Atypical presentation of progressive multifocal leukoencephalopathy in a patient with ataxic syndrome and retrovirus with infra-tentorial involvement: a case report

Abstract

Introduction: Progressive Multifocal Leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by reactivation of the JC virus. It usually occurs in immunocompromised patients. The majority of PML cases present with supratentorial involvement. In contrast, infratentorial involvement is exceedingly rare in PML. The aim to report an atypical presentation of PML in a patient with ataxic syndrome and retrovirus with infra-tentorial involvement. This is a case report study. The information of this work was obtained through review of the medical record. Case report: A 38-year-old, female, HIV-infected since 2015 and started antiretroviral therapy only in 2018. In June 2019, she developed paresthesia in lower limbs, imbalance and difficulty walking, requiring support. Two months later she evolved with dysphagia, dysarthria, and limitation in activities of daily living. In September 2019, she presented for evaluation in a wheelchair. On neurological examination, she had bi-directional horizontal nystagmus, cervical dystonic tremor, global ataxia, altered deep sensation and spastic gait. Her metabolic profile was normal. Brain magnetic resonance imaging (MRI) showed atrophy of the cerebellar leaflets and vermis associated with extensive T2/FLAIR inversion recovery hyperintensities involving the cerebellum and bilateral middle cerebellar peduncle. Cerebrospinal fluid analysis revealed hyperproteinorraquia and posterior examination revealed positive JC virus DNA. Conclusion: In the diagnosis approach of acquired ataxias, it is essential to establish a propaedeutic directed to the patient’s clinical context. Investigating opportunistic infectious diseases is essential in care and treatment especially for immunosuppressed patients.