Basilar artery hypoplasia associated with changes of brainstem potential, transcranial Doppler and perfusion-weighted imaging.

OBJECTIVE: The aim of this study was to observe brainstem hemodynamic alterations associated with basilar artery hypoplasia (BAH). METHODS: Nine hundred and fifty-two consecutive patients received emergency multimodal computed tomography; magnetic resonance imaging and magnetic resonance angiogram during the period of January 2011 to December 2014 were included. The vascular risk factors, brainstem auditory evoked potential (BAEP), blink reflex (BR), transcranial Doppler (TCD) and dynamic susceptibility contrast-enhanced perfusion-weighted imaging were completed. RESULTS: There was significant difference in the abnormal rates of TCD and BAEP between BAH and non-BAH patients. A positive correlation between basilar artery diameter and systolic velocity among BAH patients was suggested. V-wave value was used to predict posterior circulation infarction (PCI) with the sensitivity of 0.933 and specificity of 0.50 with the cutoff value of 5.97 s. Abnormal BR rate was also significantly different in BAH and non-BAH patients. The latency of R2 was used to predict PCI with the sensitivity of 0.933 and specificity of 0.50 with the cutoff value of 46.4 ms. The incidence of hypoperfusion was higher in BAH than non-BAH group and it was significant difference. CONCLUSION: BAH is closely associated with hemodynamic alterations within the pons, which might contribute to vascular vertigo due to regional hypoperfusion.

Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort.

Transcranial Doppler (TCD) is used to detect children with sickle cell anemia (SCA) who are at risk for stroke, and transfusion programs significantly reduce stroke risk in patients with abnormal TCD. We describe the predictive factors and outcomes of cerebral vasculopathy in the Créteil newborn SCA cohort (n = 217 SS/Sß(0)), who were early and yearly screened with TCD since 1992. Magnetic resonance imaging/magnetic resonance angiography was performed every 2 years after age 5 (or earlier in case of abnormal TCD). A transfusion program was recommended to patients with abnormal TCD and/or stenoses, hydroxyurea to symptomatic patients in absence of macrovasculopathy, and stem cell transplantation to those with human leukocyte antigen-genoidentical donor. Mean follow-up was 7.7 years (1609 patient-years). The cumulative risks by age 18 years were 1.9% (95% confidence interval [95% CI] 0.6%-5.9%) for overt stroke, 29.6% (95% CI 22.8%-38%) for abnormal TCD, which reached a plateau at age 9, whereas they were 22.6% (95% CI 15.0%-33.2%) for stenosis and 37.1% (95% CI 26.3%-50.7%) for silent stroke by age 14. Cumulating all events (stroke, abnormal TCD, stenoses, silent strokes), the cerebral risk by age 14 was 49.9% (95% CI 40.5%-59.3%); the independent predictive factors for cerebral risk were baseline reticulocytes count (hazard ratio 1.003/L × 10(9)/L increase, 95% CI 1.000-1.006; P = .04) and lactate dehydrogenase level (hazard ratio 2.78/1 IU/mL increase, 95% CI1.33-5.81; P = .007). Thus, early TCD screening and intensification therapy allowed the reduction of stroke-risk by age 18 from the previously reported 11% to 1.9%. In contrast, the 50% cumulative cerebral risk suggests the need for more preventive intervention.

Unique anomalous origin of the left anterior cerebral artery.

We present the case of an anomalous origin of the left anterior cerebral artery (ACA) from the supraclinoid segment of the right internal carotid artery. Because of improved imaging quality, anomalies of the ACA-anterior communicating artery (AComA) complex are increasingly recognized on transaxial images. Although most of these anomalies are incidental findings of little clinical significance, some ACA-AComA complex anomalies are clinically significant. Recognition of these anomalies may be instrumental in developing a differential diagnosis or for improved surgical planning.

Anomalous internal carotid anastomosis to contralateral anterior cerebral artery.

BACKGROUND: Many anomalies and variants in vascular anatomy have been reported in relation to the anterior cerebral artery (ACA). PATIENTS AND METHODS: We encountered an apparently novel anomaly in a 30-year-old man admitted for disturbance of consciousness following a traffic accident. Computed tomography revealed an acute subdural hematoma and subarachnoid hemorrhage. RESULTS AND CONCLUSIONS: No vascular abnormalities related to the hemorrhage were detected by conventional angiography, so we concluded that the bleeding was of traumatic origin. Anomalous origin of the ACA was disclosed incidentally, with both A1 segments arising from the right internal carotid artery; no normal A1 segment of the left ACA was visualized. We discuss possible bases for this anomalous origin.

Subcortical lacunar lesions: an MR imaging finding in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

PURPOSE: To assess the prevalence and distribution of subcortical lacunar lesions (SLLs) in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), to determine whether SLLs are an abnormal finding by studying their prevalence in healthy subjects, and to assess whether SLLs occur in other conditions associated with small vessel disease and white matter areas of high signal intensity (WMH). MATERIALS AND METHODS: The presence of SLLs, their location, and their relation to other abnormalities were assessed on magnetic resonance (MR) images (T1-weighted, T2-weighted, and fluid-attenuated inversion-recovery) obtained in 34 CADASIL patients and 20 healthy family members. Three additional control groups of healthy volunteers, elderly patients with vascular risk factors, and patients with another hereditary small vessel disease were also screened for the presence and location of SLLs. Sensitivity and specificity of the presence of SLLs for the diagnosis of CADASIL were assessed. RESULTS: SLLs were found in 20 (59%) of CADASIL patients. Incidence of SLLs increased with age (20%, <30 years; 50%, 30-50 years; 80%, >50 years). SLLs invariably occurred in the anterior temporal lobes and in areas where diffuse WMH expanded into arcuate fibers. From the anterior temporal lobe, the lesions could extend dorsally into the temporal lobes and rostrally into the frontal lobes. Lesions were not found in the parietal and occipital lobes. None of the control subjects had SLLs. Specificity and sensitivity of SLLs for CADASIL were 100% and 59%, respectively. CONCLUSION: SLLs are an abnormal finding at MR imaging that frequently occur in CADASIL patients.

Cerebral artery stenoses in Williams syndrome cause strokes in childhood.

Extensive narrowing of lumens of many cerebral arteries caused strokes with brain damage and chronic hemipareses in two children with Williams syndrome. Increased irritability, loss of consciousness, and seizures were initial signs. Arterial stenoses are not limited to the supravalvular aorta and pulmonary arteries in patients with Williams syndrome.

Lipoma of the corpus callosum associated with distal anterior cerebral artery aneurysm. A case report.

We report what may be the first case of lipoma of the corpus callosum associated with a distal anterior cerebral artery saccular aneurysm. The pathogenetic correlation between the callosal lipoma and the distal anterior cerebral artery saccular aneurysm is discussed.

[Relation between pulsed and continuous Doppler velocimetry of the anterior cerebral artery in newborn infants]. / Beziehung zwischen gepulster und kontinuierlicher Doppler-Velozimetrie der Arteria cerebri anterior bei Neugeborenen.

Continuous wave (cw) and pulsed wave (pw) Doppler velocimetry are both used to measure blood flow velocity in cerebral arteries. We examined whether data obtained with the two methods are interchangeable and equally useful for clinical application. We studied 20 infants at an age between 1 day and 2 1/2 months and a body weight between 1.2 and 3.4 kg. Cw Doppler measurements were performed using an Angiomatic (Medimatic) and pw measurements using a Mark 600 Duplex Scanner (ATL). We applied both methods within an interval as short as possible. For the data analysis the pulsatility index (PI) was used, a parameter fairly independent of the emission angle. PI was 0.66 +/- 0.06 (mean +/- SD) using the cw and 0.8 +/- 0.08 using the pw method. The linear correlation between the pulsatility indices obtained with the cw and the pw method respectively was statistically significant (r = 0.53, n = 20, p less than 0.0025), the variation around the regression line rather large. The slope of the regression line differed significantly from that of the identity line (y = 0.391x + 0.54). Therefore the PI values obtained with the two methods are not interchangable and require to be judged according to normal values. Both methods, however, yield PI values which correlate and can thus be equally used. The choice of the method rather depends on the goal to be achieved. The pw method seems to be more suitable for precise momentaneous descriptions and the cw method for continuous monitoring of cerebral blood flow.

[Antenatal cerebral artery occlusions (4 new cases)]. / Occlusions artérielles cérébrales anténatales (4 nouvelles observations).

Cerebral arterial occlusion, occurring during monozygotic twin pregnancies, is a well known entity, specially when one twin is stillborn or macerated. Most of the authors consider that the aetiology is either embolization from the dead fetus or intravascular coagulation causing embolization in the surviving co-twin via placental vascular connections. Some data suggest an infectious endarteritis on the basis of angiographic and histological findings. Clinical features may appear several weeks after birth. CT scan and pathological findings depend on the date of the arterial occlusion. Four recent cases are described, two of them concerning monozygotic twin pregnancies. Pathological, CI scan, clinical features and pathogenesis are discussed taking account of the others data in the literature.