Date: 12 Sep 2013

Spinocerebellar Ataxia Type 10: From Amerindians to Latin Americans

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Introduction

Autosomal dominant cerebellar ataxias, currently denominated spinocerebellar ataxias (SCAs), constitute a large, complex group of heterogeneous autosomal dominant degenerative diseases characterized by progressive degeneration of the cerebellum and its afferent and efferent connections [1, 2]. Other nervous system structures are usually affected, including the basal ganglia, brainstem nuclei, pyramidal tracts, and posterior column and anterior horn of the spinal cord, as well as the peripheral nerves. SCAs are clinically characterized by the presence of cerebellar gait, limb ataxia (with dysmetria,dysdiadochokinesia, intention tremor), scanning dysarthria, dysphagia, nystagmus, and ocular motility abnormalities, which may be accompanied by extracerebellar signs such as ophthalmoplegia, pyramidal signs, movement disorders (including parkinsonism, dystonia, myoclonus, and chorea), dementia, epilepsy, visual disorders (including pigmentary retinopathy), lower motor neuron dise