Lhermitte-Duclos disease (LDD; dysplastic cerebellar gangliocytoma) is a rare hamartomatous lesion of the cerebellar cortex and this was first described in 1920. LDD is considered to be part of the autosomal-dominant phacomatosis and cancer syndrome Cowden disease (CS). We examined the brain of a 46-year-old man, who displayed the manifestations of CS, with 7 Tesla (T) and 1.5T MRI and 1.5T MR spectroscopy (1H-MRS). We discuss the possible benefits of employing ultrahigh-field MRI for making the diagnosis of this rare lesion.
Cerebellar Cortex/pathology ; Cerebellar Neoplasms/complications/*pathology ; Diagnosis, Differential ; Gait Ataxia/etiology ; Hamartoma Syndrome, Multiple/complications/*pathology ; Humans ; Image Processing, Computer-Assisted/methods ; Magnetic Resonance Imaging/*methods ; Magnetic Resonance Spectroscopy/methods ; Magnetics ; Male ; Middle Aged ; Vertigo/etiology

Purpose: Multi-sac aneurysms (MSAs) are not uncommon, but studies on their management are scarce. This study aims to evaluate and compare the feasibility, safety, and efficacy of MSAs treated with either clipping or coiling after interdisciplinary case discussion at our center. Materials and Methods: We retrospectively analyzed MSAs treated by microsurgical clipping, coiling, or stent-assisted coiling (SAC). Treatment modalities, complications, angiographic results, and clinical outcomes were evaluated. Major neurological events were defined as a safety endpoint and complete occlusion as an efficacy endpoint. Results: Ninety patients (mean age, 53.2±11.0 years; 73 [81.1%] females) with MSAs met our inclusion criteria (clipping, 50; coiling, 19; SAC, 21). Most aneurysms were located in the middle cerebral artery (48.9%). All clipping procedures were technically successful, but endovascular treatment failed in 1 coiling case, and a switch from coiling to SAC was required in 2 cases. The major event rates were 4.0% after clipping (1 major stroke and 1 intracranial hemorrhage) and 0% after endovascular therapy (P=0.667). At mid-term angiographic follow-up (mean 12.0±8.9 months), all 37 followed clipped aneurysms were completely occluded, compared to 8/17 (41.7%) after coiling and 11/15 (73.3%) after SAC (P<0.001). Coiling was significantly associated with incomplete occlusion in the adjusted analysis (odds ratio, 11.7; 95% confidence interval, 2.7–52.6; P=0.001). Conclusion Both endovascular and surgical treatment were feasible and safe for MSAs. As coiling was associated with comparatively high recanalization rates, endovascular treatment may be preferred with stent support.

Background: and Purpose The Save ChildS Study demonstrated that endovascular thrombectomy (EVT) is a safe treatment option for pediatric stroke patients with large vessel occlusions (LVOs) with high recanalization rates. Our aim was to determine the long-term cost, health consequences and cost-effectiveness of EVT in this patient population. Methods: In this retrospective study, a decision-analytic Markov model estimated lifetime costs and quality-adjusted life years (QALYs). Early outcome parameters were based on the entire Save ChildS Study to model the EVT group. As no randomized data exist, the Save ChildS patient subgroup with unsuccessful recanalization was used to model the standard of care group. For modeling of lifetime estimates, pediatric and adult input parameters were obtained from the current literature. The analysis was conducted in a United States setting applying healthcare and societal perspectives. Probabilistic sensitivity analyses were performed. The willingness-to-pay threshold was set to $100,000 per QALY. Results: The model results yielded EVT as the dominant (cost-effective as well as cost-saving) strategy for pediatric stroke patients. The incremental effectiveness for the average age of 11.3 years at first stroke in the Save ChildS Study was determined as an additional 4.02 lifetime QALYs, with lifetime cost-savings that amounted to $169,982 from a healthcare perspective and $254,110 when applying a societal perspective. Acceptability rates for EVT were 96.60% and 96.66% for the healthcare and societal perspectives. Conclusions EVT for pediatric stroke patients with LVOs resulted in added QALY and reduced lifetime costs. Based on the available data in the Save ChildS Study, EVT is very likely to be a cost-effective treatment strategy for childhood stroke.

Background: and Purpose Data on safety and efficacy of intra-arterial (IA) fibrinolytics as adjunct to mechanical thrombectomy (MT) are sparse. Methods: INtra-arterial FIbriNolytics In ThrombectomY (INFINITY) is a retrospective multi-center observational registry of consecutive patients with anterior circulation large-vessel occlusion ischemic stroke treated with MT and adjunctive administration of IA fibrinolytics (alteplase [tissue plasminogen activator, tPA] or urokinase [UK]) at 10 European centers. Primary outcome was the occurrence of symptomatic intracranial hemorrhage (sICH) according to the European Cooperative Acute Stroke Study II definition. Secondary outcomes were mortality and modified Rankin Scale (mRS) scores at 3 months. Results: Of 5,612 patients screened, 311 (median age, 74 years; 44.1% female) received additional IA after or during MT (194 MT+IA tPA, 117 MT+IA UK). IA fibrinolytics were mostly administered for rescue of thrombolysis in cerebral infarction (TICI) 0-2b after MT (80.4%, 250/311). sICH occurred in 27 of 308 patients (8.8%), with an increased risk in patients with initial TICI0/1 (adjusted odds ratio [aOR], 2.3; 95% confidence interval [CI], 1.1 to 5.0 per TICI grade decrease) or in those with intracranial internal carotid artery occlusions (aOR, 3.7; 95% CI, 1.2 to 12.5). In patients with attempted rescue of TICI0-2b and available angiographic follow-up, 116 of 228 patients (50.9%) showed any angiographic reperfusion improvement after IA fibrinolytics, which was associated with mRS ≤2 (aOR, 3.1; 95% CI, 1.4 to 6.9). Conclusions Administration of IA fibrinolytics as adjunct to MT is performed rarely, but can improve reperfusion, which is associated with better outcomes. Despite a selection bias, an increased risk of sICH seems possible, which underlines the importance of careful patient selection.