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Progressive Vestibulocerebellar Syndromes 

Progressive Vestibulocerebellar Syndromes
Progressive Vestibulocerebellar Syndromes

Tracey D. Graves

and Joanna C. Jen

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date: 30 July 2021

Vestibulocerebellar syndromes which lead to dizziness and imbalance are heterogeneous in their clinical features and underlying aetiology. There is ongoing effort to improve the diagnosis, unravel the pathomechanisms, and develop treatment for vestibulocerebellar disorders. Acute ataxia generally results from damage to the cerebellum from a broad range of acquired causes, including infection, neoplasm, inflammation, and vascular injury. Vestibulocerebellar syndromes of insidious onset and gradual progression are most commonly caused by idiopathic or hereditary neurodegeneration involving the cerebellum. Symptoms of oscillopsia or diplopia and ataxia may be transient at first then become progressive with time. In this chapter, we discuss the evaluation and diagnosis of treatable causes of progressive vestibulocerebellar syndromes. We also review recent advances in the elucidation of genetic causes of a growing number of autosomal dominant, autosomal recessive, as well as X-linked disorders presenting with abnormal eye movement control and imbalance, with an emphasis on the episodic ataxias. The chapter concludes with current treatment options and ongoing research. A concerted effort between patients and researchers will likely facilitate new gene discovery to improve the diagnosis and understanding of idiopathic disorders. Those with a firm genetic diagnosis may take part in clinical trials, as researchers continue to develop new tools and approaches to measure treatment outcome and patient satisfaction.

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