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[γ球蛋白病中的临床肌萎缩侧索硬化综合征:临床、电生理和组织免疫方面]

[Clinical amyotrophic lateral sclerosis syndromes in gammapathies: clinical, electrophysiologic and histoimmunologic aspects].

作者信息

Brudon F, Bady B, Chauplannaz G, Joyeux O, Vial C, Chan V, Guillaud-Barbaret C, Chazot G

出版信息

Rev Electroencephalogr Neurophysiol Clin. 1986 Feb;15(4):369-73. doi: 10.1016/s0370-4475(86)80044-2.

Abstract

Eleven patients with motor neuron disease associated with a monoclonal gammopathy were studied. One patient had a previously known multiple myeloma. In the other patients neurological symptoms preceded diagnosis of the gammopathy by one month to 8 years. They were 5 multiple myeloma, 1 macroglobulinemia, 1 chronic lymphatic leukemia, 1 lymphoma and 3 benign monoclonal gammopathies. EMG and motor conduction velocities results were consistent with anterior horn cell disease but sensory conduction studies were abnormal in seven cases out of eight. Superficial peroneal nerve biopsy performed in all patients confirmed peripheral nerve involvement. The relations between gammopathy, clinical symptoms and electrophysiological data are discussed. The interest of sensory conduction studies in every patient with motor neuron disease is emphasized.

摘要

对11例与单克隆丙种球蛋白病相关的运动神经元病患者进行了研究。1例患者先前已知患有多发性骨髓瘤。在其他患者中,神经症状先于丙种球蛋白病诊断1个月至8年出现。他们分别为5例多发性骨髓瘤、1例巨球蛋白血症、1例慢性淋巴细胞白血病、1例淋巴瘤和3例良性单克隆丙种球蛋白病。肌电图和运动传导速度结果与前角细胞疾病一致,但8例中有7例感觉传导研究异常。对所有患者进行的腓浅神经活检证实存在周围神经受累。讨论了丙种球蛋白病、临床症状和电生理数据之间的关系。强调了对每例运动神经元病患者进行感觉传导研究的重要性。

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