A. Gravos et al present a case of a 65-year-old Greek woman with Guillain-Barré syndrome who developed cardiomyopathy shortly after admission to the intensive care unit due to respiratory deterioration. Her estimated left ventricular ejection fraction upon admission was 20%. The result of coronary angiography was negative for coronary artery disease, and cardiac magnetic resonance imaging excluded myocarditis. Her clinical condition improved with supportive therapy, and her estimated left ventricular ejection fraction at discharge was normal.
We present a case of a 65-year-old Greek woman who presented to the neurology ward of our hospital with a 1-week history of symmetrical weakness of her lower limbs, numbness, and paresthesia of her upper limbs, and dysarthria. Her medical, family and psychosocial histories were unremarkable. She was not receiving any medication at the time of her presentation, and she had no allergies. She only reported an upper respiratory viral infection 2 weeks ago.
On neurological examination, the patient’s motor strength was 4/5 in her upper extremities and 1/5 in her lower extremities. The tendon reflexes were absent, and there was no cranial nerve involvement. Initially, there were no associated cardiac symptoms, no neuromuscular respiratory weakness (vital capacity [VC] > 20 ml//kg and maximal inspiratory pressure [MIP] > 30 cm H2O), and no hypercapnia (partial pressure of carbon dioxide [PCO2] = 38 mmHg) in arterial blood gas analysis. The patient was afebrile (36.8 °C), had normal ECG findings (sinus rhythm ~ 80 beats/min), and was hemodynamically stable (mean arterial pressure [MAP] = 70 mmHg).
Initial cerebral magnetic resonance imaging (MRI) findings were normal. Both neurophysiological and cerebrospinal fluid (CSF) examinations were consistent with the diagnosis of GBS. Thus, CSF examination showed elevated protein level (450 mg/L) with normal cells (2/mm3), and electrodiagnostic testing showed temporal dispersion, significantly slow conduction velocities, prolonged distal and F-wave latencies, and abnormal upper extremity sensory nerve conduction. The patient’s laboratory test results upon admission were normal. Treatment with intravenous immunoglobulin on day 0 over a 5-day period (400 mg/kg/day) was started.
One day after admission to the neurology ward, intubation was necessary because of progressive respiratory failure (VC < 15 ml/kg and MIP < 20 cm H2O, PCO2 = 60 mmHg, pH = 7.24) due to muscle weakness and mucus plugging, and the patient was transferred to the intensive care unit (ICU). Shortly after uncomplicated intubation (for which she received midazolam 10 mg and propofol 150 mg, without cytochalasin), a marked increase in heart rate (sinus rhythm ~ 150 beats/min) was noted, and the patient became hemodynamically unstable (MAP = 50 mmHg), despite fluid loading.
To rule out pulmonary embolism, computed tomography (CT) was performed, which only revealed atelectasis of the left lower lobe and no signs of pulmonary embolism. In the following hours, antibiotics, additional fluids, high-dose norepinephrine (80 μg/min), and hydrocortisone were administered. The patient’s MAP remained low (60 mmHg), tachycardia persisted (sinus rhythm ~ 120 beats/min), and urine output ceased. ECG revealed sinus tachycardia with nonspecific ST-T segment changes.
Blood culture results and control for viral infections were negative. Laboratory tests revealed normal white blood cells; normal platelets and hematocrit; normal liver, thyroid, and kidney function; normal creatine kinase (CK = 56 U/L, normal < 145 U/L), but elevated troponin I (598 ng/L, normal < 14 ng/L) and N-terminal pro-brain natriuretic peptide (1391 pmol/L, normal < 15 pmol/L). Urgent TTE was performed, which revealed dilated and severe hypokinetic left ventricle, normal heart valves, normal right ventricle, and lack of pericardial effusion.
The estimated left ventricular ejection fraction (LVEF) was 20%. A new ECG was performed, which showed inverted T-waves in leads I, avL, and V2–V6. Urgent coronary angiography to exclude coronary artery disease was performed, which was normal, but ventriculography revealed severe diffuse hypokinesis of the left ventricle. To exclude myocarditis, the patient underwent cardiac MRI on the tenth day of admission, which showed no signs of ischemia, fibrosis, or edema.
On the basis of echocardiographic findings, it was concluded that the patient had stress cardiomyopathy or fulminant myocarditis. Dobutamine infusion was initiated (15 μg/kg/min) to assist left ventricular contractility, to reduce norepinephrine infusion, and to reduce afterload, despite persistent tachycardia. This resulted in a gradual increase in blood pressure and the return of diuresis. The next day, additional furosemide was given because of a positive fluid balance and signs of pulmonary congestion on a chest x-ray. In the next 48 h, dobutamine was tapered because the patient gradually became hemodynamically stable. Due to persistent sinus tachycardia, metoprolol was introduced stepwise over the next 3 days to reduce the sympathetic tone and improve myocardial work/oxygen consumption ratio; the maintenance dose was 50 mg twice daily. After hemodynamic stabilization, a low dose of ramipril (2.5 mg/day) was also introduced. The diagnosis of myocarditis was excluded by cardiac MRI on the tenth day of admission.
Follow-up and outcomes
Repeat follow-up TTE showed gradual normalization of LVEF in the next few days. Because of difficult weaning, the patient underwent tracheostomy on day 15, and she was discharged from the ICU on day 28 on spontaneous breathing. ECG and LVEF at discharge were normal, but the patient still had heavy peripheral, symmetrical, and especially motor polyneuropathy. New neurophysiological testing was not performed.