Hyperechogenicity of substantia nigra (SNh) is a frequent finding in amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD) and other movement disorders (MD) patients, but its meaning is unclear. To ascertain the contribution of different factors to SNh area, we measured it in 108 ALS, 102 PD, 91 other MD patients and 91 healthy controls. Demographical data were collected in all patients and controls. In ALS patients, we also recorded clinical variables, performed genetic analysis and measured baseline levels of ferritin. After family history and genetic testing, ALS patients were classified as familial (15) or sporadic (93). ALS, PD and other MD patients had a larger SNh area than controls. Left SNh and male gender, but not age, associated with larger SNh area in both patients and controls. Familial ALS patients showed larger SNh area than sporadic ones and familial ALS was the only clinical variable in the multivariate analysis to be associated with larger SNh area in ALS patients. Our results suggest that SNh associates with genetic and constitutional factors (male gender, handedness), some of which predispose to certain neurodegenerative diseases. This evidence supports the idea of SNh as an inborn marker of unspecific neuronal vulnerability.
Introduction Bifurcation aneurysms represent an ongoing endovascular challenge with a variety of techniques and devices designed to address them. We present our multicenter series of the pCONUS2 and pCONUS2 HPC devices when treating bifurcation aneurysms. Methods We performed a retrospective review of our prospectively maintained databases at 3 tertiary neurointerventional centers to identify all patients who underwent coil embolization with the pCONUS2 or pCONUS2 HPC device between February 2015 and August 2021. We recorded baseline demographics, aneurysm data, complications, immediate and delayed angiographic results. Results We identified 55 patients with 56 aneurysms, median age 63 years (range 42–78 years), 67.3% female (n = 37). The commonest aneurysm location was the MCA bifurcation (n = 40, 71.4%). Average dome height was 8.9 ± 4.2 mm (range 3.2–21.5 mm), average neck width 6.4 ± 2.5 mm (range 2.6–14 mm), and average aspect ratio 1.3 ± 0.6 (range 0.5–3.3). The pCONUS2 was used in 64.3% and the pCONUS2 HPC in 35.7%. The procedural technical success rate was 98.2%. Intraoperative complications occurred in 5 cases (8.9%), 4 of which were related to the coils with partial thrombus formation on the pCONUS2 HPC seen in 1 case that was resolved with heparin. In relation to the procedure and treatment of the aneurysm the overall permanent morbidity was 1.8% (n = 1/55) and mortality 0%. Delayed angiographic follow-up (48 aneurysms) at median 12 months postprocedure (range 3–36 months) demonstrated adequate occlusion of 83.4% of aneurysms. Conclusion The pCONUS2 and pCONUS2 HPC devices carry a high technical success rate, low complication and retreatment rate, and good rates of adequate occlusion. Larger prospective confirmatory studies are required.
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