Post-thymectomy myasthenia gravis: a case report and systematic review of literature

Myasthenia gravis (MG) is an autoimmune condition affecting the neuromuscular junction characterised by weakness and fatiguability, carrying a high morbidity if treatment is delayed. A clear association with thymoma has led to management with thymectomy as a common practice, but MG presenting post-thymectomy has rarely been reported. We present a case of an 82- year-old woman developing fatigue, ptosis and dysarthria 3 months after thymectomy. After a clinical diagnosis of MG was made, she responded well to prompt treatment with prednisolone and pyridostigmine. Her anti-acetylcholine receptor antibody (anti-AChR) subsequently came back positive. Our systematic review reveals that post-thymectomy MG can be categorised as early-onset or late-onset form with differing aetiology, and demonstrated correlation between preoperative anti-AChR titres and post-thymectomy MG. The postulated mechanisms for post-thymectomy MG centre around long-lasting peripheral autoantibodies. Clinicians should actively look for MG symptoms in thymoma patients and measure anti-AChR preoperatively to aid prognostication.

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Clinical and therapeutic features of myasthenia gravis in adults based on age at onset

Neurology ◽

10.1212/wnl.0000000000008903 ◽

2020 ◽

Vol 94 (11) ◽

pp. e1171-e1180 ◽

Cited By ~ 8

Author(s):

Elena Cortés-Vicente ◽

Rodrigo Álvarez-Velasco ◽

Sonia Segovia ◽

Carmen Paradas ◽

Carlos Casasnovas ◽

...

Keyword(s):

Myasthenia Gravis ◽

Early Onset ◽

Late Onset ◽

Age At Onset ◽

Neuromuscular Diseases ◽

Cross Sectional ◽

Life Threatening ◽

Onset Group ◽

Anti Acetylcholine

ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis observational cross-sectional multicenter study was based on information in the neurologist-driven Spanish Registry of Neuromuscular Diseases (NMD-ES). All patients were >18 years of age at onset of MG and onset occurred between 2000 and 2016 in all cases. Patients were classified into 3 age subgroups: early-onset MG (age at onset <50 years), late-onset MG (onset ≥50 and <65 years), and very-late-onset MG (onset ≥65 years). Demographic, immunologic, clinical, and therapeutic data were reviewed.ResultsA total of 939 patients from 15 hospitals were included: 288 (30.7%) had early-onset MG, 227 (24.2%) late-onset MG, and 424 (45.2%) very-late-onset MG. The mean follow-up was 9.1 years (SD 4.3). Patients with late onset and very late onset were more frequently men (p < 0.0001). Compared to the early-onset and late-onset groups, in the very-late-onset group, the presence of anti–acetylcholine receptor (anti-AChR) antibodies (p < 0.0001) was higher and fewer patients had thymoma (p < 0.0001). Late-onset MG and very-late-onset MG groups more frequently had ocular MG, both at onset (<0.0001) and at maximal worsening (p = 0.001). Although the very-late-onset group presented more life-threatening events (Myasthenia Gravis Foundation of America IVB and V) at onset (p = 0.002), they required fewer drugs (p < 0.0001) and were less frequently drug-refractory (p < 0.0001).ConclusionsPatients with MG are primarily ≥65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.

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Effects of thymectomy on late-onset non-thymomatous myasthenia gravis: systematic review and meta-analysis

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01860-y ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Jinwei Zhang ◽

Yuan Chen ◽

Hui Zhang ◽

Zhaoyu Yang ◽

Peng Zhang

Keyword(s):

Systematic Review ◽

Conservative Treatment ◽

Myasthenia Gravis ◽

Early Onset ◽

Odds Ratio ◽

Late Onset ◽

Meta Analysis ◽

Therapeutic Strategies ◽

Cochrane Library ◽

Study Objective

Abstract Background The effects of thymectomy on late-onset non-thymomatous myasthenia gravis (NTMG) remain controversial. The objective of this study was to conduct a systematic review in order to answer two questions pertinent to late-onset NTMG: (1) do patients with late-onset NTMG experience the same effects from thymectomy as their early-onset counterparts? (2) Compared with conservative treatment, does thymectomy have any benefits for late-onset NTMG patients? Methods We searched the PubMed, EMBASE, and Cochrane Library databases for studies published from January 1, 1950 to March 10, 2021. Outcomes were measured via clinical stable remission/pharmacological remission (CSR/PR) and improvement rates. We used Stata software to analyze the data. Results We ultimately included a total of 12 observational articles representing the best evidence answering the questions of our study objective. Of these, nine studies, which included 896 patients overall (766 early-onset and 230 late-onset), compared postoperative outcomes between early- and late-onset NTMG. The remaining three articles, which included 216 patients (75 in the thymectomy group and 141 in the conservative-treatment group), compared thymectomy with conservative treatment for late-onset NTMG. The early- versus late-onset NTMG studies demonstrated that patients in the former category were 1.95× likelier than their late-onset counterparts to achieve clinical remission (odds ratio [OR] 1.95; 95% confidence interval [CI] 1.39–2.73; I2 = 0%). No difference was seen in improvement or remission + improvement rates between these two groups. When comparing thymectomy with conservative treatments in late-onset NTMG patients, neither did we observe any difference in CSR/PR. Conclusion We found that late-onset NTMG patients had a lower chance of achieving CSR after thymectomy than early-onset patients. Thymectomy in late-onset NTMG also yielded no benefit to CSR or PR compared with conservative treatments. In late-onset NTMG patients, thymectomy should therefore be performed with caution, and the appropriate cutoff between early- and late-onset MG should be further explored in order to tailor and execute the proper therapeutic strategies.

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Well-Differentiated Papillary Mesothelioma of the Tunica Vaginalis: Case Report and Systematic Review of Literature

Clinical Genitourinary Cancer ◽

10.1016/j.clgc.2016.03.007 ◽

2016 ◽

Vol 14 (4) ◽

pp. e435-e439 ◽

Cited By ~ 5

Author(s):

Wei Keith Tan ◽

Mae-Yen Tan ◽

Wei Shen Tan ◽

Soon Ching Gan ◽

Rajadurai Pathmanathan ◽

...

Keyword(s):

Systematic Review ◽

Case Report ◽

Review Of Literature ◽

Tunica Vaginalis ◽

Well Differentiated

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An Uncommon Presentation of Myasthenia Gravis with Thymoma - A Case Report and Review of Literature

Journal of Medicine ◽

10.3329/jom.v13i1.10087 ◽

1970 ◽

Vol 13 (1) ◽

pp. 109-114

Author(s):

MA Hannan ◽

Mohammad Sayeed Hassan ◽

Ayesha Begum ◽

AKM Nurul Kabir

Keyword(s):

Case Report ◽

Neuromuscular Junction ◽

Myasthenia Gravis ◽

Diurnal Variation ◽

Rare Disease ◽

Liquid Food ◽

Review Of Literature ◽

Upper Arm ◽

Uncommon Presentation ◽

Incorrect Diagnosis

Myasthenia Gravis (MG) is a rare disease of neuromuscular junction which typically presents with fatigable weakness of cranial and limb muscles. But patient may present with vague symptoms which may mislead physicians to an incorrect diagnosis. We are reporting a 45 year old cobbler presented with the complaints of lack of energy, aching pain in shoulder, back, upper arm and difficulty in swallowing of both solid and liquid food without any diurnal variation for two and half years. He was ultimately diagnosed as a case of MG and thymectomy revealed thymoma. DOI: http://dx.doi.org/10.3329/jom.v13i1.10087 JOM 2012; 13(1): 109-114

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Faculty Opinions recommendation of Coronavirus Disease (Covid-19) Associated Mucormycosis (CAM): Case Report and Systematic Review of Literature.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.739505445.793585416 ◽

2021 ◽

Author(s):

Gavin Koh

Keyword(s):

Systematic Review ◽

Case Report ◽

Review Of Literature

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Outcome measures and treatment effectiveness in late onset myasthenia gravis

Neurological Research and Practice ◽

10.1186/s42466-020-00091-z ◽

2020 ◽

Vol 2 (1) ◽

Author(s):

Francesca Pasqualin ◽

Silvia V. Guidoni ◽

Mario Ermani ◽

Elena Pegoraro ◽

Domenico M. Bonifati

Keyword(s):

Side Effects ◽

Myasthenia Gravis ◽

Outcome Measures ◽

Clinical Features ◽

Early Onset ◽

Treatment Effectiveness ◽

Late Onset ◽

Positive Outcome ◽

Response To Treatment

Abstract Background Recently different subtypes of myasthenia gravis (MG) have been described. They differ for clinical features and pathogenesis but the prognosis and response to treatment is less clear. The aim of the study was to evaluate outcome and treatment effectiveness including side effects in late onset MG (LOMG) compared with early onset MG (EOMG). Methods We analysed retrospectively 208 MG patients. Clinical features were recorded as well as treatment and side effects. Outcome at the last follow-up was evaluated with MGSTI and MGPIS scales. Results The 208 patients included were classified as follow: 36 ocular MG, 40 EOMG, 72 LOMG, 25 thymoma-associated, 14 anti-MuSK and 21 double seronegative. Similar positive outcome was achieved in either early and late onset subgroup. We found pharmacological remission and minimal manifestations at the MGFA-PIS in the 95% and 94,4% of EOMG and LOMG respectively but in LOMG a lower dose of immunosuppressors (MGSTI< 2) was required compared to EOMG (p = 0,048). Severe side effects were present in a small percentage of patients in both group but diabetes was more frequent in LOMG vs EOMG (2,2% vs 5%, p = 0.017). Conclusions Despite LOMG has more comorbidities that might interfere with treatment and outcome, therapeutic management does not seem to differ between EOMG and LOMG. A similar positive outcome was seen in both subgroups but LOMG group seems to require lower doses of medication to control symptoms.

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