Background and objectiveDysferlinopathies are a group of muscle disorders caused by mutations in the DYSF gene. Previous muscle imaging studies describe a selective pattern of muscle involvement in smaller patient cohorts, but a large imaging study across the entire spectrum of the dysferlinopathies had not been performed and previous imaging findings were not correlated with functional tests.MethodsWe present cross-sectional T1-weighted muscle MRI data from 182 patients with genetically confirmed dysferlinopathies. We have analysed the pattern of muscles involved in the disease using hierarchical analysis and presented it as heatmaps. Results of the MRI scans have been correlated with relevant functional tests for each region of the body analysed.ResultsIn 181 of the 182 patients scanned, we observed muscle pathology on T1-weighted images, with the gastrocnemius medialis and the soleus being the most commonly affected muscles. A similar pattern of involvement was identified in most patients regardless of their clinical presentation. Increased muscle pathology on MRI correlated positively with disease duration and functional impairment.ConclusionsThe information generated by this study is of high diagnostic value and important for clinical trial development. We have been able to describe a pattern that can be considered as characteristic of dysferlinopathy. We have defined the natural history of the disease from a radiological point of view. These results enabled the identification of the most relevant regions of interest for quantitative MRI in longitudinal studies, such as clinical trials.Clinical trial registrationNCT01676077.
Background and Purpose—
Smoking is an established risk factor for stroke; however, it is uncertain whether prestroke smoking status affects clinical outcomes of acute ischemic stroke. This study aimed to elucidate the association between smoking status and functional outcomes after acute ischemic stroke.
Methods—
Using a multicenter hospital-based stroke registry in Japan, we investigated 10 825 patients with acute ischemic stroke hospitalized between July 2007 and December 2017 who had been independent before stroke onset. Smoking status was categorized into those who had never smoked (nonsmokers), former smokers, and current smokers. Clinical outcomes included poor functional outcome (modified Rankin Scale score ≥2) and functional dependence (modified Rankin Scale score 2–5) at 3 months. We adjusted for potential confounding factors using a logistic regression analysis.
Results—
The mean age of patients was 70.2±12.2 years, and 37.0% were women. There were 4396 (42.7%) nonsmokers, 3328 (32.4%) former smokers, and 2561 (24.9%) current smokers. The odds ratio (95% CI) for poor functional outcome after adjusting for confounders increased in current smokers (1.29 [1.11–1.49] versus nonsmokers) but not in former smokers (1.05 [0.92–1.21] versus nonsmokers). However, among the former smokers, the odds ratio of poor functional outcome was higher in those who quit smoking within 2 years of stroke onset (1.75 [1.15–2.66] versus nonsmokers). The risk of poor functional outcome tended to increase as the number of daily cigarettes increased in current smokers (
P
for trend=0.002). All these associations were maintained for functional dependence.
Conclusions—
Current and recent smoking is associated with an increased risk of unfavorable functional outcomes at 3 months after acute ischemic stroke.
Registration—
URL:
http://www.fukuoka-stroke.net/english/index.html
. Unique identifier: 000000800.
Although influenza vaccine is thought to be effective and safe, it occasionally causes systemic reactions such as toxic epidermal necrolysis, bullous pemphigoid, lichen planus (LP), etc. The period of increased risk of developing these events was different depending on the immune responses induced by the vaccination. We report 3 cases of LP which appeared after an influenza vaccination. Our cases indicate that the period of increased risk of developing vaccine-related LP was concentrated within 2 weeks after vaccination, and that the vaccine alone represents a triggering factor necessary for immune alteration sufficient for the development of LP. Because these adverse events tend to develop over a predictable time course, the time of onset may give an important clue to the diagnosis of vaccine-related diseases. We suggest that a history of recent vaccination should be sought in all patients presenting with linear LP.
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