Litvan I, Cummings J L, Mega M
National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-9130, USA.
J Neurol Neurosurg Psychiatry. 1998 Nov;65(5):717-21. doi: 10.1136/jnnp.65.5.717.
To characterise the neuropsychiatric symptoms of patients with corticobasal degeneration (CBD).
The neuropsychiatric inventory (NPI), a tool with established validity and reliability, was administered to 15 patients with CBD (mean (SEM), age 67.9 (2) years); 34 patients with progressive supranuclear palsy (PSP) (66.6 (1.2) years); and 25 controls (70 (0.8) years), matched for age and education. Both patient groups had similar duration of symptoms and mini mental state examination scores. Semantic fluency and motor impairment were also assessed.
Patients with CBD exhibited depression (73%), apathy (40%), irritability (20%), and agitation (20%) but less often had anxiety, disinhibition, delusions, or aberrant motor behaviour (for example, pacing). The depression and irritability of patients with CBD were more frequent and severe than those of patients with PSP. Conversely, patients with PSP exhibited significantly more apathy than patients with CBD. The presence of high depression and irritability and low apathy scale scores correctly differentiated the patients with CBD 88% of the time. The irritability of patients with CBD was significantly associated with disinhibition (r=0.85) and apathy (r=0.72). In CBD, apathy was associated with disinhibition (r=0.67); disinhibition was associated with aberrant motor behaviour (r=0.68) and apathy (r=0.67); and aberrant motor behaviour with delusions (r=1.0). On the other hand, depression was not associated with any other behaviour, suggesting that it has a different pathophysiological mechanism. Symptom duration was associated with total motor scores (r=0.69). However, total motor score was not associated with any behaviour or cognitive scores.
The findings indicate that frontosubcortical pathways mediating cognition, emotion, and motor function in CBD are not affected in parallel. Patients with CBD and PSP have overlapping neuropsychiatric manifestations, but they express distinctive symptom profiles. Evaluating the behavioural abnormalities of parkinsonian patients may help clarify the role of the basal ganglia in behaviour.
描述皮质基底节变性（CBD）患者的神经精神症状。
采用具有既定效度和信度的神经精神科问卷（NPI），对15例CBD患者（平均（标准误）年龄67.9（2）岁）、34例进行性核上性麻痹（PSP）患者（66.6（1.2）岁）和25名年龄及受教育程度相匹配的对照者（70（0.8）岁）进行评估。两组患者的症状持续时间和简易精神状态检查得分相似。同时评估语义流畅性和运动功能障碍。
CBD患者表现出抑郁（73%）、淡漠（40%）、易怒（20%）和激越（20%），但较少出现焦虑、脱抑制、妄想或异常运动行为（如踱步）。CBD患者的抑郁和易怒比PSP患者更频繁、更严重。相反，PSP患者的淡漠明显多于CBD患者。抑郁和易怒程度高以及淡漠量表得分低能在88%的情况下正确区分CBD患者。CBD患者的易怒与脱抑制（r = 0.85）和淡漠（r = 0.72）显著相关。在CBD中，淡漠与脱抑制相关（r = 0.67）；脱抑制与异常运动行为相关（r = 0.68）和淡漠相关（r = 0.67）；异常运动行为与妄想相关（r = 1.0）。另一方面，抑郁与任何其他行为均无关联，提示其具有不同的病理生理机制。症状持续时间与总运动评分相关（r = 0.69）。然而，总运动评分与任何行为或认知评分均无关联。
研究结果表明，CBD中介导认知、情感和运动功能的额颞叶皮质下通路并非同时受到影响。CBD患者和PSP患者存在重叠的神经精神表现，但他们表现出独特的症状特征。评估帕金森病患者的行为异常可能有助于阐明基底神经节在行为中的作用。
