Background: Progressive multifocal leukoencephalopathy (PML) is a demy
elinating disorder of the central nervous system found in immunodefici
ent patients, most frequently now in those infected with HIV, It may r
epresent the initial manifestation of HIV infection, Since the central
visual pathways may be affected, a variety of neuro-ophthalmic signs
and symptoms can manifest. We studied the clinical, radiographic and h
istopathological characteristics of patients with PML. Methods: The ch
arts of 13 patients in whom PML was diagnosed in the Neuro-AIDS clinic
at the Montreal Neurological Institute between November 1987 and Marc
h 1995 were reviewed. The diagnosis of PML was established by characte
ristic clinical features together with typical computed tomographic or
magnetic resonance imaging findings, such as nonenhancing low-density
(on computed tomography) or hyperintense (on T-2-weighted magnetic re
sonance imaging) white-matter lesions, without mass effect. Neuro-opht
halmic findings were based on clinical examination by an ophthalmologi
st, neuro-ophthalmologist or neurologist. Tissue for pathological exam
ination was obtained by biopsy in one case and at postmortem study in
a second case. Results: The most common finding was homonymous hemiano
pia, in five patients (38%), Other features included nystagmus (in two
patients), diplopia with cranial nerve palsy (in one) and cortical bl
indness (in one). One of the patients exhibited involvement of the bra
in stem, a site not usually affected by this demyelinating process, In
terpretation: The diagnosis of PML should be considered in immunocompr
omised patients with neuro-ophthalmic findings, particularly those wit
h homonymous hemianopia.