A 58 year old Caucasian woman developed a flu like syndrome, followed by eye movement disturbances three days later. On admission to our hospital, physical examination revealed disorientation, opsoclonus, myoclonus predominantly of the upper limbs, and severe limb and trunk ataxia. The deep tendon reflexes were normal and no paresis or sensory deficit could be found. Cranial CT and MRI scans and routine laboratory tests were normal. CSF examination revealed lymphocytic pleiocytosis, normal total protein, and positive oligoclonal bands. In serum and CSF no antineuronal antibodies but high titre AMA could be detected. High dose steroid therapy was started one week after onset of symptoms, and the patient experienced significant improvement of the OMS within one week of commencing steroid treatment. By four weeks the opsoclonus and myoclonus had disappeared, and there was only a mild trunk ataxia: the patient could walk unassisted.