Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Hosur Road, Bangalore, 560029, Karnataka, India; Clinical Neurosciences, National Institute of Mental Health & Neurosciences (NIMHANS), Hosur Road, Bangalore, 560029, Karnataka, India.
Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Hosur Road, Bangalore, 560029, Karnataka, India.
Parkinsonism Relat Disord. 2021 Apr;85:78-83. doi: 10.1016/j.parkreldis.2021.02.028. Epub 2021 Mar 13.
Cognitive impairment (CI) is reported but is poorly explored in spinocerebellar ataxia 2 (SCA2). This study was undertaken to evaluate and classify cognitive impairment in patients with SCA2 and to identify their grey matter (GM) correlates.
We evaluated the neurocognitive profile of 35 SCA2 and 30 age-, gender- and education-matched healthy controls using tests for attention, executive functions, learning and memory, language and fluency, and visuomotor constructive ability. Patients were classified into SCA2 with and without CI based on normative data from population and healthy controls. Furthermore, patients with CI were sub-classified based on the number of impaired domains into multi-domain CI (≥3 domains; MDCI) and limited domain CI (≤2 domains; LDCI). The underlying GM changes were identified using voxel based morphometry.
The mean age at onset, duration of disease, and ataxia score was 28.7 ± 8.51 years, 66.7 ± 44.1 months, and 16.1 ± 4.9 points, respectively. CI was present in 71.4% of SCA2 subjects (MDCI: 42.7%; LDCI: 28.5%). Patients with CI had significant atrophy of the posterior cerebellum, sensorimotor cortex, and superior frontal gyrus (FWE p-value <0.05). Patients with MDCI had significant GM atrophy of the angular gyrus compared to LDCI (FWE p-value <0.05).
Patients with CI had significant GM involvement of the posterior cerebellum and frontal lobe, suggestive of impairment in the cerebello-fronto-cortical circuitry.
认知障碍（CI）在脊髓小脑性共济失调 2 型（SCA2）中虽有报道，但研究甚少。本研究旨在评估和分类 SCA2 患者的认知障碍，并确定其灰质（GM）相关性。
我们使用注意力、执行功能、学习和记忆、语言和流畅性以及视动构造能力测试，评估了 35 例 SCA2 患者和 30 例年龄、性别和教育程度匹配的健康对照者的神经认知特征。根据人群和健康对照者的正常值，将患者分为 SCA2 伴 CI 和不伴 CI。此外，根据受损域的数量，将 CI 患者进一步分为多域 CI（≥3 个域；MDCI）和有限域 CI（≤2 个域；LDCI）。使用基于体素的形态计量学识别潜在的 GM 变化。
发病年龄、疾病持续时间和共济失调评分的平均值分别为 28.7±8.51 岁、66.7±44.1 个月和 16.1±4.9 分。71.4%的 SCA2 患者存在 CI（MDCI：42.7%；LDCI：28.5%）。CI 患者的后小脑、感觉运动皮层和额上回有明显萎缩（校正 FWE p 值<0.05）。与 LDCI 相比，MDCI 患者的角回 GM 萎缩更明显（校正 FWE p 值<0.05）。
CI 患者的后小脑和额叶 GM 明显受累，提示小脑-额-皮质回路受损。
