ArticleCerebellum (London, England)2017
Non-motor and Extracerebellar Features in Spinocerebellar Ataxia Type 2.
Article in Cerebellum (London, England), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 37 citations in OpenAlex.
- Unveiling the role of cerebellar alterations in the autonomic nervous system: a systematic review of autonomic dysfunction in spinocerebellar ataxias.Journal of neurology · 2023Pooled it
- From early psychiatric symptoms to an adolescent diagnosis: Clues from childhood in spinocerebellar ataxia Type-42.Dusunen adam : Bakirkoy Ruh ve Sinir Hastaliklari Hastanesi yayin organi · 2026Article
- Neuroscience of human ventral lateral thalamic nucleus related to movement and movement disorders.Journal of neurophysiology · 2025Review
- Respiratory Evaluation in Spinocerebellar ataxia Type 2.Cerebellum (London, England) · 2025Article
- A comprehensive review of iPS cell line-based disease modelling of the polyglutamine spinocerebellar ataxias 2 and 3: a focus on the research outcomes.Annals of medicine and surgery (2012) · 2024Review
- Cognitive, Emotional, and Other Non-motor Symptoms of Spinocerebellar Ataxias.Current neurology and neuroscience reports · 2024Review
- Disability in cerebellar ataxia syndromes is linked to cortical degeneration.Journal of neurology · 2023Article
- Excessive Daytime Sleepiness in Parkinson's Disease is Related to Functional Abnormalities in the Left Angular Gyrus.Clinical neuroradiology · 2023Article
- Role of cerebellum in sleep-dependent memory processes.Frontiers in systems neuroscience · 2023Review
- A Systematic Review of the Spectrum and Prevalence of Non-Motor Symptoms in Adults with Hereditary Cerebellar Ataxias.Movement disorders clinical practice · 2022Review
- Cognitive impairment and its neuroimaging correlates in spinocerebellar ataxia 2.Parkinsonism & related disorders · 2021Article
- Lower urinary tract and bowel dysfunction in spinocerebellar ataxias.Annals of clinical and translational neurology · 2021Article
- Urinary Symptoms and Urodynamic Findings in Patients with Spinocerebellar Ataxia.Cerebellum (London, England) · 2020Article
- Neuronal Activity in the Cerebellum During the Sleep-Wakefulness Transition in Mice.Neuroscience bulletin · 2020Article
- Spinocerebellar ataxia type 2 presenting with involuntary movement: a diagnostic dilemma.The Journal of international medical research · 2019Article
- Sleep Disorders in Hereditary Ataxias.Current neurology and neuroscience reports · 2019Review
- Spinocerebellar ataxia type 31 associated with REM sleep behavior disorder: a case report.BMC neurology · 2019Article
- SCA2 presenting as a focal dystonia.Journal of clinical movement disorders · 2018Article
- NESSCA Validation and Responsiveness of Several Rating Scales in Spinocerebellar Ataxia Type 2.Cerebellum (London, England) · 2017Article
- Muscle Cramps Profile among Spinocerebellar Ataxias.Annals of Indian Academy of NeurologyArticle
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Authors and funding
12 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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 syndrome. Dysphagia was present in 39.39 % (N = 13), weight loss 24.24 % (N = 8), and urinary dysfunction 27.27 % (N = 9). Cramps was present in only 6 % of patients (N = 2). This study highlighted the high frequency of non-motor symptoms and extra-cerebellar signs in SCA2. Our findings demonstrate the widespread of nervous system involvement in SCA2 patients and contribute to better understand the natural history of brain degeneration in this genetic condition.
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