Hj. Vangiesen et al., DIAGNOSTIC-CRITERIA AND CLINICAL PROCEDURES IN HIV-1 ASSOCIATED PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY, Journal of the neurological sciences, 147(1), 1997, pp. 63-72
The diagnosis of definite progressive multifocal leukoencephalopathy (
PML) has been a neuropathological domain. We reviewed all Human Immuno
deficiency Virus Type 1 (HIV-I) seropositive patients in our instituti
on between 01.01.1989 and 31.12.1994 and identified 20/823 cases with
PML by clinical and imaging criteria. Diagnosis was neuropathologicall
y confirmed in 5 cases. Diagnostic criteria included rapid onset (<2 w
eeks) of multifocal neurological signs and symptoms, advanced immunosu
ppression and asymmetric uni- or multifocal white matter lesions witho
ut mass effect, contrast enhancement or cortical atrophy in magnetic r
esonance imaging (MRI). The overall incidence of PML was stable over t
he observation period (congruent to 2.5%). The mean age at onset (41.7
years) was significantly lower compared to HIV-1 seronegative PML pat
ients (peak in the sixth decade of life), male patients prevailed (100
%). Mean survival (3.9 months) was extremely short. Human polyoma viru
s JC (JCV) polymerase chain reaction (PCR) in the cerebrospinal fluid
(CSF) demonstrated a considerable rare of possible cerebral co-infecti
on with HIV-1 and JCV as well as subclinical infection with JCV. There
fore demonstration of JCV deoxyribonucleic acid by PCR in the CSF alon
e is not sufficient for clinical PML diagnosis. We present diagnostic
criteria on the basis of epidemiological, neuroradiological and CSF pa
rameters that allow us to make the clinical diagnosis of PML. Although
quick and safe, routine stereotactic brain biopsy is not necessary to
confirm the diagnosis. (C) 1997 Elsevier Science B.V.