Lambert-Eaton Myasthenic Syndrome

Kesner, Vita; Oh, Shin J.; Dimachkie, Mazen M.; Barohn, Richard J.

doi:10.1016/j.ncl.2018.01.008

Cited by 101 publications

(137 citation statements)

References 73 publications

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“…At structural level, the interaction of nervous and musculoskeletal system occurs in the NMJ, also called motor end plate [ 54 ]. Muscles are organized in bundles, called muscle fibers, made up of myocytes (muscle cells).…”

Section: Introductionmentioning

confidence: 99%

The Impact of Mitochondrial Deficiencies in Neuromuscular Diseases

2020

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Neuromuscular diseases (NMDs) are a heterogeneous group of acquired or inherited rare disorders caused by injury or dysfunction of the anterior horn cells of the spinal cord (lower motor neurons), peripheral nerves, neuromuscular junctions, or skeletal muscles leading to muscle weakness and waste. Unfortunately, most of them entail serious or even fatal consequences. The prevalence rates among NMDs range between 1 and 10 per 100,000 population, but their rarity and diversity pose difficulties for healthcare and research. Some molecular hallmarks are being explored to elucidate the mechanisms triggering disease, to set the path for further advances. In fact, in the present review we outline the metabolic alterations of NMDs, mainly focusing on the role of mitochondria. The aim of the review is to discuss the mechanisms underlying energy production, oxidative stress generation, cell signaling, autophagy, and inflammation triggered or conditioned by the mitochondria. Briefly, increased levels of inflammation have been linked to reactive oxygen species (ROS) accumulation, which is key in mitochondrial genomic instability and mitochondrial respiratory chain (MRC) dysfunction. ROS burst, impaired autophagy, and increased inflammation are observed in many NMDs. Increasing knowledge of the etiology of NMDs will help to develop better diagnosis and treatments, eventually reducing the health and economic burden of NMDs for patients and healthcare systems.

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Section: Introductionmentioning

confidence: 99%

The Impact of Mitochondrial Deficiencies in Neuromuscular Diseases

2020

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“…Tanı klinik ve elektrofizyolojik olarak konulur. Tedavide tümoral bir neden varsa ona ait tedavi, pridostigmin, 3-4 diaminopridine, hızlı ilerleyen olgularda intravenöz ımmunglobulin, plazmaferez, yeterli kontrol sağlanamayan olgularda ise prednizolon ve azotiopurin önerilmektedir [3]. [5].…”

Section: Lambert Eaton Myastenik Sendromu (Lems)unclassified

Atrofi ve proksimal kas güçsüzlüğü olan olgu: seronegatif Lambert Eaton Myastenik Sendrom

Say¹,

Ergün²,

Karaca³

2019

Pamukkale Medical Journal

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Özet Lambert Eaton Myastenik Sendrom (LEMS) proksimal kas güçsüzlüğü, reflekslerde azalma veya kayıp, ılımlı oküler bulgular ve otonom bulgular ile seyreden presinaptik voltaj bağımlı kalsiyum kanallarına antikor gelişimi ile karekterize nadir görülen nöromuskuler kavşak hastalığıdır. Paraneoplastik veya otoimmün olabilir. Paraneoplastik LEMS en sık küçük hücreli akciğer kanseri ile birliktelik gösterir. Primer otoimmun olarak ise diyabet, romatoid artrit, sistemik lupus eritematozus, otoimmun tiroid hastalıklarıyla birliktelik gösterebilir. Tanı klinik bulgular ve elektrofizyolojik olarak ardışık sinir uyarım testi veya tek lif elektromyelografi ile konur. Tedavisinde olguya göre 3,4 diaminopiridin, ıntravenöz immunglobulin, plazmaferez, prednizolon ve azotiopürin yer alır.

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“…This enhances entry of Ca 2+ into the nerve terminal and increases ACh release. Treatments proposed for long-term modification of this disease include immunosuppressants ( Kesner et al, 2018 ).…”

Section: Concept Of Safety Factor and Pathological Muscle Weaknessmentioning

confidence: 99%

Autoregulation of Acetylcholine Release and Micro-Pharmacodynamic Mechanisms at Neuromuscular Junction: Selective Acetylcholinesterase Inhibitors for Therapy of Myasthenic Syndromes

2018

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Neuromuscular junctions (NMJs) are directly involved into such indispensable to life processes as respiration and locomotion. However, motor nerve forms only one synaptic contact at each muscle fiber. This unique configuration requires specific properties and constrains to be effective. The very high density of acetylcholine receptors (AChRs) of muscle type in synaptic cleft and an excess of acetylcholine (ACh) released under physiological conditions make this synapse extremely reliable. Nevertheless, under pathological conditions such as myasthenia gravis and congenital myasthenic syndromes, the safety factor can be markedly reduced. Drugs used for short-term symptomatic therapy of these pathological states, cause partial inhibition of cholinesterases (ChEs). These enzymes catalyze the hydrolysis of ACh, thus terminate its action on AChRs. Extension of the lifetime of ACh molecules compensates muscular AChRs abnormalities and, consequently, rescues muscle contractions. In this mini review, we will first outline the functional organization of the NMJ, and then, consider the concept of the safety factor and how it may be changed. This will be followed by a look at autoregulation of ACh release that influences the safety factor of NMJs. Finally, we will consider the morphological features of NMJs as a putative reserve to increase effectiveness of pathological muscle weakness therapy by ChEs inhibitors due to opportunity to use micro-pharmacodynamic mechanisms.

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Lambert-Eaton Myasthenic Syndrome

Cited by 101 publications

References 73 publications

The Impact of Mitochondrial Deficiencies in Neuromuscular Diseases

The Impact of Mitochondrial Deficiencies in Neuromuscular Diseases

Atrofi ve proksimal kas güçsüzlüğü olan olgu: seronegatif Lambert Eaton Myastenik Sendrom

Autoregulation of Acetylcholine Release and Micro-Pharmacodynamic Mechanisms at Neuromuscular Junction: Selective Acetylcholinesterase Inhibitors for Therapy of Myasthenic Syndromes

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