Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener's granulomatosis: case report and literature review

Abstract

Objective and importance: We present a very unusual case of diffuse spread of Wegener's granulomatosis causing hydrocephalus.

Clinical presentation: A 53-year-old man presented in 1985 with bilateral middle ear infections requiring myringotomies. During the next 18 months, he went on to develop a left Bell's palsy. The patient then began to develop recurrent occipital headaches along with left sixth and seventh nerve palsies and a green nasal discharge requiring hospitalization. Workup included magnetic resonance imaging showing pronounced enhancement of the tentorium and meninges in the occipital region with normal ventricle size. An x-ray of the chest showed multiple pulmonary nodules. A regimen of prednisone and cyclophosphamide was initiated. The patient did well for 2 years until he again developed middle ear infections and headache. Serial lumbar punctures showed increased pressures. A circulating antineutrophil cytoplasmic antibody was positive. Cyclophosphamide was administered, with acetazolamide added for treatment of the elevated intracranial pressure. The patient stabilized for another 2 years but then presented in 1994 with recurrent headache, bilateral papilledema, and mild left arm and right leg weakness. A lumbar puncture was performed with an opening pressure of 52 cm H2O. Computed tomography of the head revealed moderate enlargement of the lateral third and fourth ventricles, consistent with communicating hydrocephalus.

Intervention: A right frontal ventriculoperitoneal shunt was placed. A leptomeningeal biopsy performed at the side of catheter placement (far away from any meningeal enhancement revealed by magnetic resonance imaging) showed chronic meningitis and multinucleated giant cells. Cyclophosphamide therapy was begun again. The patient has not experienced recurrence of headache, cranial nerve deficits, or papilledema for more than 3 years.

Conclusion: This is the first reported case of diffuse involvement of the meninges from Wegener's granulomatosis. Fortunately, this patient responded well to shunting and sustained medical management. Although rare, Wegener's granulomatosis should be included in the differential diagnosis of chronic aseptic meningitis, communicating hydrocephalus, and papilledema.