Charcot –Marie–Tooth disease and neuroinflammation

Charcot –Marie–Tooth disease possibly presents neurophysiological or pathological demyelinating features resembling acquired immune‐mediated polyneuropathies. The involvement of the immune system in the development of demyelination in Charcot–Marie–Tooth disease was established by experimental ani mal studies. AbstractPatients with the misdiagnosis or co ‐occurrence of Charcot–Marie–Tooth disease (CMT) and inflammatory neuropathies have been reported previously. Both CMT and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have similar clinical features, including flaccid weakness in four limbs with areflexia, demyelinating neu rophysiological features and pathological demyelination. However, the symptoms of CIDP progress for several weeks or months, and typical CIDP shows proximal and distal muscle weakness. In contrast, CMT progresses for several decades and presents distal‐dominant muscle weakness, which is called len gth‐dependent neuropathy. On neurophysiological evaluation, CMT had been considered to show “uniform conduction slowing.” However, a recent study showed that CMT could show non‐uniform conduction abnormalities, which resemble inflammatory neuropathies. Furthermore, the incidence of inflammat ory neuropathy in CMT is estimated to be higher than that in the general population. CMT1A with CIDP was the most common, followed by CMTX1 with CIDP and CMT1A with Guillain–Barré syndrome. Some patients showed macrophage‐ass...

Source: Clinical and Experimental Neuroimmunology - February 25, 2020 Category: Neurology Authors: Norito Kokubun Tags: REVIEW ARTICLE Source Type: research