A Case Report of Progressive Multifocal Leukoencephalopathy in Pediatrics: When and How to Think About it?

Case Report

Austin J Radiol. 2023; 10(2):1214.

A Case Report of Progressive Multifocal Leukoencephalopathy in Pediatrics: When and How to Think About it?

Yahya El Harras*; Kaouthar Sfar; Rachida Chehrastane; Nazik Allali; Latifa Chat; Siham El Haddad

Pediatric and Gynecology Radiology Department, Children’s Hospital, University MOHAMMED V, Rabat Morroco

*Corresponding author: Yahya El Harras Pediatric and Gynecology Radiology Department, Children’s Hospital, University Mohammed V, Rabat Morocco. Email: elharrasyahya@gmail.com

Received: April 25, 2023 Accepted: May 19, 2023 Published: May 26, 2023

Abstract

Progressive Multifocal Leukoencephalopathy (PML) is a life-threatening demyelinating brain disease, usually caused by reactivation of a rare opportunistic infection with JC virus. This pathology is strongly associated with immunosuppressed states, with primary PML developing in an immunocompetent patient is very rare. Imaging has an important role in orientating the diagnosis. We report the case of an 8 years old girl, who was on chemotherapy, and suffered neurologically. Her MRI showed PML lesions. Through this case, we provide a review of the literature about this rare pathology to help clinicians and radiologists evoke it.

Keywords: Multifocal; Leukoencephalopathy; PML; MRI; Demyelinating

Introduction

Progressive multifocal leukoencephalopathy (PML) is a very rare demyelinating disease of the central nervous system due to reactivation of the JC virus (John Cunningham). It targets oligodendrocytes in the context of immunodepression, especially HIV-infected patients. Diagnosis combines a bundle of clinical, radiological and biological arguments (positive PCR in the CSF) or histopathological findings. Imaging shows the involvement of subcortical white matter in early stages with no enhancement after Gadolinium. We report the case of an 8-year old girl, who was diagnosed with T-lymphoblastic lymphoma who was on intensive chemotherapy. She presented to our department for an MRI due to neurological symptoms. PML diagnosis was then evoked.

Case Report

Our patient, an 8 year-old-girl, had a family history of a brother who died from leukemia associated to brain cancer. She was diagnosed two months ago with a T-lymphoblastic lymphoma, and was on intensive chemotherapy. Her family noticed some incoherent and nonsensical speech at times with agitation and irritability. Her oncologist referred her for altered mental status to our radiology department for brain MRI. The latter showed T2 and FLAIR bilateral non symmetrical hyperintensities affecting sub cortical white matter in both the supra and infra tentorial areas (Figure 1). The lesions didn’t show a restriction in diffusion weighted images and no enhancement after Gadolinium administration was noted (Figure 2). We evoked PML lesions and referred her to the pediatric oncology department for urgent medical care. She underwent a lumbar puncture and the qPCR for JCV returned positive, which further strengthened our diagnosis. The infant was started on antiretroviral therapy but unfortunately died 4 months later.

Citation: El Harras Y, Sfar K, Chehrastane R, Allali N, Chat L, et al. A Case Report of Progressive Multifocal Leukoencephalopathy in Pediatrics: When and How to Think About it?. Austin J Radiol. 2023; 10(2):1214.