A rare case of Takotsubo cardiomyopathy associated with amyotrophic lateral sclerosis

Abstract

Background: Takotsubo cardiomyopathy (TCM), also known as an apical ballooning syndrome or stress cardiomyopathy, is a rare clinical syndrome defined as a profound but reversible left ventricular dysfunction in the absence of coronary artery disease. TCM is commonly associated with physical and emotional stress, but the pathophysiology is unknown. Here we present a 42-year-old female who develops TCM due to prolonged emotional stress causing the progression of motor neuron dysfunction. Case Presentation: A 42-year-old female presented to the ER with loss of consciousness after developing several bouts of cough followed by acute onset of breathlessness triggered by a prolonged stressful event for several months. She developed cardiac arrest, for which CPR was successfully performed. The family denied that she had chest pain, palpitations, and lower extremity edema. Echocardiography showed apical ballooning of the left ventricle (LV), distal septum, and apex appeared hypokinetic, with a reduced EF of 40%. Repeated screening echo showed improvement in EF alone. Prior to this event, the patient had a history of severe muscle weakness, fatigue, and bulbar symptoms for 8 months, breathing difficulty with weakness in both upper and lower limbs, loss of appetite, and weight loss of about 9 kgs in 3 months. She noticed progressive thinning of leg and arm muscles. There were no visual symptoms, sensory symptoms of bowel and bladder, or systematic symptoms. Her nerve conduction velocity was normal. Antibody testing for AchR, as well as MUSK antibodies, was negative. The patient died approximately 3 months after the first cardiac arrest. Discussion: Prolonged stress, myasthenic crisis, and hyperthyroidism can lead to TCM. Here we report a patient with clinical signs and symptoms of bulbar onset ALS who had prolonged emotional and physical stress that led to TCM, thereby progressing neuromuscular weakness. We advocate screening, careful cardiac monitoring, and management of Motor neuron disease (MND) patients, especially ALS, regularly and, more importantly, during acute emotional or physical stress, as there is a potential risk of developing TCM and rapid progression of MND/ALS symptoms leading to fatal respiratory failure.