兰伯特-伊顿骨髓缩综合征由抗GABAB受体脑炎复杂化
Kazuki Yamada1, Hiroaki Yaguchi1, Kaede Ishikawa1
1Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
Internal medicine (Tokyo, Japan)
|September 24, 2023
概括
一名患有兰伯特-伊顿肌综合征 (LEMS) 的患者出现了不寻常的症状,揭示了同时存在的自身免疫性脑炎. 这凸显了在LEMS患者中考虑异常表现的脑炎的重要性.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 在瘤学瘤学.
背景情况:
- 兰伯特-伊顿肌综合征 (LEMS) 是一种影响神经肌肉结合的自身免疫性疾病.
- 不典型的LEMS表现可能会使诊断和治疗复杂化.
- 小细胞肺癌是LEMS的一种常见的副瘤病因.
研究的目的:
- 报告LEMS病例与同时存在的自身免疫性脑炎.
- 强调在LEMS中不典型症状所带来的诊断挑战.
- 为了突出LEMS,自身免疫性脑炎和隐性恶性瘤之间的潜在关联.
主要方法:
- 一个74岁的男性患者的临床病例介绍.
- 诊断工作包括神经学检查,免疫治疗试验,血清和脑脊液 (CSF) 分析.
- 对潜在恶性瘤的调查.
主要成果:
- 该患者出现了双眼视,肌肉衰弱和呼吸衰竭,被诊断为LEMS.
- 对LEMS的标准免疫疗法是无效的,并且出现了新的症状,如中央呼吸暂停和幻觉.
- 检测GABAB受体抗体证实了自身免疫性脑炎.
- 没有发现恶性瘤,但怀疑是隐性小细胞肺癌.
结论:
- 在LEMS患者的非典型症状需要考虑同时发生的自身免疫性脑炎.
- 在LEMS的背景下,GABAB受体抗体可以表明自身免疫性脑炎.
- 在这种情况下,即使没有明确的发现,也应该怀疑隐性小细胞肺癌.
相关概念视频
Myasthenia Gravis: Overview and Treatment
1.6K
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...
1.6K
Myasthenia Gravis: Diagnostic Tests
960
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...
960
Antiepileptic Drugs: GABAergic Pathway Potentiators
446
γ-aminobutyric acid or GABA, plays a pivotal role as an inhibitory neurotransmitter in the brain. GABA pathway potentiators, also known as GABAergic drugs, are a class of pharmaceutical agents designed to enhance the functioning of the GABAergic system. These medications primarily treat epilepsy, a neurological disorder characterized by recurrent seizures.
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for...
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for...
446
Antiepileptic Drugs: Glutamate Antagonists
407
Glutamate is a fundamental neurotransmitter in the central nervous system, playing a vital role in neuronal communication and various cognitive processes. Glutamate stands as the principal excitatory neurotransmitter in the brain. Its presence is crucial for the communication between neurons, underpinning essential processes such as synaptic transmission, neuronal excitability, and plasticity. These functions are vital for higher-order cognitive processes, including learning and memory. The...
407
Chemical Synapses
8.9K
Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
8.9K
Antiepileptic Drugs: Potassium Channel Activators
203
Ezocgabine or retigabine, an antiepileptic drug of remarkable efficacy, has revolutionized the management of seizures. It is a potassium channel activator, explicitly targeting the family of Q subtype potassium channels. It enhances the transmembrane potassium currents, regulating neuronal excitability. This action stabilizes the resting membrane potential, a pivotal factor in mitigating the hyperexcitability that characterizes epilepsy.
Ezogabine has gained approval as an adjunctive treatment...
Ezogabine has gained approval as an adjunctive treatment...
203


