Ingrid Herraets1, Marieke van Rosmalen1, Jeroen Bos1, Ruben van Eijk1, Elies Cats1, Bas Jongbloed1, Lotte Vlam1, Sanne Piepers1, Jan-Thies van Asseldonk1, H Stephan Goedee1, Leonard van den Berg1, W Ludo van der Pol2. 1. From the Department of Neurology and Neurosurgery (I.H., M.v.R., J.B., R.v.E., H.S.G., L.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.v.E.), University Medical Center Utrecht; Department of Neurology (E.C.), Gelre Hospital, Apeldoorn; Department of Neurology (B.J.), Admiraal de Ruyter Hospital, Goes; Department of Neurology (L.V.), Erasmus Medical Center, Rotterdam; Department of Neurology (S.P.), Meander Medical Center, Amersfoort; and Department of Neurology and Clinical Neurophysiology (J.-T.v.A.), Elisabeth-Tweesteden Hospital Tilburg, the Netherlands. 2. From the Department of Neurology and Neurosurgery (I.H., M.v.R., J.B., R.v.E., H.S.G., L.v.d.B., W.L.v.d.P.), UMC Utrecht Brain Center Rudolf Magnus; Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care (R.v.E.), University Medical Center Utrecht; Department of Neurology (E.C.), Gelre Hospital, Apeldoorn; Department of Neurology (B.J.), Admiraal de Ruyter Hospital, Goes; Department of Neurology (L.V.), Erasmus Medical Center, Rotterdam; Department of Neurology (S.P.), Meander Medical Center, Amersfoort; and Department of Neurology and Clinical Neurophysiology (J.-T.v.A.), Elisabeth-Tweesteden Hospital Tilburg, the Netherlands. w.l.vanderpol@umcutrecht.nl.
Abstract
OBJECTIVE: To assess the clinical course of multifocal motor neuropathy (MMN) in a large cohort of patients and to identify predictive factors of a progressive disease course. METHODS: Between May 2015 and February 2016, we collected clinical data from 100 patients with MMN, of whom 60 had participated in a nationwide cross-sectional cohort study in 2007. We documented clinical characteristics using standardized questionnaires and performed a standardized neurologic examination. We used multiple linear regression analysis to identify factors that correlated with worse outcome. RESULTS: We found that age at diagnosis (45.2 vs 48.6 years, p < 0.02) was significantly increased between 2007 and 2015-2016, whereas diagnostic delay decreased by 15 months. Seven out of 10 outcome measures deteriorated over time (all p < 0.01). Patients who had a lower Medical Research Council (MRC) sumscore and absence of 1 or more reflexes at the baseline visit showed a greater functional loss at follow-up (p = 0.007 and p = 0.016). CONCLUSIONS: Our study shows that MMN is a progressive disease. Although 87% of patients received maintenance treatment, muscle strength, reflexes, vibration sense, and the Self-Evaluation Scale score significantly deteriorated over time. Lower MRC sumscore and absence of reflexes predicted a more progressive disease course. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that lower MRC sumscore and the absence of reflexes predict a more progressive disease course in patients with MMN.
OBJECTIVE: To assess the clinical course of multifocal motor neuropathy (MMN) in a large cohort of patients and to identify predictive factors of a progressive disease course. METHODS: Between May 2015 and February 2016, we collected clinical data from 100 patients with MMN, of whom 60 had participated in a nationwide cross-sectional cohort study in 2007. We documented clinical characteristics using standardized questionnaires and performed a standardized neurologic examination. We used multiple linear regression analysis to identify factors that correlated with worse outcome. RESULTS: We found that age at diagnosis (45.2 vs 48.6 years, p < 0.02) was significantly increased between 2007 and 2015-2016, whereas diagnostic delay decreased by 15 months. Seven out of 10 outcome measures deteriorated over time (all p < 0.01). Patients who had a lower Medical Research Council (MRC) sumscore and absence of 1 or more reflexes at the baseline visit showed a greater functional loss at follow-up (p = 0.007 and p = 0.016). CONCLUSIONS: Our study shows that MMN is a progressive disease. Although 87% of patients received maintenance treatment, muscle strength, reflexes, vibration sense, and the Self-Evaluation Scale score significantly deteriorated over time. Lower MRC sumscore and absence of reflexes predicted a more progressive disease course. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that lower MRC sumscore and the absence of reflexes predict a more progressive disease course in patients with MMN.
Authors: W Ludo van der Pol; H Stephan Goedee; Marieke H J van Rosmalen; Martijn Froeling; Stefano Mandija; Jeroen Hendrikse Journal: J Neurol Date: 2022-01-06 Impact factor: 4.849
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Authors: Jeroen W Bos; Ewout J N Groen; Renske I Wadman; Chantall A D Curial; Naomi N Molleman; Marinka Zegers; Paul W J van Vught; Reinier Snetselaar; Raymon Vijzelaar; W Ludo van der Pol; Leonard H van den Berg Journal: Neurol Genet Date: 2021-06-22