نتایج جستجو برای: hereditary sensory and autonomic neuropathies

تعداد نتایج: 16848591  

Journal: :Brain : a journal of neurology 2010
Magdalena Zimoń Jonathan Baets Michaela Auer-Grumbach José Berciano Antonio Garcia Eduardo Lopez-Laso Luciano Merlini David Hilton-Jones Meriel McEntagart Andrew H Crosby Nina Barisic Eugen Boltshauser Christopher E Shaw Guida Landouré Christy L Ludlow Rachelle Gaudet Henry Houlden Mary M Reilly Kenneth H Fischbeck Charlotte J Sumner Vincent Timmerman Albena Jordanova Peter De Jonghe

Hereditary neuropathies form a heterogeneous group of disorders for which over 40 causal genes have been identified to date. Recently, dominant mutations in the transient receptor potential vanilloid 4 gene were found to be associated with three distinct neuromuscular phenotypes: hereditary motor and sensory neuropathy 2C, scapuloperoneal spinal muscular atrophy and congenital distal spinal mus...

Journal: :Journal of the peripheral nervous system : JPNS 1997
P De Jonghe V Timmerman E Nelis J J Martin C Van Broeckhoven

Soon after the description of Charcot-Marie-Tooth disease (CMT) in 1886, it became apparent that this syndrome is clinically and genetically heterogeneous. Neuropathological and electrophysiological studies have further dissected this syndrome into distinct categories that are now classified in a complex nosology of the inherited peripheral neuropathies. The recent advent of molecular genetics ...

Journal: :The Journal of heredity 1981
D P Sponenberg A deLahunta

Hypertrophic neuropathy in a family of inbred Tibetan Mastiff dogs is shown to be due to an autosomal recessive gene. The disease has an onset at 7 to 12 weeks of age and results in varying degrees of paresis. HEREDITARY hypertrophic neuropathies have been described in humans and in mice. In humans these include the autosomal dominant Charcot-Marie-Tooth disease with onset in the first or secon...

2012
B. Forest Plucknette Michael S. Brogan Joseph M. Anain Karl J. Terryberry

16 Objective: This study develops normalized values for sensation in the feet of healthy subjects. Knowledge relative to normal sensory perception and subsequent loss will enable clinicians to differentiate between normal and abnormal findings, especially in patients with or at risk for diabetes. Methods: Subjects were over age 18 and without medical complications that might compromise sensory ...

Journal: :Sexually transmitted infections 2002
R F Miller S Bunting S T Sadiq H Manji

Two HIV infected patients presented with peripheral neuropathy, in one patient this was originally ascribed to HIV associated mononeuritis multiplex and in the other to stavudine. Investigations confirmed these diagnoses and in both cases genetic analysis identified a second hereditary aetiology: in the first patient hereditary neuropathy with liability to pressure palsies and in the second her...

Journal: :Archives of disease in childhood 1999
E Mercuri J Poulton J Buck V Broadbent M Bamford H Jungbluth A Y Manzur F Muntoni

A 5 year old boy developed severe weakness after receiving vincristine for treatment of acute lymphoblastic leukaemia. Although weakness improved after the discontinuation of vincristine, other symptoms suggestive of a neuropathy persisted. Neurophysiological and genetic analysis at age 8 years indicated that vincristine had induced symptoms of a hereditary sensory motor neuropathy type 1A, whi...

Journal: :Neuron 2014
Emily Petrus Amal Isaiah Adam P. Jones David Li Hui Wang Hey-Kyoung Lee Patrick O. Kanold

Sensory systems do not work in isolation; instead, they show interactions that are specifically uncovered during sensory loss. To identify and characterize these interactions, we investigated whether visual deprivation leads to functional enhancement in primary auditory cortex (A1). We compared sound-evoked responses of A1 neurons in visually deprived animals to those from normally reared anima...

2006
Alexandre Moreau-Gaudry Anne Prince Olivier Chenu Jacques Demongeot Yohan Payan

Pressure ulcers are recognized as a major health issue in individuals with spinal cord injuries and new approaches to prevent this pathology are necessary. An innovative health strategy is being developed through the use of computer and sensory substitution via the tongue in order to compensate for the sensory loss in the buttock area for individuals with paraplegia. This sensory compensation w...

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