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To illustrate the occurrence of progressive multifocal leukoencephalopathy in a patient with immune dysregulation despite normal T-cell counts.

Progressive multifocal leukoencephalopathy associated with immune dysregulation

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To illustrate the occurrence of progressive multifocal leukoencephalopathy in a patient with immune dysregulation despite normal T-cell counts.
Case report of a woman in her 40s with autoimmune hepatitis-primary biliary cholangitis overlap syndrome, receiving low-dose corticosteroids.
MRI was used to assess brain changes associated with PML.
Cerebrospinal fluid analysis was performed to detect JC viral DNA.
Immunophenotyping assessed B-cell and immunoglobulin levels.
MRI findings showed cerebellar symptoms consistent with PML.
JC viral DNA detected in cerebrospinal fluid confirmed viral infection.
Immunophenotyping revealed significant B-cell lymphopenia and raised free light chains, indicating immune dysfunction.

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Case report highlights PML development in patients with subtle immune issues despite T-cell preservation, indicating complex diagnosis.

Yusuf et al. (2026) studied this question.

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