Significance of clinical-immunological patterns and diagnostic yield of biopsies in microscopic polyangiitis and granulomatosis with polyangiitis.

BACKGROUND: Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). OBJECTIVES: To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated. RESULTS: This retrospective study (2000-2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]-ANCA and 2.6% proteinase 3 [PR3]-ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3-ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients. CONCLUSIONS: The identification of GPA presentations associated with MPO-ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult-to-diagnose patients based on their significant diagnostic yield.

Brain abnormalities in newly diagnosed neuropsychiatric lupus: systematic MRI approach and correlation with clinical and laboratory data in a large multicenter cohort.

OBJECTIVES: To describe brain magnetic resonance imaging (MRI) abnormalities in newly diagnosed neuropsychiatric lupus (NPSLE). To correlate them with clinical and laboratory data. METHODS: This retrospective cross-sectional study included patients presenting NPSLE undergoing brain MRI within 6 months after onset between 2003 and 2012. Clinical and laboratory data were recorded. MRI findings were defined as inflammatory-like, large-vessel disease (LVD), and small-vessel disease (SVD); SVD was classified as white-matter hyperintensities (WMH), recent small subcortical infarcts, lacunes, microbleeds, and brain atrophy. RESULTS: We included 108 patients (mean 40.6 ± 14.2 years; range 14-77), 91.7% women. The most frequent syndromes were headache (28.5%), cerebrovascular disease (15.5%), seizure (15.5%), and cognitive dysfunction (11.4%). Brain abnormalities were found in 59.3%. SVD was the most common (55.6%), followed by LVD (13%) and inflammatory-like lesions (6.5%). The most frequent SVD findings were WMH (53.7%), atrophy (18.5%), microbleeds (13.7%) and lacunes (11.1%). Cerebrovascular syndrome correlated with LVD (p = 0.001) and microbleeds (p = 0.002), cognitive dysfunction with WMH (p = 0.045) and myelopathy with inflammatory-like lesions (p = 0.020). Low C4 and CH50 correlated with inflammatory-like lesions (p < 0.001, p = 0.019) and lupus anticoagulant with WMH (p = 0.018), microbleeds (p = 0.002) and atrophy (p = 0.008). CONCLUSIONS: Vascular disease is the hallmark of NPSLE. Certain syndromes and immunological patterns are prone to more extensive brain damage. MRI could provide significant clinical information and insights into the pathological substrate.

Single-center experience of cerebral artery thrombectomy using the TREVO device in 60 patients with acute ischemic stroke.

BACKGROUND AND PURPOSE: We sought to explore the safety and efficacy of the new TREVO stent-like retriever in consecutive patients with acute stroke. METHODS: We conducted a prospective, single-center study of 60 patients (mean age, 71.3 years; male 47%) with stroke lasting <8 hours in the anterior circulation (n=54) or <12 hours in the vertebrobasilar circulation (n=6) treated if CT perfusion/CT angiography confirmed a large artery occlusion, ruled out a malignant profile, or showed target mismatch if symptoms >4.5 hours. Successful recanalization (Thrombolysis In Cerebral Infarction 2b-3), good outcome (modified Rankin Scale score 0-2) and mortality at Day 90, device-related complications, and symptomatic hemorrhage (parenchymal hematoma Type 1 or parenchymal hematoma Type 2 and National Institutes of Health Stroke Scale score increment ≥ 4 points) were prospectively assessed. RESULTS: Median (interquartile range) National Institutes of Health Stroke Scale score on admission was 18 (12-22). The median (interquartile range) time from stroke onset to groin puncture was 210 (173-296) minutes. Successful revascularization was obtained in 44 (73.3%) of the cases when only the TREVO device was used and in 52 (86.7%) when other devices or additional intra-arterial tissue-type plasminogen activator were also required. The median time (interquartile range) of the procedure was 80 (45-114) minutes. Good outcome was achieved in 27 (45%) of the patients and the mortality rate was 28.3%. Seven patients (11.7%) presented a symptomatic intracranial hemorrhage. No other major complications were detected. CONCLUSIONS: The TREVO device was reasonably safe and effective in patients with severe stroke. These results support further investigation of the TREVO device in multicentric registries and randomized clinical trials.

Mammographic features of screening detected pT1 (a-b) invasive breast cancer using BI-RADS lexicon.

AIM: To describe mammographic features in screening detected invasive breast cancer less than or equal to 10mm using Breast Imaging Reporting and Data System lexicon in full-field digital mammography. PATIENTS AND METHODS: A retrospective analysis of 123 pT1 (a-b) invasive breast cancers in women aged 50-69 years from our screening program. Radiologic patterns were: masses, calcifications, distortions, asymmetries and mixed. Masses: shape, margins and density, and calcifications: morphology, number of flecks and size of the cluster were taken into account, following Breast Imaging Reporting and Data System terminology. RESULTS: We found 61 masses (49.6%), 8 masses with calcifications (6.5%), 30 groups of calcifications (24.4%), 19 architectural distortions (15.4%), 1 architectural distortion with calcifications (0.8%), 4 asymmetries (3.2%). Sixty out of 69 masses were irregular in shape, 6 lobular, 2 ovals and 1 round. Thirty-four showed ill-defined margins, 29 spiculated and 6 microlobulated. Most of them showed a density similar to surrounding fibroglandular tissue. Calcifications were pleomorphic or fine linear in 24 of 30 (80%). Most of cases showed more than 10 flecks and a size greater than 1cm. CONCLUSION: The predominant radiologic finding is an irregular, isodense mass those margins tend to share different descriptors, being ill-defined margins the most constant finding. Calcifications representing invasive cancer are predominantly pleomorphic with more than 10 flecks per cm. Architectural distortion and invasive tubular carcinoma are more common than reported in general series.

Multimodal CT-assisted thrombolysis in patients with acute stroke: a cohort study.

BACKGROUND AND PURPOSE: The value of multimodal CT to assist thrombolysis has received little attention in stroke. METHODS: We assessed prospectively the impact derived from the routine application of CT perfusion and CTA in patients with acute stroke treated consecutively with alteplase. The safety and efficacy of thrombolytic therapy were compared in 106 patients assisted with CT/CTA/CT perfusion (multimodal CT group) and 262 patients assisted without full multimodal brain imaging (control group) during a 5-year period (2005-2009). RESULTS: Good outcome (modified Rankin scale score ≤2) at 3 months was increased in the multimodal group compared with controls (adjusted OR, 2.88; 95% CI, 1.50-5.52). Multimodal-assisted thrombolysis yielded superior benefits in patients treated beyond 3 hours (adjusted OR, 4.48; 95% CI, 1.68-11.98) than treated within 3 hours (adjusted OR, 1.31; 95% CI, 0.80-2.16; interaction test P=0.043). Mortality (14% and 15%) and symptomatic hemorrhage (5% and 7%) were similar in both groups. CONCLUSIONS: Multimodal CT use in routine clinical practice may heighten the overall efficacy of thrombolytic therapy in acute ischemic stroke. The benefits seem greater in patients treated >3 hours after stroke onset, but further randomized clinical trials are needed to confirm these findings.

Higher incidence of mild cognitive impairment in familial hypercholesterolemia.

OBJECTIVE: Hypercholesterolemia is an early risk factor for Alzheimer's disease. Low-density lipoprotein (LDL) receptors might be involved in this disorder. Our objective was to determine the risk of mild cognitive impairment in a population of patients with heterozygous familial hypercholesterolemia, a condition involving LDL receptor dysfunction and lifelong hypercholesterolemia. METHODS: By using a cohort study design, patients with familial hypercholesterolemia (N=47) meeting inclusion criteria and comparison patients without familial hypercholesterolemia (N=70) were consecutively selected from academic specialty and primary care clinics, respectively. All patients were older than 50 years. Those with disorders that could affect cognition, including history of stroke or transient ischemic attacks, were excluded from both groups. Thirteen standardized neuropsychologic tests were performed in all subjects. Mutational analysis was performed in patients with familial hypercholesterolemia, and brain imaging was obtained in those with familial hypercholesterolemia and mild cognitive impairment. RESULTS: Patients with familial hypercholesterolemia showed a high incidence of mild cognitive impairment compared with those without familial hypercholesterolemia (21.3% vs 2.9%; P=.00). This diagnosis was unrelated to structural pathology or white matter disease. There were significant differences, independent of apolipoprotein E4 or E2 status, between those with familial hypercholesterolemia and those with no familial hypercholesterolemia in several cognitive measures, all in the direction of worse performance for those with familial hypercholesterolemia. CONCLUSION: Because prior studies have shown that older patients with sporadic hypercholesterolemia do not show a higher incidence of mild cognitive impairment, the findings presented suggest that early exposure to elevated cholesterol or LDL receptor dysfunction may be risk factors for mild cognitive impairment.

Varón de 52 años con inestabilidad de la marcha / A 52-year-old man with gait instability

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Functional analysis of third ventriculostomy patency by quantification of CSF stroke volume by using cine phase-contrast MR imaging.

OBJECTIVE: Endoscopic third ventriculostomy (ETV) is increasingly used as alternative treatment for obstructive hydrocephalus. The aim of this study was to determine the utility of quantitative and qualitative examinations with cine phase-contrast MR imaging to determine the efficacy of ventriculostomy across time and whether CSF pulsation is restored after ETV. METHODS: Thirty-eight patients treated with ETV were evaluated with cine phase-contrast MR within 1 month after surgery. Follow-up studies were performed after 1 year in 25 patients and after 2 years in 12. We evaluated flow void changes in the floor of the third ventricle and quantified the stroke volume at the site of the ventriculostomy. We also recorded changes in ventricular size and clinical outcome. To determine the restoration of CSF pulsation, we compared the CSF waveform at the ventriculostomy with the CSF waveform at the aqueduct in a healthy control group. RESULTS: After ventriculostomy, restoration of pulsate motion characteristics of CSF circulation was observed. The stroke volume registered at ventriculostomy was maintained with time. There was a statistically significant relationship between clinical outcome and stroke volume. Overall flow magnitude was the most effective variable to determine which patients would improve after surgery. Values >75 mm3 showed a sensitivity of 76.7% and a specificity of 87.5% There was no relationship between ventricular size changes and clinical outcome. Patients with primary aqueduct stenosis had the best response to surgery, whereas patients with Arnold Chiari malformation or communicating hydrocephalus had the worst response. CONCLUSION: Quantitative analysis with phase-contrast MR imaging indicates that ETV is an efficient technique for restoring CSF pulsation, with efficacy being maintained during the follow-up controls. Quantification of stroke volume at ventriculostomy is a good indicator of the functional status of ETV, and a high stroke volume in the ventriculostomy appears to be a positive predictor of favorable clinical outcome.

Varón de 72 años con lesiones paranasales crónicas / A 72 year-old man with chronic rhinosinusal lesions

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