成年神经肌肉胆瘤,一种罕见的外周神经病理:一个说明性的案例
Ashley Ricciardelli1, Alex Flores1, Hsiang-Chih Lu2
1Departments of1Neurosurgery.
Journal of neurosurgery. Case lessons
|January 29, 2024
概括
神经肌肉胆固醇瘤 (NMCs) 是一种罕见的神经瘤. 这项研究描述了成年人坐骨神经NMC的特征,并指出与儿科病例的相似之处和潜在的侵略性转变.
科学领域:
- 神经病理学神经病理学
- 在瘤学瘤学.
- 周围神经疾病 周围神经疾病
背景情况:
- 神经肌肉胆固醇瘤 (NMCs) 是一种罕见的瘤,在外周神经 (主要是坐骨神经) 中具有异常肌肉纤维.
- NMCs携带的风险是进展到desmoid类型纤维化 (NMC-DTFs),侵略性病变,可能需要截肢.
- 现有的关于成人NMC及其进展到NMC-DTF的文献很少,大多数病例报告在儿科患者中.
研究的目的:
- 在成年人群中表征坐骨神经神经肌肉胆固醇瘤 (NMCs).
- 调查NMCs和NMCs与成人的desmoid型纤维化 (NMC-DTFs) 的发展之间的联系.
- 提供成人NMC病例的全面审查,解决有限的当前信息.
主要方法:
- 一个案例研究的介绍,涉及一个66岁的男性,患有坐骨神经NMC.
- 执行系统文献审查,遵守系统审查和元分析 (PRISMA) 准则的首选报告项目.
- 脊椎神经带神经活检的组织病理学检查,以确定病变.
主要成果:
- 成年男性患者在10年的病史中呈现出渐进的左下肢虚弱和麻醉.
- 磁共振成像显示,近道坐骨神经略增大.
- 文献审查表明,成人NMC病例与儿科NMC有共同的特征.
结论:
- 由于对疾病进展,诊断和治疗的数据有限,对成人NMC的进一步广泛研究至关重要.
- 成人NMC需要谨慎管理,因为手术后转化为NMC-DTF的风险较高.
- 成人NMC与儿科NMC有相似之处,这表明有一个共同的潜在病理生理学.
相关概念视频
The Neuromuscular Junction
9.7K
The nervous system consists of complex motor neuron circuits, including upper motor neurons originating from the cerebral cortex and lower motor neurons starting in the spinal cord, coordinating both voluntary and involuntary movements. Among these, somatic motor neurons activate skeletal muscles and are classified into alpha, beta, and gamma types. Alpha neurons are vital for voluntary movement coordination, while gamma neurons adjust muscle spindle sensitivity, and the function of beta...
9.7K
Myasthenia Gravis: Overview and Treatment
1.4K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
1.4K
Disorders of the Skeletal Muscle
953
The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
953
Myasthenia Gravis: Diagnostic Tests
844
Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
844


