CLINICAL, BIOCHEMICAL, AND PHYSIOLOGICAL FEATURES DISTINGUISHING MYOCLONUS RESPONSIVE TO 5-HYDROXYTRYPTOPHAN, TRYPTOPHAN WITH A MONOAMINE-OXIDASE INHIBITOR, AND CLONAZEPAM
CLINICAL, BIOCHEMICAL, AND PHYSIOLOGICAL FEATURES DISTINGUISHING MYOCLONUS RESPONSIVE TO 5-HYDROXYTRYPTOPHAN, TRYPTOPHAN WITH A MONOAMINE-OXIDASE INHIBITOR, AND CLONAZEPAM
复制标题
DOI：
10.1093/brain/100.3.455
复制
发表时间：
1977-01-01
期刊：
影响因子：
14.5
通讯作者：
MARSDEN, CD
中科院分区：
文献类型：
--
作者：
CHADWICK, D;HALLETT, M;MARSDEN, CD
对15例各种肌阵挛综合征患者进行了临床、影像学和生理学研究。还测量了CSF色氨酸、5HIAA和HVA。在这些患者中，8例通过5HTP、色氨酸联合MAOI（但不是单独色氨酸）和氯硝西泮治疗得到不同程度的改善。本组包括6例缺氧后肌阵挛，1例创伤后肌阵挛和1例未确诊的非进行性局灶性肌阵挛和癫痫。在该组中，与无反应病例和对照组相比，发现CSF 5HIAA水平较低。2例小脑肌阵挛性协同失调，2例病因不明，2例原发性肌阵挛，1例腭肌阵挛对药物治疗无效。然而，即使在响应组中，改善情况也各不相同。最戏剧性的反应被认为是在那些患者的生理研究表明，肌阵挛是由脑干结构介导的。在肌阵挛似乎起源于皮质结构的患者中，观察到的反应不那么明显。讨论了肌阵挛对5-HT前体和氯硝西泮反应的神经化学基础。有人建议，这样的肌阵挛产生的5HT神经元系统，其结果在释放的感觉刺激，其特征在于这种类型的肌阵挛的异常反应的相对低活性。
Fifteen patients with a variety of myoclonic syndromes were studied clinically, pharmacologically, and physiologically. CSF tryptophan, 5HIAA, and HVA were also measured. Of these patients, 8 were improved to varying degrees by therapy with 5HTP, tryptophan in combination with MAOI (but not tryptophan alone), and clonazepam. This group included 6 cases of post-anoxic myoclonus, one case of post-traumatic myoclonus and one undiagnosed case of non-progressive focal myoclonus and epilepsy. In this group low levels of CSF 5HIAA were found compared to non-responsive cases and controls. Two cases of dysynergia cerebellaris myoclonica, 2 cases of undiagnosed aetiology, 2 cases of essential myoclonus, and one case of palatal myoclonus failed to respond to drug therapy. However, even amongst the responsive group the improvement varied. The most dramatic responses were seen in those patients in whom physiological study suggested that myoclonus was mediated by brain-stem structures. Less dramatic responses were seen in patients in whom the myoclonus appeared to originate from cortical structures. The neurochemical basis of myoclonus responding to 5HT precursors and clonazepam is discussed. It is suggested that such myoclonus arises from a relative hypoactivity of the 5HT neuronal system which results in a release of abnormal responses to sensory stimuli which characterize this type of myoclonus.