scholarly journals Emergence of Myasthenia Gravis with Myositis in a Patient Treated with Pembrolizumab for Thymic Cancer

2018 ◽  
Vol 14 (1) ◽  
pp. 115 ◽  
Author(s):  
So-Young Huh ◽  
Seong-Hoon Shin ◽  
Meyung Kug Kim ◽  
So-Young Lee ◽  
Ki Hun Son ◽  
...  
Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012050
Author(s):  
Shahar Shelly ◽  
John R Mills ◽  
Divyansu Dubey ◽  
Andrew McKeon ◽  
Anastasia Zekeridou ◽  
...  

Objective:To critically assess the clinical utility of striational antibodies (StrAbs) within paraneoplastic and myasthenia gravis serological evaluations.Methods:All Mayo Clinic patients tested for StrAbs from January 1st 2012-December 31st 2018 utilizing Mayo’s Unified Data Platform (UDP) were reviewed for neurological diagnosis and cancer.Results:38,502 unique paraneoplastic and 1,899 MG patients were tested. In paraneoplastic evaluations, the StrAbs positivity rate was higher in cancer vs without cancer (5% [321/6775] vs 4% [1154/31727]; p<0.0001; OR 1.35; CI=1.19-1.53) but ROC analysis indicated no diagnostic accuracy in cancer (AUC=0.505). No neurological phenotype was significantly associated with StrAbs in the paraneoplastic group. Positivity was more common in all MG cancers compared to paraneoplastic cancers (p<0.0001). In MG evaluations, the StrAbs positivity rate was higher in those with cancer vs without (46% [217/474] vs 26% [372/1425]; p<0.0001; OR 2.39, CI 1.9-2.96) with ROC analysis indicating poor diagnostic accuracy for thymic cancer (AUC 0.634, recommended cutoff=1:60, sensitivity=56%, specificity=71%), with worse accuracy for extrathymic cancers (AUC 0.543). In paraneoplastic or MG evaluations, the value of antibody positivity did not improve cancer predictions. Paraneoplastic evaluated patients were more likely with positive StrAbs to obtain computed tomography (CT) (p=0.0001) with 3% (12/468) cancer found.Conclusion:Despite a statistically significant association with cancer, an expansive review of performance in clinical service demonstrates that StrAbs are neither specific nor sensitive in predicting malignancy or neurological phenotypes. CT imaging is over utilized with positive StrAbs results. Removal of StrAbs from paraneoplastic or MG evaluations will improve the diagnostic characteristics of the current MG test.Classification of Evidence:This study provides Class II evidence that the presence of StrAbs do not accurately identify patients with malignancy or neurological phenotypes.


2017 ◽  
Vol 46 (6) ◽  
pp. 277-281
Author(s):  
Yuuki Kou ◽  
Hajime Matsue ◽  
Tetsuya Kajiyama ◽  
Masaru Ishida ◽  
Hisashi Satoh ◽  
...  

2016 ◽  
Vol 22 ◽  
pp. 277-278
Author(s):  
Lima Lawrence ◽  
Aleida Rodriguez ◽  
Tahira Yasmeen ◽  
Erin Drever

2009 ◽  
Vol 56 (S 01) ◽  
Author(s):  
M Bensch ◽  
RP Van Wijk ◽  
C Schimmer ◽  
JH Krannich ◽  
I Aleksic ◽  
...  
Keyword(s):  

2014 ◽  
Vol 45 (01) ◽  
Author(s):  
C Eienbröker ◽  
F Seitz ◽  
A Spengler ◽  
H Kurz ◽  
M Seipelt ◽  
...  
Keyword(s):  

2016 ◽  
Vol 35 (10) ◽  
pp. 665-672
Author(s):  
S. Zierz ◽  
B. Jordan
Keyword(s):  

ZusammenfassungDie Thymuspathologie bei der Myasthenia gravis (MG) umfasst sowohl die lymphofollikuläre Hyperplasie (LFH) bei der Frühmanifestation der Azetylcholinrezeptorantikörper (AChR-AK) positiven Form als auch Thymome. Die Diagnostik schließt die Erhebung des Antikörperstatus ein, um die Indikation zur Thymektomie bei der klassischen AChR-Antikörper positiven Form prüfen zu können. Bei radiologischem Verdacht einer mediastinalen Raumforderung kann nuklearmedizinisch die Bindung von Somatostatinrezeptoren (DOTA-TOC-PET) bei Thymomen visualisiert werden. Die Thymektomie mit Thymomresektion ist unter onkologischem Aspekt grundsätzlich indiziert. Die Diagnose der paraneoplastischen MG besitzt somit eine besondere Indikatorfunktion für den weiteren Verlauf der Thymomerkrankung.Wenngleich die thymomassoziierte MG sich im klinischen Verlauf nicht von der klassischen Myasthenie unterscheidet, bedarf die Betreuung dieser Patienten besonderer Sorgfalt. Meist ist die Fortführung der Immunsuppression erforderlich. Gleichzeitig sind dabei die erhöhte Infektneigung, das erhöhte Risiko eines Zweitmalignoms als auch eines Thymomrezidivs zu beachten.


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