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6–94 | Clinical Manual for Management of the HIV-Infected Adult/2006
Radiographic studies
CNS imaging may reveal changes typical of PML, but
is nonspecific. Magnetic resonance imaging (MRI) is
more sensitive than computed tomography (CT) for
detecting PML. PML presents as single or multiple
hypodense lesions in the subcortical white matter,
with no surrounding edema. On MRI, lesions show
increased T2 signal and little or no enhancement
with gadolinium. On CT, PML lesions typically are
nonenhancing. In some patients, and particularly
in patients taking ART, PML lesions may show
inflammatory changes, such as enhancement.
CSF evaluation
♦ CSF cell count, protein level, and glucose level
generally are normal or show mild pleocytosis and
slightly elevated protein.
♦
A JC virus polymerase chain reaction (PCR) assay
is approximately 75-85% sensitive; detection of JC
virus in a patient whose clinical presentation and
radiographic imaging results are consistent with
PML is adequate to make a diagnosis. A negative
result with JC virus PCR does not rule out PML.
Other studies
♦ Other diagnostic tests should be performed as
indicated to rule out other potential causes of the
patient’s symptoms.
♦
A brain biopsy should be considered if the diagnosis
is unclear.
Treatment
♦
♦
♦
There is no specific treatment for JC virus. Potent
ART with effective immune reconstitution is the
only treatment that may be effective for patients
with PML. Even with ART, however, mortality rates
approach 50%, and neurologic deficits are unlikely to
be reversible.
Initiate ART for patients who are not already
receiving treatment. It is not clear whether
antiretroviral agents with good CNS penetration are
more effective than those that are less likely to cross
the blood-brain barrier.
For patients who are taking ART with incomplete
virologic suppression, change the ART regimen
appropriately to achieve virologic suppression,
if possible. For patients on ART with poor
immunologic response, consider changing or
intensifying therapy with the goal of improved
immunologic recovery. (See chapter Antiretroviral
Therapy.)
♦
♦
♦
If symptoms are caused by immune reconstitution,
consider adding corticosteroids (eg, dexamethasone)
to help decrease inflammation.
Depending on the patient’s cognitive and physical
status, he or she may need a care provider in the
home to assure that medications are taken on
schedule.
The patient is likely to need supportive care for
personal hygiene, nutrition, safety, and prevention of
accidents or injury; refer as indicated.
Patient Education
♦
♦
When a diagnosis of PML has been established or
suspected, the clinician should initiate a discussion
of plans for terminal care (including wills, advanced
directives, and supportive care and services) with
the patient and family members or caregivers.
Supportive treatment will be necessary for an
undetermined period of time, and hospice referral
should be considered if the patient does not show
clinical improvement in response to ART.
If the patient is receiving ART, the clinician should
be sure that family members or friends are taught
about the medications and are able to help the
patient with adherence.
References
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♦
♦
♦
♦
Aksamit AJ. Review of progressive multifocal
leukoencephalopathy and natalizumab. Neurologist.
2006 Nov;12(6):293-8.
Centers for Disease Control and Prevention,
National Institutes of Health, HIV Medicine
Association/Infectious Diseases Society of America.
Treating Opportunistic Infections Among HIV-
Infected Adults and Adolescents. MMWR Recomm
Rep. 2004 Dec 17; 53(RR15);1-112. Available at:
http://aidsinfo.nih.gov/Guidelines/GuidelineDetail.
aspx?GuidelineID=14.
Koralnik IJ. New insights into progressive multifocal
leukoencephalopathy. Curr Opin Neurol. 2004
Jun;17(3):365-70.
Ragland J. Progressive multifocal leukoencephalopathy.
AIDS Clin Care. 1993 Mar;5(3):17-19.
Wyen C, Lehmann C, Fatkenheuer G, et al. AIDSrelated
progressive multifocal leukoencephalopathy in
the era of HAART: report of two cases and review
of the literature. AIDS Patient Care STDS. 2005
Aug;19(8):486-94.