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Revue Neurologique
Volume 173, n° 5
pages 320-325 (mai 2017)
Doi : 10.1016/j.neurol.2017.03.018
Received : 16 February 2017 ;  accepted : 29 Mars 2017
Motor neuropathies and lower motor neuron syndromes
 

A. Verschueren
 Reference Centre for Neuromuscular Diseases and ALS, University Hospital La Timone, 264 rue Saint Pierre, 13385 Marseille cedex 05, France 

Abstract

Motor or motor-predominant neuropathies may arise from disease processes affecting the motor axon and/or its surrounding myelin. Lower motor neuron syndrome (LMNS) arises from a disease process affecting the spinal motor neuron itself. The term LMNS is more generally used, rather than motor neuronopathy, although both entities are clinically similar. Common features are muscle weakness (distal or proximal) with atrophy and hyporeflexia, but no sensory involvement. They can be acquired or hereditary. Immune-mediated neuropathies (multifocal motor neuropathy, motor-predominant chronic inflammatory demyelinating polyneuropathy) are important to identify, as effective treatments are available. Other acquired neuropathies, such as infectious, paraneoplastic and radiation-induced neuropathies are also well known. Focal LMNS is an amyotrophic lateral sclerosis (ALS)-mimicking syndrome especially affecting young adults. The main hereditary LMNSs in adulthood are Kennedy's disease, late-onset spinal muscular atrophy and distal hereditary motor neuropathies. Motor neuropathies and LMNS are all clinical entities that should be better known, despite being rare diseases. They can sometimes be difficult to differentially diagnose from other diseases, particularly from the more frequent ALS in its pure LMN form. Nevertheless, correct identification of these syndromes is important because their treatment and prognoses are definitely different.

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Keywords : Motor neuropathy, Lower motor neuron syndrome, Motor neuronopathy, ALS, Spinal muscular atrophy, Benign monomelic amyotrophy




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