Kulisevsky J, Litvan I, Berthier M L, Pascual-Sedano B, Paulsen J S, Cummings J L
Movement Disorders Unit, Department of Neurology, Sant Pau Hospital, Autonomous University of Barcelona, Spain.
Mov Disord. 2001 Nov;16(6):1098-104. doi: 10.1002/mds.1225.
The role of the basal ganglia in conditions with co-occurring movement disorders and neuropsychiatric symptoms is not well known. It has been hypothesized that hyperkinesia -disinhibited behaviors and hypokinesia-inhibited behaviors result from an imbalance between the direct and indirect striatal output pathways, and that differential involvement of these pathways could account for the concurrent abnormalities in movement and behavior observed in these disorders. This study aimed to evaluate whether the pattern and the extent of the neuropsychiatric manifestations of patients with GTS, a hyperkinetic movement disorder of basal ganglia origin, differs from that of patients with other basal ganglia hyperkinetic (e.g., HD) or hypokinetic (e.g., PSP) movement disorders, and to determine whether patients with GTS show a greater frequency of hyperactive behaviors (e.g., agitation, irritability, euphoria, or anxiety) than PSP patients, and are comparable to patients with HD. The Neuropsychiatric Inventory (NPI), a scale with established validity and reliability, was administered to 26 patients with GTS (mean age, 30.2 +/- 2.2 years), and the results were compared with that of 29 patients with HD (mean age, 43.8 +/- 2 years) and 34 with PSP (mean +/- S.D. age, 66.6 +/- 1.2 years). There was no difference between the groups in the total NPI scores. However, there was a double dissociation in behaviors: patients with hyperkinetic disorders (HD and GTS) exhibited significantly more agitation, irritability, anxiety, euphoria, and hyperkinesia, whereas hypokinetic patients (PSP) exhibited more apathy. Patients with GTS showed greater scores than HD patients in all those scores differentiating HD and GTS from PSP patients (e.g., agitation, irritability, anxiety and euphoria), and were differentiated in a logistic regression analysis from both HD and PSP patients in having significantly more anxiety. We found that patients with GTS manifested predominantly hyperactive behaviors similar but more pronounced than those presented by patients with HD, while those with PSP manifested hypoactive behaviors. Based on our findings and the proposed models of basal ganglia dysfunction in these disorders, we suggest that the hyperactive behaviors in GTS are comparable to those observed in HD, being both secondary to an excitatory subcortical output through the medial and orbitofrontal cortical circuits, while in PSP the hypoactive behaviors are secondary to hypostimulation of these circuits. Abnormalities of other brain structures (e.g., amygdala, brainstem nuclei) may account for the significantly higher anxiety scores differentiating GTS from HD patients.
基底神经节在同时出现运动障碍和神经精神症状的疾病中的作用尚不清楚。有假说认为，运动增多（去抑制行为）和运动减少（抑制行为）是由纹状体直接和间接输出通路之间的失衡导致的，并且这些通路的不同参与情况可以解释这些疾病中观察到的运动和行为的并发异常。本研究旨在评估抽动秽语综合征（一种起源于基底神经节的运动增多性疾病）患者的神经精神表现模式和程度是否与其他基底神经节运动增多性（如亨廷顿舞蹈症）或运动减少性（如进行性核上性麻痹）运动障碍患者不同，并确定抽动秽语综合征患者是否比进行性核上性麻痹患者表现出更高频率的多动行为（如烦躁、易怒、欣快或焦虑），以及是否与亨廷顿舞蹈症患者相当。对26例抽动秽语综合征患者（平均年龄30.2±2.2岁）使用具有既定效度和信度的神经精神科问卷（NPI）进行评估，并将结果与29例亨廷顿舞蹈症患者（平均年龄43.8±2岁）和34例进行性核上性麻痹患者（平均年龄±标准差66.6±1.2岁）的结果进行比较。各组NPI总分无差异。然而，在行为方面存在双重分离：运动增多性疾病（亨廷顿舞蹈症和抽动秽语综合征）患者表现出明显更多的烦躁、易怒、焦虑、欣快和多动，而运动减少性患者（进行性核上性麻痹）表现出更多的冷漠。在所有区分亨廷顿舞蹈症和抽动秽语综合征与进行性核上性麻痹患者的评分中（如烦躁、易怒、焦虑和欣快），抽动秽语综合征患者的得分高于亨廷顿舞蹈症患者，并且在逻辑回归分析中，抽动秽语综合征患者因明显更多的焦虑而与亨廷顿舞蹈症和进行性核上性麻痹患者区分开来。我们发现，抽动秽语综合征患者主要表现出多动行为，与亨廷顿舞蹈症患者相似但更明显，而进行性核上性麻痹患者表现出少动行为。基于我们的研究结果以及这些疾病中提出的基底神经节功能障碍模型，我们认为抽动秽语综合征中的多动行为与亨廷顿舞蹈症中观察到的行为相当，两者均继发于通过内侧和眶额皮质回路的兴奋性皮质下输出，而在进行性核上性麻痹中，少动行为继发于这些回路的低刺激。其他脑结构（如杏仁核、脑干核）的异常可能解释了区分抽动秽语综合征与亨廷顿舞蹈症患者的明显更高的焦虑评分。
