相关实验视频
Updated: Feb 14, 2026

10:46
Diffusion Imaging in the Rat Cervical Spinal Cord
Published on: April 7, 2015
12.2K
心脏病发作还是代谢崩? 从长髓延伸到腰椎脊髓的纵向广泛的扩散受限病变
Yuka Nakaya1, Koji Hayashi1, Mamiko Sato1,2
1Department of Rehabilitation Medicine, Fukui General Hospital, 55-16-1 Egami-cho, Fukui 910-8561, Japan.
Diagnostics (Basel, Switzerland)
|February 13, 2026
概括
一种罕见的晚期出现的甲酸甲酶转基因酶 (OTC) 缺乏症,呈现出严重的神经症状和广泛的白质病变. 这种由营养不足加剧的代谢障碍导致了致命的结果.
科学领域:
- 神经学 神经学
- 代谢障碍 代谢障碍 代谢障碍
- 神经免疫学 神经免疫学
背景情况:
- 接受甲状腺素治疗的风湿性关节炎患者出现意识改变,吐和肠道穿孔.
- 最初的实验室显示高氨血和脱水;尽管氨正常化,神经状态略有改善.
研究的目的:
- 在怀疑代谢崩的患者中调查严重神经病变的原因.
- 描述广泛的白质病变,并确定导致的因素.
主要方法:
- 脑脊髓液分析 (寡克隆带,髓基蛋白).
- 血清自身免疫标记测试 (抗AQP4,抗MOG,抗GFAP).
- 大脑和脊髓的磁共振成像 (MRI),包括扩散加权成像 (DWI).
- 代谢查 (叶酸,铜,尿中耳酸,血清素).
主要成果:
- 脑脊液显示肌蛋白基本蛋白升高;自身免疫标志物是负的.
- 核磁共振扫描显示了从脑干到脊髓白质的广泛的,扩散受限制的T2/DWI-高强度病变.
- 代谢查表明晚期发病的甲酸转糖酶 (OTC) 缺乏,叶酸和铜含量低.
结论:
- 这一案例突出了潜在的代谢崩,可能是OTC缺乏,导致严重的神经毒性.
- 观察到广泛的非血管性白质病变,可能是由于代谢障碍,营养不足和药物毒性.
- 患者经历了突然死亡,这凸显了病情的严重性.
相关概念视频
Spinal Cord
2.0K
The spinal cord, a critical component of the central nervous system, extends from the base of the brainstem to the lumbar region of the vertebral column. It is essential for maintaining physical stability and facilitating communication between the brain and peripheral parts of the body.
2.0K
The Spinal Cord
31.9K
The spinal cord is the body’s major nerve tract of the central nervous system, communicating afferent sensory information from the periphery to the brain and efferent motor information from the brain to the body. The human spinal cord extends from the hole at the base of the skull, or foramen magnum, to the level of the first or second lumbar vertebra.
31.9K
Spinal Cord: Information Processing
3.6K
The spinal cord is an integral hub for motor and sensory information that enables the brain to communicate with the peripheral nervous system (PNS). This communication consists of relaying sensory data and transmission of motor commands.
Sensory Information Processing
Sensory information processing begins at the sensory receptors located in the skin and other tissues, which detect somatic sensory stimuli such as touch, temperature, or pain. These receptors function as catalysts, initiating...
Sensory Information Processing
Sensory information processing begins at the sensory receptors located in the skin and other tissues, which detect somatic sensory stimuli such as touch, temperature, or pain. These receptors function as catalysts, initiating...
3.6K
What is Metabolism?
132.6K
Overview
132.6K
Spinal Cord: Gross Anatomy
5.9K
The spinal cord resides within the protective confines of the vertebral column. It is the main pathway for information traveling between the brain and the body. It plays a fundamental role in nearly all bodily functions, from simple reflexes to complex motor movements. The spinal cord begins at the medulla oblongata at the base of the brainstem and extends downward, terminating at the conus medullaris near the first and second lumbar vertebrae. The spinal cord's length in adults is...
5.9K
Longitudinal Research
13.5K
Sometimes we want to see how people change over time, as in studies of human development and lifespan. When we test the same group of individuals repeatedly over an extended period of time, we are conducting longitudinal research. Longitudinal research is a research design in which data-gathering is administered repeatedly over an extended period of time. For example, we may survey a group of individuals about their dietary habits at age 20, retest them a decade later at age 30, and then again...
13.5K

