Abstract
An 80-year-old man with no history of an immune-compromising disorder was diagnosed with progressive multifocal leukoencephalopathy (PML). He presented with dysphagia and left-sided weakness; magnetic resonance imaging demonstrated marked signal abnormality in the subcortical white matter of the left frontal lobe and in the posterior limb of the right internal capsule. Polymerase chain reaction (PCR) analysis of the cerebrospinal fluid (CSF) was negative for John Cunningham (JC) virus. On brain biopsy, foamy macrophages infiltrating the white matter were identified, staining positive for anti-simian virus 40 antibodies. Postoperatively, PCR for JC viral DNA in the CSF was positive, establishing the diagnosis of PML. Extensive investigation for an occult immunocompromising disorder was negative. The patient's neurologic deficits rapidly increased throughout his hospital stay, and he died 3.5 months after his diagnosis.
Highlights
Progressive multifocal leukoencephalopathy (PML) is a rapidly advancing demyelinating disorder of the central nervous system almost exclusively encountered in immunocompromised individuals[1]
It is caused by reactivation of the John Cunningham virus (JCV) under conditions of cellular immunocompromise such as those encountered in patients with acquired immunodeficiency syndrome (AIDS), patients with hematologic and solid organ malignancies receiving chemotherapy, and transplant recipients under immunosuppression[2]
With the advent of the human immunodeficiency virus (HIV) epidemic, PML was recognized as a major opportunistic infection of AIDS,[8] but with effective antiretroviral therapy, its incidence and attributable mortality rates have decreased[9]
Summary
Progressive multifocal leukoencephalopathy (PML) is a rapidly advancing demyelinating disorder of the central nervous system almost exclusively encountered in immunocompromised individuals[1]. There was no history of renal insufficiency On his second hospital day, a magnetic resonance imaging (MRI) study of the brain without contrast demonstrated ill-defined white matter edema involving the right thalamus and left paramidline frontal lobe (sparing the cortex) and extending into the right cerebral peduncle. Testing for other infectious causes (toxoplasmosis, hepatitides, Cryptococcus, human T-lymphotropic virus I and II) was negative His persistent lymphopenia (absolute lymphocyte count throughout the hospital course ranged between 120 and 1077 cells/μl) raised the suspicion for a rheumatologic disease. Throughout the rest of his hospital stay, the patient’s neurologic condition continued to deteriorate His course was marked by an episode of altered mental status, progressive dementia, motor weakness, declining visual acuity, and worsening performance status. He was discharged to a skilled nursing facility closer to home, where he died 3.5 months after the diagnosis of PML
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