Department of Neurology, Division of Movement Disorders, Vanderbilt University Medical Center, A-0118 Medical Center North, Nashville, TN 37232, United States.
Department of Pediatrics, Division of Child Neurology, Vanderbilt Children's Hospital, United States.
Brain Dev. 2020 Oct;42(9):627-638. doi: 10.1016/j.braindev.2020.06.003. Epub 2020 Jun 26.
Tourette syndrome (TS) is a neurodevelopmental disorder defined by tics, but most patients also experience bothersome sensory phenomena, in the form of premonitory urges and/or sensory hypersensitivity. Whereas premonitory urges are temporally paired with tics, sensory hypersensitivity is a constant, heightened awareness of external and/or internal stimuli. The intensity of sensory hypersensitivity does not strongly correlate with the severity of tics or premonitory urges, suggesting it is a dissociable clinical phenomenon. At least 80% of TS patients report subjectively enhanced perception of various sensory stimuli. These same patients demonstrate normal static detection thresholds. However, individuals with TS habituate abnormally to repetitive stimuli, indicating incapacity to appropriately filter redundant sensory input, i.e. impaired sensory gating. Physiologic support for this hypothesis is provided by abnormal pre-pulse inhibition (PPI) and event-related potential (ERP) investigations. Preclinical data implicates parvalbumin-positive (PV+) interneuron dysfunction in altered sensory gating in TS and other neurodevelopment disorders. Studies probing TS sensory hypersensitivity must methodically account for comorbid psychiatric conditions, namely obsessive compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and autism spectrum disorder (ASD), as these entities appear to involve pathophysiologic processes shared with TS. The presence of psychiatric comorbidities in TS is associated with even more profound sensory processing dysfunction. A deepened understanding of TS sensory hypersensitivity will afford novel insights into disease mechanisms, clinical phenotype, and therapeutic management.
妥瑞氏症候群（TS）是一种神经发育障碍，其特征为抽搐，但大多数患者也会经历烦人的感觉现象，表现为预感冲动和/或感觉过敏。虽然预感冲动与抽搐在时间上相关联，但感觉过敏是对外界和/或内部刺激的持续、增强的感知。感觉过敏的强度与抽搐或预感冲动的严重程度没有很强的相关性，这表明它是一种可分离的临床现象。至少 80%的 TS 患者报告主观上增强了对各种感觉刺激的感知。这些患者同样表现出正常的静态检测阈值。然而，TS 患者对重复刺激的适应异常，表明他们无法适当地过滤冗余的感觉输入，即感觉门控受损。这一假设的生理学支持来自异常的前脉冲抑制（PPI）和事件相关电位（ERP）研究。临床前数据表明，PV+中间神经元功能障碍与 TS 和其他神经发育障碍中的感觉门控改变有关。探测 TS 感觉过敏的研究必须系统地考虑共病的精神疾病，即强迫症（OCD）、注意缺陷多动障碍（ADHD）和自闭症谱系障碍（ASD），因为这些实体似乎涉及与 TS 共享的病理生理过程。TS 中存在精神共病与更严重的感觉处理功能障碍有关。深入了解 TS 感觉过敏将为疾病机制、临床表型和治疗管理提供新的见解。
