Apical hypertrophic cardiomyopathy in encephalomyopathy

Apical hypertrophic cardiomyopathy (AHC) is associated with neurological abnormalities such as transient ischemic attack, stroke, limb-girdle muscular dystrophy, or eosinophilic myositis in single cases. The association of AHC and metabolic myopathy has not been reported. In an 84-year-old woman with long-standing gait disturbance, dementia, Parkinson syndrome, ptosis, ophthalmoparesis, tetraparesis, polyneuropathy, lactacidosis, polyarthralgia, dorsalgia, and osteoporosis, cardiac examination for long-standing anginal chest pain and palpitations, revealed supraventricular and monomorphic ventricular ectopic beats, hypertrophic signs, ST-depression and negative T waves on electrocardiogram (ECG), diastolic dysfunction with impaired relaxation, and AHC on transthoracic echocardiography. AHC was confirmed by cardiac magnetic resonance imaging, which additionally showed a small left ventricular apical aneurysm with a wall-thickness of only 3mm. The patient was suspected to additionally have a multisystem disease, most likely due to impaired oxidative metabolism. This case shows that AHC may take a mild course and be associated with a number of extracardiac abnormalities.