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Forschungsdatenbank PMU-SQQUID

Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment.
Loscher, WN; Oberreiter, EM; Erdler, M; Quasthoff, S; Culea, V; Berek, K; Embacher, N; Grinzinger, S; Hess, I; Hoger, FS; Horlings, CGC; Huemer, M; Jecel, J; Kleindienst, W; Laich, E; Muller, P; Oel, D; Ortl, W; Lenzenweger, E; Rath, J; Stadler, K; Stieglbauer, K; Thaler-Wolf, C; Wanschitz, J; Zimprich, F; Cetin, H; Topakian, R
J NEUROL. 2018; 265(12): 2834-2840.
Originalarbeiten (Zeitschrift)

PMU-Autor/inn/en

Grinzinger Susanne
Kleindienst Waltraud

Abstract

Background and objectivesMultifocal motor neuropathy (MMN) is a rare neuropathy and detailed descriptions of larger patient cohorts are scarce. The objective of this study was to evaluate epidemiological, clinical, and laboratory features of MMN patients and their response to treatment in Austria and to compare these data with those from the literature.MethodsAnonymized demographic and clinical data about MMN patients until 31.12.2017 were collected from registered Austrian neurologists. Exploratory statistics on clinical and laboratory features as well as treatment regimens and responses were performed.Results57 Patients with MMN were identified, resulting in a prevalence of 0.65/100.000. Mean age of onset was 44.113.1 years, the diagnostic delay 5.5 +/- 8.4 years. In 77% of patients, symptom onset was in the upper limbs, and in 92%, it occurred in distal muscles. Proximal onset was never observed in the lower limbs. At the final follow-up, the majority of patients had atrophy (88%) in affected regions. Definite motor conduction blocks (CB) were found in 54 patients. Anti-GM1-IgM antibodies were present in 43%. Treatment with intravenous immunoglobulins improved muscle strength and INCAT score initially, but at last follow-up, both scores deteriorated to values before treatment.Discussion p id=Par4 The findings of the present study corroborate the previous findings in MMN. Onset typically occurs in the upper limbs and mostly distal, CBs are found in the majority of cases, while anti-GM1-IgM antibodies are detected in only approximately 40%. Our study underlines that the initial good response to treatment fades over time.


Find related publications in this database (Keywords)

Multifocal motor neuropathy
Conduction block
Intravenous immunoglobulin
Anti-GM1 antibody