Article

Neuronal and / or axonal hyperactivity and hyperexcitability is an important
feature of motor neuron diseases. It results clinically in cramps and
fasciculations. It is not specific to motor neuron diseases, and can occur in
healthy subjects, as well as in various pathologies of the peripheral nervous
system, including nerve hyperexcitability syndromes. Hyperexcitability plays an
important and debated role in the pathophysiology of motor neuron diseases,
especially in amyotrophic lateral sclerosis (ALS). The mechanisms causing
hyperexcitability are not yet clearly identified. While most studies favor a
distal axonal origin site of fasciculations, some of the fasciculations could be
of cortical origin. The consequences of hyperexcitability are also discussed,
whether it is rather protective or deleterious in the disease course.
Fasciculations are depicted both clinically and using electromyogram, and more
recently the interest of ultrasound has been highlighted. The importance of
fasciculation potentials in the diagnosis of ALS led to changes in
electrophysiological criteria at Awaji consensus conference.
The contribution of
these modifications to ALS diagnosis has been the subject of several studies. In
clinical practice, it is necessary to distinguish fasciculations potentials of
motor neuron disease from benign fasciculations. In most studies of fasciculation
potentials in ALS, the presence of complex fasciculation potentials appears to be
relevant for the diagnosis and the prognosis of the disease.
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Langue : ANGLAIS