Thymoma-associated anti-LGI1 encephalitis and myasthenia gravis: A unique combination with autoantibodies

Satake, Akane; Nagasaka, Takamura; Kurita, Takafumi; Murata, Hiroaki; Hata, Takanori; Shinmura, Hiroyuki; Matsubara, Hirochika; Shindo, Kazumasa; Takiyama, Yoshihisa

doi:10.1016/j.ensci.2022.100395

Cited by 4 publications

(2 citation statements)

References 12 publications

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“…While earlier case reports have described the co-occurrence of thymomas, myasthenia gravis with AChR-antibodies, and autoimmune encephalitis associated with both AMPAR and Lgi1 antibodies (3)(4)(5), the pathological mechanisms underlying this association remain unknown. A recent study found an increased risk of autoimmune disease following thymectomy (6), and some patients with thymoma-related myasthenia gravis can experience symptom worsening and an increase in anti-AChR antibody titers following thymectomy (7,8).…”

Section: Discussionmentioning

confidence: 97%

Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1

Nes,

Haugen,

Haugland

et al. 2023

Front. Neurol.

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ObjectivesAutoantibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) and leucine-rich glioma-inactivated 1 (Lgi1) are associated with autoimmune encephalitis. We described an acetylcholine receptor (AChR)-positive patient with myasthenia gravis who developed limbic encephalitis with antibodies to AMPAR and Lgi1.MethodsA single-case report with detailed, prospective clinical and biomarker data including serial laboratory testing and histopathology.ResultsA 49-year-old woman was diagnosed with anti-AChR antibody-positive generalized myasthenia gravis in 1983. After 9 months of the removal of thymoma in 1984, she developed influenza-like symptoms and then symptoms of limbic encephalitis. Retrospective analysis of serum showed high concentrations of anti-AMPAR and lower concentrations of anti-Lgi1 antibodies. Cerebral CT was normal, EEG showed bifrontal dysrhythmia, and CSF showed mild pleocytosis. Immuno-histochemical examination of the thymoma confirmed staining for Glur2, a subunit of AMPAR. The patient recovered with mild sequelae, but low levels of anti-AMPAR and anti-Lgi1 antibodies were detectable for over 25 years subsequently.DiscussionThis case confirms earlier reports of AMPAR-associated autoimmune encephalitis co-occurring with thymoma and myasthenia gravis and is unique in its observational length. It shows, moreover, that antibodies to AMPAR and Lgi1 can persist despite clinical recovery.

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Section: Discussionmentioning

confidence: 97%

Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1

Nes,

Haugen,

Haugland

et al. 2023

Front. Neurol.

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“…The patient underwent thymectomy and immunotherapy, and the symptoms gradually improved. In 2022, a case complicated with LGI1 encephalitis and thymoma-associated AChR MG was reported by Satake et al (60). The patient first presented with memory impairment and had bilateral ptosis.…”

Section: Autoimmune Encephalitismentioning

confidence: 95%

Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases

Zhu,

Wang,

Hao

et al. 2023

Front. Immunol.

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Multiple reports on the co-existence of autoimmune diseases and myasthenia gravis (MG) have raised considerable concern. Therefore, we reviewed autoimmune diseases in MG to explore their clinical presentations and determine whether the presence of autoimmune diseases affects the disease severity and treatment strategies for MG. We reviewed all the major immune-mediated coexisting autoimmune conditions associated with MG. PubMed, Embase and Web of Science were searched for relevant studies from their inception to January 2023. There is a higher frequency of concomitant autoimmune diseases in patients with MG than in the general population with a marked risk in women. Most autoimmune comorbidities are linked to AChR-MG; however, there are few reports of MuSK-MG. Thyroid disorders, systemic lupus erythematosus, and vitiligo are the most common system autoimmune diseases associated with MG. In addition, MG can coexist with neurological autoimmune diseases, such as neuromyelitis optica (NMO), inflammatory myopathy (IM), multiple sclerosis (MS), and autoimmune encephalitis (AE), with NMO being the most common. Autoimmune diseases appear to develop more often in early-onset MG (EOMG). MS coexists more commonly with EOMG, while IM coexists with LOMG. In addition, MG complicated by autoimmune diseases tends to have mild clinical manifestations, and the coexistence of autoimmune diseases does not influence the clinical course of MG. The clinical course of neurological autoimmune diseases is typically severe. Autoimmune diseases occur most often after MG or as a combined abnormality; therefore, timely thymectomy followed by immunotherapy could be effective. In addition, thymoma-associated AChR MG is associated with an increased risk of AE and IM, whereas NMO and MS are associated with thymic hyperplasia. The co-occurrence of MG and autoimmune diseases could be attributed to similar immunological mechanisms with different targets and common genetic factor predisposition. This review provides evidence of the association between MG and several comorbid autoimmune diseases.

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Clinical study of autonomic dysfunction in patients with autoimmune encephalitis

Lian

2023

Immunobiology

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Thymoma-associated anti-LGI1 encephalitis and myasthenia gravis: A unique combination with autoantibodies

Cited by 4 publications

References 12 publications

Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1

Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1

Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases

Clinical study of autonomic dysfunction in patients with autoimmune encephalitis

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