German Center for Neurodegenerative Diseases, Bonn, Germany; Department of Neurology, University Hospital of Bonn, Bonn, Germany.
Mov Disord. 2013 Sep;28(10):1435-8. doi: 10.1002/mds.25512. Epub 2013 Jun 4.
Although it is well established that there is cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3), it is unknown whether cognition deteriorates with disease progression. We therefore prospectively studied cognitive function in patients with SCA3.
Eleven patients with SCA3 were assessed using an extensive neuropsychological test battery and retested after 3.5 ± 0.4 years.
In addition to ataxia and motor control, verbal learning and verbal and figural memory deteriorated significantly during the follow-up period. An increase in depressive symptoms was not observed.
The observation that memory and learning abilities deteriorated with disease progression suggests that cognitive dysfunction is an integral part of SCA3. Because the applied tests for memory function did not require motor responses, cognitive decline cannot be attributed to progressive cerebellar ataxia. The deterioration of verbal and figural memory can be explained either by extracerebellar pathology or by disruption of cerebellar-cerebral circuitries.
尽管脊髓小脑共济失调 3 型（SCA3）存在认知功能障碍已得到充分证实，但尚不清楚认知功能是否会随疾病进展而恶化。因此，我们前瞻性研究了 SCA3 患者的认知功能。
对 11 例 SCA3 患者进行了广泛的神经心理学测试，并在 3.5±0.4 年后进行了复测。
除了共济失调和运动控制外，在随访期间，患者的语言学习能力以及言语和图像记忆能力显著下降。未观察到抑郁症状的增加。
记忆和学习能力随疾病进展而恶化的观察结果表明，认知功能障碍是 SCA3 的一个固有组成部分。由于用于记忆功能的测试不需要运动反应，因此认知能力下降不能归因于进行性小脑共济失调。言语和图像记忆的恶化可能是由于小脑-大脑回路的破坏或小脑外病理学引起的。
