Neurological recovery after liver transplantation in a severely disabled adolescent with Wilson disease: a 1-year follow-up case report.

BACKGROUND

Neurological Wilson disease with severe motor, cognitive, and functional impairment remains a challenging and controversial indication for liver transplantation because neurological injury is often considered irreversible. The Unified Wilson Disease Rating Scale (UWDRS) is used to quantify disease severity and provides an objective framework for monitoring neurological outcomes.

CASE PRESENTATION: A 15-year-old adolescent with a 5-year history of Wilson disease presented with rapid neurological deterioration, profound malnutrition, tense ascites, hepatic decompensation, and severe neurological dysfunction including immobility, loss of speech, contractures, sialorrhea, dysphagia, and encephalopathy. The total UWDRS score at presentation was 162. Liver transplantation was performed as a life-saving procedure. The postoperative course was complicated by aspiration pneumonia requiring reintubation and prolonged enteral feeding. Continuous neuropsychosocial rehabilitation was implemented in the intensive care unit, the transplant ward, and during outpatient follow-up.

OUTCOME: At 1 year, the patient demonstrated marked recovery. UWDRS scores improved to 59, speech returned, cognitive engagement normalized, contractures decreased, and she regained the ability to walk with support and perform basic daily activities with assistance and returned to school.

CONCLUSION

This case provides evidence that significant neurological improvement is possible in adolescents with advanced neuro-Wilson disease following timely liver transplantation. Restoration of copper metabolism combined with structured rehabilitation may lead to substantial functional recovery even in patients with severe preoperative neurological compromise. This case supports the view that severe neurological impairment should not be considered an absolute contraindication to liver transplantation in carefully selected patients.