BACKGROUND: Apical ballooning syndrome (ABS) and posterior reversible encephalopathy syndrome (PRES) are recently described, seemingly unrelated, reversible conditions. The precise pathophysiology of these syndromes remains unknown. The aim of this study was to describe the clinical characteristics and outcomes of a unique series of patients with both ABS and PRES. METHODS AND RESULTS: In a retrospective study of 224 consecutive patients diagnosed with ABS between 2002 and 2010, 6 (2.7%) were also diagnosed with PRES. All were female with a mean age of 63.7 ± 12.5 years. All patients had preceding medical comorbidities and physical stress triggers that precipitated ABS and PRES. Mean peak troponin T levels and left ventricular ejection fraction at presentation were 0.47 ± 0.48 mg/dL and 31.5 ± 8.2%, respectively. Characteristic left ventricular wall motion abnormalities (regional wall motion score index 2.22 ± 0.37) were noted in all patients, and magnetic resonance imaging of the brain was significant for vasogenic edema, predominantly in the posterior circulation. All patients recovered left ventricular (ejection fraction at follow-up 60.2 ± 6.0%) and neurologic function with supportive management. Two patients had recurrence of ABS and 1 of PRES during follow-up. CONCLUSIONS: ABS and PRES can occur simultaneously during an acute illness. Patients with ABS who develop neurologic dysfunction should be evaluated for PRES and vice versa. Because transient sympathetic overactivity and microvascular dysfunction have been observed in both reversible syndromes, we speculate that they may represent the shared pathophysiologic mechanism.
BACKGROUND:Apical ballooning syndrome (ABS) and posterior reversible encephalopathy syndrome (PRES) are recently described, seemingly unrelated, reversible conditions. The precise pathophysiology of these syndromes remains unknown. The aim of this study was to describe the clinical characteristics and outcomes of a unique series of patients with both ABS and PRES. METHODS AND RESULTS: In a retrospective study of 224 consecutive patients diagnosed with ABS between 2002 and 2010, 6 (2.7%) were also diagnosed with PRES. All were female with a mean age of 63.7 ± 12.5 years. All patients had preceding medical comorbidities and physical stress triggers that precipitated ABS and PRES. Mean peak troponin T levels and left ventricular ejection fraction at presentation were 0.47 ± 0.48 mg/dL and 31.5 ± 8.2%, respectively. Characteristic left ventricular wall motion abnormalities (regional wall motion score index 2.22 ± 0.37) were noted in all patients, and magnetic resonance imaging of the brain was significant for vasogenic edema, predominantly in the posterior circulation. All patients recovered left ventricular (ejection fraction at follow-up 60.2 ± 6.0%) and neurologic function with supportive management. Two patients had recurrence of ABS and 1 of PRES during follow-up. CONCLUSIONS:ABS and PRES can occur simultaneously during an acute illness. Patients with ABS who develop neurologic dysfunction should be evaluated for PRES and vice versa. Because transient sympathetic overactivity and microvascular dysfunction have been observed in both reversible syndromes, we speculate that they may represent the shared pathophysiologic mechanism.
Authors: Stephan Grimaldi; Emilie Doche; Caroline Rey; Nadia Laksiri; Salah Boussen; Jacques Quilici; Emmanuelle Robinet; Fabien Devemy; Jean Pelletier Journal: Case Rep Neurol Date: 2017-07-11