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The various forms of hereditary motor neuron disorders and their historical descriptions.

Authors :
Mathis, Stéphane
Beauvais, Diane
Duval, Fanny
Solé, Guilhem
Le Masson, Gwendal
Source :
Journal of Neurology. Jul2024, Vol. 271 Issue 7, p3978-3990. 13p.
Publication Year :
2024

Abstract

Motor neuron disorders comprise a clinically and pathologically heterogeneous group of neurologic diseases characterized by progressive degeneration of motor neurons (including both sporadic and hereditary diseases), affecting the upper motor neurons, lower motor neurons, or both. Hereditary motor neuron disorders themselves represent a vast and heterogeneous group, with numerous clinical and genetic overlaps that can be a source of error. This narrative review aims at providing an overview of the main types of inherited motor neuron disorders by recounting the stages in their historical descriptions. For practical purposes, this review of the literature sets out their various clinical characteristics and updates the list of all the genes involved in the various forms of inherited motor neuron disorders, including spinal muscular atrophy, familial amyotrophic lateral sclerosis, hereditary spastic paraplegia, distal hereditary motor neuropathies/neuronopathies, Kennedy's disease, riboflavin transporter deficiencies, VCPopathy and the neurogenic scapuloperoneal syndrome. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
03405354
Volume :
271
Issue :
7
Database :
Academic Search Index
Journal :
Journal of Neurology
Publication Type :
Academic Journal
Accession number :
178341014
Full Text :
https://doi.org/10.1007/s00415-024-12462-6