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[运动神经元病合并帕金森病——病例报告]

[Motor neuron disease with Parkinson's disease--case report].

作者信息

Nishinaka T, Kuroda S, Hayashi Y, Fujisawa Y

机构信息

Department of Neurology, Okayama National Hospital.

出版信息

Rinsho Shinkeigaku. 1990 Nov;30(11):1252-5.

PMID:2085932
Abstract

The patient was a 64-year-old woman who showed muscle weakness and tremor of upper extremities and gait disturbance at the age of 62 years. The symptoms progressed and she was admitted to our hospital. Neurological examination revealed muscle weakness, muscle atrophy and fasciculation bilaterally in the upper extremities. The deep tendon reflexes were reduced in the upper extremities and increased in the lower extremities, but Babinski's sign was not present. There was mild hand tremor at rest (right greater than left). Muscle rigidity was also evident. Her gait was small-stepped and her trunk was bent forward. She showed hypomimia, but no dementia was detected. She died of respiratory failure 7 months after admission. The duration of the illness was about 2 years. At autopsy, macroscopic examination showed depigmentation of the substantia nigra and locus ceruleus, and atrophy of the anterior roots of the spinal cord. Microscopic examination revealed a few senile plaques in the temporal cortex. In the substantia nigra, the number of melanin-containing cells was decreased in its central parts. A few Lewy bodies were found in some of the remaining neurons, and melanin pigment migrated into the parenchyma. In the locus ceruleus and dorsal motor nucleus of vagus, abundant Lewy bodies and mild astrocytosis were seen. A few Lewy bodies were also seen in the nucleus raphe, nucleus basalis of Meynert and hypothalamic nuclei. Severe neuronal loss of the anterior horn cells was observed in the cervical segment, and to a lesser degree, in the lumbo-sacral segments. segments.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

患者为一名64岁女性,62岁时出现上肢肌肉无力、震颤及步态障碍。症状进展,遂入住我院。神经系统检查发现双侧上肢肌肉无力、肌肉萎缩及肌束震颤。上肢腱反射减弱,下肢腱反射增强,但未引出巴宾斯基征。静息时可见轻度手部震颤(右侧大于左侧)。肌肉强直也很明显。她步态小步,躯干前倾。面部表情减少,但未发现痴呆。入院7个月后死于呼吸衰竭。病程约2年。尸检时,大体检查显示黑质和蓝斑色素脱失,脊髓前根萎缩。显微镜检查发现颞叶皮质有少量老年斑。在黑质,其中心部位含黑色素细胞数量减少。在一些残留神经元中发现少量路易小体,黑色素色素迁移至实质内。在蓝斑和迷走神经背运动核,可见大量路易小体和轻度星形细胞增生。在中缝核、Meynert基底核和下丘脑核中也可见少量路易小体。在颈段观察到前角细胞严重神经元丢失,腰骶段程度较轻。

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