Contributions
Abstract: EP1440
Type: ePoster
Abstract Category: Clinical aspects of MS - 11 Comorbidity
Background: Takotsubo is very rare in neurological diseases, where a release of catecholamine due to bulbar vegetative areas lesions could lead to sympathetic overactivation. Demyelinating diseases were not classically associated with takotsubo cardiomyopathy. Recently, Androdias et al. reported five cases of multiple sclerosis (MS) with takotsubo associated with a new demyelinating lesion in the medulla oblongata on brain MRI were reported.
Objective: To report the case of a reverse takotsubo during an acute tetraplegia and brainstem syndrome revealing neuromyelitis optica (NMO).
Results: A 49 year-old woman without significant medical history, developed tetraparesia with dysarthria, dysphagia, oculomotor impairment, urine retention, tachycardia and hypotension leading to mechanical ventilation over one week. CSF analysis revealed a lymphocytic cellular reaction (210/mm3), an elevated protein level (2 g/L) and presence of oligoclonal bands with elevated IgG index. Electrocardiogram showed a sinus tachycardia without ST-segment modification associated with elevated troponine levels (1327 ng/L). Echocardiography revealed a severe left ventricular dysfunction (ejection fraction of 20%) with basal akinesia and residual apical mobility, typical of reverse takotsubo. Cardiac MRI showed no argument in favor of myocardial ischemia or myocarditis. MRI revealed the presence of longitudinal extensive transverse myelitis (LETM), lesions of the medulla oblongata, corpus callosum and left temporal lobe with gadolinium enhancement. Anti-aquaporin 4 antibodies (anti-AQP4) were positive, confirming the diagnosis of NMO. Cardiac dysfunction fully recovered in three days while neurologic outcome at 2 months was very poor despite plasma exchange, high dose steroids and Rituximab.
Discussion: To our knowledge, this is the first reported case of takotsubo occurring in NMO. Only two cases of LETM with brainstem involvement and takotsubo were previously described in a 10 year-old boy and in a 27 year-old woman, without confirmation of NMO diagnosis. The five cases of MS with takotsubo recently reported were also younger (16-27 year-old) with good neurological recovery. As in our case, reverse takotsubo was observed in 3 of them.
Conclusion: This observation suggests that MS and NMO share some common features in relationship with takotsubo, possibly due to the localization of inflammatory lesions in the medulla oblongata in both demyelinating diseases.
Disclosure:
P Prin: nothing to disclose
Y Rodriguez: nothing to disclose
C Heroum: nothing to disclose
C Molinero: nothing to disclose
E Thouvenot has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Biogen Idec, Genzyme-Sanofi, Merck, Novartis, Roche, and TEVA.
Abstract: EP1440
Type: ePoster
Abstract Category: Clinical aspects of MS - 11 Comorbidity
Background: Takotsubo is very rare in neurological diseases, where a release of catecholamine due to bulbar vegetative areas lesions could lead to sympathetic overactivation. Demyelinating diseases were not classically associated with takotsubo cardiomyopathy. Recently, Androdias et al. reported five cases of multiple sclerosis (MS) with takotsubo associated with a new demyelinating lesion in the medulla oblongata on brain MRI were reported.
Objective: To report the case of a reverse takotsubo during an acute tetraplegia and brainstem syndrome revealing neuromyelitis optica (NMO).
Results: A 49 year-old woman without significant medical history, developed tetraparesia with dysarthria, dysphagia, oculomotor impairment, urine retention, tachycardia and hypotension leading to mechanical ventilation over one week. CSF analysis revealed a lymphocytic cellular reaction (210/mm3), an elevated protein level (2 g/L) and presence of oligoclonal bands with elevated IgG index. Electrocardiogram showed a sinus tachycardia without ST-segment modification associated with elevated troponine levels (1327 ng/L). Echocardiography revealed a severe left ventricular dysfunction (ejection fraction of 20%) with basal akinesia and residual apical mobility, typical of reverse takotsubo. Cardiac MRI showed no argument in favor of myocardial ischemia or myocarditis. MRI revealed the presence of longitudinal extensive transverse myelitis (LETM), lesions of the medulla oblongata, corpus callosum and left temporal lobe with gadolinium enhancement. Anti-aquaporin 4 antibodies (anti-AQP4) were positive, confirming the diagnosis of NMO. Cardiac dysfunction fully recovered in three days while neurologic outcome at 2 months was very poor despite plasma exchange, high dose steroids and Rituximab.
Discussion: To our knowledge, this is the first reported case of takotsubo occurring in NMO. Only two cases of LETM with brainstem involvement and takotsubo were previously described in a 10 year-old boy and in a 27 year-old woman, without confirmation of NMO diagnosis. The five cases of MS with takotsubo recently reported were also younger (16-27 year-old) with good neurological recovery. As in our case, reverse takotsubo was observed in 3 of them.
Conclusion: This observation suggests that MS and NMO share some common features in relationship with takotsubo, possibly due to the localization of inflammatory lesions in the medulla oblongata in both demyelinating diseases.
Disclosure:
P Prin: nothing to disclose
Y Rodriguez: nothing to disclose
C Heroum: nothing to disclose
C Molinero: nothing to disclose
E Thouvenot has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Biogen Idec, Genzyme-Sanofi, Merck, Novartis, Roche, and TEVA.