Central nervous system graft versus host disease causing intracranial vasculopathy

Abstract

Background: Graft-versus-host-disease (GVHD can complicate allogeneic bone marrow transplantation (BMT) and is due to a donor T-cell response that usually targets host skin, liver and gut. Reports of central nervous system (CNS) GVHD are very rare. Diagnosis of CNS-GVHD is challenging due to the wide range of potential neurological complications following BMT. We report an unusual case of CNS-GVHD causing intracranial vasculopathy with associated ischaemic and haemorrhagic changes. Method(s): Case report and literature review. Result(s): A 20 year-old male, on cyclosporin following allogeneic BMT 18 months previously for acute myeloid leukaemia (AML), presented with acute onset right hemiparesis. He previously had cutaneous and hepatic GVHD which had stabilised following manipulation of his immunotherapy. Magnetic resonance imaging (MRI) brain demonstrated acute infarction in the left basal ganglia and left periventricular white matter and chronic infarction in both basal ganglia. Magnetic resonance angiography (MRA) demonstrated irregularity of middle and anterior cerebral arteries suggestive of vasospasm or a vasculitic process. Cerebrospinal fluid (CSF) protein and leukocytes were not elevated and there was no evidence of bacterial, viral or fungal infection. CSF cytology was negative. Vasculitic and thrombophilia screen were negative. Repeat bone marrow aspiration revealed that his haematological malignancy had changed morphology. Despite maximising his immunotherapy, sequential brain imaging and blood films continued to show aggressive progression of his disease. His cutaneous and hepatic GVHD recurred and biopsies confirmed inflammatory vascular changes at both sites. Conclusion(s): The progressive course, sequential imaging findings and lack of evidence for alternative diagnoses is compatible with CNS-GVHD. It is important to consider CNS-GVHD in BMT patients presenting with neurological symptoms as it has significant treatment and prognostic implications.