ECTRIMS eLearning

Reverse takotsubo cardiomyopathy in the setting of active multiple sclerosis lesion involving the medulla oblongata
Author(s): ,
F. London
Affiliations:
Neurology, Université Catholique de Louvain, Yvoir
,
N. Gonzalez Rodriguez de Azero
Affiliations:
Neurology
,
M. Philippart
Affiliations:
Neurology
N. Mulquin
Affiliations:
Radiology, Université Catholique de Louvain, YVOIR, Belgium
ECTRIMS Learn. London F. 10/10/18; 229220; EP1381
Frédéric London
Frédéric London
Contributions
Abstract

Abstract: EP1381

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS symptoms

Background: Takotsubo cardiomyopathy (TTC) has been sporadically related to multiple sclerosis (MS) relapses involving the medulla oblongata. From a pathophysiological point of view, it is believed that the demyelinating brainstem lesions involve bulbar autonomic regulatory areas and lead to acute sympathetic pathway overactivation and subsequent massive release of catecholamines, triggering a transient myocardial stunning. The underlying mechanism remains however incompletely understood. To date, only 11 well-documented cases of TTC during MS exacerbations have been described.
Objective: To describe the case of a reverse TTC, a variant of classic TTC phenomenon, in the setting of acute brainstem MS relapse.
Results: A 30-year-old woman was diagnosed with relapsing-remitting MS in the beginning of March 2018 after she had a second clinical event requiring intravenous corticosteroids. Three weeks later, she presented to the emergency department with acute chest pain, palpitations and shortness of breath. She had hypoxemia and hypotension. Electrocardiogram showed repolarization abnormalities and atrial fibrillation with rapid ventricular response (150/min) but there was no ST-segment modification. Blood tests showed elevated serum troponin levels (peak value 6,71 ng/mL; N < 0,12). Echocardiography revealed a severe left ventricular dysfunction with decreased ejection fraction (20%; normal > 50%). Cardiac angiography demonstrated normal coronary arteries, but left ventriculography showed basal and midventricular akinesia with apical hyperkinesia, suggesting reverse TTC. Intensive care management was required. Extensive serologic workup for myocarditis was negative and urine catecholamine levels were normal. Neurological examination showed vertical nystagmus and cerebellar ataxia. Brain magnetic resonance imaging was performed and showed a newly formed acute MS lesion in the medulla oblongata with gadolinium-enhancement and also revealed 2 new left cerebellar lesions. The patient completed a seven-day course of high-dose intravenous corticosteroids (1000 mg/day). Cardiac dysfunction fully recovered in less than 3 weeks. The patient was tested negative for anti-JC virus antibodies and natalizumab was started.
Conclusion: Acute brainstem MS relapse should be considered in patients presenting with TTC, especially in the absence of other identifiable cause of acute heart failure occurring in young patients.
Disclosure: F London received travel grants from Biogen, Merck and Sanofi. His institution receives honoraria for consultancy and lectures from Biogen, Merck, and Sanofi. N Gonzalez Rodriguez de Azero: nothing to disclose. M Philippart: nothing to disclose. N Mulquin: nothing to disclose.

Abstract: EP1381

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS symptoms

Background: Takotsubo cardiomyopathy (TTC) has been sporadically related to multiple sclerosis (MS) relapses involving the medulla oblongata. From a pathophysiological point of view, it is believed that the demyelinating brainstem lesions involve bulbar autonomic regulatory areas and lead to acute sympathetic pathway overactivation and subsequent massive release of catecholamines, triggering a transient myocardial stunning. The underlying mechanism remains however incompletely understood. To date, only 11 well-documented cases of TTC during MS exacerbations have been described.
Objective: To describe the case of a reverse TTC, a variant of classic TTC phenomenon, in the setting of acute brainstem MS relapse.
Results: A 30-year-old woman was diagnosed with relapsing-remitting MS in the beginning of March 2018 after she had a second clinical event requiring intravenous corticosteroids. Three weeks later, she presented to the emergency department with acute chest pain, palpitations and shortness of breath. She had hypoxemia and hypotension. Electrocardiogram showed repolarization abnormalities and atrial fibrillation with rapid ventricular response (150/min) but there was no ST-segment modification. Blood tests showed elevated serum troponin levels (peak value 6,71 ng/mL; N < 0,12). Echocardiography revealed a severe left ventricular dysfunction with decreased ejection fraction (20%; normal > 50%). Cardiac angiography demonstrated normal coronary arteries, but left ventriculography showed basal and midventricular akinesia with apical hyperkinesia, suggesting reverse TTC. Intensive care management was required. Extensive serologic workup for myocarditis was negative and urine catecholamine levels were normal. Neurological examination showed vertical nystagmus and cerebellar ataxia. Brain magnetic resonance imaging was performed and showed a newly formed acute MS lesion in the medulla oblongata with gadolinium-enhancement and also revealed 2 new left cerebellar lesions. The patient completed a seven-day course of high-dose intravenous corticosteroids (1000 mg/day). Cardiac dysfunction fully recovered in less than 3 weeks. The patient was tested negative for anti-JC virus antibodies and natalizumab was started.
Conclusion: Acute brainstem MS relapse should be considered in patients presenting with TTC, especially in the absence of other identifiable cause of acute heart failure occurring in young patients.
Disclosure: F London received travel grants from Biogen, Merck and Sanofi. His institution receives honoraria for consultancy and lectures from Biogen, Merck, and Sanofi. N Gonzalez Rodriguez de Azero: nothing to disclose. M Philippart: nothing to disclose. N Mulquin: nothing to disclose.

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