First report of acute haemorrhagic leukoencephalitis, or Weston-Hurst syndrome, post-bone marrow transplant in a young person

Abstract

Objective: AHLE is thought to be an immune mediated process that can occur in a parainfectious context, often considered as a variant and fulminant form of ADEM with high mortality & morbidity. The few reports in the literature mostly describe adults with AHLE. We describe the first case of AHLE, or Weston-Hurst syndrome, post-bone marrow transplant (BMT) in a young person Method: This is a descriptive case report with serial MRI imaging to illustrate the very rare occurrence of AHLE in an 18-year old girl post-bone marrow transplant (BMT). Results: The patient received an unrelated transplant for HbE/beta thalassaemia and required immunosuppression postoperatively. Her recovery was marked by skin graft-versus-host disease, refractory thrombocytopenia & idiopathic pneumonia syndrome (IPS) which necessitated prolonged intensive care including high frequency oscillation. On day 25 post-transplant following a cytokine storm and whilst on PICU she had rhythmic jerking of her lower jaw accompanied by a fall in BP, with no concomitant EEG seizure activity. Brain MRI showed extensive symmetrical punctate microhemorrhages mostly in the deep white matter (WM) and WM changes in keeping with AHLE. A series of brain MRI scans as obtained over the following days-weeks will be illustrated. MRI spine was normal. Extensive investigations including vasculitic screen, cultures & PCRs were unremarkable. The young lady had received immunomodulation for IPS including toculizimab, etanercept and methylprednisolone. Following the diagnosis of AHLE she was aggressively treated with further steroids, IVIG, ongoing high dose acyclovir, broadspectrum antibiotics and antifungal treatment. In the immediate post-extubation period (D+29) she was markedly hypotonic and hypophonic. By D+37 she had recovered significantly, ambulating with support and communicating coherently. Conclusion: This report, which is the first to describe AHLE in a post-BMT context, adds to the sparse literature on this devastating condition and highlights the need for aggressive management.