NEUROOPHTHALMIC MANIFESTATIONS OF MENINGOCEREBRAL INFLAMMATION FROM THE LIMITED FORM OF WEGENERS GRANULOMATOSIS

PURPOSE: Wegener's granulomatosis is classically a systemic necrotizin g granulomatous vasculitis that involves the upper and lower respirato ry tracts and kidneys, Ophthalmologic and neurologic manifestations ar e common. The limited form of Wegener's granulomatosis may have pathol ogic characteristics consistent with the disease but lacks the complet e clinical triad, We studied the clinical, pathologic, laboratory, and radiologic findings of four patients with Wegener's granulomatosis so that others will recognize the disease, even when it occurs in its li mited form. METHODS: From three clinical centers, a chart review discl osed four patients with neuroophthalmic findings and the limited form of Wegener's granulomatosis.RESULTS: Three men and one woman, ranging in age from 40 to 73 years, were studied. All four patients had ocular motor abnormalities (one with oculomotor and trochlear nerve palsies, one with oculomotor nerve palsy, one with trochlear and abducens nerv e palsies, and one with horizontal gaze deviation) in addition to othe r cranial nerve and cerebral abnormalities, Neuroimaging showed promin ent meningeal, as well as intraparenchymal, abnormalities. In all pati ents, results of antineutrophil cytoplasmic antibody tests were initia lly negative but in one patient were positive at a late stage of the d isease. In all patients, results of a biopsy demonstrated necrotizing granulomatous inflammation consistent with Wegener's granulomatosis. C ONCLUSIONS: Neuro-ophthalmic findings may be the earliest manifestatio ns of the limited form of Wegener's granulomatosis. Extensive meningoc erebral inflammation can occur before systemic involvement or laborato ry confirmation. Early diagnosis by biopsy of affected tissues may fac ilitate appropriate treatment and prevent progression of the disease.