Progressive multifocal leukoencephalopathy in the absence of immunosuppression

Benjamin E Zucker,Sybil R L Stacpoole

doi:10.1007/s13365-017-0592-2

Abstract

A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely.

Highlights

Progressive multifocal leukoencephalopathy (PML) is potentially fatal demyelinating encephalopathy of gradual onset caused by the John-Cunningham virus (JCV)
progressive multifocal leukoencephalopathy (PML) is relatively commonly seen in immunosuppressed patients, it has, very infrequently, been reported in
There are two different types of JCV: the archetypal form which is transmitted between people in childhood and resides in the kidney and the neurotropic type which evolves from the archetype and causes PML

Summary

Introduction

Progressive multifocal leukoencephalopathy (PML) is potentially fatal demyelinating encephalopathy of gradual onset caused by the John-Cunningham virus (JCV). Human immunodeficiency virus (HIV) infection is the most typical context in which JCV reactivation, and subsequent development of PML, occurs. Another more recent but relatively common situation in which JCV reactivation and consequent development of PML can occur, is as a result of treatment of multiple sclerosis (MS) with Natalizumab. A 69-year-old right-handed retired head teacher presented with fatigue and left hand weakness of insidious onset over 6 weeks Her past medical history was unremarkable apart from hypertension and mild COPD. She developed a dysarthria 7 months after initial presentation and died shortly after that

Discussion

Findings

Compliance with ethical standards