMIM entries ) respectively, while Dejerine-Sottas syndrome (DSS,OMIM entry ) is included in HMSN type III. M olecular genetic studies have been very successful in the field of the inherited peripheral neuropathies. Up to date, about 30 loci and 14 genes have been identified. The peripheral myelin protein 22 gene ) on chromosome 17p11.2, the

88. CMT - Scientific Progress The inherited peripheral neuropathies were classified by Dyck et al. (1993) as hereditarymotor and sensory neuropathies (HMSN), hereditary motor neuropathies http://www.molgen.ua.ac.be/CMT/ScientificProgress/CMTProgress.cfm

Recent scientific progress made in inherited peripheral neuropathies: T he inherited peripheral neuropathies were classified by Dyck et al. (1993) as hereditary motor and sensory neuropathies (HMSN), hereditary motor neuropathies (HMN), hereditary sensory neuropathies (HSN) and hereditary sensory and autonomic neuropathies (HSAN). G enetic linkage studies were very successful in the study of hereditary neuropathies identifying at least 17 gene loci M utations in the peripheral myelin protein 22 gene ( ) at chromosome 17p11.2 and the myelin protein zero gene MPZ ) gene at chromosome 1q21-q22 are responsible for the autosomal dominant form of Charcot-Marie-Tooth neuropathy type 1 (CMT1), Dejerine-Sottas syndrome (DSS) and congenital hypomyelination (CH). The gene is also involved in hereditary neuropathy with liability to pressure palsies (HNPP). M utations in the connexin 32 gene ( ) on the X chromosome, Xq13, are associated with the X-linked dominant form of CMT1 (CMTX). The identification of mutations in these peripheral myelin protein genes was greatly facilitated since these genes resided in the linked chromosomal regions and therefore became ideal functional and positional candidate genes. T he identification in CMT1 of a 1.5 Mb tandem duplication in chromosome 17p11.2, containing the

89. Neuromuscular Diseases - Internet Handbook Of Neurology Washington University, St. Louis; hereditary motor sensory NeuropathiesCharcotMarie-Tooth - Washington University, St. Louis; Congenital http://www.neuropat.dote.hu/nmd.htm

Internet Handbook of Neurology Compiled by K atalin H Department of Neurology University of Debrecen, Hungary Neuromuscular Diseases Chapters: A Collection of High Quality Online Resources for Health Professionals Pathology see Pathology of Nerve and Muscle Overview Neuromuscular Diseases - Washington University, St. Louis Neuromuscular Diseases - Muscular Dystrophy Association (MDA), AU Neuromuscular Diseases - Wake Forest University Neuromuscular Disease Info Center - Neuroland Muscle - University of Oklahoma Muskelkrankheiten - Neurologische Klinik Zentralkrankenhaus Bremen Ost, DE (in German) Diagnostic Criteria for Neuromuscular Disorders - European Neuro Muscular Centre Ion Channel Diseases - Washington University, St. Louis HIV-1 Associated Myopathies - eMedicine/Neurology EMG - TeleEMG Single Fiber EMG - eMedicine/Neurology Nerve Conduction Studies and Electromyography in the Evaluation of Peripheral Nerve Injuries - Othopaedic Journal, Spring 1999 Nerve Conduction Studies - Journal of Neurological Sciences (Turkish), April-June 2000

90. AAPM&R - EMG Case No. 59, Cont Sjogren s syndrome, infectious (including HIV), hereditary sensory Autonomic Neuropathies(HSAN) Type I whether the child has any motor symptoms, such http://www.aapmr.org/education/emgcases/emg5903b.htm

What is a Physiatrist? Legislative, Business and Clinical Practice Issues Annual Assembly Medical Education ... EMG EMG CASE No. 59, January 2003, continued History A 13-year-old African American boy was admitted to the hospital for swelling of the left knee. This started 3 months earlier after having jumped from the second story of a building. There was a significant delay in seeking medical attention. He had been in foster care for the last 11 years for reported neglect, physical and sexual abuse on several occasions. The child had a longstanding history of behavioral problems and self-mutilation. His medical history consisted of numerous injuries including electrical burns and scalds on the hands, face, torso, and lower extremities, some of which required skin grafting. Around the age of 3 years, he developed ataxia and an occipital headache. An MRI revealed Arnold-Chiari Type I malformation. He was treated with a suboccipital craniectomy and C1 laminectomy. Prior to continuing, please develop a differential diagnosis and list each possible diagnosis in order of likelihood.

91. BOFFS - Development Of Charcot Joint Following Surgery In Hereditary Sensory Mot Development of Charcot joint following surgery in hereditary sensorymotor neuropathy (CharcotMarie-Tooth disease). *M. Nyska MD http://www.bofss.org.uk/html/development_of_charcot_joint_following_surgery_in_h

1999 Annual Scientific Meeting Abstracts 1999 abstracts Outcome of tarsometatarsal arthrodesis Wound healing following partial closure of the Cincinnati incision for surgery of congenital talipes equinovarus Does addition of footblock for daycase foot surger ... Subtalar distraction fusion after calcaneal fractures using RAMP cage Development of Charcot joint following surgery in hereditary sensory motor neuropathy (Charcot-Marie-Tooth disease) *M. Nyska M.D., **M. Myerson M.D. *Department of Orthopaedic Surgery, Hadassah Medical Centre, Hebrew University, Jerusalem, Israel. **Foot and Ankle Services, Union Memorial Hospital, Baltimore Maryland USA. Charcot-Marie-Tooth (CMT) disease is a spectrum of peripheral neuropathy affecting motor and sensory nerves of the extremity. Most of these patients manifest with progressive distal weakness, pes cavovarus and family history. In advanced cases surgical treatment to correct the foot deformity tendon transfers, soft tissue release, osteotomies, and arthrodesis is needed. Destructive joint process compatible with Charcot changes may rarely appear, mainly in weight bearing joints. The mechanism responsible for activation of the Charcot process is not known but has been associated with neuropathy and fractures in diabetics. We present 3 cases having CMT who developed Charcot destructive process of ankles in two patients and midfoot in another patient. The process developed during recovery period for reconstructive surgery and may have been the trigger for initiating the process. The possible mechanism for development of Charcot process in these patients are discussed.

92. Journal Articles Written About Charcot-Marie-Tooth Disease 1999 May;66(5)560. New form of autosomalrecessive axonal hereditarysensory motor neuropathy Eckhardt SM, et al; Pediatr Neurol. http://www.geocities.com/dgosling_rn/journal1.html

******PLEASE READ and We subscribe to the HONcode principles of the Health On the Net Foundation and the Medinex Code Of Online Excellence Please note that ALL of the articles listed on these pages are old. The reason I have left them here as a reference is that some of the old research done a number of years ago, has not been repeated or updated. Some people may find these articles have some value in their search for information about Charcot Marie Tooth Disease. The Full Text of the Following Articles Can Be Found At Your Local Medical Library, Some of the Articles Or Their Abstracts May Be Found By Searching Medline for "Charcot Marie Tooth" or "Hereditary Motor Sensory Neuropathy" Text Only and Version To Print Pages: General Overviews of CMT Anaesthesia And The CMT Patient Autosomal Recessive CMT Bone Density and CMT ... Vocal Cords In CMT Autosomal Recessive CMT "Autosomal recessive Charcot Marie Tooth disease", Malcolm S.; J Neurol Neurosurg Psychiatry. 1999 May;66(5):560. "New form of autosomal-recessive axonal hereditary sensory motor neuropathy" Eckhardt SM, et al; Pediatr Neurol. 1998 p;19(3):234-5 "The human neuregulin-2 (NRG2) gene: cloning, mapping and evaluation as a candidate for the autosomal recessive form of Charcot-Marie-Tooth disease linked to 5q. " Ring HZ, et al. Hum Genet. 1999 Apr;104(4):326-32.

93. Neurology -- Abstracts: Kessali Et Al. 48 (4): 867 F. BONO, M. MUGLIA, P. VALENTINO, and A. QUATTRONE Autosomal Recessive HereditaryMotor and sensory neuropathy with Focally Folded Myelin Sheaths (CMT4B) Ann. http://www.neurology.org/cgi/content/abstract/48/4/867

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Citation Map Email this article to a friend Correspondence: Submit a response to this article Similar articles found in: Neurology Online PubMed PubMed Citation This Article has been cited by: other online articles Search PubMed for articles by: Kessali, M. Grid, D. Alert me when: new articles cite this article Download to Citation Manager ARTICLES A clinical, electrophysiologic, neuropathologic, and genetic study of two large Algerian families with an autosomal recessive demyelinating form of Charcot-Marie-Tooth disease M Kessali, R Zemmouri, A Guilbot, T Maisonobe, A Brice, E LeGuern and D Grid Service de Neurologie, CHU Mustapha, Algiers, Algeria. The hereditary sensory and motor neuropathies form a clinically heterogenous group of disorders, the most frequent of which is Charcot- Marie-Tooth disease (CMT). The autosomal dominant forms of CMT are well characterized, but the nosology of autosomal recessive CMT is still controversial. We report two large consanguineous Algerian families with an

94. ECR 97 Presentation 11-037 Multiple cranial nerve involvement in hereditary motor and sensoryneuropathy type 3 (DejerineSottas disease) MR imaging findings. http://www.ecr.org/T/ECR97/sciprg/abs/9711037p.htm

Obuz F. Multiple cranial nerve involvement in hereditary motor and sensory neuropathy type 3 (Dejerine-Sottas disease): MR imaging findings A 19 year-old male who had been suffering from severe sensory-motor complaints for 17 years, was admitted to neurology department. Neurologic examination revealed, foot deformities, limb atrophies, trunk ataxia, left upper limb dysmetria and absence of DTR's. Sensory and motor neuropathy was detected on EMG and also confirmed by sural nerve biopsy. Cranial MRI demonstrated bilaterally, symmetric, diffuse hypertrophy of trigeminal, facial, statoacustic and hypoglossal nerves. After Gd-DTPA injection the hypertrophic cranial nerves showed mild signal enhancement on fat saturated T1W MR images. To our knowledge, in hereditary motor and sensory neuropathies cranial nerve involvement has not been reported in literature previously. The presented case is the first MRI description of hereditary motor and sensory neuropathy which involves cranial nerves.

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