Litvan I, Paulsen J S, Mega M S, Cummings J L
Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892-9130, USA.
Arch Neurol. 1998 Oct;55(10):1313-9. doi: 10.1001/archneur.55.10.1313.
The role of the basal ganglia in neuropsychiatric behaviors is not well known. Anatomical, neurophysiological, and neurochemical evidence supports the notion of parallel direct and indirect basal ganglia thalamocortical motor systems, the differential involvement of which accounts for the hypokinesia or hyperkinesia observed in basal ganglia disorders.
To evaluate the neuropsychiatric manifestations of patients with a hyperkinetic movement disorder, such as Huntington disease (HD), vs a hypokinetic disease, such as progressive supranuclear palsy (PSP). To verify if patients with HD show a greater frequency of hyperactive behaviors (eg, agitation, irritation, euphoria, or anxiety), while those with PSP exhibit hypoactive behaviors (eg, apathy).
The Neuropsychiatric Inventory, a tool with established validity and reliability, was administered to 29 patients with HD (mean +/- SD age, 43.8 +/- 2 years) and 34 with PSP (mean +/- SD age, 66.6 +/- 1.2 years), matched for education, symptom duration, and overall degree of dementia.
There was no difference between the groups in the total Neuropsychiatric Inventory scores. However, there was a double dissociation in behaviors: patients with HD exhibited significantly more agitation (45%), irritability (38%), and anxiety (34%), whereas patients with PSP exhibited more apathy (82%) (P < .01). Euphoria was present only in patients with HD.
We found that patients with HD manifested predominantly hyperactive behaviors, while those with PSP manifested hypoactive behaviors. Based on our findings and the anatomical lesions known to occur in these disorders, we suggest that the hyperactive behaviors in HD are secondary to an excitatory subcortical output through the medial and orbitofrontal cortical circuits, while in PSP the hypoactive behaviors are secondary to hypostimulation.
基底神经节在神经精神行为中的作用尚不明确。解剖学、神经生理学和神经化学证据支持平行的直接和间接基底神经节丘脑皮质运动系统的概念，两者的不同参与情况解释了在基底神经节疾病中观察到的运动减少或运动增多。
评估运动增多性运动障碍患者（如亨廷顿病[HD]）与运动减少性疾病患者（如进行性核上性麻痹[PSP]）的神经精神表现。验证HD患者是否表现出更高频率的多动行为（如激动、易怒、欣快或焦虑），而PSP患者是否表现出少动行为（如冷漠）。
使用效度和信度已得到确立的工具神经精神量表，对29例HD患者（平均±标准差年龄，43.8±2岁）和34例PSP患者（平均±标准差年龄，66.6±1.2岁）进行评估，这些患者在教育程度、症状持续时间和痴呆总体程度方面相匹配。
两组的神经精神量表总分无差异。然而，在行为方面存在双重分离：HD患者表现出明显更多的激动（45%）、易怒（38%）和焦虑（34%），而PSP患者表现出更多的冷漠（82%）（P<0.01）。欣快仅出现在HD患者中。
我们发现HD患者主要表现为多动行为，而PSP患者表现为少动行为。基于我们的发现以及已知在这些疾病中发生的解剖学病变，我们认为HD中的多动行为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PSP中少动行为继发于刺激不足。
