Cerebellar Involvement in an Immunocompetent Patient Presenting with Progressive Multifocal Leukoencephalopathy

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus, a polyomavirus that can be reactivated under certain immunosuppressive conditions, such as AIDS, immunomodulatory therapy, and haematological malignancies. However, a few cases of immunocompetent patients have been reported in which no immunodeficiency was present. We describe the case of an 83-year-old immunocompetent man who presented with severe cerebellar symptoms with an MRI scan suggestive of severe demyelinating disease. We were not able to identify any occult immunosuppression or malignancy in our patient.

Isolated cerebellar progressive multifocal leukoencephalopathy after allogeneic bone marrow transplantation: focus on imaging

“…3,7 Isolated posterior fossa involvement is uncommon. 2,[4][5][6][7][8][9] There is little or no mass effect or enhancement except in immune reconstitution inflammatory syndrome. 3 Among other possible differential diagnoses in an immunocompromised hematologic patient with an isolated cerebellar lesion, we considered ischemic, infectious, inflammatory, and oncologic diseases.…”

“…11 Despite commonly occurring with subcortical white matter lesions, PML is a pleomorphic disease with described histologic and radiologic variants, including perivascular inflammation with punctate enhancement, mass effect, and supratentorial and infratentorial location. [1][2][3]8,11 Besides early suspicion by clinical and radiologic features, JC virus detection by PCR or by immunohistochemistry in CNS samples remain the most sensitive methods for PML diagnosis. 11 Treatment options for PML are limited.…”

“…1,3 Isolated posterior fossa involvement is rarely described. 2,[4][5][6][7][8][9] Here we illustrate a case of relapsed acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplant (HSCT) and isolated cerebellar PML with par-ticular focus on its computed tomography (CT) and magnetic resonance imaging (MRI) features.…”

Slowly progressive fatal PML-IRIS following antiretroviral initiation at CD4+ nadir of 350 cells/mm3 despite CD4+ cell count rise to 900 cells/mm3

“…Zucker et al 10 69/F HTN and COPD JC virus in CSF Fatal Garcia-Carretero et al 11 83/M Atrial fibrillation JC virus in CSF Fatal Christakis et al 12 69/M DM Type 2 Brain biopsy Patient stable van der Kolk et al 5 49/M Unremarkable Brain biopsy Fatal Grewal et al 13 55/M COPD, chronic alcoholism, and hypothyroidism Brain biopsy Fatal Gourineni et al 14 62/F Unremarkable Brain biopsy Fatal Tan et al 15 62/M Thoracic shingles, CAD and HTN Brain biopsy Patient stable Naess et al 16 35/M Unremarkable Brain biopsy Patient stable Di Giambenedetto et al 17 37/M HIV-1 infection Brain biopsy Fatal Delobel et al 18 47/M HIV-1 infection Brain biopsy Fatal HTN: hypertension, DM: diabetes mellitus, COPD: chronic obstructive pulmonary disorder; CAD: coronary artery disease, JC Virus: John Cunningham virus, HIV: human immunodeficiency virus.…”

Progressive multifocal leukoencephalopathy in an immunocompetent patient: A case report and review of literature

“…PML rarely presents in patients without obvious factors of immunosuppression. We reviewed the literature and found over 21 cases of PML in immunocompetent patients with no underlying immunosuppressive condition 3–18 . Here, we report a case of PML in a patient without known factors of suppressed immunity, with recent exposure to COVID‐19 before the onset of symptoms.…”