Non-Progressive Leukoencephalopathy with Bilateral Temporal Cysts

We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. Cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.

Coronary Revascularization Induces a Shift From Cardiac Toward Noncardiac Mortality Without Improving Survival in Vascular Surgery Patient

OBJECTIVE: Although evidence has shown that ischemic heart disease (IHD) in vascular surgery patients has a negative impact on the prognosis after surgery, it is unclear whether directed treatment of IHD may influence cause-specific and overall mortality. The objective of this study was to determine the prognostic implication of coronary revascularization (CR) on overall and cause-specific mortality in vascular surgery patients. METHODS: Patients undergoing surgery for abdominal aortic aneurysm, carotid artery stenosis, or peripheral artery disease in a university hospital in The Netherlands between January 2003 and December 2011 were retrospectively included. Survival estimates were obtained by Kaplan-Meier and Cox regression analysis. RESULTS: A total of 1104 patients were included. Adjusted survival analyses showed that IHD significantly increased the risk of overall mortality (hazard ratio [HR], 1.50; 95% confidence interval, 1.21-1.87) and cardiovascular death (HR, 1.93; 95% confidence interval, 1.35-2.76). Compared with those without CR, patients previously undergoing CR had similar overall mortality (HR, 1.38 vs 1.62; P = .274) and cardiovascular mortality (HR, 1.83 vs 2.02; P = .656). Nonrevascularized IHD patients were more likely to die of IHD (6.9% vs 35.7%), whereas revascularized IHD patients more frequently died of cardiovascular causes unrelated to IHD (39.1% vs 64.3%; P = .018). CONCLUSIONS: This study confirms the significance of IHD for postoperative survival of vascular surgery patients. CR was associated with lower IHD-related death rates. However, it failed to provide an overall survival benefit because of an increased rate of cardiovascular mortality unrelated to IHD. Intensification of secondary prevention regimens may be required to prevent this shift toward non-IHD-related death and thereby improve life expectancy.