Pediatric onset multiple sclerosis (POMS) commonly occurs at the time of various endocrine changes. Evaluation of the impact of endocrine status on disease severity in POMS has not been previously ...explored.
This study sought to evaluate if sex and stress hormones in children with POMS impact motor and non-motor diseases severity.
A single-center case control study was performed. Individuals with POMS were compared to individuals without neurologic disease. Each individual had three blood draws assessing stress and sex hormones between 07:00 and 09:00. Measures of fatigue (Epworth sleepiness scale), depression (PHQ-9), and quality of life (PedsQL) assessed at each visit.
Forty individuals with POMS and 40 controls were enrolled. Individuals with POMS had lower free testosterone (
= 0.003), cortisol (
< 0.001), and ACTH (
< 0.001) and had higher progesterone (
= 0.025) levels than controls. Relapses and EDSS were not impacted by endocrine variables. The POMS cohort had a significantly higher Epworth score (
< 0.001), PHQ-9 score (
< 0.001), and lower PQL score (
< 0.001) than controls. Non-motor measures were not associated with endocrine status.
Free testosterone, cortisol, ACTH, and progesterone were abnormal in children with POMS although there was no association between endocrine status and markers of disease severity or non-motor symptoms of MS.
Down syndrome regression disorder (DSRD) is a clinical symptom cluster consisting of neuropsychiatric regression without an identifiable cause. This study evaluated the clinical effectiveness of IVIg ...and evaluated clinical characteristics associated with relapse after therapy discontinuation. A prospective, multi-center, non-randomized, observational study was performed. Patients met criteria for DSRD and were treated with IVIg. All patients underwent a standardized wean-off therapy after 9-12 months of treatment. Baseline, on-therapy, and relapse scores of the Neuropsychiatric Inventory Total Score (NPITS), Clinical Global Impression-Severity (CGI-S), and the Bush-Francis Catatonia Rating Scale (BFCRS) were used to track clinical symptoms. Eighty-two individuals were enrolled in this study. Patients had lower BFCRS (MD: -6.68; 95% CI: -8.23, -5.14), CGI-S (MD: -1.27; 95% CI: -1.73, -0.81), and NPITS scores (MD: -6.50; 95% CI: -7.53, -5.47) while they were on therapy compared to baseline. Approximately 46% of the patients (n = 38) experienced neurologic relapse with wean of IVIg. Patients with neurologic relapse were more likely to have any abnormal neurodiagnostic study (χ
= 11.82, P = 0.001), abnormal MRI (χ
= 7.78, P = 0.005), and abnormal LP (χ
= 5.45, P = 0.02), and a personal history of autoimmunity (OR: 6.11, P < 0.001) compared to patients without relapse. IVIg was highly effective in the treatment of DSRD. Individuals with a history of personal autoimmunity or neurodiagnostic abnormalities were more likely to relapse following weaning of immunotherapy, indicating the potential for, a chronic autoimmune etiology in some cases of DSRD.
Purpose： Trauma is an inevitable part of the health burden in every country. Both the preventive and rehabilitative aspects of traumatic injuries are expensive. Since most of the injuries happen in ...low- and middle-income developing countries, a judicious allocation of the limited resources to the most cost- efficient strategies is necessary. The present study was designed to report the causes of trauma, injured body regions, trauma severity scores and the one year survival rate of a randomly selected sample of trauma patients in a major referral hospital in Tehran, Iran. Methods： We chose and analyzed a random subgroup of traumatic patients admitted during the one- year period of May 2012 to May 2013 to Shariati Hospital, a major University Teaching Hospital in Tehran, Iran. Patients who stayed at the hospital for less than 24 h were excluded. In total, 73 traumatic patients were registered. The mean age was （40.19 ＋ 20.34） years and 6Z1% of them were male. Results： In general, the most common cause of injury was falls （47.9%）, followed by road traffic crashes （RTCs, 40.8%）. Assault and exposure to inanimate mechanical forces each were only associated with 5.6% of all injuries. The only cause of injury in ages of more than 65 years was fall. The most common cause of injury in ages between 15 and 45 years was RTCs. During the study, two deaths occurred： one was at ICU and the other was at home. The most commonly injured body region was the head （23.8%）, followed by the elbow and forearm （19%）, hip and thigh （15.9%）, and multiple body regions （14.3%）. The mean abbreviated injury score was 2.23 ± 1.02; injury severity index was 7.26 ± 7.06; and revised trauma score was 7.84, calculated for 38 patients. Conclusion： Prevention strategy of traumatic injury should focus on falls and RTCs, which are respec- tively the most common cause of trauma in older aged people and young males.
Objective：Trauma is one of the leading causes of mortality and morbidity in adults and a major contributor to health care expenditures.Although spine-related injuries constitute a small proportion of ...trauma cases,they need special consideration due to poor functional outcomes and substantial burden.Despite relatively extensive previous studies on traumatic spinal injuries,there is still obscurity in some aspects of the issue.The purpose of this study is to establish a regional multicenter traumatic spine fracture/dislocation registry.Methods：This is a prospective case series study,including all patients with acute traumatic spine lesions admitted to a regional multicenter since 2014.Data is extracted based upon a form developed by Sina Trauma and Surgery Research Center,Tehran University of Medical Sciences,Tehran,Iran.Novel electronic data entry software is initiated and data will be entered to the software.Information remains confidential and security considerations will be taken based on standards of data entry systems.Results：The results of this study will include age and gender distribution of the patients,causes of injury,location of pain and neurological deficit,the American Spinal Injury Association score and Frankel grade on admission,at discharge,after 6 and 12 months and at the latest annual follow-up,radiologic findings,details of operative procedures and methods of external fixation.Conclusion：This study will identify prognostic factors that influence the ultimate fate of spine fracture patients and determine short and long-term outcome of different treatment methods.It can lead to a considerable improvement in patient care and will have a great national and transnational impact.
To determine the prevalence of neuroimaging abnormalities in individuals with Down syndrome regression disorder (DSRD) and evaluate if neuroimaging abnormalities were predictive of therapeutic ...responses.
A multicenter, retrospective, case-control study which reviewed neuroimaging studies of individuals with DSRD and compared them to a control cohort of individuals with Down syndrome (DS) alone was performed. Individuals aged 10-30 years and meeting international consensus criteria for DSRD were included. The presence of T1, T2/FLAIR, and SWI signal abnormalities was reviewed. Response rates to various therapies, including immunotherapy, were evaluated in the presence of neuroimaging abnormalities.
In total, 74 individuals (35%) had either T2/FLAIR and/or SWI signal abnormality compared to 14 individuals (12%) without DSRD (p < 0.001, 95%CI: 2.18-7.63). T2/FLAIR signal abnormalities were not appreciated more frequently in individuals with DSRD (14%, 30/210) than in the control cohort (9%, 11/119) (p = 0.18, OR: 1.63, 95%CI: 0.79-3.40). SWI signal abnormalities were appreciated at a higher frequency in individuals with DSRD (24%, 51/210) compared to the control cohort (4%, 5/119) (p < 0.001, OR: 7.31, 95%CI: 2.83-18.90). T2/FLAIR signal abnormalities were localized to the frontal (40%, 12/30) and parietal lobes (37%, 11/30). SWI signal abnormalities were predominantly in the bilateral basal ganglia (94%, 49/52). Individuals with DSRD and the presence of T2/FLAIR and/or SWI signal abnormalities were much more likely to respond to immunotherapy (p < 0.001, OR: 8.42. 95%CI: 3.78-18.76) and less likely to respond to benzodiazepines (p = 0.01, OR: 0.45, 95%CI: 0.25-0.83), antipsychotics (p < 0.001, OR: 0.28, 95%CI: 0.11-0.55), or electroconvulsive therapy (p < 0.001, OR: 0.12; 95%CI: 0.02-0.78) compared to individuals without these neuroimaging abnormalities.
This study indicates that in individuals diagnosed with DSRD, T2/FLAIR, and SWI signal abnormalities are more common than previously thought and predict response to immunotherapy.
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