Zusammenfassung
Die multifokale motorische Neuropathie (MMN) gehört zur Gruppe der immunologisch vermittelten entzündlichen Neuropathien. Sie ist klinisch gekennzeichnet durch langsam progrediente, distal betonte, asymmetrische Paresen besonders an den oberen Extremitäten ohne begleitendes sensibles Defizit. Charakteristische Zusatzbefunde sind multifokale persistierende Leitungsblöcke (LB) und eine regelmäßige Assoziation mit hochtitrigen Anti-GM1-IgM-Antikörpern. Wichtige Differenzialdiagnosen umfassen die Motoneuronerkrankungen (spinale Muskelatrophie, amyotrophe Lateralsklerose) sowie andere entzündliche Neuropathien, insbesondere die chronisch-inflammatorische demyelinisierende Neuropathie (CIDP) und das Lewis-Sumner-Syndrom (MADSAM-Neuropathie). Therapie der Wahl sind hoch dosierte intravenöse Immunglobuline (IVIG); Kortikosteroide und Plasmapherese sind gänzlich unwirksam. Die exakte nosologische Einordnung innerhalb der Immunneuropathien ist weiterhin offen, hinsichtlich der optimalen Langzeittherapie fehlen bislang kontrollierte klinische Studien.
Abstract
Multifocal motor neuropathy (MMN) is an immune-mediated disorder which is clinically characterised by slowly progressive, predominantly distal, asymmetrical limb weakness without sensory deficit. The upper limbs are more often affected than the lower limbs. Diagnostic features are persistent multifocal partial conduction blocks (CB) and high-titre anti-GM1 IgM-antibodies. The most important differential diagnoses include motor-neuron diseases (spinal muscle atrophies, amyotrophic lateral sclerosis) and other chronic dysimmune neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) and the Lewis-Sumner syndrome (MADSAM neuropathy). High-dose intravenous immunoglobulins (IVIG) are the first-line treatment while corticosteroids and plasma exchange are generally not effective. In spite of significant successes in elucidating the underlying disease mechanisms, important aetiopathological issues and the optimum long-term therapy remain to be clarified.
Key words
multifocal motor neuropathy - motor conduction block - anti-GM1-antibodies - therapy
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Dr. Christoph Kleinschnitz
Universität Würzburg, Neurologische Klinik und Poliklinik des Universitätsklinikums Würzburg
Josef-Schneider-Straße 11
97080 Würzburg
Email: christoph.kleinschnitz@mail.uni-wuerzburg.de
