Progressive multifocal leukoencephalopathy with gastrointestinal disease in a pediatric kidney transplant recipient

Area of Science:

• Neurology
• Infectious Diseases
• Transplantation

Background:

• Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system.
• PML is caused by John Cunningham virus (JCV) reactivation, particularly in immunocompromised individuals.
• While PML is documented in transplant recipients, symptomatic gastrointestinal involvement is rarely reported.

Observation:

• A nine-year-old kidney transplant recipient presented with severe gastrointestinal illness mimicking pseudo-obstruction.
• The patient was diagnosed with PML, with high JCV titers detected in cerebrospinal fluid and blood.
• Simian virus 40 (SV40) was positively stained in the colon, suggesting intestinal JCV infection.

Findings:

• This case represents the first reported instance of PML associated with symptomatic gastrointestinal pseudo-obstruction in a kidney transplant recipient.
• JCV was identified as the likely causative agent for both PML and the severe gastrointestinal symptoms.
• The presence of SV40 in the colon supports the hypothesis of JCV intestinal infection.

Implications:

• JCV intestinal infection should be considered in kidney transplant recipients presenting with unexplained gastrointestinal pseudo-obstruction.
• Early recognition and diagnosis of JCV-related gastrointestinal complications may improve patient outcomes.
• This finding expands the spectrum of clinical manifestations of JCV infection in immunocompromised hosts.