Non-triggered Takotsubo-syndrome in metabolic myopathy.

Takotsubo-syndrome (TTS) is characterized by sudden onset chest-pain and dyspnea, electrocardiographic changes resembling acute coronary syndrome, minimal elevation of cardiac enzyme levels and transient wall-motion abnormalities in the absence of coronary artery obstruction [1]. Although TTS occurs frequently after emotional or physical stress, non-triggered TTS, is reported in 10–66% of the cases [2,3]. We report a female with TTS in whom the trigger remained speculative despite extensive investigations but neurological investigations suggested a metabolic myopathy. A 74-year-old Caucasian female, with a height of 159 cm, weight of 53 kg, and with a history of arterial hypertension was hospitalized at age 71 years because of sudden onset of severe anginal chest pain, dyspnea, vertigo, and diarrhea. Her blood pressurewas 185/115 mmHg. The ECG showed ST-elevations in leads V5 and V6 and negative T-waves in leads II, V3–V6. Echocardiography revealed a moderately reduced left ventricular systolic function with akinesia of the septal and apical parts. Since troponin-T and serum CK (175, normal b141 U/l) were elevated, a non-ST-elevation myocardial infarction was diagnosed. Two days later she developed pulmonary edema with atrial fibrillation, and transient disturbance of consciousness. Since chest X-ray showed an infiltrate in the right upper lobe and C-reactive proteinwas increased to 13.4 mg/dl (normal b0.5 mg/dl), ciprofloxacin was started. After stabilization of her hemodynamic situation and restoration of sinus rhythm with diuretics, ATII-blockers and beta-blockers, she was referred to another hospital for cardiac catheterization three days after admission. Surprisingly, coronary arteries were without stenoses but ventriculography showed apical ballooning (Fig. 1). Pro-BNP was 40,700 pg/ml (n,b95 pg/ml). Thedosagesof diuretics and sartaneswere increased. The further course was uneventful and she was discharged after 7 days. Follow-up echocardiography and ECG-investigations showed normalization of both. She was referred to the neurologist because of easy fatigability, muscle aching, irregularly occurringmuscle cramps since age 22 years and slightly elevated muscle enzymes. She also reported pain on both foot soles when staying or walking. She complained about depressive mood and had a history of a number of non-neurological problems, such as hepatopathy, steatosis hepatis, renal insufficiency, hyperlipidemia, hyperuricemia, previous hypothyroidism, paroxysmal atrial fibrillation, arterial hypertension, anemia, thrombopenia, psoriasis, previous cataract surgery, previous resection of a lipoma on the right upper arm, glaucoma, compression fractures of vertebra Th11 and L5, and polyarthralgia. At age 64 years she experienced recurrent diarrhea due to diphyllobothriasis requiring adequate treatment. Clinical neurologic investigation at age 70 and 71 years revealed narrow lid fissures, weak head anteflexion, sore neck muscles, proximal weakness of the upper and lower limbs, distal wasting on the upper limbs, flexion contractures of the fingers (camptodactylia), reduced Achilles tendon reflexes, achillodynia, and abnormal fat distribution resembling lipodystrophy. Cerebral CT showed old ischemic lesions in the internal capsule bilaterally. MRI of the lumbar spine revealed scoliosis, multi-segmental osteochondrosis, and fractures of vertebrae Th11 and L5. Though nerve conduction studies and electromyography were normal, metabolic myopathy was suspected and muscle biopsy recommended but refused by the patient for unknown reasons. During the following 3 years she remained cardiorespiratory stable without recurrence of TTS. She had been hospitalized two times because of emesis, diarrhea, renal failure and elevation of hepatic enzymes, which resolved after volume replacement. Repeated echocardiograms did not show any wall motion abnormalities but stress testing had to be discontinued because of muscle weakness at 63% of the expected exercise level. Pharmacotherapy at the last follow-up comprised of simvastatin (20 mg/d), furosemide (30 mg/ d), acetylsalicylic acid (100 mg/d), losartan (50 mg/d), pantoprazol (40 mg/d), and carvedilol (12.5 mg/d). Though the pathogenesis of TTS is unclear, several speculations have been put forward to explain the phenomenon. The most widely accepted pathogenetic theory assumes that TTS results from coronary microvessel spasms following catecholamine overstimulation and International Journal of Cardiology 154 (2012) e16–e18