There are many types of autosomal dominant cerebellar ataxia (ADCA). Pathological changes involve the cerebellum and its afferent and efferent pathways. In addition to loss of cerebellar neurons, lesions of the spinal cord, pons, olive nucleus, basal ganglia, optic nerve, retina and peripheral nerves can also be seen. The clinical characteristics are simple cerebellar symptoms such as progressive truncal ataxia, dysarthria, dysmetria, intention tremor, etc., and involuntary movements, visual or auditory disorders, extraocular muscle paralysis, pyramidal tract signs, paresthesias, cranial nerve paralysis, etc. Common combinations of various clinical symptoms: simple cerebellar signs, cerebellar signs and brainstem signs, cerebellar and basal ganglia syndrome, spinal cord or peripheral neuropathy signs, cerebellar signs and special sensory (hearing, vision) disorders, cerebellar and pituitary dysfunction, cerebellar and myoclonus syndrome, cerebellar and pyramidal hypertonia, etc. In recent years, several types of mutations involving trinucleotide repeat expansion have been discovered, called dominant spinocerebellar ataxia (SCA), which is divided into more than 10 subtypes, with CAG trinucleotide repeat expansion being the most common.