Abstract
Case Report 23-year-old dextral man was referred to the Neuroimmunology clinic following a subacute onset asymmetric central cerebellar syndrome over preceding 4 months. He was diagnosed with T-cell acute lymphoid leukemia in August 2022 after presenting with confusion and hyperviscosity syndrome. He underwent leukapheresis, standard induction and consilidation therapy. He had myeloablative chemotherapy followed by matched unrelated ASCT in March 2023.
Initial treatment for the CNS GvHD was with rituximab 700 mg intravenously weekly for a month, intrathecal hydrocortisone twice weekly, intravenous immunoglobulins induction followed by maintenance 4 weekly, intravenous methylprednisolone 1g two doses followed by oral prednisolone with a slow wean to 12.5 mg daily. He was also on cyclosporin 300mg BD and ruxolitinib.
He had pan-cerebellar signs with superimposed long tract signs on examination. A magnetic resonance imaging (MRI) of the brain with gadolinium demonstrated bilateral leukoencephalopathic process with tract involvement. An MRI spine was unremarkable. Serum anti-glutamic acid decarboxylase, voltage-gated potassium channel antibodies, anti-MOG antibodies and JCV serology were negative. Cerebrospinal fluid (CSF) examination was non-inflammatory, CSF viral PCR including EBV, JCV, herpes simplex and antineuronal antibodies were negative. The patient was diagnosed with probable multifocal CNS GvHD with concurrent cerebellitis. Ciclosporin was recommended to be increased to 6mg/kg and an option of mesenchymal stem cell therapy was discussed.
Discussion CNS GvHD following ASCT is a rare condition. The management of CNS GvHD is unclear but focuses on cytotoxic T-cell antagonism. The treatment usually involves steroids and in resistant cases cyclophosphamide, mesenchymal stem cells and JAK inhibitors.
