Sonographic evaluation of cervical nerve roots in ALS and its clinical subtypes

Abstract

: Morphological assessment of peripheral nerves in amyotrophic lateral sclerosis (ALS) has been available by sonography. Detection of possible axonal atrophy could be important in predicting progression. Research on correlation between sonographic findings and clinical presentation has been sparse. The aim of the study was to assess possible motor axon loss in patients with ALS by sonography and to correlate the imaging features with clinical subtypes. Patients with either definite or probable ALS and control subjects had sonographic evaluation of the cervical nerve roots (C5, C6, and C7). Each diameter and their sums were measured. The ALS patients were classified by their clinical onset and progression (arm-onset, leg-onset, bulbar, and flail-arm variant) and the sonographic features were compared. Overall, the cervical nerve roots were thinner in ALS than in the controls, but the diagnostic sensitivity was low. The patients with arm dysfunctions tended to show thinner nerve roots than those with normal or relatively preserved arm functions. The four ALS subtypes showed similar diameters of the nerve roots. There was no correlation between the disease duration and the diameters of the nerve roots. Sonography of the cervical nerve roots showed axonal atrophy in ALS and potentially reflects subtle arm dysfunctions.

Toward in vivo determination of peripheral nervous system immune activity in amyotrophic lateral sclerosis

“…High prevalence of a degenerative constellation is in line with experimental and human studies in which PNS axonal degeneration and reduction of myelinated fibers dominate the histopathological picture . It is also in agreement with most reports of nerve ultrasound studies in describing significant nerve atrophy at a group level in ALS . Our findings provide evidence that peripheral nerve degeneration relates to a more detrimental disease course, as indicated by its association to shorter disease duration and classic ALS.…”

“…[28][29][30][31] It is also in agreement with most reports of nerve ultrasound studies in describing significant nerve atrophy at a group level in ALS. 13,16,17,32 Our findings provide evidence that peripheral nerve degeneration relates to a more detrimental disease course, as indicated by its association to shorter disease duration and classic ALS. These findings are in agreement with those of several Proposed peripheral nerve inflammatory constellation was found in about 20% of our ALS sample, which is comparable to an MRI study of the brachial or lumbosacral plexus in ALS in which nerve enlargement was reported in 30% of the patients.…”

Ultrasound in the Diagnosis and Monitoring of Amyotrophic Lateral Sclerosis: A Review

“…Mori et al . (2016) revisited the cervical nerve root thinning in ALS . This study was a follow up on the group's initial publication in 2014 .…”

Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis

“…High-resolution ultrasound is a useful tool to quantify structural nerve pathology and thus has a role in the diagnostic workup of peripheral nerve disorders, such as hereditary and immune-mediated neuropathies. 1 Recently, nerve sonography has been applied to amyotrophic lateral sclerosis (ALS) demonstrating evidence of both nerve root and distal peripheral nerve atrophy [2][3][4][5][6][7][8] ; this offers potential for ultrasound-based differentiation of ALS and ALSmimicking disorders characterized by enlarged nerves, particularly multifocal motor neuropathy. 6,9,10 Thus far, sonographic nerve measures, however, have failed to show robust associations with clinical data and other biomarkers in ALS, such as disease duration, revised ALS Functional Rating Scale (ALSFRS-R), disease progression rate, and electrophysiological data.…”

“…6,9,10 Thus far, sonographic nerve measures, however, have failed to show robust associations with clinical data and other biomarkers in ALS, such as disease duration, revised ALS Functional Rating Scale (ALSFRS-R), disease progression rate, and electrophysiological data. [2][3][4][5]7,8,10 As such, although sonographic peripheral nerve atrophy has obvious potential to act as an ALS biomarker, it remains unclear how it relates to other aspects of the pathological process.…”