Incidence and presentation of the central neurological manifestations of Wegener's granulomatosis: a monocentric study of 14 cases

Central nervous system manifestations are rare clinical features of Wegener's granulomatosis, and occur in 4-8% of the patients, but few studies were dedicated to them. This retrospective study (1988-2001) include 14 consecutive patients suffering from Wegener's granulomatosis. Involvement of central nervous system was defined as follows: suggestive neurological signs, compatible cerebral imaging, efficacy of the specific treatment of the granulomatosis. Four patients had a central nervous system manifestation (29%), including three women (average age 51 years). The signs were inaugural in a case. The manifestations were the following: sensibility disturbance (three cases), motor weakness (two cases), aphasia (one case), and mood disorders (two cases). Cranial nerves were constantly involved. Cerebral magnetic resonance imaging findings were: pachymeningitis and venous thrombosis (one case), vasculitis (two cases). Under steroid therapy associated with cyclophosphamide, in spite of a recurrence at 27 months in one patient, all patients had a complete remission. There was no death, with a median follow-up of 66 months. Except the ocular signs, that were more common (three cases), these patients had the usual characteristics of the Wegener's granulomatosis: rhinosinusitis (four cases), pulmonary (three cases), renal (three cases), and peripheral nervous system involvement (three cases). Our study, based on precise criteria, indicates that the frequency of the central neurologic manifestations of Wegener's granulomatosis is probably under estimated. Cranial nerves involvement is highly evocative. The long-term prognosis seems good, in spite of the associated multivisceral disorder.