Cytomegalovirus ventriculoencephalitis in AIDS. A syndrome with distinct clinical and pathologic features.

Cytomegalovirus ventriculoencephalitis is a late and terminal complication of AIDS. Cytomegalovirus retinitis was diagnosed before the onset of encephalitis in all but 1 of the 7 patients in this series. A distinct clinical presentation was observed, with encephalitis often associated with cranial nerve deficits and gaze-directed nystagmus. Examination of CSF demonstrated pleocytosis with elevated protein and hypoglycorrhachia. Increased signal of periventricular white matter was visualized by MRI soon after the development of encephalitis, and progressive ventriculomegaly was detected by serial CT scanning. Cytomegalovirus ventriculoencephalitis developed in some patients while receiving ganciclovir or foscarnet maintenance therapy, and the response to higher doses of these agents was limited in the 2 patients so treated. Death ensued a median of 4 weeks after the onset of neurologic symptoms. Pathologic examination showed extensive necrotizing periventriculitis involving ependymal and subependymal regions with spread to the meninges and adjacent cranial nerve roots. The infection was associated with characteristic CMV inclusion-bearing cells. This entity should be considered in AIDS patients with encephalitis, particularly in the presence of cranial nerve impairment or ascending muscle weakness. With the improvement in survival of patients with AIDS it is expected that this manifestation of CMV infection will become increasingly common.