Central and peripheral nervous system involvement as a manifestation of graft versus host disease after liver transplantation: case report

The incidence of graft versus host disease (GVHD) after liver trasplantation (LT) has been estimated at 0.1–2%, but the mortality rate is 80–100%. Clinical series show that nervous system (NS) complications following liver trasplantation (LT) occur in 8 to 47% cases and include diffuse encephalopathy, cerebrovascular disorder, infection, immunosuppression-induced neurotoxicity, and peripheral nerve damage; however the involvement of the central and peripheral NS as manifestation of GVHD after LT has not been demonstrated conclusively. Here we report a 57 years old male patient who received a liver transplant for HBV related-epatocirrosis. On post operative day (POD) 20 he developed a pattern of asymmetric severe painful dysestesias of upper limbs. A week after he presented a stepwise progressive weakness of upper limbs followed days later by numbness and profound weakness in left distal peroneal distribution. Elettrofisiological findings showed a pattern of axonal sensori-motor multiplex mononeuropathy. High titers of anti-neutrophil cytoplasmic antibodies (ANCA) were detected in his serum. Cerebrospinal fluid examination and serological tests were performed to exclude differential diagnosis such as drug-induced toxicities or opportunistic infections. High dose intravenous metilprednisolone resulted in remission of painful symptomatology and improved overall neurological examination. His subsequent clinical course was complicated by acute disartria ad emiparesis on POD 246 due to ischaemic stroke detected with diffusion-weighted MRI. We propose that multineuropathy and ischaemic stroke occured in this patient as major manifestation of recurrent acute GVHD, suggesting that peripheral and central NS can be targets of a vasculitic immunomediated disorder in GVHD.