Proton magnetic resonance spectroscopy and single photon emission CT in patients with olivopontocerebellar atrophy

Using proton magnetic resonance spectroscopy (1H-MRS) and single photon emission CT (SPECT), the cerebellum of patients with olivopontocerebellar atrophy (OPCA) and of age-matched control subjects was studied. A spectrum was collected from a 27 cm3 (3 x 3 x 3 cm) voxel in the cerebellum containing white and gray matters in order to measure the distribution and relative signal intensities of N-acetylaspartate (NAA), creatine (Cre) and choline (Cho). In the cerebellum of the patients with OPCA, mean NAA/Cre ratios for OPCA patients were significantly decreased compared with normal control subjects (OPCA, 1.01±0.247; controls, 1.526±0.144: p<0.001). Mean NAA/Cho ratios for OPCA patients were slightly decreased (OPCA, 1.285±0.228; controls 1.702±0.469: p<0.06). Cho/Cre ratios valued in the cerebellum of OPCA patients were not significantly different from those in normal controls (OPCA, 0.793±0.186; controls, 0.946±0.219). The ratio of RI count in the cerebellum to that in the occipital lobe was significantly decreased in OPCA patients (OPCA, 0.947±0.096; controls, 1.06±0.063: p<0.01). Cerebellar signs were assessed including gait ataxia, limb ataxia, dysarthria, saccadic pursuit, and nystagmus separately or in combination. In patients with more severe ataxic gait and dysarthria, MRS revealed slightly lowered NAA/Cre ratio. There was no significant correlation between NAA/Cre ratio and severity of other clinical signs. The MRS and SPECT findings give a confirmative evidence of hypofunction in cerebellum of patients with OPCA. (author)