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Peripheral neuropathies and motor neuron disorders 

Peripheral neuropathies and motor neuron disorders
Chapter:
Peripheral neuropathies and motor neuron disorders
Author(s):

Stéphane Mathis

and Jean-Michel Vallat

DOI:
10.1093/med/9780198701590.003.0112_update_001

Update:

For diabetic neuropathy, we added further details about its prognostic.

Some information about nodo-paranodopathies (acute and chronic) were provided.

We highlighted the difficulties for classifying Charcot-Marie-Tooth, due to the too many implicated genes (nowadays more than 10 genes were found).

For amyotrophic lateral sclerosis, we added a sentence about edaravone, an antioxidant treatment now used in addition to riluzole.

Updated on 24 September 2020. The previous version of this content can be found here.
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date: 26 November 2020

Peripheral nervous system disorders are common in older people. Most peripheral neuropathies are secondary to metabolic (diabetes) or toxic causes (medications, alcohol), but several other aetiologies are possible, requiring clinical, electrophysiological, biological, and sometimes pathological (nerve biopsy) examinations to make the right diagnosis and instigate effective treatments. However, despite using this strategy, no cause is found in some axonal polyneuropathies, leading to the concept of chronic idiopathic axonal polyneuropathy (CIAP). Amyotrophic lateral sclerosis (ALS) affects the peripheral nervous system, but is also characterized by central nervous system involvement: it is a strictly motor disorder affecting both the first and second motor neurons, usually presenting during the sixth or seventh decade. This rapidly progressive, still incurable neurodegenerative disease leads to death in all cases.

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