[Computer tomography of the head in the diagnosis of various forms of hereditary familial ataxia]

Abstract

Clinical examinations of 34 patients wit hereditary ataxies was carried out using computerized tomography. Potentialities of the computerized tomography for specifying the diagnosis in patients with Friedreich's ataxy (12 observations) and cerebellar degenerations (22 observations) were determined. The examinations of the 12 patients with Friedreich's hereditary ataxy performed by the computerized tomography method revealed signs of the cerebellar atrophy only in one patient. In 21 out of the 22 patients with various forms of the cerebellar ataxy, the tomographic examinations revealed symptoms of atrophy of the cerebellum and the brain stem both in the initial stages of the disease and in the stages characterized with gross coordination disturbances. The more pronounced the atactic disturbances, the greater the degree of the cerebellum atrophy, this increase being statistically valid (p less than 0.001), when assessed from the number of the hemispheric grooves). On the other hand, no relationship between the disease duration and the degree of the cerebellum atrophy symptoms was revealed by the computerized tomography method. In family cases of the cerebellar degeneration, the symptoms of the cerebellum atrophy were disclosed by the computerized tomography in all the family members examined, however, the degree of their manifestation was different. The data of the computerized tomography can be used for diagnostic differentiation between Friedreich's disease and cerebellar ataxies--this is of great importance for medico-genetic consulting.