Abstract
The clinical significance of CT was investigated in group of 25 patients with non-progressive ataxic syndromes. The age of the patients ranged from 3 to 19 (11 males and 14 females). All patients were evaluated from the points of the neurological findings and their motor developmental history, and also examined radiologically with CT.
Six view points about the hemisphere and vermis of the cerebellum in CT were graded from 0 through 3; 0-normal, 1-mild, 2-moderate, 3-severe. The degree of cerebellar atrophy was expressed as the total score the score of cerebellar atrophy, SCA11 cases whose total scores were above seven had distinct abnormalities in the vermis, and markedly retarded in their motor development. Although the cases without any abnormal findings in the vermis were below seven in SCA, three cases in the group had severely retarded milestones (24 months were needed for them to sit up and crawl). No close correlation was found between SCA and the motor developmental retardation.
Thirteen cases, over 5 years, were classified into dysequilibrium syndrome (DES) and congentital cerebellar ataxia (CCA) according to Hagberg's criteria. Six cases who had abnormalities in the vermis were diagnosed as DES, while seven cases who had no abnormalities in the vermis consisted of four cases of DES and three cases of CCA. Therefore, in the cases of DES diagnosed on the clinical findings, there were two different types DES with or without abnormalities in the vermis. There were no differences of IQ and motor development between these two types.
In summary, CT allowed the diagnosis of DES with abnormal findings in the vermis, but when the vermis has no abnormalities it may be difficult to distinguish DES from CCA. There was no correlation between SCA and motor developmental retardation.
