Thymoma-associated Myasthenia Gravis in a Young Adult with Development of Paraneoplastic Limbic Encephalitis and Systemic Lupus Erythematosus Post-thymectomy: A Case Report

Liu, Robin; Dar, A. Rashid; Tay, Keng Yeow; Nicolle, Mike W; Inculet, Richard

doi:10.7759/cureus.3581

Cited by 7 publications

(5 citation statements)

References 17 publications

(40 reference statements)

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“…Fourteen cases in 22 patients with anti-AMPAR encephalitis had tumors, such as lung cancer, thymoma, breast cancer, or ovarian teratoma, indicating that paraneoplastic autoimmunity plays a key role in the pathogenic mechanisms of anti-AMPAR encephalitis [10,14]. Our case is more likely paraneoplastic autoimmune encephalitis even if encephalitis emerged 2 years after thymoma diagnosis.…”

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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“…The initial search identified 465 articles, of which 407 titles and abstracts were screened and excluded (Figure 1). Fifty‐eight papers 5,7–63 eventually included that enrolled a total of 68 patients.…”

Section: Resultsmentioning

confidence: 99%

“…The initial search identified 465 articles, of which 407 titles and abstracts were screened and excluded (Figure 1 ). Fifty‐eight papers 5 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 <...…”

Section: Resultsmentioning

confidence: 99%

Thymoma‐associated autoimmune encephalitis: Analysis of factors determining prognosis

Song

et al. 2023

CNS Neurosci Ther

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Introduction: Autoimmune encephalitis (AE) is a heterogeneous group of inflammatory central nervous system disorders caused by a misdirected immune response against self-antigens expressed in the central nervous system. The thymus is a central organ in the immune system and thymic tumors are thought to be possible initiators of many neurological disorders. Recently, there is growing evidence that thymomas are associated with autoimmune encephalitis.Aims: Our study initially explored the characteristics of patients with autoimmune encephalitis combined with thymoma. Methods:We used patient data from January 1, 2011 to October 1, 2021 from the PubMed, Web of Science, Ovid, and CNKI platforms to analyze overall demographics, frequency of symptoms and associations, and treatment prognosis outcomes.Results: A total of 68 patients were included. There were 39 female cases (57.4%).The mean age was 50 years (IQR 40-66 years). All had acute and subacute onset.The clinical manifestations were mostly cognitive changes (70.6%), mental disorders (57.4%), and epilepsy (50.0%). The most common neuronal antibody was alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA). Magnetic resonance imaging (MRI) abnormalities were present in 81.0% of patients, mostly in the hippocampus, temporal lobe, and some in cortical and subcortical areas. Abnormalities in the electroencephalogram (EEG) in 69.8% of patients. Treatment involved immunotherapy and thymoma treatment, with 79.7% of patients improving after treatment. While 20.3% of patients had a poor prognosis. Further, 14.8% of patients relapsed. Mental disorders, autonomic dysfunction, sleep disturbances, anti-Ma2, and thymoma untreated were more frequent in patients with poor prognosis. Conclusion:Thymoma-associated autoimmune encephalitis is a unique disease entity.Long-term follow-up of chest CT findings is recommended for patients with autoimmune encephalitis.

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“…Of the previously reported anti-AMPAR encephalitis associated with ovarian tumors, one patient developed cognitive deficits following the surgery for ovarian adenocarcinoma [ 6 , 9 , 32 ]. Notably, we identified three cases in which patients developed encephalitis symptoms only a few days after tumor resection: two patients with breast cancer and another with thymoma [ 14 , 15 , 33 ]. Generally, patients with paraneoplastic AE develop encephalitis before detecting concurrent tumors or suggesting recurrent tumors [ 34 – 39 ].…”

Section: Discussionmentioning

confidence: 99%

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis developed after ovarian cancer cytoreduction surgery: a case report and literature review

Huang,

Zhou,

Zhou

et al. 2023

BMC Women's Health

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Background Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis (AE), is often found associated with tumors such as thymoma, lung cancer, ovarian tumors, and breast cancer, and the tumors were generally detected during the screening process after the encephalitis initiated. The tumor is considered a trigger of AE, but the mechanism remains unclear. Case Presentation A 53-year-old woman presented short-term memory loss two days after the primary cytoreduction for high-grade serous ovarian cancer (HGSOC, FIGO stage IC3). Cell-based assay found AMPAR CluA2 IgG positive in both serum (1:3.2) and cerebrospinal fluid (1:32). Moreover, mild AMPAR GluA1 and strong GluA2 expressions were also found positive in the paraffin sections of ovarian tumor tissue, indicating the ovarian cytoreduction surgery might stimulate the release of receptor antigens into the circulation system. The patient’s condition deteriorated within two weeks, developing consciousness and autonomic dysfunction, leading to ICU admission. With oral steroids, intravenous immunoglobulin, plasmapheresis, and rituximab treatment, the patient’s consciousness markedly improved after three months. Conclusion We presented the first case of anti-AMPAR encephalitis developed right after the primary cytoreduction of a patient with HGSOC and retrieved paraneoplastic anti-AMPAR encephalitis cases (n = 66). Gynecologists should pay attention to patients who develop cognitive dysfunction or psychiatric symptoms shortly after the ovarian tumor resection and always include AE in the differentiation diagnosis.

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Thymoma-associated Myasthenia Gravis in a Young Adult with Development of Paraneoplastic Limbic Encephalitis and Systemic Lupus Erythematosus Post-thymectomy: A Case Report

Cited by 7 publications

References 17 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

Thymoma‐associated autoimmune encephalitis: Analysis of factors determining prognosis

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis developed after ovarian cancer cytoreduction surgery: a case report and literature review

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