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The development of ataxia is a neurologic sign that may provide a clue to the nature of the underlying disorder. Interruption of afferent and efferent connections within the spinocerebellar system results in ataxic gait, scanning dysarthria, explosive speech, intention tremor, dysdiadochokinesia, dysmetria, and abnormalities of eye movements. Many variations are encountered in the clinical phenotype, ranging from findings of pure cerebellar dysfunction to mixed patterns of involvement reflecting extrapyramidal, brainstem, and cerebral cortical involvement. Spinocerebellar Ataxia represents a group of hereditary ataxia of varying inherited degrees of rarity, which is in contrast to a related group of neurological disorders that are acquired following traumatic injuries or other external agents. Until recently, all autosomal dominant ataxias were called Marie Ataxia and all autosomal recessive ataxias were called Friedreich Ataxia. They may be present at almost any time between infancy and adulthood. (Table S-2)

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Table S-2 Spinocerebellar Ataxia: An Overview of Autosomal Dominant Types

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