scholarly journals Granulomatous Myositis Associated with Extremely Elevated Anti-striated Muscle Antibodies in the Absence of Myasthenia Gravis

Cureus ◽  
2020 ◽  
Author(s):  
Sreenath Meegada ◽  
Hamza Akbar ◽  
Suman Siddamreddy ◽  
David Casement ◽  
Rajanshu Verma
Keyword(s):  
Myasthenia Gravis ◽  
Striated Muscle ◽  
Granulomatous Myositis

Paraneoplastic myasthenia gravis: Detection of anti-MGT30 (titin) antibodies predicts thymic epithelial tumor

Neurology ◽  
1997 ◽  
Vol 49 (5) ◽  
pp. 1454-1457 ◽  
Author(s):  
R. D. Voltz ◽  
W. C. Albrich ◽  
A. Nägele ◽  
F. Schumm ◽  
M. Wick ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Predictive Value ◽  
Striated Muscle ◽  
Immunofluorescence Assay ◽  
Epithelial Tumor ◽  
Thymic Epithelial Tumors ◽  
Thymic Epithelial Tumor ◽  
Antibody Assays ◽  
Better Than ◽  
Titin Antibodies

It has been suggested that antibodies against nonacetylcholine receptor proteins of striated muscle are markers of the presence of a thymic epithelial tumor in patients with myasthenia gravis (MG). These antibodies may be measured using an immunofluorescence assay against striated muscle(anti-STR) or an ELISA with a recombinant 30-kd titin fragment (anti-MGT30). To directly compare anti-STR with anti-MGT30, we examined the sera of 276 consecutive patients with known or suspected MG. Definite diagnoses and thymic histology, if available, were correlated with the antibody assays. Of the 276 patients, 164 had MG. Thymic histology was obtained in 44 patients: 18 had lymphofollicular hyperplasia, 13 thymic epithelial tumors, 8 atrophy, and 5 were normal. When compared with anti-STR, anti-MGT30 showed a sensitivity of 69% (STR 77%), specificity of 100% (STR 56%, p = 0.026), negative predictive value of 82% (STR 77%), and positive predictive value of 100% (STR 56%, p = 0.003) for the identification of a thymic epithelial tumor versus thymic hyperplasia. We conclude that the anti-MGT30 ELISA is better than the anti-STR immunofluorescence assay for the diagnosis of paraneoplastic MG.

scholarly journals Myasthenia Gravis in Pregnancy Treated With Daily Massive Vitamin D Dose

2020 ◽  
Vol 3 (2) ◽  
pp. 171-177
Author(s):  
Silva LFF ◽  
Martins MC ◽  
Caplum MC ◽  
Luz MS ◽  
Amaral VM ◽  
...  
Keyword(s):  
Vitamin D ◽  
T Cells ◽  
Myasthenia Gravis ◽  
Striated Muscle ◽  
First Trimester ◽  
Effector T Cells ◽  
Myasthenic Crisis ◽  
First Trimester Of Pregnancy ◽  
Muscle Groups ◽  
In Pregnancy

Myasthenia gravis (MG) is an autoimmune disease affecting the motor endplate of striated muscle. It is caused by antibodies that act in the cholinergic receptors at the post-synaptic portion of the neuromuscular junction, which results in asthenia and fatigue in some muscle groups. In pregnancy, it’s unpredictable, because pregnant women can present MG exacerbation, remission, stability, or even a myasthenic crisis during pregnancy. Complications are more frequent in the first trimester of pregnancy and the first 30 days of puerperium. Vitamin D and its metabolites are potent immunomodulators since their immuno-regulatory effect directly inhibits effector T cells and induces regulatory T cells (Treg) to decrease the production of inflammatory cytokines. The authors present a case report of a patient with MG who was treated throughout pregnancy with massive doses of vitamin D, obtaining good results.

NCMP-18. FIXED PTOSIS AS A SYMPTOM OF MYOSITIS SECONDARY TO CHECKPOINT INHIBITION

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi150-vi150
Author(s):  
Ashley Aaroe ◽  
Sudhakar Tummala
Keyword(s):  
Myasthenia Gravis ◽  
Striated Muscle ◽  
Rapid Onset ◽  
Cancer Center ◽  
High Dose ◽  
Post Discharge ◽  
Institutional Review ◽  
Number Of Patients ◽  
Average Maximum

Abstract BACKGROUND A growing number of patients with cancer receive checkpoint inhibitor (CPI) based immunotherapy. Peripheral nervous system toxicities including myositis, myasthenia gravis and inflammatory neuropathy are distinct from their non immunotherapy-related equivalents, with unique clinical presentations and therapeutic considerations. METHODS Patients with CPI myositis and non-fatigable ptosis were identified, and clinical data was retrospectively extracted from the electronic medical record in compliance with MD Anderson Cancer Center Institutional Review Board guidelines. RESULTS 14 patients were identified. Average maximum creatine kinase (CK) was 3571 U/L (range 20-19,794). 9 patients had electromyography and nerve conduction studies documented in our system; all had electrodiagnostic evidence of myopathy and two had evidence of concomitant myasthenia gravis by electrodecrement. Two muscle biopsies revealed myositis with inflammatory T-cell infiltrate. 6 had positive anti-striated muscle antibody titers, 9/14 had concomitant myocarditis, 2 had hepatitis, 2 had pneumonitis and 1 had thyroiditis. All received high dose steroids, 11 received plasma exchange, 4 received rituximab, 4 received tacrolimus and 2 received tocilizumab. 7/14 patients died (50%). Mean time from initial neurology consultation for symptoms to death was 6.46 months (range .3-24). 5 patients had documented clinical stabilization or mild improvement on post-discharge follow-up. CONCLUSIONS While ptosis in patients receiving CPI can initially suggest myasthenia gravis, in patients with rapid onset and ultimately fixed deficits there should be a high index of suspicion for immunotherapy related restricted localized myositis. Myositis may coincide with other toxicities, such as myocarditis and pneumonitis, and can be fatal despite aggressive treatment. Deficits persist on post-discharge follow-up, suggesting protracted recovery for patients who survive.

MYASTHENIA GRAVIS IMMUNOLOGICAL RELATIONSHIP BETWEEN STRIATED MUSCLE AND THYMUS

The Lancet ◽  
1966 ◽  
Vol 287 (7428) ◽  
pp. 57-60 ◽  
Author(s):  
HugoW.R. Van Der Geld ◽  
ArthurJ.L. Strauss
Keyword(s):  
Myasthenia Gravis ◽  
Striated Muscle ◽  
Immunological Relationship

scholarly journals Granulomatous Myositis Associated with Myasthenia Gravis: A Rare Case

Cureus ◽  
2019 ◽  
Author(s):  
Shumaila M Iqbal ◽  
Linda Burns ◽  
Cassandra Zhi
Keyword(s):  
Myasthenia Gravis ◽  
Rare Case ◽  
Granulomatous Myositis

scholarly journals Myasthenia gravis and thymoma: evaluation of 41 patients

1999 ◽  
Vol 57 (1) ◽  
pp. 6-13 ◽  
Author(s):  
JOSÉ LAMARTINE DE ASSIS ◽  
ANTONIO ALBERTO ZAMBON ◽  
PATRICIA S. SOUZA ◽  
PAULO EURIPEDES MARCHIORI
Keyword(s):  
Myasthenia Gravis ◽  
Striated Muscle ◽  
Clinical Laboratory ◽  
Anterior Mediastinum ◽  
Clinical Findings ◽  
Malignant Thymoma ◽  
Results Of Treatment ◽  
Clinical Investigations ◽  
Immunological Tests ◽  
And Control

We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36%) were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.

scholarly journals Contracturing Granulomatous Myositis with Recurrent Malignant Thymoma and Myasthenia Gravis: A Case Report

2021 ◽  
pp. 1-5
Author(s):  
Ching On Luk ◽  
Wai Ting Joyce Lo ◽  
Kitty Yan Kwok ◽  
Moon Ho Leung ◽  
Wing Chi Fong ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Case Reports ◽  
Immunoglobulin A ◽  
Chemotherapy Treatment ◽  
Malignant Thymoma ◽  
History Of ◽  
Antibody Levels ◽  
Granulomatous Myositis ◽  
Anti Acetylcholine

Contracturing granulomatous myositis (CGM) is a very rare myopathic syndrome where patients present with slowly progressive muscle pain or weakness affecting mainly proximal muscles together with flexion contractures of the limbs. To date, there have only been several case reports of CGM, which have been reported to occur most commonly in association with sarcoidosis. We report a case of CGM associated with recurrent malignant thymoma and myasthenia gravis (MG). A gentleman with history of treated malignant thymoma presented with new onset proximal muscle weakness and pain that later evolved into contractures that persisted despite treatment with steroids and intravenous immunoglobulin. A malignancy workup revealed recurrence of his malignant thymoma warranting chemotherapy treatment. His contractures only resolved after addition of chemotherapy treatment. He later developed clinical features of MG 1 year after his initial elevation in Anti-acetylcholine (Anti-AChR) antibody levels. We postulate CGM to be a paraneoplastic phenomenon of underlying malignant thymoma. Treatment of thymoma together with immunosuppressive therapies led to clinical improvement of our patient’s CGM. The sole elevation of Anti-AChR levels without the accompanying clinical features does not equate to a MG diagnosis, but should raise the clinician’s vigilance for future development of the condition.

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