Atypical presentations and course of JC virus infection

Area of Science:

• Neurovirology
• Immunocompromised Host Infections
• Central Nervous System (CNS) Diseases

Background:

• Human polyomavirus 2 (JCV) is increasingly recognized to cause a spectrum of central nervous system (CNS) diseases beyond the typical progressive multifocal leukoencephalopathy (PML).
• Understanding these diverse JCV manifestations is critical, particularly in patients with immunodeficiency or those undergoing immunosuppressive therapies like multiple sclerosis (MS) treatments.
• This study aims to increase clinician awareness of unusual presentations of JCV infection.

Observation:

• Case 1: An HIV-positive male with suppressed viral load presented with headache and MRI findings of PML, despite a history of similar resolved episodes.
• Case 2: A 61-year-old male with newly diagnosed HIV experienced subacute binocular vision loss, with CSF positive for JCV DNA and MRI showing chiasmo-hypothalamic enhancement.
• Case 3: A lung transplant recipient developed progressive confusion and behavioral changes, with increasing JCV DNA in CSF but no MRI evidence of PML, ultimately leading to fatal encephalopathy.

Findings:

• JCV infection can manifest atypically, including chronic relapsing courses and unusual patterns of CNS involvement.
• Symmetrical visual pathway disease and non-localizing encephalopathy without MRI evidence of PML represent less common but significant presentations of JCV infection.
• These cases highlight the diagnostic challenges posed by JCV in immunocompromised individuals.

Implications:

• Heightened clinical suspicion for JCV is warranted in patients with unexplained neurological symptoms, especially those with altered immune status.
• Early recognition of diverse JCV presentations can guide appropriate diagnostic workup, including CSF analysis for JCV DNA.
• Management strategies may involve adjusting immunosuppression and considering specific antiviral or immunomodulatory therapies.