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Patients with adult onset non-familial progressive ataxia are classified in sporadic ataxia group. There are several disease categories that may manifest with sporadic ataxia: toxic causes, immune-mediated ataxias, vitamin deficiency, infectious diseases, degenerative disorders and even genetic conditions. Considering heterogeneity in the clinical spectrum of sporadic ataxias, the correct diagnosis remains a clinical challenge. In this review, the different disease categories that lead to sporadic ataxia with adult onset are discussed with special emphasis on their clinical and neuroimaging features, and diagnostic criteria. The term ataxia traditionally means loss of order, and clinically denotes loss of balance and coordination.
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Overview of Adult Onset Cerebellar Ataxia
Ataxia - Symptoms and causes - Mayo Clinic
NCBI Bookshelf. The hereditary ataxias are a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. In this GeneReview the hereditary ataxias are categorized by mode of inheritance and gene or chromosome locus in which pathogenic variants occur.
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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Sporadic adult-onset ataxia of unknown etiology describes a group of non-hereditary degenerative ataxias characterized by a slowly progressive cerebellar syndrome with ataxia of stance and gait, upper limb dysmetria and intention tremor, ataxic speech, and oculomotor abnormalities , presenting in adulthood at around 50 years of age , that is not due to a known cause.
The ataxias are clinically heterogenous disorders caused by pathological processes affecting the cerebellum and cerebellar pathways resulting in impaired coordination. Lesions of the cerebellum and its connections can result in breakdown and incoordination of movement. The pathophysiology of cerebellar ataxias is as diverse as the various neurological and systemic diseases affecting the cerebellum. Broadly classifying ataxias into genetic and non-genetic conditions is a first step in discovering their underlying mechanism.