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Background:
Myasthenia gravis associated takotsubo syndrome is a rare condition. This study aimed to explore its typical presentation, investigations and treatment through a systematic review of previously reported cases.
Methods:
Databases and reference lists of the selected articles were searched for case reports on Myasthenia gravis associate...
Context in source publication
Citations
... MC-induced TC is a rare and deadly combination [7] . The majority of cases (88%) were contributed from the Western Pacific, American, and European regions [8] . No cases from Nepal and Southern Asia are described in published literature. ...
Introduction and importance
Myasthenic Crisis(MC) is characterized by severe weakness in bulbar and respiratory muscles.Takotsubo Cardiomyopathy (TC) is a rare clinical entity mainly associated with postmenopausal woman. We report a case of both these condition in a premenopausal woman.
Case presentation
A 31-year-old woman with hypothyroidism presented with dyspnea. Bedside echocardiography in the intensive care unit revealed an apical ballooning with an ejection fraction of 25%,and she was treated with losartan, furosemide, and spironolactone. She was intubated after 2 days as she developed respiratory distress and type II respiratory failure. Upon investigation, the patient tested positive for anti-acetylcholine receptor antibody. Treatment with 5 doses of IVIg was given and she made a remarkable recovery. Repeat echocardiography revealed her ejection fraction is normal and cardiac function is resolved.
Clinical Discussion
The association between Takotsubo cardiomyopathy and myasthenic crisis is unusal and not commonly observed. Myasthenic crisis can be a natural progression of myasthenia gravis or due to stressors, such as infection, medicine, pregnancy, and surgery. Stressful events can lead to TC. This leads to the possibility of TC along with other cardiac complications in patients with MC.
Conclusion
Patients with MC may be at potential risk of developing TC, thus careful cardiac monitoring is necessary while treating them for a better prognosis.
... History of diabetes mellitus, hypertension, atrial fibrillation as in [25] and smoking have been found in minority of Open Access Library Journal the cases, most cases have been cured by anticholinergic (pyridostigmine), NSA-IDs (prednisone), immunoglobulines, plasma exchange as in [1] and ventilatory support for mytheniccrisis as in [4] and [26], and dobutamine for takotsubo cardiomyopathy, vasopressor and inotropes have been led to a poor outcomes including death and intractable heart failure as in [12]. Broken heart syndrome have been occurred on second day of admission with mythenia gravis as in [27] and [28], vital signs have been demonstrated normal blood pressure without tachycardia or bradycardia as in [12]. ...
... Most of the cases have been occurred among patients from Western pacific, American and European regions as in [27], and there were no cases reported from African and Eastern Mediterranean regions. Most of the cases have been occurred among female (Table 1) because takotsubo cardiomyopathy is most likely associated with postmenopausal female, but it can be occurred among male gender also. ...
... Occurrence of takotsubo cardiomyopathy in patients with myasthenia gravis is a rare condition but a life threatening, so this study is made to find out the treatment and methods of prevention of it by analyzing the previous cases reported, and it must be understood clearly and avoided by avoiding stressful conditions as in [27] and triggering of mythenic crisis, and further studies should be focused in understanding the mechanism of occurrence for controlling it well. ...
... [27][28][29] Patients with myasthenic crisis or GBS can develop takotsubo (stress) cardiomyopathy. 30,31 Supportive measures used in other patients in the ICU should be implemented for patients with neuromuscular respiratory failure including but not limited to infection prevention measures, sedation and analgesia balanced with daily awakening, and VTE prophylaxis. Physical therapy consultation can be extremely beneficial. ...
... Its pathogenesis is debated and attributed to a coronary and peripheral vasospasm caused by an adrenergic overstimulation, metabolic alterations, or inflammatory mechanisms all triggered by emotional and physical stressors [1]. Myasthenia gravis (MG) has been associated with TC, especially when a myasthenic crisis (MC) occurs [2]. ...
... Whether MC and TC can be considered causatively associated or concomitant events due to partial overlapping of common triggers is difficult to unveil [3], and the pathophysiology of TC during a MC is yet not fully understood [2]. The role of catecholamine surge triggered by emotional and physical stressors has been proposed, as well as a cardiac dysfunction secondary to MG-related autoimmune mechanisms, reversible vasospasm, microcirculatory dysfunction, and respiratory infections [4]. ...
... Therapy includes supportive and symptomatic treatment [1]. Beta-adrenergic and calcium channel antagonists should be handled carefully for the risk of triggering a MC [2]. ...
... Broken Heart Syndrome is also known as s t r e s s c a r d i o m y o p a t h y , s t r e s s -i n d u c e d cardiomyopathy, takotsubo cardiomyopathy, reversible left ventricular dysfunction, Apical Ballooning Cardiomyopathy or Apical Ballooning Syndrome (ABS), or neurogenic myocardial stunning. In 1998, a left ventriculogram in the Circulation journal caught the attention of many doctors due to the term "Broken heart" given by [1][2][3] the author. ...
Broken Heart Syndrome (BHS) is the weakness of the heart muscle due to emotional stress or physical stress called cardiomyopathy. The main etiology is a sudden release of stress hormones (catecholamines), such as norepinephrine, epinephrine, and dopamine. About 90% of BHS patients are female with average age of 67-70"‰ years, most of them are post-menopausal females. The most widely supported pathological theories are catecholamine-induced cardiotoxicity and microvascular dysfunction. The clinical condition resembles that of acute myocardial infarction, consisting of chest pain, electrocardiographic changes, elevated cardiac biomarkers, and abnormalities of heart wall motion. There is transient systolic dysfunction in the apical and/or middle segment of the left ventricle resembling acute myocardial infarction but absence of coronary artery obstructive disease. There are BHS criteria according to Mayo Clinic. Laboratory tests can be performed by examining Natriuretic Peptides, cardio myonecrosis markers (Troponin I and T, creatinine kinase, and myoglobin), and catecholamines. There is no single established biomarker for initial diagnosis of BHS that distinguishes it from STEMI. It was found that the most accurate ratio as a marker capable of differentiating BHS from STEMI in early stages was NTproBNP/TnI ratio. The InterTAK diagnostic score was used to predict the probability of BHS, differentiating it from ACS in an acute stage, prior to coronary angiography. The main differential diagnosis of BHS is ACS, besides acute myocarditis infectious. Patients with BHS should be treated as ACS until proven otherwise. The prognosis for BHS patients is generally very good.
... In earlier observational reports, IVIG was effective in nearly 70 to 75 percent of patients with MG [23,24]. Compared with AChR-Abpositive MG, IVIG is less effective in MuSK-Ab-positive MG, with only 20% to 61% of muscle-specific kinase antibody-positive MG patients in previous studies improved with IVIG [25][26][27]. In terms of the results of this study, compared with the clinical score, the improvement rate of the PE +immunoglobulin group was higher (P < 0:05). ...
Background:
Myasthenia gravis (MG) is an acquired autoimmune disease. The main clinical features of MG are skeletal muscle fatigue and pathological fatigue, which worsen at night or after fatigue, such as dyspnea, dysphagia, and systemic weakness. Plasma exchange (PE) is often used in patients with acute exacerbation of MG. Intravenous immunoglobulin (IVIG) is a collection of immunoglobulins from thousands of donors. IVIG can replace a variety of immunosuppressants or PE. However, the effect of PE or IVIG on patients' consciousness, immune function, and prognosis is not clear.
Objective:
A prospective randomized test of the effects of PE combined with immunoglobulin on consciousness, immune function, and prognosis in patients with myasthenia gravis crisis (MGC).
Methods:
Sixty patients with MGC treated from February 2019 to April 2021 were enrolled in our hospital. The cases who received PE were set as the PE group, and those who received PE combined with immunoglobulin were set as the PE+immunoglobulin group. The efficacy, clinical score, state of consciousness, immune function, acetylcholine receptor antibody (AChR-Ab), lymphocyte (LYM), albumin (ALB) levels, and the incidence of adverse reactions were compared.
Results:
The improvement rate was 100.005% in the treatment group and 83.33% in the PE group. After treatment, the clinical score of the PE+immunoglobulin group was lower than that of the PE group, and the clinical relative score of the PE+immunoglobulin group was higher than that of the PE group (P < 0.05). The number of conscious people in the PE+immunoglobulin group was more than that in the PE group (P < 0.05). Immunoglobulin A, immunoglobulin M, immunoglobulin G, and immunoglobulin G in the PE+immunoglobulin group were higher than those in the PE group (P < 0.05). The levels of AChR-Ab and ALB in the PE+immunoglobulin group were higher than those in the PE group, while the level of LYM in the PE+immunoglobulin group was lower than that in the PE group. The incidence of skin system, gastrointestinal system, nervous system, and systemic damage in the PE+immunoglobulin group was lower than that in the PE group (P < 0.05).
Conclusion:
The treatment of MGC with PE combined with immunoglobulin can not only effectively enhance the consciousness and immune function of patients but also effectively promote the prognosis, and the safety of treatment can be guaranteed.
... Myasthenic crisis was thought to be superimposed on Takotsubo syndrome. A small number of cases have been reported describing a combination of these two diseases, all of which are Takotsubo syndrome associated with myasthenic crisis [9,10]. Takotsubo syndrome following myasthenic crisis had a 15-fold higher prevalence, nearly two-fold higher allcause mortality, and higher resource utilization than Takotsubo syndrome caused by other etiologies [10]. ...
Background Takotsubo syndrome and myasthenic crisis can be triggered by physical stress. We present the case of a woman who developed Takotsubo syndrome and myasthenic crisis following radiocontrast media-induced anaphylaxis. Case Report A 39-year-old woman presented with diplopia and ptosis. After chest computed tomography scan, her consciousness was stupor and her oxygen saturation decreased. Electrocardiography showed ST elevation, and cardiac enzyme levels increased. Echocardiography revealed severe left ventricular dysfunction. Myasthenia gravis was diagnosed based on anti-acetylcholine receptor antibody and repetitive nerve stimulation test. Extubation failed, and her weakness worsened. Her neurological condition gradually improved after steroid therapy. Repeat echocardiography demonstrated complete recovery of left ventricular dysfunction. Conclusion Takotsubo syndrome can be triggered by anaphylaxis and can occur in patients with neurological disorders; therefore, neurologists need to know about this disorder. The combination of Takotsubo syndrome and myasthenic crisis is rare but may be associated with a poor prognosis.
... Our patient, however, had a myasthenic crisis and there have been case reports published showing a link between MG and TCM. In a systematic review of all documented cases of TCM with MG [5], 88% of cases were found in the Western Pacific, American, and European regions with 81% females affected, thus mirroring that TCM is a disease of mainly post-menopausal women [6]. This retrospective case review further observed that in more than half the cases, there were left systolic dysfunction with elevated troponin levels and T-wave inversions. ...
Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction that affects skeletal muscles causing weakness, typically the ocular, facial, oropharyngeal, respiratory, and limb muscles. Patients can present as either an MG exacerbation with weakness of any muscle group or an MG crisis which is a life-threatening weakness of the respiratory muscles that usually requires intubation and mechanical ventilation; however, though rare, cardiac manifestations must be considered in the management of such patients.
... A high level of catecholamine in the blood triggers the transformation of signaling in the ventricular myocytes from Gs (stimulatory) to Gi (inhibitory) proteins via beta-2-adrenergic receptors, which has a negative inotropic effect and thus contributes to Takutsubo or stress cardiomyopathy. TC and MC share similar precipitants like emotional or physical stress, infection, and pain, among others [16]. Therefore, a common precipitant could have triggered them together. ...
A literature review shows scarce reports of myasthenic crises (MC) complicated by Takotsubo cardiomyopathy (TC). This patient cohort (0.11%) has higher all-cause mortality and prolonged in-hospital course. We present a rare case of a 72-year-old man who developed cardiogenic shock post-plasmapheresis for myasthenia crisis. He became hemodynamically unstable and developed acute respiratory failure requiring intubation 30 minutes after completion of plasma exchange. Serum troponin peaked at 3.19 ng/mL while an emergent 12-lead electrocardiogram (EKG) showed new-onset diffuse ST-segment elevation. Hypokinesis of the entire apex, anterior septum, mid-and apical inferior septum, and mid-and apical inferior wall consistent with Takotsubo cardiomyopathy was seen on bedside echocardiogram. The patient received a continuous infusion of norepinephrine and vasopressin. The hospital course was complicated by multiorgan failure and eventual demise. This case highlights MC and the potential of plasma exchange therapy to induce TC.
... The goal of the inflammatory immune response is to inactivate and eliminate the antigen (173). Acute TTS attacks have been reported in patients with the infections of organ systems, including sepsis, chronic inflammation, autoimmune and demyelinating diseases (174)(175)(176)(177)(178)(179)(180)(181)(182). Immunopathological mechanisms have also been detected behind the attacks induced by allopurinol, influenza B, transfusion reaction, or influenza vaccination (56, 57, [183][184][185]. ...
Indirect evidences in reviews and case reports on Takotsubo syndrome (TTS) support the fact that the existence of oxidative stress (OS) might be its common feature in the pre-acute stage. The sources of OS are exogenous (environmental factors including pharmacological and toxic influences) and endogenous, the combination of both may be present, and they are being discussed in detail. OS is associated with several pathological conditions representing TTS comorbidities and triggers. The dominant source of OS electrones are mitochondria. Our analysis of drug therapy related to acute TTS shows many interactions, e.g., cytostatics and glucocorticoids with mitochondrial cytochrome P450 and other enzymes important for OS. One of the most frequently discussed mechanisms in TTS is the effect of catecholamines on myocardium. Yet, their metabolic influence is neglected. OS is associated with the oxidation of catecholamines leading to the synthesis of their oxidized forms – aminochromes. Under pathological conditions, this pathway may dominate. There are evidences of interference between OS, catecholamine/aminochrome effects, their metabolism and antioxidant protection. The OS offensive may cause fast depletion of antioxidant protection including the homocystein-methionine system, whose activity decreases with age. The alteration of effector subcellular structures (mitochondria, sarco/endoplasmic reticulum) and subsequent changes in cellular energetics and calcium turnover may also occur and lead to the disruption of cellular function, including neurons and cardiomyocytes. On the organ level (nervous system and heart), neurocardiogenic stunning may occur. The effects of OS correspond to the effect of high doses of catecholamines in the experiment. Intensive OS might represent “conditio sine qua non” for this acute clinical condition. TTS might be significantly more complex pathology than currently perceived so far.
