Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment

Wolfgang N Löscher, Eva-Maria Oberreiter, Marcus Erdler, Stefan Quasthoff, Valeriu Culea, Klaus Berek, Norbert Embacher, Susanne Grinzinger, Isolde Hess, Franz Stefan Höger, Corinne G C Horlings, Michael Huemer, Julia Jecel, Waltraud Kleindienst, Eva Laich, Petra Müller, Dierk Oel, Wolfgang Örtl, Eva Lenzenweger, Jakob RathKlaus Stadler, Karl Stieglbauer, Claudia Thaler-Wolf, Julia Wanschitz, Fritz Zimprich, Hakan Cetin, Raffi Topakian

Research output: Journal article (peer-reviewed)Journal article

Abstract

BACKGROUND AND OBJECTIVES: Multifocal 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.

METHODS: Anonymized 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.

RESULTS: 57 Patients with MMN were identified, resulting in a prevalence of 0.65/100.000. Mean age of onset was 44.1 ± 13.1 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: 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.

Original languageEnglish
Pages (from-to)2834-2840
Number of pages7
JournalJournal of Neurology
Volume265
Issue number12
DOIs
Publication statusPublished - 1 Dec 2018

Keywords

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Austria/epidemiology
  • Autoantibodies/metabolism
  • Female
  • Follow-Up Studies
  • G(M1) Ganglioside/immunology
  • Humans
  • Immunoglobulin M/metabolism
  • Male
  • Middle Aged
  • Motor Neuron Disease/epidemiology
  • Neurologists
  • Prevalence
  • Surveys and Questionnaires
  • Young Adult

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