Increased muscle echointensity correlates with clinical disability and muscle strength in chronic inflammatory demyelinating polyneuropathy

Abstract

Background and purpose We evaluated muscle echointensity as a marker for secondary axonal damage in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) using ultrasonography. Findings were correlated with clinical disability and muscular strength. Methods Eighty patients with CIDP (40 with typical and 40 with atypical CIDP) were examined clinically, including assessment of Medical Research Council (MRC) sum score and Inflammatory Neuropathy Cause and Treatment Overall Disability Sum Score (INCAT‐ODSS). Echointensity in eight proximal and distal muscles of the arms and legs was evaluated by muscle ultrasonography using the Heckmatt scale. Results Alterations of echointensity occurred most frequently in the distal leg muscles, with a median (range) Heckmatt score of 1.5 (1–4). There were no differences between typical and atypical CIDP patients with regard to Heckmatt score. Alterations of echointensity correlated to disability and muscle strength. The arm score of the INCAT‐ODSS correlated to Heckmatt score for the distal arm muscles (r = 0.23, p = 0.046) and the leg score of the INCAT‐ODSS correlated to Heckmatt scores for the proximal (r = 0.34, p = 0.002) and distal leg muscles (r = 0.33, p = 0.004). MRC sum score, as well as individual MRC scores for arm and leg muscles, correlated to Heckmatt scores of the corresponding muscle groups (r = −0.25, p = 0.02 for MRC sum score). Conclusion Increased muscle echointensity, reflecting fibrosis and fatty infiltration due to secondary axonal damage, correlated to muscular strength and disability in a large cohort of CIDP patients. Alterations of echointensity occur in both typical and atypical CIDP patients and are pronounced in the distal leg muscles.

Axonal damage determines clinical disability in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): A prospective cohort study of different CIDP subtypes and disease stages

“…Second, evidence of PSA on electromyography in CIDP has been associated with a worse long‐term outcome [11]. Third, increased muscle echointensity, reflecting fibrosis and fatty infiltration due to secondary axonal damage, correlated with muscular strength and disability in CIDP patients [29].…”

Different Tendencies in Muscle Ultrasound Characteristic in Amyotrophic Lateral Sclerosis and Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…13,14 However, only a few studies have investigated the MUS findings of demyelinating neuropathies. 5,15 Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common demyelinating neuropathy. Our previous study and the study by Fisse et al 5 reported that morphologic changes in MUS reflect secondary axonal degeneration in the disease.…”

“…[1][2][3] Recently, several studies have shown that MUS can be a promising biomarker for neuromuscular diseases. [3][4][5][6] The utility of quantitative MUS evaluation of EI and MT has been reported in various axonal degenerative diseases like motor neuron diseases, brachial plexopathies, and several axonal neuropathies. 2,4,7,8 In amyotrophic lateral sclerosis (ALS), e.g., EI and MT have a fair correlation with muscle weakness and electrophysiological findings.…”

Moderner Ultraschall in der Diagnostik Neuromuskulärer Erkrankungen

“…Dies tritt z. B. bei Motoneuronerkrankungen und schweren chronischen Radikulopathien auf, kann aber auch bei Immunneuropathien wie der CIDP infolge eines sekundären axonalen Schadens erscheinen [35]. Das Muster der Muskelbeteiligung im Ultraschall kann verwendet werden, um die zugrundeliegende neurogene Schädigung z.…”

“…In der dynamischen Untersuchung können neurogene Faszikulationen bei Polyneuropathien im Ultraschall beobachtet werden, deren Bedeutung klinisch noch nicht geklärt ist. Diese könnten jedoch in longitudinalen Beobachtungen einen prognostischen Krankheitswert haben [35].…”