Computerized tomography in hereditary ataxias

Abstract

Patients [39] with hereditary ataxia (HA), hereditary spastic paraplegia or Charcot-Marie-Tooth disease were investigated with computerized cranial tomography (CT). Infratentorial as well as supratentorial atrophies were registered and scored. These were compared with the patient''s neurological symptoms, which were related to the lesion in the CNS and scored. There was correlation between the distribution of brainstem/cerebellar symptoms and the distribution and degree of infratentorial atrophy. HA cases with cerebral cortical atrophy had significantly higher dementia scores than those without wide sulci. I.v. injection with contrast medium was of no diagnostic aid, as no focal changes were found. CT is an aid in the diagnosis of hereditary ataxias and hereditary spastic paraplegia.