Dados do Trabalho

Título

Progressive Multifocal Leukoencephalopathy: a case report in a immunocompetent patient

RESUMO

Case presentation: A 23-years-old men, consanguineous parents, present vertigo and left visual cloudiness in November 2020 evolving in 07 days with paraesthesia and weakness in the right hand and dizziness. Pulse therapy was done for 05 days without improvement. He evolved with incoordination, severe dysarthria, right hemiplegia. CSF and serology were performed without changes; MRI of the skull that showed defects of semiovarian centers, bilateral periatrial, posterior horns of lateral skulls, splenium of the corpus callosum and cerebellar with hyposignal in T1 and hypersignal in T2/FLAIR in the white substance of the cerebral tissue.

Immunoglobulin was performed without improvement of the condition, with disease progression. Investigation of the immune system without alterations, positive JC virus, stereotactic biopsy and anatomopathological examination characterizing Progressive Multifocal Leukoencephalopathy (PML). Despite therapy, the patient passed away within 7 months.

Discussion: PML is a central demyelinating disease, related to JC virus reactivation, usually occuring in immunodepressed patients and less usual in immunocompetent patients. The physiopathology is based on the oligodendrocytes and astrocytes lesions of the subcortical hemispheric white matter and cerebellar peduncles, and may also affect a gray matter of the basal ganglia. The prognosis depends on variables such as the degree of contrast enhancement on neuroimaging, CD4 T lymphocyte count and neurological recovery.

Final considerations: In the case under analysis, there was a peculiarity of the pathology affecting a previously healthy individual, with no history of Acquired Immunodeficiency Syndrome or use of medications that could adjust his immunological competence, making the diagnosis even more challenging. Restoration of the adaptive immune system is the main objective given the fact that the disease still has no specific treatment.

Palavras Chave

Progressive Multifocal Leukoencephalopathy, PML, JC virus, immunocompetent