Multifokale motorische Neuropathie: Klinische und elektrophysiologische Charakteristika

Zusammenfassung

Die multifokale motorische Neuropathie (MMN) ist eine behandelbare, vermutlich immunvermittelte Neuropathie. Die klinische Symptomatik ist durch asymmetrische, rein motorische Defizite charakterisiert, die dem Verteilungsmuster peripherer Nerven entsprechen und vorwiegend distale Abschnitte an den oberen Extremitäten betreffen. Die typischen elektrophysiologischen Befunde der MMN sind multifokale, häufig persistierende Leitungsblöcke, die die Abgrenzung der MMN von den nichtbehandelbaren Motoneuronerkrankungen erlauben. Da die Demyelinisierung bei der MMN nicht so ausgedehnt ist wie bei der chronisch-entzündlichen demyelinisierenden Polyneuropathie (CIDP), sind die Nervenleitgeschwindigkeiten oft normal. Um die Nachweismöglichkeit eines Leitungsblockes zu verbessern, sollten mittels spezieller elektrophysiologischer Stimulationstechniken proximale Nervenabschnitte mituntersucht werden. Antikörpertiter gegen GM₁-Ganglioside können bei der MMN erhöht sein, reichen aber zur Diagnosesicherung nicht aus. Hoch dosierte intravenöse Immunglobuline (ivIg) und Zyklophosphamid sind die beiden einzigen gesicherten effektiven Therapieverfahren der MMN. IvIg sind aufgrund ihres günstigen Nebenwirkungsprofils die Therapie der ersten Wahl. Kortikosteroide und Plasmapherese können die neurologischen Symptome verschlechtern. Die schnelle Besserung nach einer Immunglobulintherapie könnte durch die Neutralisierung funktionell blockierender Antikörper bedingt sein. Immunglobuline sind auch im Langzeitverlauf effektiv. Bei einigen Patienten kann jedoch die Erkrankung trotz regelmäßiger Immunglobulinbehandlungen fortschreiten. Da eine Immunglobulinlangzeittherapie teuer ist, kann sie auch in Kombination mit Zyklophosphamid durchgeführt werden, um Immunglobuline einzusparen.

Abstract

Multifocal motor neuropathy (MMN) is a treatable, probably immune-mediated neuropathy. Typical clinical symptoms are asymmetrical pure motor deficits in a peripheral nerve distribution, predominantly affecting distal parts of upper limbs. The characteristic electrophysiological findings of MMN are multifocal and often persistent conduction blocks that can be differentiated from untreatable motor neuron disease. Since demyelination in MMN is not as widespread as in chronic inflammatory demyelinating polyneuropathy (CIDP), nerve conduction velocities are often normal. To improve the diagnosis of conduction blocks, electrophysiological studies should include proximal nerve segments using special stimulation techniques. Antibody titres against GM₁ gangliosides may be elevated in MMN, but are not sufficient for diagnostic values. High dose intravenous immunoglobulins (IVIG) and cyclophosphamide are the only effective therapies in MMN, IVIG being the treatment of choice because of its less serious side effects. Corticosteroids and plasma exchange therapy may aggravate neurological deficits. Rapid improvement after IVIG application may be caused by neutralisation of functional blocking antibodies. IVIG is also effective in the long-term course. In some patients, however, disease progression may be observed despite of regular IVIG treatment. As long-lasting IVIG treatment is expensive, combination with cyclophosphamide may be performed to reduce the IVIG dosage. Application of further immunomodulatory drugs in MMN should be investigated in the future.

Key words

Multifocal motor neuropathy - MMM - conduction block - anti-GM₁ - antibodies - immunoglobulin therapy - IVIG

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