Extractions: Home What's New Browse Handbook ... Search Hereditary neuropathy with liability to pressure palsies On this page: Hereditary neuropathy with liability to pressure palsies is a disorder that affects peripheral nerves. These nerves connect the brain and spinal cord to muscles as well as sensory cells that detect touch, pain, and temperature. In people with this disorder, the peripheral nerves are unusually sensitive to pressure, which can cause recurrent episodes of numbness and loss of muscle strength (pressure palsies). Hereditary neuropathy with liability to pressure palsies causes episodes of numbness, tingling, and/or muscle weakness. An episode can last from several minutes to several months, but recovery is usually complete. Repeated incidents, however, can cause permanent weakness or loss of sensation. This disorder is also associated with painful limbs, especially the hands.
Extractions: AllRefer Channels :: Yellow Pages Reference Health Home ... Contact Us Quick Jump ADD/ADHD Allergies Alzheimer's Disease Arthritis Asthma Back Pain Breast Cancer Cancer Colon Cancer Depression Diabetes Gallbladder Disease Heart Attack Hepatitis High Cholesterol HIV/AIDS Hypertension Lung Cancer Menopause Migraines/Headaches Osteoporosis Pneumonia Prostate Cancer SARS Stroke Urinary Tract Infection 1600+ More Conditions Alternative Medicine Health News Symptoms Guide Special Topics ... Medical Encyclopedia Go To Main Page Alternate Names : Hereditary Motor and Sensory Neuropathy, Hereditary Peroneal Nerve Dysfunction, Neuropathy - Peroneal (Hereditary), Progressive Neuropathic (Peroneal) Muscular Atrophy Definition Charcot-Marie-Tooth disease defines a group of inherited, slowly progressive disorders that result from progressive damage to nerves. Symptoms include numbness and wasting of muscle tissue, first in the feet and legs, then in the hands and arms.
Hereditary Motor And Sensory Neuropathy hereditary motor and sensory neuropathy. Used for group of peripheral nervous system hereditary disorders with varying course, symptoms, neuropathology http://crisp.cit.nih.gov/Thesaurus/00003732.htm
Extractions: Next Term: hereditary neuropathy Charcot Marie Tooth disease Charcot Marie Tooth muscular atrophy Dejerine Sottas disease Hanel's syndrome neuropathic muscular atrophy peroneal muscular atrophy progressive neural muscular atrophy Roussy Levy syndrome HMSN hereditary peripheral nervous system disorder sensory neuropathy Refsum disease Send your comments to: Melody Lowe
DermIS / Menu Principal / PeDOIA / Table Des Matières / 2 Génodermatoses / 2. Translate this page Hered motor/sens neuropathy IV, hereditary motor and sensory neuropathy type hereditary sensory-motor neuropathy, type IV, hereditary Type IV motor http://dermis.multimedica.de/doia/diagnose.asp?zugr=p&lang=f&diagnr=757106&topic
Extractions: Vol. 56 No. 10, October 1999 Featured Link E-mail Alerts Editorial Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Related articles in this issue Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Pleasure D Contact me when this article is cited Topic Collections Neurogenetics Topic Collection Alerts Hereditary Motor and Sensory Neuropathy The Plot Thickens Arch Neurol. Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. IN THIS issue of the ARCHIVES, De Jonghe and colleagues describe a family with autosomal dominant hereditary motor and sensory neuropathy (HMSN). Whereas the proband had bilateral pes cavus, slight peroneal weakness, and loss of Achilles reflexes, 12
Immunological Study Of Hereditary Motor And Sensory Neuropathy Abbreviations HMSN1a, hereditary motor and sensory neuropathy type 1a; PMP22, peripheral nerve myelin protein 22; IL6, interleukin-6; sTNF R1, http://jnnp.bmjjournals.com/cgi/content/abstract/72/2/230
Extractions: This Article Figures Only Full Text Full Text (PDF) ... Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Hartung, H.-P. Neurology Articles K. Wagner, PhD V. Timmerman, PhD P. De Jonghe, MD, PhD and H.-P. Hartung, MD 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 marked distal sensory loss, frequent toe and foot ulcerations, osteomyelitis, and necrosis, which may be complicated by toe
Extractions: Peripheral neuropathies can be hereditary (passed within families) or acquired. Hereditary neuropathies often have characteristic clinical, electrodiagnostic, and genetic markers. An example of a hereditary neuropathy is Charcot-Marie Tooth (CMT) disease or hereditary sensory and motor neuropathy (HSMN). Acquired neuropathies, on the other hand, are more common and can be more difficult to diagnose. The most common cause of acquired polyneuropathy is diabetes mellitus. Other causes include Guillain-Barre syndrome or acute inflammatory demyelinating polyradiculoneuropathy (AIDP), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), nerve compression, nutritional deficiencies, organ failure, tumors, and a variety of generalized disorders (including, but not limited to amyloidosis, connective tissue diseases, and vasculitis). Diagnosis The treatment of peripheral neuropathies depends on the underlying cause. For instance, therapy for peripheral neuropathy caused by diabetes involves control of the diabetes. A multi-disciplinary approach is used for all peripheral neuropathies, including treatment of pain and maintenance of strength and flexibility. This is accomplished by using appropriate neuropathic medications, immune-modulating agents (such as corticosteroids, azathioprine, cyclosporine, cyclophosphamide, mycophenolate, and rituximab), IVIG, therapeutic apheresis, and physical and occupational therapy.
Extractions: (advertisement) Synonyms, Key Words, and Related Terms: 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 Background: Charcot-Marie-Tooth (CMT) disease was first recognized independently in France and Great Britain (Charcot and Marie, 1886; Tooth, 1886). A few years later, a more severe form of inherited neuropathy was described (Dejerine and Sottas, 1893). More recent nomenclature designated Charcot-Marie-Tooth disease as a hereditary motor and sensory neuropathy (HMSN). Recent advances in genetic research have identified several types of HMSN, which correspond with specific genetic mutations. In 1968, Dyck and Lambert created a broader classification system, which is as follows: HMSN types 1A and B (dominantly inherited hypertrophic neuropathies) HMSN type 2 (neuronal type of peroneal muscular atrophy) HMSN type 3 (hypertrophic neuropathy of infancy [Dejerine-Sottas]) HMSN type 4 (hypertrophic neuropathy [Refsum] associated with phytanic acid excess)
Extractions: var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Brain, Spinal Cord, and Nerve Disorders Chapter Peripheral Nerve Disorders Topics Introduction Hereditary Neuropathies Mononeuritis Multiplex Mononeuropathy Muscle Stimulation Disorders Neuromuscular Junction Disorders ... Thoracic Outlet Syndromes Hereditary Neuropathies Buy The Book Print This Topic Email This Topic Pronunciations atrophy Charcot-Marie-Tooth disease mononeuropathy muscular atrophy ... thoracic Hereditary neuropathies affect the peripheral nerves, causing subtle symptoms that worsen gradually. Hereditary neuropathies may affect only motor nerves (motor neuropathies), only sensory nerves (sensory neuropathies), or both sensory and motor nerves (sensory-motor neuropathies). Some hereditary neuropathies are relatively common but often are not recognized. Hereditary sensory neuropathies are especially rare.
Hereditary Motor-Sensory Neuropathies (HMSN) hereditary motorsensory Neuropathies (HMSN) England, JD Entrapment Neuropathies. Current Opinion in Neurology 1999; 12597-602. Request http://www.athenadiagnostics.com/site/content/diagnostic_ed/references/hmsn.asp
Extractions: Auer-Grumbach, M. et al., Roussy-Levy syndrome is a phenotypic variant of Charcot-Marie-Tooth syndrome IA associated with a duplication on chromosome 17p11.2. Journal of Neurological Sciences 1998; 154:72-75. Chapon, F.P. et al., Axonal phenotype of Charcot-Marie-Tooth disease associated with a mutation in the myelin protein zero gene. Journal of Neurology, Neurosurgery, and Psychiatry 1999; 66:779-782. England, J.D. Entrapment Neuropathies. Current Opinion in Neurology 1999; 12:597-602. Request Reprint Graf, W. et al., Severe Vincristine Neuropathy in Charcot-Marie-Tooth Disease Type 1A. Cancer 1996; 77:1356-1362. Ohnishi, A.T. et al., Denervation of eccrine glands in patients with familial amyloidotic polyneuropathy type I. Neurology 1998; 51:714-721. Parnell, K.J. and Pourmand, R. Evaluation of a Patient Presenting with Foot Drop. Journal of Clinical Neuromuscular Disease 1999; 1(1):41-46. N/A Poloni, T.E. et al., Facial nerve is liable to pressure palsy. Neurology 1998; 51:321-322. Sahenk, Z.L. et al., A novel PMP22 point mutation causing HNPP phenotype. Neurology 1998; 51:702-707.
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NINDS Forwarding Page Therefore, doctors may use terms such as predominately motor neuropathy, predominately sensory neuropathy, sensorymotor neuropathy, or autonomic neuropathy http://www.ninds.nih.gov/health_and_medical/pubs/peripheral_neuropathy.htm
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Extractions: the word the part of word in MeSH term in MeSH term and description Information on "Hereditary Motor and Sensory Neuropathies": Medical hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Hierarchy English French German Spanish Portuguese Hereditary Motor and Sensory Neuropathies Definition: A group of slowly progressive inherited disorders affecting motor and sensory peripheral nerves. Subtypes include HMSNs I-VII. CHARCOT-MARIE-TOOTH DISEASE and II both refer to CHARCOT-MARIE-TOOTH DISEASE HMSN VII refers to hypertrophic neuropathy of infancy, HMSN VII refers to HMSN VII refers to a condition marked by a hereditary motor and sensory neuropathy associated with spastic paraplegia (see SPASTIC PARAPLEGIA, HEREDITARY HMSN VII refers to HMSN associated with an inherited optic atrophy ( OPTIC ATROPHIES, HEREDITARY ), and HMSN VII refers to HMSN associated with retinitis pigmentosa. (From Adams et al., Principles of Neurology, 6th ed, p1343)
Baylor Neurology Case Of The Month Diagnosis Multifocal motor neuropathy with conduction block However, rare cases of motor involvement without significant sensory signs have been http://www.bcm.edu/neurology/challeng/pat26/summary.html
Extractions: Diagnosis: Multifocal Motor Neuropathy with conduction block Patient #26 presented with slowly progressive asymmetrical limb weakness, atrophy, and fasciculations without evidence of sensory or upper motor neuron involvement. The pattern of weakness was primarily distal, and cranial nerves were not affected. These findings indicate a lower motor neuron syndrome or asymmetrical motor neuropathy. The differential diagnosis includes the various causes of motor neuron disease, chronic inflammatory demyelinating neuropathy, motor neuropathies, and mononeuritis multiplex. CIDP usually causes a symmetrical, distal, sensorimotor neuropathy primarily affecting the legs (though arms may be prominently affected, and proximal involvement is not unusual). Sensory symptoms are extremely common, and sensory signs are almost universal. However, rare cases of motor involvement without significant sensory signs have been described. In most cases, however, the motor involvement is symmetrical at onset and throughout the course of the disease. The clinical course is progressive with episodes of relapse. Reflexes are universally decreased and often absent. These features argue against CIDP as the cause of neuropathy in this case. The EMG/NCV findings also argue against CIDP, as motor conduction velocities are markedly slowed even in areas without conduction blocks in CIDP findings not present in this case. Mononeuritis multiplex (multiple mononeuropathies) may be caused by diabetes mellitus, vasculitis, sarcoidosis, infectious causes (HIV, Lyme, leprosy), HNPP (hereditary neuropathy with predisposition to pressure palsies), multiple nerve tumors (neurofibromatosis), and perineuromas. Clinically, mononeuritis multiplex typically presents with asymmetrical motor and sensory involvement and variable involvement of reflexes. It would be highly unusual for mononeuritis multiplex to present as a pure motor syndrome, as in this case. The long course of the disease without other accompanying features would also be highly unusual for any of the causes of multiple mononeuropathies.
Extractions: Neuropathic Muscular Atrophy Polyneuropathy Hereditary Sensory and Autonomic Neuropathies ... Neural Tube Defects Hereditary Motor and Sensory Neuropathies Hereditary Sensory and Autonomic Neuropathies ... Neuronal Ceroid-Lipofuscinosis Hereditary Motor and Sensory Neuropathies Hereditary Sensory and Autonomic Neuropathies ... Mental Retardation, X-Linked
Neuromuscular Diseases Clinical Features of motor Neuron Disease Bethlehem Health Care, Inc. (AU). motor Neuron Disease and hereditary sensory and Autonomic Neuropathies http://www.mic.ki.se/Diseases/c10.668.html
Extractions: Diseases and Disorders Links pertaining to Neuromuscular Diseases Alert! Patients and laypersons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider. Start Page Contents: Amyotrophic Lateral Sclerosis Brachial Plexus Neuritis Brachial Plexus Neuritis Brachial Plexus Neuropathies ... Ulnar Nerve Compression Syndromes Neuromuscular Diseases Motor Cortex illustration [Earles et al.] - Furman Univ. (US) EMG and Nerve Conductions Homepage [JF Jabre] The Neuromuscular Disease Information Center - REHABinfo Network/UC-Davis (US) Neuromuscular Disorders Information - MDA Assoc. of Canada Neuromuscular Diseases - MDA (US) Neuromuscular Disorders [Nadeau and Valenstein] - Univ of Florida (US) Neuromuscular Disorders Information - Muscular Dystrophy Group of (UK)+ N. Ireland Info about the Creatine Kinase (CK, CPK) test - MDA (US), and some more - ADAM/MedlinePLUS Neuromuscular Disease Center , and a collection of one-page summaries of
Neurosurgery - UserLogin Topics such as familial amyloid polyneuropathies, the hereditary sensory motor neuropathies, leukodystrophies, and hereditary liability to pressure palsies http://www.neurosurgery-online.com/pt/re/neurosurg/fulltext.00006123-199608000-0
HighWire -- Browse Journals - Hereditary Motor And Sensory Neuropathies Journals focusing on hereditary motor and sensory Neuropathies (in order by highest focus). American Journal of Neuroradiology info free ISSUES http://highwire.stanford.edu/lists/topic_dir/602925/602926/602998/603011/focus.d
Extractions: Charcot-Marie-Tooth Disease Hereditary Spastic Paraplegia Sort by: Alphabet Frequency of articles in Hereditary Motor and Sensory Neuropathies Focus of journal on Hereditary Motor and Sensory Neuropathies What's this? Journals focusing on Hereditary Motor and Sensory Neuropathies (in order by highest focus) American Journal of Neuroradiology info free ISSUES New England Journal of Medicine ... Nervous System Malformations Hereditary Motor and Sensory Neuropathies Home Adv. Search For Institutions For Publishers ... partners/suppliers