Yasuoka T, Ikeda M, Maki N, Hokoishi K, Komori K, Tanabe H
Department of Neuropsychiatry, Ehime University School of Medicine, Shigenobu-cho, Onsen-gun, Ehime 791-0295, Japan.
No To Shinkei. 2001 Aug;53(8):781-5.
Corticobasal degeneration(CBD) is a neurodegenerative disorder characterised clinically by apraxia, cortical sensory loss, alien limb, dementia, oculomotor abnormalities, dysarthria, postural instability, akinesia, rigidity, and pyramidal signs. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. We reported a case of corticobasal degeneration of which praxic impairments were improved by administration of amantadine. The patient was a 63-year-old right-handed woman. She showed marked dysfunction including rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. A brain MRI scan revealed bilateral cortical atrophy centered in the postcentral gyrus, more pronounced in the left hemisphere than the right. A SPECT scan showed a decrease in blood flow in the temporo-parieto-occipital regions, more pronounced in the left hemisphere than the right. An EEG showed a diffuse slowness. L-dopa had no effect on the symptoms of rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. By administration of amantadine, rigidity and cortical sensory loss did not improve, but some praxic impairments, such as dressing apraxia and ideomotor apraxia, and the EEG improved. Upon withdrawal of amantadine, the improved symptoms deteriorated. Amitriptyline did not improve the deteriorated symptoms. After amantadine was re-administered, the same praxic impairments and the EEG improved again. This suggested that administration of amantadine had some effect on certain praxic impairments and the EEG.
皮质基底节变性(CBD)是一种神经退行性疾病,临床特征为失用症、皮质感觉丧失、异己肢体、痴呆、眼球运动异常、构音障碍、姿势不稳、运动不能、强直和锥体束征。脑成像可能显示病变较重一侧对侧的异常更为明显。我们报告了一例皮质基底节变性患者,给予金刚烷胺后失用性损害得到改善。患者为一名63岁右利手女性。她表现出明显的功能障碍,包括强直、肢体运动性失用、皮质感觉丧失、观念运动性失用和穿衣失用。脑部MRI扫描显示双侧皮质萎缩,以中央后回为中心,左侧半球比右侧更明显。SPECT扫描显示颞顶枕区血流减少,左侧半球比右侧更明显。脑电图显示弥漫性减慢。左旋多巴对强直、肢体运动性失用、皮质感觉丧失、观念运动性失用和穿衣失用症状无效。给予金刚烷胺后,强直和皮质感觉丧失没有改善,但一些失用性损害,如穿衣失用和观念运动性失用以及脑电图有所改善。停用金刚烷胺后,改善的症状恶化。阿米替林未能改善恶化的症状。再次给予金刚烷胺后,相同的失用性损害和脑电图再次改善。这表明给予金刚烷胺对某些失用性损害和脑电图有一定作用。