Tako-tsubo cardiomyopathy in a patient with bilateral lesions in the dorsal medulla
- First Online:
- Cite this article as:
- Berganzo, K., Ciordia, R., Gómez-Esteban, J.C. et al. Clin Auton Res (2011) 21: 65. doi:10.1007/s10286-010-0088-0
- 124 Views
Tako-tsubo-like cardiomyopathy (TTC) is much more common than originally thought. The exact pathophysiology of TTC is unclear. The most accepted theory proposes myocardial stunning of neurogenic origin, supported by the frequent antecedent of emotional or physical stress, suggesting a catecholamine-mediated mechanism. We present a patient with this syndrome and bilateral damage of the dorsal medulla oblongata likely affecting both solitary tract nuclei. Our case points to a link between baroreflex failure and TTC, highlighting the important role of sympathetic discharge in the pathophysiology of TTC.
KeywordsTako-tsubo cardiomyopathySolitary tract nucleusBaroreflex failure
The autonomic nervous system plays an essential role in regulating blood pressure and heart rate variability. The most important autonomic cell groups are located in the brainstem .
Autonomic dysfunction is rarely reported in focal brainstem lesions, such as tumors, syringobulbia, paraneoplastic encephalitis and stroke. Extensive brainstem damage in the region of the solitary tract nuclei may result in baroreflex dysfunction [1, 2, 3].
We report the case of a 37-year-old woman who in the course of an inflammatory lesion of the medulla oblongata developed acute myocardial dysfunction despite normal coronariography findings. We discuss some pathophysiological issues derived from this observation.
A 37-year-old woman with no medical antecedents was admitted to our service because of a sudden episode of dizziness. Physical examination revealed vertical nystagmus, with no other abnormal findings. Approximately 20 h later the patient developed a rapidly ascending sensory disturbance of the lower limbs. Both the spinal cord and brain magnetic resonance imaging (MRI) were normal. The cerebrospinal fluid was normal without oligoclonal bands. Iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy was performed to rule out the presence of pheochromocytoma. Neurophysiological tests (electromyography, electroencephalography, visual evoked potentials, somatosensory evoked potentials and brainstem auditory evoked potentials) were normal. Studies to rule out occult malignancy (body computed tomography, breast ultrasound, body positron emission tomography and tumor markers) yielded normal results. The serum titer of antibodies to Mycoplasma pneumonia by IgM ELISA was 1:320. The neurological symptoms and signs improved spontaneously during the first week after admission. Nevertheless, the patient received a course of levofloxacin 500 mg/12 h for 10 days. She was discharged free of symptoms.
Approximately 48 h later, while jogging, she experienced a sudden episode of palpitations, breathlessness and sensory disturbance in all four extremities. Initially, she presented high blood pressure (170/100 mmHg) without bradycardia (85 bpm), but a few minutes later she became hypotensive (80/50 mmHg) with bradycardia (60 bpm). The electrocardiogram tracing upon admission showed ST-segment elevation in the high lateral leads, ST-segment depression in V3-V6 and inferior leads and bradycardia (58 bpm) compatible with myocardial infarction. Both serum creatine kinase MB (90.4 U/l) and troponin T (0.65 ng/ml) were increased. The echocardiogram showed global hypokinesia of the left ventricular wall with severe mitral insufficiency and an estimated ejection fraction of 35%, but coronariography detected no significant changes in the coronary arteries. The condition was thus taken to be suggestive of tako-tsubo cardiomyopathy (TTC). The patient required vasoactive drugs for 36 h to maintain a systolic blood pressure of 90 mmHg (dopamine 7 μg/h).
High-dose intravenous methylprednisolone was administered, with improvement of the neurological symptoms. Complete recovery of the electrocardiographic and echocardiographic alterations occurred over time, confirming TTC. Three months after discharge the patient remained asymptomatic, and control MRI showed disappearance of the FLAIR hyperintensities.
The final diagnosis was probable acute disseminated encephalomyelitis associated to Mycoplasma pneumoniae infection, with brainstem involvement and TTC.
Our patient met the diagnostic criteria of TTC: akinesis or dyskinesia of the apical and/or midventricular segments of the left ventricle, with regional wall motion abnormalities that extended beyond the distribution of a single epicardial vessel and the absence of obstructive coronary artery disease . TTC syndrome is much more common than originally thought . It occurs mainly in middle aged and elderly women (over 80% of all cases), and is often preceded by physical or mental stress, as in our patient.
The exact pathophysiology of TTC is unclear. The most widely accepted theory proposes myocardial stunning of neurogenic origin, supported by the frequent antecedent of emotional or physical stress, suggesting a catecholamine-mediated mechanism . To date, TTC has been reported secondary to subarachnoid hemorrhage, epilepsy, electroconvulsive therapy, pheochromocytoma  and hyperthyroidism. Our patient presumably had a bilateral inflammatory lesion in the dorsal medulla oblongata that preceded TTC, likely affecting both solitary tract nuclei, which are involved in regulating blood pressure. Impulses arising from baroreceptors converge in this center. Bilateral lesions of these nuclei, as in our case, lead to loss of baroreflex control of blood pressure . The manifestations of baroreflex failure in humans range from an acute fulminant hypertensive crisis to blood pressure and heart rate lability syndrome [3, 8]. Our patient suffered a hypertensive crisis in the emergency department before developing heart failure.
In conclusion, our case contributes two important aspects on this topic: it is the first published case of TTC caused by focal brainstem injury and, most importantly, it points to a link between baroreflex failure and TTC. This finding would highlight the important role of sympathetic discharge in the pathophysiology of TTC.
The authors report no conflicts of interest.