[运动神经病变之前的初级系统性 Amyloidosis]
Maor Mermelstein1, Felix Benninger1, Idit Tamir2
1Department of Neurology, Rabin Medical Center, Petach Tikva, Israel, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Harefuah
|November 21, 2024
概括
主要系统性粉粉症 (AL) 通常会导致影响感觉神经的神经病变. 这种病例独特地显示了系统AL氨基粉症之前的运动神经病变,突出了这种不常见疾病的罕见表现.
科学领域:
- 神经学 神经学
- 血液学 血液学 血液学
- 病理学 病理学 病理学
背景情况:
- 初级 (AL) 系统性粉症涉及免疫球蛋白轻链沉积,导致器官功能障碍.
- 常见的症状包括疲劳,体重减轻,性综合征,心肌病和神经病变.
- 虽然中枢神经系统不受影响,但外围神经病变会影响17-36%的AL患者,通常会影响小的感觉纤维.
研究的目的:
- 报告一个独特的临床运动轴突神经病变的病例,该病例早于诊断出系统性粉样化症.
- 要突出AL氨基粉症的非典型表现,强调神经参与的变异性.
主要方法:
- 病例报告详细介绍患者的临床表现和诊断旅程.
- 关于AL氨基粉症及其神经表现的文献综述.
主要成果:
- 该患者在AL氨基粉症的全身表现之前出现了运动轴突神经病变.
- 这与AL氨基粉症的典型表现形成鲜明对比,这种表现通常涉及感官神经病变.
结论:
- 临床运动轴突神经病变可能是AL氨基粉症的初始表现.
- 这一案例凸显了在患者中考虑AL粉样粉症的重要性 无法解释的运动神经病变,甚至在系统性症状明显之前.
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