Purpose: Wegener's granulomatosis is classically a systemic necrotizing granulomatous vasculitis that involves the upper and lower respiratory tracts and kidneys. Ophthalmologic and neurologic manifestations are common. The limited form of Wegener's granulomatosis may have pathologic characteristics consistent with the disease but lacks the complete 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 limited form.
Methods: From three clinical centers, a chart review disclosed four patients with neuro-ophthalmic 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 nerve palsies, and one with horizontal gaze deviation) in addition to other cranial nerve and cerebral abnormalities. Neuroimaging showed prominent meningeal, as well as intraparenchymal, abnormalities. In all patients, results of antineutrophil cytoplasmic antibody tests were initially negative but in one patient were positive at a late stage of the disease. In all patients, results of a biopsy demonstrated necrotizing granulomatous inflammation consistent with Wegener's granulomatosis.
Conclusions: Neuro-ophthalmic findings may be the earliest manifestations of the limited form of Wegener's granulomatosis. Extensive meningocerebral inflammation can occur before systemic involvement or laboratory confirmation. Early diagnosis by biopsy of affected tissues may facilitate appropriate treatment and prevent progression of the disease.