We present the case of a 51-year-old man with a 5-year history of slowly progressive
gait ataxia and
dysarthria who showed a wide-based gait requiring assistance. The patient's score on the Revised Hasegawa
Dementia Scale (HDS-R) was 22/30 and constructional
apraxia was also evident. Cerebrospinal fluid analysis showed 3 cells/microl, and the
protein concentration was 58 mg/dl. Brain MRI showed no evidence of cerebellar
atrophy, and SPECT-eZIS showed no decrease in cerebellar blood flow. However, voxel based morphometry (VBM) and FineSRT revealed cortical cerebellar
atrophy and reduced cerebellar blood flow. In addition, the patient tested positive for anti-
gliadin (
IgA) and anti-SS-A/
Ro antibodies, and was thus diagnosed as having autoimmune
cerebellar ataxia. The patient showed positive response to
intravenous immunoglobulins (
IVIg) and regained the ability to walk unassisted. The HDS-R score also improved to 27/30. If cortical cerebellar
atrophy can be diagnosed in the early stages in patients with progressive
cerebellar ataxia by imaging techniques such as MRI-VBM and FineSRT, and if such patients test positive for anti-
gliadin, anti-GAD or anti-thyroid
antibodies, it is possible that they have autoimmune
cerebellar ataxia. The commencement of
immunotherapy including
IVIg should be considered in such