Article

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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Langue : ANGLAIS