We report 4 autopsy-proven cases of cytomegalovirus ventriculoencephal
itis (CMV-VE) and a further case with dramatic clinical and radiologic
al response to ganciclovir therapy, The diagnoses were based upon clin
ical features, cerebrospinal fluid (CSF) examination and either brain
computerized tomography (CT) or magnetic resonance imaging (MRT), and
confirmed by autopsy findings in 4 cases. All patients had previously
had an AIDS-defining condition. CMV retinitis was diagnosed in 3 patie
nts, 2 of them before the onset of encephalitis. CMV viremia was prese
nt in 4 patients. Examination of CSF demonstrated elevated protein and
hypoglycorachia in all cases. CSF culture was negative for CMV in 3 o
f 3 patients, Periventricular enhancement was detected by MRI in 2 of
3 patients, but in only 1 of 5 patients by CT, Three patients received
ganciclovir and 2 patients foscarnet therapy, All 4 patients died. Pa
thologic examination revealed periventriculitis with ependymal necrosi
s and CMV intranuclear inclusion bodies all 4 patients. One showed a m
arked neurological improvement and radiological resolution by MRI afte
r 4 weeks of ganciclovir therapy. We conclude that CMV-VE should be su
spected in HIV-infected patients who present with altered neurological
status, CMV viremia or retinitis, hypoglycorachia and ventriculitis a
s demonstrated by MRI. Although more effective therapy is needed, ganc
iclovir may be beneficial, as shown in one of our patients.