[Relationship between cognitive function and regional CBF in hereditary spinocerebellar degeneration--comparison between Joseph disease and OPCA of Menzel type].

Regional cerebral blood flow (rCBF) was measured using positron emission tomography (PET) in patients with Machado-Joseph disease (Joseph disease), hereditary OPCA of Menzel type (Menzel type), and in normal control subjects in order to investigate relationship between cognitive function and rCBF in 2 subgroups of hereditary spinocerebellar degeneration (SCD). Though absolute rCBF in the cerebellar hemisphere and pons was significantly low in each of 2 subgroups of SCD compared to the control group, no difference was seen between 2 subgroups of SCD in these regions. In addition, absolute rCBF in the cerebral cortices did not show significant difference among all groups. However, when the rCBF data was normalized to the occipital cortex for relative rCBF, in the patient group with Menzel type, relative rCBF in the thalamus was significantly low value compared to the control group and relative rCBF in the frontal, temporal, and parietal cortex, especially in the frontal, was significantly low value compared to the patient group with Joseph disease. Moreover, clinically cognitive impairments were seen in patients of Menzel type, but not in patients of Joseph disease. These findings suggest that the measurement of rCBF by PET is useful for the understanding of the pathophysiology of hereditary SCD subgroups as well as for the differential diagnosis of these diseases.