Die Beteiligung des zentralen Nervensystems (ZNS) im Rahmen von Erkrankungen aus dem rheumatischen Formenkreis stellt eine ernsthafte Bedrohung für den Patienten dar. Sie rechtfertigt in der Regel eine aggressive immunsuppressive Therapie. Die klinische Manifestation hängt von der Größe und Lokalisation der betroffenen Arterien ab. Im Falle einer Vaskulitis großer und mittelgroßer Arterien dominieren neurologische Herdsymptome, die apoplektiform auftreten und zumeist auf ischämische Hirninfarkte (Territorialinfarkte) zurückzuführen sind. Häufiger handelt es sich aber um eine Vaskulitis der kleinen Arterien und die klinische Symptomatik ist eher diffus. Kopfschmerzen und ein hirnorganisches Psychosyndrom sind dann die Leitsymptome. Die paraklinischen Befunde (Liquor, MRT) sind unspezifisch, erlauben aber nicht selten den Beweis eines entzündlichen ZNS-Prozesses. Bildgebende Verfahren können hilfreich sein, andere zentralnervöse Manifestationen wie granulomatöse Enzephalitiden oder Meningitiden von rein vaskulären Syndromen abzugrenzen. Die Prognose der Grunderkrankung verschlechtert sich im Allgemeinen durch das Auftreten einer ZNS-Beteiligung.
Cerebral Vasculitis in Rheumatic Disorders
Involvement of the central nervous systems in rheumatic disorders is a serious threat to patients. Normally it justifies a reinforcement of immunosuppressive treatment. Clinical manifestations depend on the size and localisation of involved vessels. In case of vasculitis of large arteries, focal neurological signs and symptoms like hemiparesis determine the clinical picture. These symptoms appear suddenly and are a consequence of local ischaemic brain infarctions. More often small vessels are involved, which leads to diffuse neurological symptoms such as headache and organic brain syndrome with e. g. disturbed memory or personality changes. Diagnostic procedures include analysis of the cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI). Both may allow the proof of inflammatory CNS disease, but are not specific for vasculitis. MRI may be helpful to distinguish granulomatous encephalitis and meningitis from vasculitis. In general, CNS involvement in systemic rheumatic diseases worsens the prognosis and should therefore be taken seriously.
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