Myasthenia gravis and congenital myasthenic syndromes

Gilhus, Nils Erik

doi:10.1016/b978-0-323-98818-6.00010-8

Cited by 3 publications

(2 citation statements)

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“…Autoantibodies are responsible for the immune-mediated disease known as acquired MG. These antibodies are frequently directed against the acetylcholine receptors found at the NMJ [10].…”

Section: Congenital Vs Acquired Mgmentioning

confidence: 99%

Myasthenia Gravis: A Systematic Review

Mishra,

Varma

2023

Cureus

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Myasthenia gravis (MG), a rare disease, is the most common neuromuscular junction problem. It's the quintessential autoimmune disease with ocular, bulbar, respiratory, axial, and limb muscles exhibiting a typical fatigable weakening due to the development of antibodies against the acetylcholine receptor (AChR). Infections, stress, surgeries, thymus gland anomalies, and pharmaceutical side effects can also cause it. Ocular symptoms are initially experienced by most of the sufferers. The majority of the sufferers will go through at least one episode of symptom exacerbation during their illness. The immune system in MG interferes with nerve-muscle communication, causing muscles to become weak and tired quickly. The actual cause is not yet known, but a problem in the thymus gland may be the cause. In a person suffering from this disease, the size of the thymus becomes larger than normal, which is also called thymic hyperplasia. It is more common for women to have early-onset MG (EOMG) than for males to have late-onset MG (LOMG). Merely clinical evidence, encompassing the patients' medical history and physical indications of fluctuating muscle weakness in a specific region, is utilized to diagnose MG. Complementary diagnostic procedures and lab techniques aid in confirming the synaptic dysfunction and characterizing its kind and degree. Early diagnosis and the availability of effective treatments have reduced the burden of severe impairment and high mortality previously associated with MG. Current immunomodulation-based therapies come with side effects brought on by persistent immune suppression. Improved knowledge of this relatively uncommon but curable condition is required among primary carers. The objective of this review is to provide information about MG and to help people recognize its symptoms and start treatment without panic so that the progression of this disease can be stopped and complications can be avoided.

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“…Autoantibodies are responsible for the immune-mediated disease known as acquired MG. These antibodies are frequently directed against the acetylcholine receptors found at the NMJ [10].…”

Section: Congenital Vs Acquired Mgmentioning

confidence: 99%

Myasthenia Gravis: A Systematic Review

Mishra,

Varma

2023

Cureus

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“…Myasthenia gravis (MG) is an autoimmune disorder affecting the post-synaptic side of the neuromuscular junction, resulting in a characteristic muscle weakness [ 1 , 2 ]. MG patients are classified according to a combination of features: age of onset, i.e., early-onset (EOMG) and late-onset MG (LOMG); the affected muscle district, i.e., ocular (if symptoms remain confined to this region for at least 2 years), axial, limb, or bulbar; presence of specific auto-antibodies, i.e., mainly anti-acetyl-choline receptor (AchR, the most common), anti-muscle-specific kinase (MuSK), and anti-lipoprotein receptor-related peptide 4 (LRP4), abnormalities of the thymus (e.g., hyperplasia, thymoma), and other autoimmune disorders [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

The Incidence of Myasthenia Gravis in the Province of Ferrara, Italy, in the Period of 2008–2022: An Update on a 40-Year Observation and the Influence of the COVID-19 Pandemic

Antonioni,

Raho,

Carlucci

et al. 2023

JCM

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Myasthenia gravis (MG) is the most common neuromuscular junction disorder. We evaluated the MG incidence rate in the province of Ferrara, Northern Italy, over two time frames (2008–2018 and 2019–2022, i.e., the COVID-19 pandemic) and considered early-onset (EOMG), late-onset (LOMG), and thymoma- and non-thymoma-associated MG. Moreover, in the second period, we assessed its possible relationship with SARS-CoV-2 infection or COVID-19 vaccination. We used a complete enumeration approach to estimate the MG incidence and its temporal trend. For the period of 2008–18, 106 new cases were identified (mean incidence rate 2.7/100,000 people). The highest rates were observed for the over-70 age group and in rural areas, with 17% of thymoma-associated MG. During the COVID-19 period, 29 new cases were identified (average incidence rate 2.1/100,000 people), showing a marked (though not statistically significant) decrease in the mean annual incidence compared to the previous period. Again, the highest rate was observed for the over-70 age group. The first period was in line with our previous observations for the period between 1985 and 2007, highlighting a rising incidence of LOMG and a marked decrease in EOMG. During the COVID-19 period, incidence rates were lower in the first years whereas, when the pandemic ended, the previous trend was confirmed.

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