LAUSR

Demyelinating diseases are not classically associated with takotsubo cardiomyopathy. Recently, Androdias et al. reported on 5 cases of multiple sclerosis (MS) with takotsubo cardiomyopathy associated with new demyelinating lesions in the medulla oblongata on brain magnetic resonance imaging (MRI). We report on the case of a 49-year-old woman without medical history presenting with takotsubo cardiomyopathy during a rapidly progressive tetraplegia and brainstem syndrome, which was attributed to neuromyelitis optica (NMO). She developed tetraplegia over 1 week associated with hyporeflexia, dysarthria, dysphagia, oculomotor impairment, urine retention, tachycardia, hypotension, and respiratory failure leading to mechanical ventilation. Cerebrospinal fluid analysis showed 210 lymphocytes/mm, elevated protein level (2.0g/l; N< 0.5), and the presence of oligoclonal bands with elevated immunoglobulin G index (0.75; N< 0.70). An electrocardiogram showed sinus tachycardia without ST-segment modification, associated with elevated serum troponin levels (1,327ng/l). Echocardiography revealed severe left ventricular dysfunction (ejection fraction, 20%) with basal akinesia and residual apical mobility, typical of reverse takotsubo cardiomyopathy. Cardiac MRI showed no evidence of myocardial ischemia or myocarditis. Cardiac dysfunction fully recovered after 3 days. MRI revealed the presence of longitudinally extensive transverse myelitis (LETM), and lesions of the medulla oblongata, corpus callosum, and left temporal lobe with gadolinium enhancement, suggestive of NMO (Figure). Antiaquaporin 4 (antiAQP4) antibodies were positive in the serum (cell-based assay), confirming the NMO diagnosis. Despite five sessions of plasma exchange, high-dose intravenous methylprednisolone (12g total), and rituximab therapy, neurological outcome was poor at 2 months with persisting tetraplegia, facial diplegia, and tracheostomy. Takotsubo cardiomyopathy is very rare in neurological diseases. Catecholamine release attributed to lesions involving bulbar autonomic regulatory areas is thought to cause sympathetic overactivation responsible for heart failure. To the best of our knowledge, this is the first case of takotsubo cardiomyopathy occurring in NMO. Only 2 cases of LETM of unknown origin with brainstem involvement and takotsubo cardiomyopathy were previously described, one in a 10-year-old boy and another in a 27-year-old woman. However, in these reports, the localization of cardiac hypokinesia (basal/apical) and antiAQP4 antibodies were not assessed. Only a few cases of takotsubo cardiomyopathy were described in central nervous system (CNS) demyelinating diseases, especially in young MS patients with good neurological recovery (16to 27-year-old). In these reports, active inflammatory lesions involving the medulla oblongata and a reverse takotsubo cardiomyopathy were most frequently observed, as in our case. This observation suggests that MS and NMO share some common features occasionally causing takotsubo cardiomyopathy, possibly attributed to the localization of demyelinating lesions in the medulla oblongata.