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Título

Progressive Multifocal Leukoencephalopathy: An Atypical Presentation

RESUMO

Presentation: A 45-year-old woman, with no known comorbidities, presents to our service
with progressive onset of weakness in her right upper and lower limb. Concomitantly, she
developed tingling in her right hemiface and dysarthria. Neurological examination showed right
peripheral facial nerve palsy, soft right palatal palsy, in addition to complete and proportionate
hemiparesis on the right, with signs of ipsilateral pyramidal release. At investigation, anti-HIV
1/2 reagent serology was found. CT brain scan showed subcortical hypoattenuation in pre and
postcentral gyrus on the left. Brain MRI had evidence of hypointense signal on T1 and
hypersignal in T2-FLAIR at the topography of the pons and cerebellar peduncles, as well as
in the supratentorial topography mentioned above at head CT, involving subcortical U-fibers,
and without water diffusion restriction or contrast enhancement. The possibility of progressive
multifocal leukoencephalopathy (PML) was then raised, with confirmation after positive JC
Virus research in cerebrospinal fluid.

Discussion: PML is a progressive demyelinating
disease of the Central Nervous System (CNS) caused by the JC Virus in immunocompromised
patients. Among the conditions most associated with this disease, the best known is HIV
infection, in the AIDS phase, however cases of PML have already been described in other
contexts of immunodeficiency, such as cancer, organ transplantation, and autoimmune
diseases. Concerning the diagnosis, in addition to the clinical history, the patients present, on
head CT with asymmetric areas of hypoattenuation in white matter. On MRI, it
characteristically presents with hypointensity on T1 and hyperintensity on T2-FLAIR in white
matter, as well as the absence of water restriction diffusion and contrast enhancement,
typically in supratentorial topographies. The diagnosis is confirmed by the positivity of JC Virus
in cerebrospinal fluid of suspected patients.

Final comments: Our patient had an atypical
presentation of PML, with infratentorial lesions. It is necessary to consider this diagnosis in
the face of an adequate clinical context. Despite the atypical presentation, there are similar
cases described in the literature, particularly in the context of immunodeficiencies other than
those associated with HIV. We hope that this case report can help other colleagues in the
future who are faced with the same radiological images.

Palavras Chave

PML; Progressive Multifocal Leukoencephalopathy; HIV;