COMMUNICATING HYDROCEPHALUS SECONDARY TO DIFFUSE MENINGEAL SPREAD OF WEGENERS-GRANULOMATOSIS - CASE-REPORT AND LITERATURE-REVIEW

OBJECTIVE AND IMPORTANCE: We present a very unusual case of diffuse sp read of Wegener's granulomatosis causing hydrocephalus. CLINICAL PRESE NTATION: A 53-year-old man presented in 1985 with bilateral middle ear infections requiring myringotomies, During the next 18 months, he wen t 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 ventri cle size. An x-ray of the chest showed multiple pulmonary nodules, A r egimen of prednisone and cyclophosphamide was initiated. The patient d id well for 2 years until he again developed middle ear infections and headache. Serial lumbar punctures showed increased pressures. A circu lating antincutuophil cytoplasmic antibody was positive. Cyclophospham ide was administered, with acetazolamide added for treatment of the el evated intracranial pressure. The patient stabilized for another 2 yea rs but then presented in 1994 with recurrent headache, bilateral papil ledema, and mild left arm and right leg weakness. A lumbar puncture wa s performed with an opening pressure of 52 cm H2O. Computed tomography of the head revealed moderate enlargement of the lateral third and fo urth ventricles, consistent with communicating hydrocephalus, INTERVEN TION: A right frontal ventriculoperitoneal shunt was placed. A leptome ningeal biopsy performed at the side of catheter placement (far away f rom any meningeal enhancement revealed by magnetic resonance imaging) showed chronic meningitis and multinucleated giant cells. Cyclophospha mide therapy was begun again. The patient has not experienced recurren ce of headache, cranial nerve deficits, or papilledema for more than 3 years. CONCLUSION: This is the First reported case of diffuse involve ment of the meninges from Wegener's granulomatosis. Fortunately, this patient responded well to shunting and sustained medical management. A lthough rare, Wegener's granulomatosis should be included in the diffe rential diagnosis of chronic aseptic meningitis, communicating hydroce phalus, and papilledema.