Thelansis

  Spinocerebellar ataxia (SCA) is an extensive and heterogeneous group of autosomal dominant degenerative diseases characterized by progressive cerebellum degeneration and its afferent and efferent connections. The basal ganglia, brainstem nuclei, pyramidal tracts, posterior column, anterior horn, peripheral nerve, and other nervous system structures are typically also affected. SCAs are clinically characterized by the presence of cerebellar gait and limb ataxia (with dysmetria, dysdiado chokinesia, intention tremor, dysarthria, and nystagmus), which may be accompanied by extracerebellar signs such as ophthalmoplegia, pyramidal signs, movement disorders (including parkinsonism, dystonia, myoclonia, and chorea), dementia, epilepsy, visual disorders (including pigmentary retinopathy), lower motor neuron disease and peripheral neuropathy. SCA accounts for 3% to 5% of the ataxic diagnosed population, with an estimated prevalence of 1 to 5 cases in 100,000 people. Thelan...