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         Hereditary Sensory Motor Neuropathy:     more detail
  1. Hereditary Motor and Sensory Neuropathy: Webster's Timeline History, 1980 - 2007 by Icon Group International, 2009-04-16
  2. Genetics of hereditary motor and sensory neuropathy and the Costa Rican contribution.(Charcot-Marie-Tooth (CMT)): An article from: Revista de Biología Tropical by Alejandro Leal, 2004-09-01
  3. Charcot-Marie-Tooth Disease: A Practical Guide. Also Known as Hereditary Motor and Sensory Neuropathy and Peroneal Muscular Atrophy. by (No Author), 2000
  4. 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
  5. Lipidoses: An entry from Thomson Gale's <i>Gale Encyclopedia of Children's Health: Infancy through Adolescence</i> by Tish, A.M. Davidson, Altha Edgren, 2006

21. Vincristine Treatment Revealing Asymptomatic Hereditary Motor Sensory Neuropathy
Keywords vincristine; hereditary sensory motor neuropathy type 1A; neuropathy; acute lymphoblastic leukaemia. © 1999 by Archives of Disease in Childhood
http://adc.bmjjournals.com/cgi/content/abstract/81/5/442

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Arch Dis Child 442-443 ( November )
Vincristine treatment revealing asymptomatic hereditary motor sensory neuropathy type 1A
E Mercuri a , J Poulton b , J Buck c , V Broadbent c , M Bamford d , H Jungbluth a , A Y Manzur a , F Muntoni a a b Department of Genetics, Radcliffe Hospital, Oxford OX9 3DU, UK, c Department of Paediatric Oncology, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK, d Ipswich Hospital, Heath Road, Ipswich IP4 5PD, UK

22. Vincristine Treatment Revealing Asymptomatic Hereditary Motor Sensory Neuropathy
(Arch Dis Child 1999;81442443) Keywords vincristine; hereditary sensory motor neuropathy type 1A; neuropathy; acute lymphoblastic leukaemia
http://adc.bmjjournals.com/cgi/content/full/81/5/442

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Pharmacology and toxicology

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Arch Dis Child 442-443 ( November )
Vincristine treatment revealing asymptomatic hereditary motor sensory neuropathy type 1A
E Mercuri a , J Poulton b , J Buck c , V Broadbent c , M Bamford d , H Jungbluth a , A Y Manzur a , F Muntoni a a b Department of Genetics, Radcliffe Hospital, Oxford OX9 3DU, UK, c Department of Paediatric Oncology, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK, d Ipswich Hospital, Heath Road, Ipswich IP4 5PD, UK

23. Search Forbes.com Book Club
Hereditary Motor Sensory Neuropathy, Hereditary Motor Sensory Neuropathy I, Hereditary peroneal nerve dysfunction, hereditary sensory motor neuropathy.
http://www.forbesbookclub.com/SearchResults.asp?pagenum=5&ProdCat=HEA

24. Hereditary Motor Sensory Neuropathies: Charcot-Marie-Tooth
hereditary sensorymotor neuropathy with Ataxia26 l Chromosome 7q22-q32; Dominant. Epidemiology Single American family of Irish ancestry; Onset
http://www.neuro.wustl.edu/neuromuscular/time/hmsn.html
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HEREDITARY MOTOR SENSORY NEUROPATHIES (HMSN; CMT)
Dominant
CMT 1A
: PMP-22; 17p11
CMT 1B
: P protein; 1q22
CMT 1C
: LITAF; 16p13
CMT 1D

CMT 1E
: P protein; 1q22
CMT 1F
: Neurofilament light chain; 8p21 HNPP : PMP-22 deletion; 17p11 HMSN 3 (Dejerine-Sottas) PMP-22; P Thermosensitive Sensory PN + hearing loss : Connexin-31; 1p35 Hypomyelin, No symptoms Recessive CMT 4A : GDA P1; 8q21.1 CMT 4B CMT 4B2 CMT 4C CMT 4D (Lom) ... CMT 4F : Periaxin; 19q13 HMSN-Russe CMT 4H HMSN 3 (Dejerine-Sottas) P PMP-22 Periaxin HMSN + Juvenile glaucoma ... Congenital hypomyelinating P Farber's lipogranulomatosis : Ceramidase; 8p22 Glycosylation deficient, Ia Krabbe : GALC; 14q31 MLD : ARSA; 22q13 PMP-22 point mutations Refsum's disease Childhood : PHYH; 10pter-p11.2 Adolescent-Adult Infant HMSN + CNS : Heterogeneous X-linked Connexin-32 Pyramidal signs Axonal Dominant CMT 2A1 CMT 2A2 CMT 2B CMT 2C ... CMT 2D : GARS; 7p14 CMT 2E : Neurofilament light chain; 8p21 CMT 2F CMT 2G CMT 2I : P CMT 2J : P CMT 2L HMSN-Proximal HMSN 5 : Pyramidal signs HMSN + Optic atrophy HMSN + Deafness HMSN 6 P ... AR-CMT2A : Lamin A/C; 1q21

25. Neuromuscular Large+Small Sensory
Ulceromutilating Neuropathies Dominant. hereditary sensory motor neuropathy with Ulcero-mutilation1 l Autosomal Dominant. Genetics
http://www.neuro.wustl.edu/neuromuscular/sensory-large small.html

26. EMedicine - Charcot-Marie-Tooth And Other Hereditary Motor And Sensory Neuropath
hereditary motor and sensory neuropathy type 3 (DejerineSottas syndrome). This is a rare hypertrophic neuropathy of infancy inherited as an autosomal
http://www.emedicine.com/neuro/topic468.htm
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Charcot-Marie-Tooth and Other Hereditary Motor and Sensory Neuropathies
Last Updated: January 16, 2004 Rate this Article Email to a Colleague Synonyms and related keywords: Charcot-Marie-Tooth, Charcot-Marie-Tooth neuropathy, Charcot-Marie-Tooth disorder, Charcot-Marie-Tooth neuromuscular disease, Charcot-Marie-Tooth neurologic disease, Charcot-Marie-Tooth syndrome, Charcot-Marie-Tooth disease type 1B, CMT1B, CMT type 1B, hereditary motor and sensory neuropathy 1B, hereditary motor and sensory neuropathy type 1B, HMSN1B, peroneal muscular atrophy, Dejerine-Sottas syndrome, DSS, hereditary motor and sensory neuropathy type 3 AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Aamir Hashmat, MD

27. Neuropathy, Hereditary Sensory, Type I
The hereditary sensory neuropathies (HSN) include 4 6 similar but distinct inherited, The resulting symptoms may involve sensory, motor, reflex,
http://my.webmd.com/hw/brain_nervous_system/nord799.asp
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Who We Are About WebMD Site Map Health Topics Symptoms ... For a Complete Report Neuropathy, Hereditary Sensory, Type I Important It is possible that the main title of the report Neuropathy, Hereditary Sensory, Type I 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
  • Neuropathy, Hereditary Sensory and Autonomic, Type I HSAN1 Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant
Disorder Subdivisions
  • None
General Discussion The hereditary sensory neuropathies (HSN) include 4-6 similar but distinct inherited, degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. The classification of the hereditary sensory neuropathies is complicated, and the experts do not always agree. This report deals with HSN type I. There is a separate report in NORD’s Rare Disease Database dealing with HSN type II. One other type of hereditary sensory neuropathy, HSN-III, is related to, or identical with, familial dysautonomia (Riley-Day syndrome). Another type, HSN-IV, is related to, or identical with, a form of Charcot-Marie-Tooth disorder.

28. THE MERCK MANUAL, Sec. 14, Ch. 183, Disorders Of The Peripheral Nervous System
hereditary motor and sensory neuropathy types I and II (CharcotMarie-Tooth disease, peroneal muscular atrophy) is a relatively common, usually autosomal
http://www.merck.com/pubs/mmanual/section14/chapter183/183f.htm
var locationOverride = "http://www.merck.com/pubs/";
This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 14. Neurologic Disorders Chapter 183. Disorders Of The Peripheral Nervous System Topics [General] Lower And Upper Motor Neuron Disorders Nerve Root Disorders Plexus Disorders ... Disorders Of Neuromuscular Transmission
Peripheral Neuropathy
A syndrome of sensory loss, muscle weakness and atrophy, decreased deep tendon reflexes, and vasomotor symptoms, alone or in any combination.
Etiology
Trauma is the most common cause of a localized injury to a single nerve. Violent muscular activity or forcible overextension of a joint may produce a focal neuropathy, as may repeated small traumas (eg, tight gripping of small tools, excessive vibration from air hammers). Pressure or entrapment paralysis usually affects superficial nerves (ulnar, radial, peroneal) at bony prominences (eg, during sound sleep or during anesthesia in thin or cachectic persons and often in alcoholics) or at narrow canals (eg, in carpal tunnel syndrome). Pressure paralysis may also result from tumors, bony hyperostosis, casts, crutches, or prolonged cramped postures (eg, in gardening). Hemorrhage into a nerve and exposure to cold or radiation may cause neuropathy. Mononeuropathy may result from direct tumor invasion. Acute Viral Encephalitis and Aseptic Meningitis in Ch. 176).

29. Neuropathy, Hereditary Sensory, Type I
The hereditary sensory neuropathies (HSN) include 46 similar but distinct inherited, The resulting symptoms may involve sensory, motor, reflex,
http://www.bchealthguide.org/kbase/nord/nord799.htm
var hwPrint=1;var hwDocHWID="nord799";var hwDocTitle="Neuropathy, Hereditary Sensory, Type I";var hwRank="1";var hwSectionHWID="nord799-Header";var hwSource="en-caQ2_05";var hwDocType="Nord";
National Organization for Rare Disorders, Inc.
Neuropathy, Hereditary Sensory, Type I
Important
It is possible that the main title of the report Neuropathy, Hereditary Sensory, Type I 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
  • Neuropathy, Hereditary Sensory and Autonomic, Type I HSAN1 Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant
Disorder Subdivisions
  • None
General Discussion
Hereditary sensory neuropathy Type I (HSN1) is a rare genetic disorder characterized by the loss of sensation (sensory loss), especially in the feet and legs and, less severely, in the hands and forearms. The sensory loss is due to abnormal functioning of the sensory nerves that control responses to pain and temperature and may also affect the autonomic nervous system that controls other involuntary or automatic body processes. other involuntary or automatic body processes.
The disorder is inherited as an autosomal dominant trait, and the mutated gene has been identified and tracked to a site on chromosome 9.

30. BBC - Health - Conditions - Hereditary Motor And Sensory Neuropathy
Discover the causes, symptoms and treatments of this neurological disorder.
http://www.bbc.co.uk/health/conditions/hmsn1.shtml
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Hereditary motor and sensory neuropathy
Dr Trisha Macnair Discover the symptoms and treatments of this inherited neurological disorder, plus links to some useful support groups in the UK and abroad.
In this article
What is it? Symptoms Who's affected? Diagnosis and treatment
What is it?
Charcot-Marie-Tooth disease - also known as hereditary motor and sensory neuropathy (HMSN) or peroneal muscular atrophy - is the name given to a group of conditions where the nerves to the muscles, particularly in the lower leg and hands, don't work properly. The part of the nerves carrying sensory signals back towards the brain may also be affected, leading to altered sensation in the affected areas. There are at least four different types of HMSN, which were originally classified on the basis of electrical tests of nerve conduction. But as genetic causes for these different conditions are discovered, so HMSN conditions are being reclassified into a wider group of disorders. Other features include high arched feet in many cases and, in some less common types, deafness, problems with vision, paralysis of the vocal cords and breathing difficulties.

31. Peripheral Neuropathy
Sarnat, HB hereditary motor sensory Neuropathies. In Textbook of Pediatrics, ed. WE Nelson, et al., 17581759. 1996. Philadelphia WB Saunders Company.
http://www.athenadiagnostics.com/site/content/diagnostic_ed/neuro_disorders/peri
Peripheral Neuropathy
Peripheral neuropathy (PN) affects two million people in the U.S., typically middle-aged and elderly individuals. It is a neurological disorder that affects the sensory, motor and/or autonomic nerves, and is caused by abnormal function of these nerves due to various etiologies. These disorders can originate from numerous causes, such as diabetes, alcoholism, HIV, toxin exposure, metabolic abnormalities, vitamin deficiency, or adverse effects of certain drugs. However, after evaluations for the etiologies of these PNs are performed, 32-70% of all peripheral neuropathies remain idiopathic. With the development of autoimumme and genetic tests, these idiopathic peripheral neuropathies can often be diagnosed. Autoimmune Peripheral Neuropathy
Symptoms of an autoimmune peripheral neuropathy may include weakness, cramping, decreased tendon reflexes, numbness, tingling, and pain affecting the arms and/or legs. Clinically, peripheral neuropathies are classified according to various characteristics: symmetric or asymmetric, proximal or distal, acute or chronic, slowly progressive or rapid onset, affecting one (mononeuropathy) or many nerves (polyneuropathy). Upon electrophysiological examination, assessment of a neuropathy can be further classified, depending on which part of the peripheral nervous system is affected, such as the axon or myelin sheath. Peripheral neuropathy can be demyelinating, axonal, or both, as determined by electromyography (EMG) and nerve conduction studies (NCS).

32. Dorlands Medical Dictionary
motor and sensory neuropathy, hereditary, (HMSN) any of a group of hereditary sensory and motor neuropathy, hereditary, hereditary motor and sensory n.
http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

33. ANNALS ONLINE -- Sign In Page
hereditary motor and sensory neuropathies. In Peripheral neuropathy. PJ Dyck, PK Thomas, JW Griffin, PA Low, JF Podulso, Eds. 10941136. Saunders.
http://www.annalsnyas.org/cgi/content/full/883/1/186

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34. An Algorithm For The Evaluation Of Peripheral Neuropathy - February 15, 1998 - A
hereditary motor sensory neuropathy Vitamin B12 deficiency hereditary amyloid neuropathy type II* Lead neuropathy. HIV=human immunodeficiency virus;
http://www.aafp.org/afp/980215ap/poncelet.html

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Journals Vol. 57/No. 4 (February 15, 1998) ... Patient Information
An Algorithm for the Evaluation of Peripheral Neuropathy
ANN NOELLE PONCELET, M.D.,
University of California, San Francisco, San Francisco, California
The diagnosis of peripheral neuropathies can be frustrating, time consuming and costly. Careful clinical and electrodiagnostic assessment, with attention to the pattern of involvement and the types of nerve fibers most affected, narrows the differential diagnosis and helps to focus the laboratory evaluation. An algorithmic approach to the evaluation and differential diagnosis of a patient with peripheral neuropathy is presented, based on important elements of the clinical history and physical examination, the use of electromyography and nerve conduction studies, autonomic testing, cerebrospinal fluid analysis and nerve biopsy findings. The underlying cause of axonal neuropathies can frequently be treated; demyelinating neuropathies are generally managed with the assistance of a neurologist. T he incidence of peripheral neuropathy is not known, but it is a common feature of many systemic diseases. Diabetes and alcoholism are the most common etiologies of peripheral neuropathy in adults living in developed countries. The primary worldwide cause of treatable neuropathy is leprosy.

35. Acrodystrophic Neuropathy,Hereditary Sensory Neuropathy Type I
hereditary sensory Neuropathies of various types may attack a single nerve These symptoms may involve sensory, motor, reflex, or blood vessel
http://www.icomm.ca/geneinfo/acrodystr.htm

36. Hereditary Neuropathies Information Page: National Institute Of Neurological Dis
including hereditary motor and sensory neuropathy, hereditary sensory neuropathy, hereditary motor neuropathy, and hereditary sensory and autonomic
http://www.ninds.nih.gov/disorders/neuropathy_hereditary/neuropathy_hereditary.h
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The nation's leading supporter of biomedical research on disorders of the brain and nervous system More about Hereditary Neuropathies
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You are here: Home Disorders Hereditary Neuropathies NINDS Hereditary Neuropathies Information Page
Synonym(s): Neuropathy - Hereditary Get Web page suited for printing Email this to a friend or colleague Table of Contents (click to jump to sections) What is Hereditary Neuropathies? Is there any treatment? What is the prognosis? What research is being done? ... Related NINDS Publications and Information What is Hereditary Neuropathies? Hereditary neuropathies are a group of inherited disorders of the peripheral nervous system. Within the group there are 4 subcategories of disorders, including hereditary motor and sensory neuropathy, hereditary sensory neuropathy, hereditary motor neuropathy, and hereditary sensory and autonomic neuropathy.

37. Core Curriculum - POSNA
Define hereditary motor sensory neuropathy and list several types The hereditary motor sensory neuropathies (HMSN) are a heterogeneous group of
http://www.posna.org/index?service=page/coreCurriculum&article=hereditaryMotorSe

38. Clinical Neurosciences - Hereditary Motor And Sensory Neuropathy
hereditary motor sensory neuropathy (HMSN). Synonyms. Peroneal muscular atrophy; CharcotMarie-Tooth disease; Roussy-Levy syndrome; Dejerine-Sottas
http://medweb.bham.ac.uk/http/depts/clin_neuro/teaching/tutorials/hmsn/hmsn.html
Synonyms Peroneal muscular atrophy; Charcot-Marie-Tooth disease; Roussy-Levy syndrome; Dejerine-Sottas disease HMSN is the commonest cause of the peroneal muscular atrophy syndrome consisting of distal leg muscle wasting and weakness, usually with a pes cavus foot deformity.
THE DIFFERENTIAL DIAGNOSIS OF PES CAVUS AND LOWER LEG WASTING INCLUDES:
  • HMSN
  • Old polio infection
  • Friederich's ataxia
  • Spina bifida
    CLINICAL FEATURES
    The characteristic clinical features include distal wasting of the lower limb muscles (the so-called 'inverted champagne bottle' appearance). The feet show pes cavus and clawing of the toes, with weakness of the feet extensors. The ankle jerks are absent and the plantar reflexes show no response (occasionally they can be extensor). Palpable nerve thickening is found in about 25% of cases and is specific for the demyelinating forms of HMSN. The patient may have a 'high stepping gait' due to bilateral foot drop. There may be wasting of the small muscles of the hand. In general, the presenting symptoms are due to difficulty walking or a foot deformity. The inheritance is usually autosomal dominant, but recessive forms also occur. The video clip (video not ready yet) shows some of these clinical features in 2 affected brothers.
  • 39. HONselect - Hereditary Motor And Sensory Neuropathies
    Translate this page hereditary, Type III, motor and sensory neuropathy - hereditary, Type VII, motor and sensory neuropathy. Français, Neuropathies héréditaires motrices et
    http://www.hon.ch/HONselect/RareDiseases/C10.500.300.html
    List of rare diseases: English Deutsch
    Language:
    MeSH term:
    Accepted terms:
    English: Hereditary Motor and Sensory Neuropathies - Dejerine-Sottas Disease
    - HMSN
    - HMSN Type III
    - HMSN Type VII
    - Hereditary, Type III, Motor and Sensory Neuropathy
    - Hereditary, Type VII, Motor and Sensory Neuropathy
    Français: Neuropathies héréditaires motrices et sensorielles - Neuropathies héréditaires motrices et sensitives Deutsch: Hereditäre motorische und sensorische Neuropathien - Hereditäre motorische und sensorische Neuropathie, Typ III - HMSN Typ III - Déjerine-Sottas-Krankheit - Hereditäre motorische und sensorische Neuropathie, Typ VII - HMSN Typ VII - HMSN Español: Neuropatías Motoras y Sensoriales Hereditarias - Neuropatía Motora y Sensorial Hereditaria Tipo III - Enfermedad de Dejerine-Sottas - HMSN Tipo III - Neuropatía Motora y Sensorial Hereditaria Tipo VII - HMSN Tipo VII - HMSN Português: Neuropatias Motoras e Sensoriais Hereditárias - Neuropatia Motora e Sensorial Hereditária Tipo III - Doença de Dejerine-Sottas - HMSN Tipo III - Neuropatia Motora e Sensorial Hereditária Tipo VII - HMSN Tipo VII - HMSN HONselect ressources Definition: Yes Articles: Yes Images: No News: No Conferences: No Clinical trials: No Web sites: English Yes Français Yes Deutsch No Español Yes Português No Home About us Site map Search ... Contact http://www.hon.ch/HONselect/RareDiseases/C10.500.300.html

    40. Charcot Marie Tooth (hereditary Motor Sensory Neuropathy
    have hereditary motor sensory neuropathy; correction without long-term recurrence can be achieved with bracing, and, for progressive curves,
    http://www.wheelessonline.com/ortho/charcot_marie_tooth_hereditary_motor_sensory
    Error in query [ ] Column count doesn't match value count at row 1 Duke Orthopaedics presents Wheeless' Textbook of Orthopaedics Site Index A - Z Search Site by Word Home Contact Us My Account
    Charcot Marie Tooth: (hereditary motor sensory neuropathy)
    - Discussion:
    type I
    type II , and type III are most common;
    - type IV:
    - is also referred to as Refsum disease;
    - associated w/ excess phytanic acid;
    - type V:
    - inherited spastic paraplegia with distal weakness in the limbs;
    - usually presents in the second decade of life or later;
    - patients develop awkward gait and equinus foot deformities;
    - type VI involves optic atrophy in association with peroneal muscular atrophy;
    - type VII is characterized by retinitis pigmentosa associated with distal muscle weakness in the limbs and atrophy; - Clinical Features: - patients may present w/ muscle cramps, difficulty with gait or w/ deformities of the feet; upper extremity in CMT: - hip joint: look for proximal muscle weakness, and hip dysplasia;

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