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

Abstract

Purpose: 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.

Methods: 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.

Results: 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).

Conclusions: 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.