SHM Converge Scientific Abstract ePoster Gallery-Altered Mental Status to Progressive Multifocal Leukoencephalopathy: An Unexpected Diagnosis

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This document describes progressive multifocal leukoencephalopathy (PML), a rare and often fatal demyelinating disease of the central nervous system caused by reactivation of JC polyomavirus. PML commonly occurs in people with profound cellular immunosuppression, including those with hematologic malignancies, organ transplants, or immunomodulatory therapy. Because symptoms are nonspecific and presentation depends on lesion location, diagnosis is often delayed. Typical signs include subacute neurologic deficits such as altered mental status, weakness, and ataxia. In immunosuppressed patients, PML should be suspected when brain MRI shows white matter lesions, usually without mass effect.<br /><br />The case presented involves a 62-year-old woman with Crohn’s disease and rheumatoid arthritis who was taking vedolizumab and prednisone. She came to the hospital with altered mental status and initially had leukocytosis and acute kidney injury. After empiric antibiotics and fluids, her lab abnormalities improved, but her cognition worsened. An extensive workup, including infectious, autoimmune, EEG, and lumbar puncture studies, was largely negative except for elevated CMV PCR. She was treated with valganciclovir, but her condition continued to decline. Repeat MRI showed white matter abnormalities in the left frontal and occipital lobes. Eventually, cerebrospinal fluid PCR was positive for JC virus, confirming PML. Steroid taper and supportive measures were started, but she did not improve, later developed septic shock, and died.<br /><br />The key conclusions are that PML is a devastating disease with no definitive cure, survival depends on the patient’s ability to mount a cellular immune response, and median survival in patients without HIV or natalizumab exposure is about three months. Diagnosis requires a combination of clinical, imaging, and virologic evidence, and a negative JC virus PCR does not exclude the disease. Management focuses on reducing immunosuppression and, in HIV-related cases, optimizing antiretroviral therapy.

Asset Subtitle

Philicia Duncan

Meta Tag

Author List Evelyn Goodyear, Philicia Duncan

Category Clinical Vignettes

Concept Progressive Multifocal Leukoencephalopathy

Concept Cellular Immunosuppression

Concept JC Virus

Concept Subacute Neurologic Deficits

Concept Magnetic Resonance Imaging White Matter Lesions

Distinguished Non-Finalist

Presenter Organization The Ohio State University Wexner Medical Center

Presenting Author Philicia Duncan

Track Adult

Keywords

progressive multifocal leukoencephalopathy

PML

JC polyomavirus

immunosuppression

white matter lesions

altered mental status

MRI brain

cerebrospinal fluid PCR

rheumatoid arthritis

vedolizumab

Progressive Multifocal Leukoencephalopathy

Cellular Immunosuppression

JC Virus

Subacute Neurologic Deficits

Magnetic Resonance Imaging White Matter Lesions