Echocardiographic Evidence of Innate Aortopathy in the Human Intracranial Aneurysm

<div><p>Background</p><p>Intracranial aneurysm (IA) is significantly more prevalent in patients with coarctation of the aorta or bicuspid aortic valve than in the general population, suggesting a common pathophysiology connecting IA and aortopathy. Here, we analyzed echocardiographic aortic root dimension (ARD) in patients with IA to confirm this possibility.</p><p>Methods</p><p>From January 2008 to December 2010, 260 consecutive patients with IA who were admitted to our institution for coil embolization or for acute stroke management and who also underwent echocardiography were enrolled. We hypothesized that patients with large, ruptured, or multiple IAs are more likely to harbor co-prevalent aortopathy as measured by ARD compared to patients with small, isolated, unruptured IAs. Eccentric group was defined as patients aged <55 years with at least one ruptured aneurysm, an aneurysm ≥7 mm in size, or multiple aneurysms; the remainder was classified into a non-eccentric group. Clinical, angiographic, and echocardiographic findings of the two groups were compared.</p><p>Results</p><p>ARD was significantly larger in the eccentric group than in the non-eccentric group (<i>P</i> = 0.049), and the difference was confirmed by multivariable analysis (<i>P</i> = 0.02). Subgroup analysis of patients aged <55 years showed similar result for ARD (<i>P</i> = 0.03), whereas hypertension was more associated with the non-eccentric group (<i>P</i> = 0.01). In addition, height was inversely related to aneurysm size after adjustment for age, sex, weight, ARD, smoking status, and number of aneurysms (<i>P</i> = 0.004).</p><p>Conclusions</p><p>A certain group of IA patients share a common intrinsic wall defect with aortopathy. Shared neural crest cell origin may give rise to this phenomenon.</p></div

Age distribution of the study population.

<p>(a) Age distribution of the total population (<i>n</i> = 260) was bimodal; there were a large number of patients aged 50–54 years. (b) The stroke patients (<i>n</i> = 57) were relatively old, and the distribution was unimodal. (c) Age distribution of the coiled patients (<i>n</i> = 206) demonstrate another peak which contributed to the bimodality of the total population. Patients with (d) large IAs (≥7, <i>n</i> = 64), (e) multiple aneurysms (<i>n</i> = 75), and (f) a ruptured aneurysm (<i>n</i> = 41) also showed bimodal age distributions. The labels on the <i>x</i>-axis indicate mean ages of 5-year age groups starting at age 20.</p

Scatter plot of aneurysm size versus height.

<p>Aneurysm size tended to decrease with increasing height. The fitted regression line for the association is shown as a solid line with the 95% confidence intervals shown as dotted lines (<i>R</i>² = 0.046).</p

Results of multivariable logistic regression analysis of determinants of eccentricity.

<p>*Patients with ischemic stroke, transient ischemic attack, or coronary artery disease (angina pectoris or myocardial infarction) were included.</p><p>BSA: body surface area; OR: odds ratio; CI: confidence interval; CAD: coronary artery disease; ARD: aortic root dimension; LA: left atrial.</p

Subgroup analysis of patients aged <55 years.

<p>OR: odds ratio; CI: confidence interval; ARD: aortic root dimension.</p

Identification of cerebral perfusion using arterial spin labeling in patients with seizures in acute settings

<div><p>This study aimed to explore the utility of arterial spin labeling perfusion-weighted imaging (ASL-PWI) in patients with suspected seizures in acute settings. A total of 164 patients who underwent ASL-PWI for suspected seizures in acute settings (with final diagnoses of seizure [n = 129], poststroke seizure [n = 18], and seizure mimickers [n = 17]), were included in this retrospective study. Perfusion abnormality was analyzed for: (1) pattern, (2) multifocality, and (3) atypical distribution against vascular territories. Perfusion abnormality was detected in 39% (50/129) of the seizure patients, most (94%, 47/50) being the hyperperfusion pattern. Of the patients with perfusion abnormality, multifocality or hemispheric involvement and atypical distribution against vascular territory were revealed in 46% (23/50) and 98% (49/50), respectively. In addition, seizures showed characteristic features including hyperperfusion (with or without non-territorial distribution) on ASL-PWI, thus differentiating them from poststroke seizures or seizure mimickers. In patients in whom seizure focus could be localized on both EEG and ASL-PWI, the concordance rate was 77%. The present study demonstrates that ASL-PWI can provide information regarding cerebral perfusion status in patients with seizures in acute settings and has the potential to be used as a non-invasive imaging tool to identify the cerebral perfusion in patients with seizures.</p></div

Perfusion patterns in seizure patients (convulsive vs. nonconvulsive) with known time intervals between the last seizure events and MR scans.

<p>Perfusion patterns in seizure patients (convulsive vs. nonconvulsive) with known time intervals between the last seizure events and MR scans.</p

A 33-year-old woman who visited the emergency department for a nonconvulsive seizure.

<p>(A) Brain parenchyma appears normal on the T2 FLAIR image. (B) A tiny dot-like diffusion restriction is noted at the left parietal cortex (arrowhead). (C) Arterial spin labeling perfusion-weighted MR image depicts hyperperfusion at the left middle cerebral artery territory (arrow). Note the combined hypoperfusion portion adjacent to the hyperperfused area (arrowhead). (D) MR angiography reveals occlusion at the left M2 segment (arrowhead).</p

Perfusion patterns in seizure patients (generalized vs. partial) with known time intervals between the last seizure events and MR scans.

<p>Perfusion patterns in seizure patients (generalized vs. partial) with known time intervals between the last seizure events and MR scans.</p

A 71-year-old man with underlying cavernous malformation who presented to the emergency department with a seizure.

<p>(A) T2 FLAIR MR image demonstrates a cavernous malformation (arrow) with a hemoderin rim and typical popcorn ball appearance secondary to multiple locules containing hemorrhage at the left frontal lobe. (B) Diffusion-weighted image shows no abnormal signal change around the cavernous malformation (arrow). (C) Arterial spin labeling perfusion-weighted MR image, however, reveals prominent perilesional hyperperfusion (arrowheads) surrounding the cavernous malformation (arrow) at the left frontal lobe. (D) Additional hyperperfusion foci (arrowheads) are also noted at the left parietal lobe.</p