Monza D, Ciano C, Scaioli V, Soliveri P, Carella F, Avanzini G, Girotti F
Department of Neurology I, C. Besta National Neurological Institute, Via Celoria 11, I-20133 Milan, Italy.
Neurol Sci. 2003 Apr;24(1):16-23. doi: 10.1007/s100720300016.
We investigated the association between clinical and neurophysiological characteristics in patients with a clinical diagnosis of probable corticobasal degeneration (CBD), and searched for neurophysiological features supporting the diagnosis in life. Ten patients with clinically probable CBD underwent comprehensive neurological evaluation and brain MRI. Long latency reflexes (LLR), upper limb somatosensory (SEP) and motor evoked (MEP) potentials were recorded. The mini-mental state examination (MMSE), the phonemic verbal fluency test (PVFT) and the De Renzi ideomotor apraxia test were also performed. Polygraphic EEG was performed in the six patients with myoclonus. The SEP N30 frontal component was absent bilaterally in four patients, was absent on the left side in one, and had increased latency in other three. MEPs were abnormal in four patients (three had prolonged central motor conduction time, one of whom also had increased MEP threshold, and one had increased MEP threshold). All six patients with myoclonus had enhanced LLRs at rest, which were also of abnormally increased amplitude during motor activation; latencies were generally shorter than in classic cortical reflex myoclonus. On back-averaging, no EEG spikes time-locked to EMG activity were found in any myoclonus patient. Five patients were demented by MMSE, eight had ideomotor apraxia scores in the ideomotor apraxia range and five had defective verbal fluency. Brain MRI revealed asymmetric cortical atrophy in all patients, particularly evident frontoparietally. Neurophysiological techniques, particularly LLR, can assist CBD diagnosis especially in patients with myoclonus. Patients with evident parkinsonism had greater SEP N30 (frontal) abnormalities, while most patients with marked paresis had slower MEP times.
我们研究了临床诊断为可能的皮质基底节变性（CBD）患者的临床特征与神经生理特征之间的关联，并寻找支持生前诊断的神经生理特征。10例临床可能为CBD的患者接受了全面的神经学评估和脑部MRI检查。记录了长潜伏期反射（LLR）、上肢体感诱发电位（SEP）和运动诱发电位（MEP）。还进行了简易精神状态检查（MMSE）、语音流畅性测试（PVFT）和德雷尼观念运动性失用症测试。对6例有肌阵挛的患者进行了多导脑电图检查。4例患者双侧SEP N30额部成分缺失，1例左侧缺失，另3例潜伏期延长。4例患者MEP异常（3例中枢运动传导时间延长，其中1例MEP阈值也升高，1例MEP阈值升高）。所有6例有肌阵挛的患者静息时LLR增强，运动激活时振幅也异常增加；潜伏期一般比经典皮质反射性肌阵挛短。在反向平均时，任何肌阵挛患者均未发现与肌电图活动时间锁定的脑电图棘波。5例患者MMSE显示痴呆，8例观念运动性失用症评分在观念运动性失用症范围内，5例语言流畅性有缺陷。脑部MRI显示所有患者均有不对称皮质萎缩，以额顶叶尤为明显。神经生理技术，尤其是LLR，可辅助CBD诊断，特别是对有肌阵挛的患者。有明显帕金森综合征的患者SEP N30（额部）异常更明显，而大多数有明显轻瘫的患者MEP时间更慢。
