首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Concomitant alpha-synuclein pathology in an autopsy case of amyotrophic lateral sclerosis presenting with orthostatic hypotension and cardiac arrests.
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Concomitant alpha-synuclein pathology in an autopsy case of amyotrophic lateral sclerosis presenting with orthostatic hypotension and cardiac arrests.

机译:在肌萎缩性侧索硬化的尸检病例中伴有α-突触核蛋白病理,表现为体位性低血压和心脏骤停。

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A 74-year-old man gradually developed muscular weakness in the upper extremities, followed by dyspnea and dysarthria over a 6-month period. He was admitted to our facility and diagnosed as having amyotrophic lateral sclerosis (ALS) based on clinical and neurophysiological findings. Two months later, transtracheal positive pressure ventilation (TPPV) was started. During his clinical course, orthostatic hypotension occurred a few times. He also had two episodes of transient cardiac arrest, and he died 15 months after disease onset. At autopsy, the brain, weighing 850?g, showed diffuse cortical atrophy, preferentially involving the frontal lobes. Microscopic findings included severe loss of neurons in the motor cortex, the motor nuclei of the brainstem and the anterior horns of the spinal cord, and mild loss of axons and myelin in the corticospinal tract. Trans-activation response DNA protein 43 (TDP-43) immunoreactive cytoplasmic inclusions, the pathognomonic findings for ALS, were noted in the nucleus facialis, nucleus ambiguus, and in the anterior horn of the spinal cord. In addition, Lewy bodies and Lewy neurites were found in the brainstem and in the nucleus intermediolateralis of the thoracic cord. The concomitant alpha-synuclein pathology may have been partly related to possible autonomic dysfunction underlying the two episodes of cardiac arrest.
机译:一名74岁的男子在6个月内逐渐出现上肢肌肉无力,随后出现呼吸困难和构音障碍。根据临床和神经生理学发现,他被送进了我们的医院,并被诊断出患有肌萎缩性侧索硬化症(ALS)。两个月后,开始经气管正压通气(TPPV)。在他的临床过程中,体位性低血压发生过几次。他还出现了两次短暂性心脏骤停,并在疾病发作后15个月死亡。尸检时,重850微克的大脑显示出弥漫性皮质萎缩,优先累及额叶。显微镜下的发现包括运动皮层,脑干和脊髓前角神经元的严重丧失,以及皮质脊髓束中轴突和髓鞘的轻度丧失。反式激活DNA蛋白43(TDP-43)免疫反应性胞质内含物,是ALS的病理学发现,见于面神经核,歧核和脊髓前角。此外,在脑干和胸中叶外侧核中发现了路易体和路易神经突。伴随的α-突触核蛋白病理可能与心脏骤停的两次发作潜在的自主神经功能障碍有关。

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