Zusammenfassung
Als immunvermittelte und damit prinzipiell gut behandelbare Polyneuropathie sollte die CIDP möglichst frühzeitig und sicher diagnostiziert werden. Die typische klinische Symptomatik besteht in progredienten oder schubförmigen, oft proximal-betonten, symmetrischen, vorwiegend motorischen peripheren Defiziten. Grundsätzlich basiert die Diagnosesicherung aber auf elektrophysiologischen Parametern. Während entsprechend der diagnostischen Kriterien der „American Academy of Neurology” eine signifikante Demyelinisierung in zahlreichen Nerven mittels verschiedener Parameter nachgewiesen werden muss, sollte - aufgrund atypischer Sonderformen der CIDP mit mehr umschriebenen, asymmetrischen oder distal-betonten klinischen Defiziten - mittlerweile besonderer Wert auf den Nachweis umschriebener Entmarkungen wie Leitungsblock oder fokale temporale Dispersion gelegt werden. Sind gerade zu Beginn der Erkrankung die Läsionen nur sehr umschrieben, kann die proximale Nervenstimulation zum Nachweis fokaler Entmarkungen im Bereich von Plexus oder Wurzel von besonderer Bedeutung sein. Von der idiopathischen CIDP abgegrenzt werden sollten symptomatische Formen wie die CIDP bei monoklonalen Gammopathien, da sich hier häufig therapeutische Besonderheiten ergeben. Therapeutisch kommen bei der CIDP neben den drei Basistherapieverfahren (Kortison, hoch dosierte Immunglobuline, Plasmapherese) auch verschiedene klassische immunsuppressive Medikamente in Betracht. Zur Therapiekontrolle sollte neben einer validierten klinischen Befunderhebung auch der elektrophysiologische Verlauf mit besonderer Berücksichtigung der Leitungsblockdiagnostik herangezogen werden.
Abstract
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune and treatable disease. Therefore, CIDP should be diagnosed as early as possible. Typical clinical symptoms of CIDP are progressive or stepwise, often proximal, symmetrical deficits with predilection of motor nerves. Diagnosis of CIDP is predominantly based on electrophysiological criteria. According to the diagnostic criteria of the American Academy of Neurology, significant demyelination must be demonstrated in multiple nerves by different parameters. However, most acquired demyelinating polyneuropathies with more focal, asymmetrical or distally accentuated clinical deficits are also considered to be variants of CIDP. Therefore, diagnostic evidence of focal demyelination with conduction block or focal temporal dispersion is more important. In early CIDP, focal demyelination might be limited to proximal nerve segments as plexus or dorsal roots and proximal nerve stimulation can be necessary to demonstrate focal demyelinating lesions. Symptomatic forms of CIDP as in monoclonal gammopathy should be differentiated from idiopathic CIDP because of differences in treatment response. Therapy of CIDP comprises three alternative basic regimens (steroids, immunoglobulins, plasmapheresis). In non-responders, different immunosuppressive agents can be applied. Treatment response should be evaluated by standardised and scored clinical examinations as well as electrophysiological tests including conduction block studies.
Key words
Chronic inflammatory demyelinating polyneuropathy - CIDP criteria - conduction block - temporal dispersion - monoclonal gammopathy
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PD Dr. Holger Grehl
Neurologische Klinik Evangelisches und Johanniter-Klinikum
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