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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