Abstract
A 65 year-old male patient had been treated for Late cortical cerebellar atrophy (LCCA) for 7 years. His neuro-otological findings and a review of the literature on LCCA are presented in this paper.
In 1985, this patient was found to have down-beat nystagmus and gaze nystagmus. Electronystagmography (ENG) showed a very abnormal pattern. His eye tracking test (ETT) showed saccadic pursuit, no optokinetic nystagmus and no visual suppression. The neuro-otological findings suggested a cerebellar lesion, although he had no neurological abnormality.
In 1986, trunkal ataxia and speech disturbance appeared. MRI showed atrophy of the vermis. These findings led us to the clinical diagnosis of LCCA.
It was interesting that the neuro-otological findings preceded the neurological signs. Probably the oculomotor system and the equilibrium system of this patient with LCCA are degenerating separately.
