The role of parliamentarians in promoting self-care interventions for sexual and reproductive health and rights: applying COVID-19 lens in the Eastern Mediterranean region.

Innovative people-centered care modalities including self-care interventions offer an opportunity to ensure continuity of healthcare services during COVID-19 and in post-COVID-19, as well as contribute to the achievement of universal health coverage. Parliamentarians are uniquely positioned to promote self-care interventions for sexual and reproductive health and rights through their legislative, budget allocation, oversight, and advocacy roles. However, existing health systems governance challenges in the Eastern Mediterranean region such as weak institutions setups, fragmentation of health programs, and limitation of resources could impede parliamentarians' progress. To address these challenges, the following recommended actions should be considered: (1) promote the adaptation of sexual and reproductive health and rights service packages at primary healthcare level to integrate self-care interventions (2) govern innovative people-centered care channels including self-care interventions; and (3) engage in a dialogue with civil society and communities to meet needs, raise public awareness and generate demand.

The NEALS primary lateral sclerosis registry.

BACKGROUND AND OBJECTIVE: Primary lateral sclerosis (PLS) is a neurodegenerative disease characterized by progressive upper motor neuron dysfunction. Because PLS patients represent only 1 to 4% of patients with adult motor neuron diseases, there is limited information about the disease's natural history. The objective of this study was to establish a large multicenter retrospective longitudinal registry of PLS patients seen at Northeast ALS Consortium (NEALS) sites to better characterize the natural progression of PLS. Methods: Clinical characteristics, electrophysiological findings, laboratory values, disease-related symptoms, and medications for symptom management were collected from PLS patients seen between 2000 and 2015. Results: The NEALS registry included data from 250 PLS patients. Median follow-up time was 3 years. The mean rate of functional decline measured by ALSFRS-R total score was -1.6 points/year (SE:0.24, n = 124); the mean annual decline in vital capacity was -3%/year (SE:0.55, n = 126). During the observational period, 18 patients died, 17 patients had a feeding tube placed and 7 required permanent assistive ventilation. Conclusions: The NEALS PLS Registry represents the largest available aggregation of longitudinal clinical data from PLS patients and provides a description of expected natural disease progression. Data from the registry will be available to the PLS community and can be leveraged to plan future clinical trials in this rare disease.

Update on Muscular Dystrophies with Focus on Novel Treatments and Biomarkers.

PURPOSE OF REVIEW: Muscular dystrophies are a heterogeneous group of inherited muscular disorders characterized by progressive muscle weakness and in many cases cardiac and respiratory muscle involvement. Historically, these disorders are considered incurable with grave prognoses. The genes responsible for most muscular dystrophies are known, and early diagnosis is achievable with proper clinical recognition and advanced genetic testing. This article reviews recent advances in the development of novel treatments and biomarkers in the realm of muscular dystrophies commonly encountered in pediatric population. RECENT FINDINGS: The therapeutic landscape of muscular dystrophies has changed with the development of new approved treatments for Duchenne muscular dystrophy (DMD), the most common and severe muscular dystrophy. This has paved the way for the development of novel therapeutic strategies for not only DMD but also other muscular dystrophies. This article reviews recent advances in the development of novel treatments and biomarkers in the realm of muscular dystrophies commonly encountered in pediatric population.

Migraine History: A Predictor of Negative Diffusion-Weighted Imaging in IV-tPA-Treated Stroke Mimics.

BACKGROUND: Migraine, seizures, and psychiatric disorders are frequently reported as "stroke mimics" in patients with negative diffusion-weighted imaging (DWI) after IV-tPA. We sought to determine predictors of negative DWI in suspected stroke patients treated with IV-tPA. METHOD: A retrospective case-control study encompassing all acute stroke patients treated with IV-tPA (at our hospital or "dripped and shipped") from January 2013 to December 2014 was con- ducted. A total of 275 patients were identified with 47 negative DWI cases and 228 positive DWI controls. Variables including demographic factors, stroke characteristics, and clinical comorbidities were analyzed for statistical significance. A multivariate logistic regression was performed (SPSS-24) to identify predictors of negative DWI. RESULTS: Approximately 17% of patients had negative DWI after IV-tPA. Compared to controls, migraine history independently predicted negative DWI (odds ratio [OR] 5.0 95% confidence interval [CI] 1.03-24.6, P = .046). Increasing age (OR .97 95% CI .94-.99, P = .02) and atrial fibrillation (OR .25 95% CI .08-.77, P = .01) predicted lower probability of negative DWI. Gender, admission NIHSS, treatment location, preadmission modified Rankin scale, diabetes mellitus, hypertension, hyperlipidemia, symptom side, seizure history, and psychiatric history did not predict negative DWI status. CONCLUSIONS: In our study, roughly 1 in 6 patients treated with IV-tPA were later found to be stroke mimics with negative DWI. Despite a high proportion of suspected stroke mimics in our study, only preexisting migraine history independently predicted negative DWI status after IV-tPA treatment in suspected stroke patients.

Trends in Endovascular Treatment of Aneurysmal Subarachnoid Hemorrhages.

INTRODUCTION: Aneurysmal subarachnoid hemorrhage (aSAH) accounts for 5% of all strokes; 30-day mortality is as high as 40%. We sought to evaluate outcomes of aSAH patients treated 2004-2014 by endovascular therapy (EVT), to demonstrate associated trends, and to evaluate angioplasty use for aSAH-related cerebral vasospasm. METHODS: The Nationwide Inpatient Sample (NIS) database 2004-2014 was used to derive a study cohort using ICD-9 codes. Survey procedures were used to adjust for stratified cluster design of NIS. NIS trend weights were used to generate national estimates. Mortality during hospitalization and use of angioplasty for aSAH-induced cerebral vasospasm trends were evaluated with multivariate regression analysis. RESULTS: We identified n = 10,822 (weighted n = 52,062) EVT-treated aSAH hospitalizations. Increasing years independently predicted decreased mortality (odds ratio [OR] 0.926, 95% confidence interval [CI] 0.905-0.948, p < 0.0001), decreased utilization of angioplasty (age ≥50 years [OR 0.916, 95% CI 0.867-0.968, p = 0.0019] and age <50 years [OR 0.922, 95% CI 0.879-0.967, p = 0.0009]) after controlling for increasing age, Charlson comorbidity index, and external ventricular drain placement. Angioplasty rates were higher in age <50 years compared to age ≥50 years (5 vs. 3.63%, p < 0.001). CONCLUSION: It is notable that EVT for aSAH management will be an integral and increasingly useful tool for initial aneurysm management. Advances in procedural techniques, operator experience, and periprocedural management could be significant contributors of decreasing mortality and reducing the need for angioplasty for cerebral vasospasm in patients admitted with aSAH.

Spanning from the West to East: An Updated Review on Endovascular Treatment of Intracranial Atherosclerotic Disease.

Ischemic stroke is a major cause of morbidity and mortality, incurring significant cost. Intracranial atherosclerotic disease (ICAD) accounts for 10-15% of ischemic stroke in Western societies, but is an underlying pathology in up to 54% of ischemic strokes in Asian populations. ICAD has largely been treated with medical management, although a few studies have examined outcomes following endovascular treatment. Our objective was to summarize the major trials that have been performed thus far in regard to the endovascular treatment of ICAD and to provide direction for future management of this disease process. Systematic review of the literature from 1966 to 2015, was conducted in regard to intracranial angioplasty and stenting. Studies were analyzed from PubMed, American Heart Association and Society of Neurointerventional Surgery databases. SAMMPRIS and VISSIT are the only randomized controlled trials from which Western guidelines of intracranial stenting have been derived, which have displayed the superiority of medical management. However, pooled reviews of smaller studies and other nonrandomized trials have shown better outcomes with endovascular therapy in select patient subsets, such as intracranial vertebrobasilar stenosis or in the presence of robust collaterals. Suboptimal cases, including longer lesions, bifurcations and significant tortuosity tend to fair better with medical management. Medical management has been shown to be more efficacious with less adverse outcomes than endovascular therapy. However, the majority of studies on endovascular management included a diverse patient population without ideal selection criteria, resulting in higher adverse outcomes. Population analyses and selective utilization of endovascular therapy have shown that the treatment may be superior to other management in select patients.

Does the Fibula Need to be Fixed in Complex Pilon Fractures?

OBJECTIVES: To review a series of patients with complex plafond injuries with a metadiaphyseal dissociation who did not have the fibula fixed and compare with patients who had their fibula fixed using patients without a fibula fracture as a control group. DESIGN: Retrospective case-control study. SETTING: Level 1 Trauma center at a university hospital. PATIENTS/PARTICIPANTS: Skeletally mature patients with a complete metadiaphyseal plafond fracture, and adequate presentation, postreduction, and healed radiographs to measure varus and valgus alignment. INTERVENTION: Surgical treatment [external fixator or open reduction internal fixation (ORIF)] of high energy pilon fractures. MAIN OUTCOME MEASUREMENTS: Metaphyseal alignment at the time of presentation, after fixation, and at union, surgical procedures performed, and complications. METHODS: From 364 patients with plafond fractures, 111 had high energy injuries with metadiaphyseal dissociation and form the basis of the study. Radiographs and charts were reviewed for fracture characteristics, metaphyseal alignment at the time of presentation, after fixation, and at union, surgical procedures performed, and complications. RESULTS: Of the 111 study patients, 93 patients were treated definitively with ORIF of the tibia and 18 patients were treated definitively in an external fixator. Within the 93 patients treated definitively with ORIF of the tibia, we identified 3 groups of patients those with a fibula fracture that was fixed (26 patients), those with a fibula fracture that was not fixed (37 patients), and those without a fibula fracture acting as the control group (30 patients). Between the 2 groups having a fibula fracture treated with ORIF of the tibia, there was no difference in fibula fracture pattern or location. For the 26 patients who had fibular fixation, it was performed in 11 patients at an average of 17 days for inability to hold length and alignment and in 15 patients to augment fixation in poor bone stock or to aid in the reduction. Patients with initial valgus deformity were more likely to have their fibula fixed. There was no difference in the postoperative or final alignment between the patients with fibula fractures (with or without fixation) and those without fibula fractures (P = 0.92). When comparing the 3 groups, the only statistical finding between the 2 groups was that those with fibula fixation required plate removal (P < 0.0001). CONCLUSIONS: Fibular fixation is not a necessary step in the reconstruction of pilon fractures, although it may be helpful in specific cases to aid in tibial plafond reduction or augment external fixation. We found a higher rate of plate removal if the fibula was fixed. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

A new SETX mutation producing AOA2 in two siblings.

OBJECTIVE: In this paper, we document two cases of a new SETX mutation (820:A>G) combined with an established recessive SETX mutation (5927:T>G) causing ataxia with oculomotor apraxia type 2 (AOA2). METHODS: The patients had a detailed neurological history and examination performed. Radiological imaging was obtained and genetic analysis was obtained. RESULTS: Both siblings demonstrated healthy and normal growth until adolescence. At that time, slowed speech, hypophonia, dysarthria, extraocular muscle dysfunction and some mild choreiform movements began to appear. Family history included some movement disorder difficulties in second degree relatives. The diagnosis of AOA2 was confirmed by genetic testing. CONCLUSIONS: We describe a new SETX gene mutation, which when combined with a recognized SETX mutation results in AOA2. The clinical, radiographic and ancillary testing are described.

Efficacy of lower-extremity venous thrombolysis in the setting of congenital absence or atresia of the inferior vena cava.

PURPOSE: A rare but described risk factor for deep venous thrombosis (DVT), predominantly in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia. MATERIALS AND METHODS: Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound. RESULTS: All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12-72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 ± 20.2 months (range 3.8-54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome. CONCLUSIONS: PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.