Opsoclonus myoclonus following allogeneic haematopoietic stem cell transplantation

Abstract

A 30-year-old woman with relapsed acute myeloid leukaemia (AML) underwent allogeneic haematopoietic stem cell transplantation (HSCT) in second complete remission, from a male matched unrelated donor in August 2004. There was a single mismatch at the DQB1 allele. The patient was conditioned with total body irradiation 14.4 Gy in six fractions, cyclophosphamide 120 mg/kg and CAMPATH 1H, with short-course methotrexate and cyclosporin A for graft-versus-host disease prophylaxis. She was readmitted at day 60 post transplant complaining of a week history of vague abdominal pain and diarrhoea, as well as feeling 'shaky'. On examination, she had a coarse jerking tremor, ataxia, dysdiadochokinesis and a marked inability to follow movement with her eyes. Abdominal examination was normal. The cyclosporin level was acceptable at 122 nmol/l, and brief discontinuation made no improvement in her symptoms. CT and MRI brain were normal, as was CSF taken at lumbar puncture. There was marked further deterioration in both speech and tremor, and the empirical use of foscarnet to cover the possibility of HHV 6 infection was also of no benefit. Bone marrow examination showed her to still be in morphological remission, although chimerism was 83% donor. A neurology opinion was sought, and a clinical diagnosis of opsoclonus myoclonus (OM) was made. Anti-Hu antibody was tested and was negative. A slow spontaneous recovery in symptoms occurred over the next 4 weeks and the patient was transferred to a neurological rehabilitation unit. Unfortunately, she suffered a frank haematological relapse of her AML a month later and ultimately died of her disease.