Zacharia Sophie J, Sokratous Giannis, Samuel Mike, Costello Angela, Ashkan Keyoumars, Shotbolt Paul
Psychology, King's College Hospital, London, GBR.
Neurosurgery, King's College Hospital, London, GBR.
Cureus. 2018 Oct 29;10(10):e3507. doi: 10.7759/cureus.3507.
Cognitive deficits and psychiatric morbidities are commonly detected in dystonia. Psychiatric disturbances are of particular clinical concern as they not only contribute to poor quality of life and disease associated burden, but also exacerbate motor and cognitive symptoms. Bilateral deep brain stimulation of the globus pallidus internus improves motor symptoms in treatment-resistant dystonia, but its implications for non-motor manifestations are poorly understood. Improved prediction of cognitive and neuropsychiatric outcomes is important in deep brain stimulation (DBS) research and we aim to assess the latter through established assessment tools. We document the cognitive and neuropsychiatric profiles in 11 primary and 10 secondary dystonia patients attending our DBS clinic. We performed routine multidisciplinary assessments including a comprehensive battery of neuropsychometric tests and detailed neuropsychiatric evaluations. Post-operative assessment outcomes are reported for three patients in case series. The main cognitive deficit was on the Brixton test of spatial anticipation in primary dystonia. Background medical history included psychiatric illness in 38.1% of the patients with 76% of patients having mood abnormalities confirming elevated psychiatric morbidity in this population. Depressive illness was more prominent in primary, whereas clinically relevant histories in secondary dystonia were varied. Of the 21 patients three were able to perform on selected tests due to extensive limitations of their dystonia. No obvious alteration in intellectual functioning following DBS surgery relative to performance at the time of initial assessment was observed. The frequency of individual impairments suggests that difficulties associated with dystonia are likely to be of clinical relevance to cognitive functions in the majority of patients. In particular, current findings suggest that executive difficulties related to inductive processes and spatial learning may be a common in primary dystonias. Psychiatric disturbances demand recognition as a central aspect of dystonia as they contribute to overall disease burden, poor quality of life and exacerbated motor disabilities. The available evidence provides overwhelming suggestion that vulnerability to depression is inherent to the dystonia phenotype.
肌张力障碍患者常伴有认知缺陷和精神疾病。精神障碍尤其值得临床关注,因为它们不仅会导致生活质量下降和疾病相关负担,还会加重运动和认知症状。双侧苍白球内侧核深部脑刺激可改善难治性肌张力障碍的运动症状,但其对非运动表现的影响尚不清楚。在深部脑刺激(DBS)研究中,更好地预测认知和神经精神结果很重要,我们旨在通过既定的评估工具来评估后者。我们记录了在我们的DBS诊所就诊的11例原发性和10例继发性肌张力障碍患者的认知和神经精神特征。我们进行了常规的多学科评估,包括一系列全面的神经心理测试和详细的神经精神评估。在病例系列中报告了3例患者的术后评估结果。原发性肌张力障碍的主要认知缺陷在于布里克斯顿空间预期测试。背景病史包括38.1%的患者患有精神疾病,76%的患者有情绪异常,证实该人群的精神疾病发病率较高。抑郁症在原发性肌张力障碍中更为突出,而继发性肌张力障碍的临床相关病史则各不相同。在这21例患者中,3例由于肌张力障碍的广泛限制而无法进行某些测试。与初次评估时的表现相比,未观察到DBS手术后智力功能有明显改变。个体损伤的频率表明,与肌张力障碍相关的困难可能在大多数患者中与认知功能具有临床相关性。特别是,目前的研究结果表明,与归纳过程和空间学习相关的执行困难在原发性肌张力障碍中可能很常见。精神障碍需要被视为肌张力障碍的一个核心方面,因为它们会导致整体疾病负担、生活质量下降和运动残疾加剧。现有证据提供了压倒性的提示,即抑郁症易感性是肌张力障碍表型所固有的。