And remaining myoclonus was completely resolved by clonazepam 0


And remaining myoclonus was completely resolved by clonazepam 0.5?mg a day for 7?days. Open in a separate window Fig. unexplained nausea, vomiting, and cough. Myoclonus on the limbs developed afterward. Taken with suspicion of area postrema syndrome (APS), the brain MRI showed a demyelinating lesion in the medulla oblongata. APS and severe heart failure by stress cardiomyopathy were completely resolved by ECMO and hydrocortisone therapy. However, the CNS demyelinating lesion recurred after 1?month. The patient was diagnosed with NMO evident by the presence of aquaporin-4 antibody; Steroid therapy improved her symptoms. Conclusion NMO should be considered as one of the differential diagnoses in patients with APS preceding severe cardiopulmonary distress. strong class=”kwd-title” Keywords: Neuromyelitis optica, Stress cardiomyopathy, Takotsubo cardiomyopathy, Area postrema syndrome, Aquaporin-4 antibody Main text Background A rare but relevant association between stress cardiomyopathy (Takotsubo Goat polyclonal to IgG (H+L)(Biotin) cardiomyopathy) and multiple sclerosis (MS) has been reported in several case series [1C4]. Neuromyelitis Optica (NMO) is a rarer autoimmune demyelinating disorder of the central nervous system (CNS) by a different immunological mechanism with MS. Herein, we on report a patient with NMO who presented with area postrema syndrome (APS) and a catastrophic cardiopulmonary crisis by stress cardiomyopathy. Case presentation A 30-year-old woman without underlying disease suffered from unexplained nausea, vomiting, and cough without fever. After 1?month, she was admitted to the emergency department with suddenly deteriorated dyspnea 3-Hydroxyvaleric acid and myoclonic jerks on all limbs. At admission, the chest X-ray showed multifocal patchy ground-glass opacity in the bilateral lungs. ST elevation of the inferior leads on the electrocardiography and increased cardiac markers (creatine kinase-MB, 33.1?ng/mL; troponin I, 10989.0?pg/mL, and N-terminal-pro b-type natriuretic peptide, 3837?pg/mL) were observed. The echocardiography showed severe left ventricular (LV) systolic 3-Hydroxyvaleric acid dysfunction (LV Ejection fraction, LVEF 13%) and regional wall motion abnormalities (Fig.?1a,b). Coronary angiography conducted to exclude ischemic etiology was normal. Veno-arterial extracorporeal membrane oxygenation (ECMO) was performed to maintain her vitality under the suspicion of stress cardiomyopathy or acute myocarditis. After 2?days of ECMO with the administration of a vasopressor, hydrocortisone, and antibiotics, dyspnea and lung infiltration were rapidly improved. After 12?days, the LVEF was improved to 40% and was normal after 1?month. Cardiac magnetic resonance imaging (MRI) showed a high signal intensity in the T2-weighted image suggesting focal edema at 1?month after ECMO therapy. Pheochromocytoma was not detected on the abdominal computed tomography (CT) scan and in the 24-h urine collection. Open in a separate window Fig. 1 a, b Thoracic echocardiography showing akinesia on the base to the mid left ventricle (left, end-diastole; right, end-systole) With suspicion of nausea, vomiting, and myoclonus caused by medullary lesion, a brain MRI was done at 1?week after stopping ECMO. The brain MRI showed high signal 3-Hydroxyvaleric acid lesions in the medulla oblongata on the T2 and fluid attenuated inversion recovery (FLAIR) image (Fig.?2a,b,c,d). The cerebrospinal fluid (CSF) analysis was normal. Fluorescent antinuclear antibody (FANA) was positive with a homogenous pattern, but anti-dsDNA antibodies and Anti Ro/La-Ab were negative. There was no evidence of lupus or paraneoplastic syndrome in the clinical examination and abdomen and chest CT scan. Acute disseminated encephalomyelitis was the initial diagnosis for the medullary lesion and myoclonus. Her initial symptoms including nausea, vomiting, myoclonus, and cough were rapidly improved after the introduction of ECMO and hydrocortisone. And remaining myoclonus was completely resolved by clonazepam 0.5?mg a day for 7?days. Open in a separate window Fig. 2 a, b, c, d Brain MRI taken at 1?week after admission (a, b) Fluid attenuated inversion recovery (FLAIR) MRI showed high signal lesions in the medulla oblongata. (c, d) FLAIR with contrast enhancement MRI showed mild enhancement in the same area A predisposing neurologic event, rapid recovery.