Non-motor and Extracerebellar Features in Spinocerebellar Ataxia Type 2

The Cerebellum
José Luiz PedrosoOrlando Graziani Povoas Barsottini

Abstract

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant degenerative disease. Pathological studies have demonstrated not only cerebellar and brainstem atrophy, but substantia nigra, motoneurons, basal ganglia, thalamus, and peripheral nerves involvement. These findings may explain non-motor and extra-cerebellar features in SCA2. We accessed the non-motor symptoms and extra-cerebellar signs in SCA2 patients in order to provide a better understanding on pathophysiological mechanisms and natural history of brain degeneration in the disease. Thirty-three SCA2 patients were evaluated and compared with 26 healthy subjects. We investigated the following variables: sleep disorders, cognitive deficit, olfactory impairment, urinary dysfunction, psychiatric symptoms, cramps, pain, movement disorders, and weight loss. SCA2 had a high frequency of REM sleep behavior disorder (48.48 %, N = 16) as well as excessive daytime sleepiness (42.42 %, N = 14). Chorea was present in 15.15 % (N = 5), dystonia in 27.27 % (N = 9), and parkinsonism in 27.27 % (N = 9). Slow saccadic pursuit was present in 87.87 % (N = 29) and ophtalmoparesis in 78.78 % (N = 26) of patients. Regarding sleep disorders, 18.18 % (N = 6) of patients had restless leg syndr...Continue Reading

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Citations

Mar 4, 2017·Movement Disorders : Official Journal of the Movement Disorder Society·Luis Velázquez-PérezJosé Miguel Laffita-Mesa
Jul 26, 2019·Current Neurology and Neuroscience Reports·Lucio HuebraJosé Luiz Pedroso
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Mar 24, 2021·Parkinsonism & Related Disorders·Albert StezinPramod Kumar Pal
Aug 24, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Vrutangkumar V ShahChristopher M Gomez

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Software Mentioned

Statistical Package for the Social Science

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