Ultrasound is useful for non-invasive visualization of focal nerve pathologies probably resulting from demyelination, remyelination, edema or inflammation. In patients with progressive muscle weakness, differentiation between multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) is essential regarding therapy and prognosis. Therefore, the objective of this study was to investigate whether nerve ultrasound can differentiate between ALS and MMN. Systematic ultrasound measurements of peripheral nerves and the 6th cervical nerve root (C6) were performed in 17 patients with ALS, in 8 patients with MMN and in 28 healthy controls. Nerve conduction studies of corresponding nerves were undertaken in MMN and ALS patients. Electromyography was performed in ALS patients according to revised El-Escorial criteria. ANOVA and unpaired t test with Bonferroni correction revealed significant differences in cross-sectional areas (CSA) of different nerves and C6 diameter between the groups. Nerve enlargement was found significantly more frequently in MMN than in other groups (p < 0.001). Receiver operating characteristics analysis revealed detection of enlarged nerves/roots in at least four measurement points to serve as a good marker to differentiate MMN from ALS with a sensitivity of 87.5% and a specificity of 94.1%. Ultrasonic focal nerve enlargement in MMN was often not colocalized with areas of conduction blocks found in nerve conduction studies. Systematic ultrasound measurements in different nerves and nerve roots are valuable for detecting focal nerve enlargement in MMN, generally not found in ALS and thus could serve as a diagnostic marker to differentiate between both entities in addition to electrodiagnostic studies.

CSF-based miRNAs were differentially regulated in patients with MS as compared with OND and in different MS disease courses. Despite the preliminary character of our case-control study, the results provide rationale for a confirmation study in larger MS cohorts.

As reliable biomarkers of disease activity are lacking, monitoring of therapeutic response in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remains a challenge. We sought to determine whether nerve ultrasound and electrophysiology scoring could close this gap. In CIDP patients (fulfilling EFNS/PNS criteria), we performed high-resolution nerve ultrasound to determine ultrasound pattern sum scores (UPSS) and predominant echotexture nerve conduction study scores (NCSS) as well as Medical Research Council sum scores (MRCSS) and inflammatory neuropathy cause and treatment disability scores (INCAT) at baseline and after 12 months of standard treatment. We retrospectively correlated ultrasound morphology with nerve histology when available. 72/80 CIDP patients featured multifocal nerve enlargement, and 35/80 were therapy-naïve. At baseline, clinical scores correlated with NCSS (r = 0.397 and r = 0.443, p < 0.01), but not or hardly with UPSS (Medical Research Council sum scores MRCSS r = 0.013, p = 0.332; inflammatory neuropathy cause and treatment disability scores INCAT r = 0.053, p = 0.048). Longitudinal changes in clinical scores, however, correlated significantly with changes in both UPSS and NCSS (r = 0.272-0.414, p < 0.0001). Combining nerve/fascicle size with echointensity and histology at baseline, we noted 3 distinct classes: 1) hypoechoic enlargement, reflecting active inflammation and onion bulbs; 2) nerve enlargement with additional hyperechogenic fascicles/perifascicular tissue in > 50% of measured segments, possibly reflecting axonal degeneration; and 3) almost no enlargement, reflecting "burned-out" or "cured" disease without active inflammation. Clinical improvement after 12 months was best in patients with pattern 1 (up to 75% vs up to 43% in pattern 2/3, Fisher's exact test p < 0.05). Nerve ultrasound has additional value not only for diagnosis, but also for classification of disease state and may predict treatment response.

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Ultrasonography can be used to visualize peripheral nerve abnormalities in immune-mediated neuropathies. The objective of this study was to prove the role of ultrasonography (US) in acute phase of Guillain-Barré syndrome (GBS). Systematic ultrasonic measurements of several peripheral nerves including the vagal nerve as well as the sixth cervical nerve root were performed in 18 patients with GBS at days 1-3 after symptom onset and compared to 21 healthy controls. Nerve conduction studies (NCS) of corresponding nerves were undertaken. Consequently, significant differences between the groups were found in compound muscle action potential amplitudes, F-wave latency, and persistency. Ultrasonic cross-sectional areas (CSAs) showed significant enlargement in all nerves except of the ulnar nerve (upper arm) and the sural nerve compared to healthy controls, most prominent in proximal and middle median nerve (p < 0.01). The vagal nerve also showed enlargement compared to controls (p < 0.05), which was most pronounced in patients with autonomic dysfunction compared to patients without (p < 0.05). C6 root diameter showed a significant correlation to the amount of cerebrospinal fluid (CSF)-protein (Pearson correlation, p < 0.05). US shows nerve enlargement in several peripheral nerves including vagal nerve and C6 root in acute phase of GBS and could be an additional diagnostic tool for example, in GBS of atypical onset and autonomic dysfunction.

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To investigate the use of nerve ultrasound (NUS) along with the European Federation of Neurological Societies (EFNS) guidelines and clinical scores in untreated, recently diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) vs. long-lasting treated CIDP. NUS and nerve conduction studies (NCS) of predefined nerves/cervical roots were performed in CIDP on diagnostic onset and "chronic-CIDP" (diagnosis and therapy >6 months), compared to controls. Nerve morphology was quantified using the modified ultrasound pattern sum score mUPSS, which is the sum of 3 ultrasound scores derived at 12 predefined measurement points and the homogeneity score (HS) in ulnar, median, and tibial nerve. 21 onset-CIDP, 21 "chronic-CIDP", and 21 age-matched controls were included. No differences in clinical scores or in the number of electrophysiologically affected nerves existed between the groups. Significantly enlarged cross-sectional areas of the nerves and diameters of the roots ensued already in onset-CIDP; however, with proximal predominance, whilst in chronic-CIDP, nerve enlargement was more prominent and ubiquitous. Increased UPS scores were shown in both patient groups compared to the controls. Significant differences between the patient groups were found particularly in the peripheral nerve score UPSA. Evaluation by means of HS revealed that the nerves in onset-CIDP were mostly regionally enlarged (often sparing distal segments) whereas in chronic-CIDP, nerves were more generalized enlarged. Onset- and chronic-CIDP show enlarged nerves and therefore increased mUPSS, however, nerve enlargement shows a more generalized pattern in chronic-CIDP compared to disease onset and correlates with disease duration and delayed therapy start, but not with disability.