Spongiform encephalopathy following allogeneic cord blood transplant

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Hematopoietic stem cell transplantation (HSCT) is a life-saving procedure.
• Complications following HSCT can be diverse and challenging to diagnose.
• Progressive encephalopathy is a rare but severe post-transplant complication.

Observation:

• A 6-year-old boy experienced rapid, fatal encephalopathy 5 months post-unrelated cord blood transplant.
• Autopsy revealed characteristic spongiform changes in the brain.
• Clinical presentation mimicked transmissible spongiform encephalopathy (TSE).

Findings:

• Etiology remained undetermined despite pre-mortem and post-mortem investigations.
• Spongiform encephalopathies encompass both TSEs and mitochondrial disorders.
• Differential diagnosis is crucial in post-HSCT progressive encephalopathy.

Implications:

• This case highlights the importance of considering rare neurological disorders post-HSCT.
• Broadening the differential diagnosis for encephalopathy after transplantation is critical.
• Further research into post-transplant neurological complications is warranted.