T W Rattay1,2, N Winter1, B F Décard3, N M Dammeier1, F Härtig1, M Ceanga4, H Axer4, A Grimm1. 1. Department of Neurology, Tübingen University Hospital, Tübingen, Germany. 2. Hertie Institute for Clinical Brain Research and German Center of Neurodegenerative Diseases (DZNE), University of Tübingen, Tübingen, Germany. 3. Department of Neurology, Basel University Hospital, Basel, Switzerland. 4. Hans Berger Department of Neurology, Jena University Hospital, Jena, Germany.
Abstract
BACKGROUND AND PURPOSE: High-resolution ultrasound is a valuable tool in supporting the diagnosis of multifocal motor neuropathy (MMN) but longitudinal data under therapy are lacking. METHODS: The change in peripheral nerve ultrasound pattern in patients with MMN was assessed over time. Patients with MMN received a thorough initial examination and follow-up over a period of 6-12 months using high-resolution ultrasound of the cervical roots and the nerves of the arms and legs, nerve conduction studies, Medical Research Council Sum Score (MRCSS) and Rotterdam Inflammatory Neuropathy Cause and Treatment Group (INCAT) score to evaluate changes under treatment. The Ultrasound Pattern Sum Score (UPSS) was used as standardized peripheral nerve ultrasound protocol. RESULTS: Seventeen patients with MMN received initial examinations of whom 12 were successfully followed up. All patients with MMN showed at least localized but often multifocal peripheral nerve enlargement. An enlarged overall cross-sectional area as well as enlarged single fascicles (>3 mm²) in clinically and electrophysiologically affected (>90%) and unaffected (>70%) nerves were found. The UPSS did not correlate with clinical disability at both visits. However, the change in clinical disability (evaluated as difference in MRCSS) and the change in UPSS correlated significantly inversely (P = 0.004). CONCLUSIONS: High-resolution sonography of peripheral nerves revealed multifocal nerve enlargement in MMN. Distinct enlargement patterns may support the diagnosis. Ultrasound findings did not correlate well with clinical severity or electrophysiological findings at initial presentation. As changes in UPSS correlated significantly with the clinical course in terms of muscle strength (MRCSS), sonographic assessment may represent a useful tool for therapeutic monitoring.
BACKGROUND AND PURPOSE: High-resolution ultrasound is a valuable tool in supporting the diagnosis of multifocal motor neuropathy (MMN) but longitudinal data under therapy are lacking. METHODS: The change in peripheral nerve ultrasound pattern in patients with MMN was assessed over time. Patients with MMN received a thorough initial examination and follow-up over a period of 6-12 months using high-resolution ultrasound of the cervical roots and the nerves of the arms and legs, nerve conduction studies, Medical Research Council Sum Score (MRCSS) and Rotterdam Inflammatory Neuropathy Cause and Treatment Group (INCAT) score to evaluate changes under treatment. The Ultrasound Pattern Sum Score (UPSS) was used as standardized peripheral nerve ultrasound protocol. RESULTS: Seventeen patients with MMN received initial examinations of whom 12 were successfully followed up. All patients with MMN showed at least localized but often multifocal peripheral nerve enlargement. An enlarged overall cross-sectional area as well as enlarged single fascicles (>3 mm²) in clinically and electrophysiologically affected (>90%) and unaffected (>70%) nerves were found. The UPSS did not correlate with clinical disability at both visits. However, the change in clinical disability (evaluated as difference in MRCSS) and the change in UPSS correlated significantly inversely (P = 0.004). CONCLUSIONS: High-resolution sonography of peripheral nerves revealed multifocal nerve enlargement in MMN. Distinct enlargement patterns may support the diagnosis. Ultrasound findings did not correlate well with clinical severity or electrophysiological findings at initial presentation. As changes in UPSS correlated significantly with the clinical course in terms of muscle strength (MRCSS), sonographic assessment may represent a useful tool for therapeutic monitoring.
Authors: Tim W Rattay; Jennifer Just; Benjamin Röben; Holger Hengel; Rebecca Schüle; Matthis Synofzik; Anne S Söhn; Natalie Winter; Nele Dammeier; Ludger Schöls; Alexander Grimm Journal: Orphanet J Rare Dis Date: 2018-11-03 Impact factor: 4.123
Authors: H Stephan Goedee; Ingrid J T Herraets; Leo H Visser; Hessel Franssen; Jan-Thies H van Asseldonk; W Ludo van der Pol; Leonard H van den Berg Journal: Muscle Nerve Date: 2019-07-24 Impact factor: 3.217
Authors: Kai F Loewenbrück; Robin Werner; René Günther; Markus Dittrich; Robert Klingenberger; Heinz Reichmann; Alexander Storch; Andreas Hermann Journal: J Neurol Date: 2020-12-23 Impact factor: 4.849