Cerebellar-onset Progressive Multifocal Leukoencephalopathy (PML) Associated with Hepatitis B-related Liver Cirrhosis

Area of Science:

• Neurology
• Infectious Diseases
• Hepatology

Background:

• Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system.
• It is typically associated with severe immunosuppression, often seen in conditions like HIV/AIDS or during treatments with immunomodulatory drugs.
• Hepatitis B-related liver cirrhosis can lead to a state of relative immunodeficiency, increasing susceptibility to opportunistic infections.

Purpose of the Study:

• To report a case of PML in a patient with hepatitis B-related liver cirrhosis.
• To emphasize the diagnostic challenges and key imaging findings suggestive of PML in this context.
• To highlight the potential efficacy of mefloquine and mirtazapine in treating PML.

Main Methods:

• Case report of a woman in her 70s with hepatitis B-related liver cirrhosis presenting with cerebellar symptoms.
• Initial diagnostic workup included magnetic resonance imaging (MRI) of the brain.
• Diagnosis was confirmed through re-evaluation of brain biopsy and JC virus (JCV) polymerase chain reaction (PCR) analysis of cerebrospinal fluid (CSF).

Main Results:

• The patient presented with ataxia and dysarthria, suggestive of cerebellar involvement.
• Initial MRI revealed an asymmetric cerebellar white matter lesion, but PML diagnosis was delayed.
• Cerebrospinal fluid (CSF) analysis confirmed JC virus (JCV) infection.
• Treatment with mefloquine and mirtazapine led to viral clearance from CSF and clinical stabilization.

Conclusions:

• Crescent-shaped cerebellar lesions on MRI should raise suspicion for PML, even in patients without overt severe immunodeficiency.
• Hepatitis B-related liver cirrhosis can be an underlying condition predisposing to PML.
• Prompt diagnosis and treatment, including antiviral therapy, are essential for managing PML and improving patient outcomes.