Leucoencefalopatía multifocal progresiva: recursos diagnósticos y evolución en una serie de casos

Abstract

Introduction

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the reactivation of the John Cunningham virus (JCV) in the central nervous system (CNS) in patients with several degrees of immunocompromised status. Its clinical and imaging manifestations are well described, but a high index of suspicion for the disease is necessary to arrive at a diagnosis.

Objectives

To provide a clinical description and complementary studies in patients with confirmed PML.

Patients and methods

A retrospective descriptive multicentre study of patients with confirmed PML, obtaining demographic, clinical, imaging, laboratory, and biopsy data from medical records.

Results

Twelve patients with a definitive diagnosis of PML: 10 with HIV/AIDS (human immunodeficiency virus/acquired immunodeficiency syndrome), 1 with non-Hodgkin lymphoma (NHL) and treatment with rituximab and 1 with HIV/AIDS, NHL and rituximab. In cranial MRI, all lesions appeared hyperintense on FLAIR and T2 and hypointense on T1, with 7 being bilateral, and 4 enhancing with EV contrast. The most affected sites were the frontal lobe, parietal lobe, and brainstem, correlating with the most frequent symptoms (motor disorders and incoordination). CSF showed no significant alterations. The two patients with survival greater than 1 year were those with negative JC PCR in CSF.

Conclusions

PML is a disease that requires high suspicion to be studied and diagnosed. We highlight the considerable involvement of both deep and cortical gray matter evidenced in this study. It is important to note the observation of increased survival in those with negative JC PCR in CSF.