European Neurology · 2018 · 20 citations · 23 references
Life SciencesNeurologyNeuroscienceClinical NeuroscienceNeurogeneticsNeuropsychiatryDepressive DisordersSpinocerebellar AtaxiasAtaxia Severity ScalesAtaxia SyndromesDisease Severity EstimationHereditary AtaxiasMultivariable Prediction ModelsMultivariable Regression AnalysisSpinocerebellar Ataxia Type 3
BACKGROUND: Spinocerebellar ataxia type 3 (SCA3), which is the most common subtype of SCA worldwide, exhibits common neuropsychological symptoms such as depression. However, the contribution of depression to the severity of SCA3 has not yet been thoroughly investigated. METHODS: The present study investigated the prevalence of depression using Beck depression inventory in 104 molecularly confirmed SCA3 patients from China. The putative risk factors for depression and whether the depression could affect the severity of ataxia were established by multivariable linear regression models. RESULTS: The frequency of depression in the study subjects was 57.69% (60/104), which was higher than that in SCA3 patients from a subset of other populations. The gender (p = 0.03) and severity (p < 0.01) of ataxia were those risk factors that could affect depression. Conversely, depression (p < 0.01) together with the duration (p < 0.01) of SCA3 could also play a positive role in the severity of ataxia. CONCLUSIONS: The extremely common depression results from motor disability caused by ataxia; it also affects the disease severity of SCA3. These findings suggested that depression was a part of neurodegeneration in SCA3 and necessitated intensive focus and interventions while caring for SCA3 patients.
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The cerebellar cognitive affective syndrome
Jeremy D. Schmahmann · Brain · 1998 · 3.2K citations · Full text
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1
Yoshiya Kawaguchi, Toshihiro Okamoto, Masafumi Taniwaki et al. · Nature Genetics · 1994 · 1.8K citations