Alexander Grimm1, Debora Vittore2, Victoria Schubert2, Christina Lipski2, Bianka Heiling3, Bernhard F Décard4, Hubertus Axer3. 1. Department of Neurology, Tübingen University Hospital, Tübingen, Germany. Electronic address: alexander.grimm@usb.ch. 2. Department of Neurology, Tübingen University Hospital, Tübingen, Germany. 3. Hans Berger Department of Neurology, Jena University Hospital, Jena, Germany. 4. Department of Neurology, Basel University Hospital, Basel, Switzerland.
Abstract
OBJECTIVE: To investigate the use of nerve ultrasound in the differentiation between Charcot-Marie Tooth hereditary neuropathy (CMT1) and chronic inflammatory demyelinating polyradiculoneuropathies (CIDP), multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensory and motor neuropathies (MADSAM). METHODS: Ultrasound/electrophysiology of predefined nerves was performed in CMT1a/b, immunoneuropathies, and healthy controls. Ultrasound pattern sum score (UPSS, sum of the amount of 12 predefined measurement points), homogeneity score (HS) and regional nerve enlargement index (RNEI) in ulnar, median, and tibial nerve were used for evaluation of morphology. RESULTS: 13 CMT1, 27 CIDP, 10 MADSAM, 12 MMN, and 23 controls were included. Significant enlargement was shown in all neuropathies compared to the controls, (p<0.001), however the amount of enlargement as evaluated by the UPSS was most prominent in CMT compared to the others (median UPSS 18 vs. 11/8.5/5 in CIDP/MADSAM/MMN, p<0.001). Homogeneous enlargement was significantly more often seen in CMT (67%, HS 6 vs. 2-3 in immune-mediated PNP, p<0.001), while in CIDP the enlargement was regional, homogeneous or inhomogeneous with equal contribution. In MMN and MADSAM regional enlargement (48%/40%) next to normal segments (∼20%) predominated (RNEI in MMN=2, in MADSAM=1 vs. 0 in the others). CSAs were inversely correlated with motor conduction velocity. CONCLUSION: Ultrasound, quantified by UPSS, HS, and RNEI facilitates a reliable and reproducible differentiation of immunoneuropathies and hereditary neuropathies by the use of boundary values. SIGNIFICANCE: By the use of quantitative scores, ultrasound differentiation of demyelinating neuropathies is operationalized and ameliorated compared to CSA measurements only.
OBJECTIVE: To investigate the use of nerve ultrasound in the differentiation between Charcot-Marie Tooth hereditary neuropathy (CMT1) and chronic inflammatory demyelinating polyradiculoneuropathies (CIDP), multifocal motor neuropathy (MMN) and multifocal acquired demyelinating sensory and motor neuropathies (MADSAM). METHODS: Ultrasound/electrophysiology of predefined nerves was performed in CMT1a/b, immunoneuropathies, and healthy controls. Ultrasound pattern sum score (UPSS, sum of the amount of 12 predefined measurement points), homogeneity score (HS) and regional nerve enlargement index (RNEI) in ulnar, median, and tibial nerve were used for evaluation of morphology. RESULTS: 13 CMT1, 27 CIDP, 10 MADSAM, 12 MMN, and 23 controls were included. Significant enlargement was shown in all neuropathies compared to the controls, (p<0.001), however the amount of enlargement as evaluated by the UPSS was most prominent in CMT compared to the others (median UPSS 18 vs. 11/8.5/5 in CIDP/MADSAM/MMN, p<0.001). Homogeneous enlargement was significantly more often seen in CMT (67%, HS 6 vs. 2-3 in immune-mediated PNP, p<0.001), while in CIDP the enlargement was regional, homogeneous or inhomogeneous with equal contribution. In MMN and MADSAM regional enlargement (48%/40%) next to normal segments (∼20%) predominated (RNEI in MMN=2, in MADSAM=1 vs. 0 in the others). CSAs were inversely correlated with motor conduction velocity. CONCLUSION: Ultrasound, quantified by UPSS, HS, and RNEI facilitates a reliable and reproducible differentiation of immunoneuropathies and hereditary neuropathies by the use of boundary values. SIGNIFICANCE: By the use of quantitative scores, ultrasound differentiation of demyelinating neuropathies is operationalized and ameliorated compared to CSA measurements only.
Authors: Kai F Loewenbrück; Markus Dittrich; Josef Böhm; Jürgen Klingelhöfer; Petra Baum; Jochen Schäfer; Heinz Reichmann; Andreas Hermann; Alexander Storch Journal: J Neurol Date: 2017-11-28 Impact factor: 4.849
Authors: Angela Puma; N Azulay; N Grecu; C Suply; E Panicucci; C Cambieri; L Villa; C Raffaelli; S Sacconi Journal: J Neurol Date: 2019-06-07 Impact factor: 4.849