Thymoma-associated myasthenia gravis and LGI1-encephalitis, with nephrotic syndrome post-thymectomy

Hor, Jyh Yung; Lim, Thien Thien; Cheng, Mei Chih; Chia, Yuen Kang; Wong, Chee Keong; Lim, Su Min; Cheah, Chun Fai; Tan, Kenny; Easaw, P.E.S.; Leite, Maria Isabel

doi:10.1016/j.jneuroim.2018.01.011

Cited by 12 publications

(10 citation statements)

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“…Recently, a patient with anti-AMPAR encephalitis who was reported to be in remission for 34 months showed clinical relapse three months after the detection of recurrent thymoma [11]. Hor et al reported a case of thymoma-associated myasthenia gravis and LGI1encephalitis with nephrotic syndrome after thymectomy [13]. Our case raises the questions of whether the autoantigens could be expressed by the thymus after thymectomy, and how these autoantigens from thymic tumors could trigger immune disorders.…”

Section: Discussionmentioning

confidence: 70%

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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Background: Autoimmune encephalitis (AE) is a newly recognized autoimmune disorders in which the targets are proteins or receptors involved in synaptic transmission and neuronal excitability. α-amino-3-hydroxy-5-methyl-4isoxazolepropionic acid receptor (AMPAR) is a subtype of glutamate receptor that mediates most of the fast excitatory neurotransmission in the brain. Case presentation: A 50-year-old woman presented with subacute onset of memory loss and behavioral changes. High levels of serum (1:1000) and CSF (1:32) antibodies against the AMPAR GluR2 were detected. A wide range of abnormalities in 6-8 Hz low to middle slow waves was found by electroencephalographs, and high-intensity signals on fluid-attenuated inversion recovery in both the medial temporal lobe and hippocampus were identified on brain magnetic resonance images. This patient presented with myasthenia gravis and type B2 thymoma (World Health Organization Thymoma Classification) at age 48. This case was unique in that the patient initiated with the symptom of myasthenia gravis and thymoma two years prior to encephalitis, and a complete thymectomy was performed before AE onset without recurrence of the thymoma when encephalitis occurred. Conclusions: Thymoma was reported to be associated with paraneoplastic neurological disease. This is the first time a thymectomy has been applied in a myasthenia gravis patient with thymoma two years prior to the onset of anti-AMPAR2 encephalitis. This case highlights the complexity of autoimmune encephalitis associated with thymoma.

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Section: Discussionmentioning

confidence: 70%

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

2019

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“…According to previous case series, PNS has a 0–31% chance of revealing tumors ( 77 – 83 , 87 ), among which thymoma and lung cancer were considered the most common ones ( 1 ). Nonetheless, 5.58% of our included cases showed carcinogenesis, including oral squamous cell carcinoma and locally advanced lung cancer ( 30 , 70 ), breast cancer ( 25 ), prostate cancer ( 37 ), thymoma ( 65 ), renal cell carcinoma ( 76 ), etc., which are inconsistent with the former results. It is likely the tumor types mentioned above were not included, so further investigations are needed to gather more complete information.…”

Section: Discussionmentioning

confidence: 95%

Clinical Features and Therapeutic Effects of Anti-leucine-rich Glioma Inactivated 1 Encephalitis: A Systematic Review

Teng

Zeng

et al. 2022

Front. Neurol.

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Background: Clinical presentations and treatment programs about anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis still remain incompletely understood.Objective: This study analyzed the clinical features and therapeutic effects of anti-LGI1 encephalitis.Methods: PubMed, EMBASE, and the Cochrane Library were searched to identify published English and Chinese articles until April 2021. Data were extracted, analyzed, and recorded in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines.Results: A total of 80 publications detailing 485 subjects matched our inclusion criteria. Short-term memory loss (75.22%), faciobrachial dystonic seizures (FBDS) (52.53%), other seizures excluding FBDS (68.48%), psychiatric symptoms (57.67%), and sleep disturbances (34.30%) were the most frequently described symptoms in anti-LGI1 encephalitis. Hyponatremia (54.90%) was the most common hematologic examination change. The risk of incidence rate of malignant tumors was higher than in healthy people. The positive rate of anti-LGI1 in serum (99.79%) was higher than CSF (77.38%). Steroids (93.02%), IVIG (87.50%), and combined use (96.67%) all had a high remission rate in the initial visit. A total of 35 of 215 cases relapsed, of which 6/35 (17.14%) did not use first-line treatment, and 21 (60.00%) did not maintain long-term treatment. Plasma exchange (PE) could be combined in severe patients, immunosuppressant could be used for refractory patients or for recurrence and using an anti-epileptic drug to control seizures may benefit cognition.Conclusions: Short-term memory loss, FBDS, psychiatric symptoms, and hyponatremia were key features in identifying anti-LGI1 encephalitis. Serum and CSF antibody tests should be considered in diagnosis criteria. Steroids with IVIG should be recommended, PE was combined for use in severe patients, immunosuppressant therapy might improve outcomes if recurrence or progression occurred, and control seizures might benefit cognition. The useful ways to reduce relapse rate were early identification, clear diagnosis, rapid treatment, and maintaining long-term treatment. The follow-up advice was suggested according to the research of paraneoplastic syndrome, and concern about tumors was vital as well.

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“…Both, non-proliferative and proliferative glomerulopathies were associated with systemic autoimmune features in thymomas, namely minimal change disease, membranous nephropathy and focal segmental glomerulosclerosis as well as membranoproliferative glomerulonephritis and crescentic glomerulonephritis (rapidly progressive glomerulonephritis) as outlined in a former review by Bacchetta et al (90) and in recent case reports (91)(92)(93)(94)(95). Pronounced in the nonproliferative glomerulopathies, nephrotic syndrome is frequently encountered as complication which itself bears the risk of secondary thrombosis or infections due to renal protein loss.…”

Section: Glomerulopathiesmentioning

confidence: 87%

“…Pronounced in the nonproliferative glomerulopathies, nephrotic syndrome is frequently encountered as complication which itself bears the risk of secondary thrombosis or infections due to renal protein loss. Responses to immunosuppressive agents in combination with thymoma-specific treatments were described as quite favourable (90)(91)(92)(93)(94)(95). The underlying pathophysiologies are still inadequately understood but seem to involve different mechanisms of AID as well as PNS eliciting the distinct glomerulopathy variants (96).…”

Section: Glomerulopathiesmentioning

confidence: 99%

Autoimmune disorders and paraneoplastic syndromes in thymoma

Blum¹,

Misch²,

Kollmeier³

et al. 2020

J Thorac Dis

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Thymomas are counted among the rare tumour entities which are associated with autoimmune disorders (AIDs) and paraneoplastic syndromes (PNS) far more often than other malignancies. Through its complex immunological function in the context of the selection and maturation of T cells, the thymus is at the same time highly susceptible to disruptive factors caused by the development and growth of thymic tumours. These T cells, which are thought to develop to competent immune cells in the thymus, can instead adopt autoreactive behaviour due to the uncontrolled interplay of thymomas and become the trigger for AID or PNS affecting numerous organs and tissues within the human body. While myasthenia gravis is the most prevalent PNS in thymoma, numerous others have been described, be they related to neurological, cardiovascular, gastrointestinal, haematological, dermatological, endocrine or systemic disorders. This review article sheds light on the pathophysiology, epidemiology, specific clinical features and therapeutic options of the various forms as well as courses and outcomes of AID/PNS in association with thymomas.Whenever suitable and backed by the limited available evidence, the perspectives from both the thymoma and the affected organ/tissue will be highlighted. Specific issues addressed are the prognostic significance of thymectomy on myasthenia gravis and other thymoma-associated AID/PND and further the impact and safety of immunotherapies on AID and PND relating to thymomas.

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Thymoma-associated myasthenia gravis and LGI1-encephalitis, with nephrotic syndrome post-thymectomy

Cited by 12 publications

References 10 publications

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor GluR2 encephalitis in a myasthenia gravis patient with complete thymectomy: a case report

Clinical Features and Therapeutic Effects of Anti-leucine-rich Glioma Inactivated 1 Encephalitis: A Systematic Review

Autoimmune disorders and paraneoplastic syndromes in thymoma

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