Antibodies to the aquaporin-4 (AQP4) water channel in neuromyelitis optica spectrum disorder (NMOSD) are reported to trigger the complement cascade, which is implicated in astrocyte damage and subsequent neuronal injury. The PREVENT study demonstrated that the terminal complement inhibitor eculizumab reduces adjudicated relapse risk in patients with anti-AQP4 immunoglobulin G-positive (AQP4+) NMOSD. The objective of this analysis was to evaluate the efficacy of eculizumab in reducing relapse risk and its safety in AQP4+ NMOSD across clinically relevant subgroups in PREVENT.In the randomized, double-blind, time-to-event, phase 3 PREVENT trial, 143 adults received eculizumab (maintenance dose, 1200 mg/2 weeks) or placebo (2:1), with stable-dose concomitant immunosuppressive therapy (IST) permitted (except rituximab and mitoxantrone). Post hoc analyses of relapses and adverse events were performed for prespecified and post hoc subgroups based on concomitant IST and prior rituximab use, demographic and disease characteristics, and autoimmune comorbidity.The significant reduction in relapse risk observed for eculizumab versus placebo in the overall PREVENT population was consistently maintained across subgroups based on concomitant IST and previous rituximab use, age, sex, region, race, time since clinical onset of NMOSD, historical annualized relapse rate, baseline Expanded Disability Status Scale score, and history of another autoimmune disorder. The serious infection rate was lower with eculizumab than placebo regardless of rituximab use in the previous year, concomitant IST use, or history of another autoimmune disorder.Across a wide range of clinically relevant AQP4+ NMOSD patient subgroups in PREVENT, eculizumab therapy was consistently effective versus placebo in reducing relapse risk, with no apparent increase in serious infection rate.NCT01892345 (ClinicalTrials.gov).
Lambert–Eaton myasthenic syndrome (LEMS) is a rare immune-mediated disorder of neuromuscular junctions. The knowledge of the effects of pregnancy on the course of patients with LEMS is limited. Here we describe a patient without a history of previous known illness who has complained of weakness during the last trimester of her pregnancy, delivered a healthy baby and was diagnosed with non-paraneoplastic LEMS during the postpartum period. With this case we wanted to emphasize the effects of pregnancy on the course of patients with LEMS.
Central nervous system (CNS) involvement in patients with familial Mediterranean fever (FMF) is considerably rare. Patients with FMF may exhibit clinical and radiologic symptoms similar to multiple sclerosis (MS). However, the impact of the Familial Mediterranean Fever Gene (MEFV) mutations on the clinical course of MS is not fully understood as yet.In our study, we investigated the presence of probable MEFV mutations in patients diagnosed with definite MS and the association of these mutations with the clinical course, radiologic characteristics and disability status of the individuals. A total of 105 patients diagnosed with definite MS according to the McDonald criteria and a control group of 112 non-symptomatic individuals were included in the study.Thirty-seven patients (35.2%) had MEFV gene mutations; three were compound heterozygotes (M694V/E148Q; M694V/V726A; P369S/E148Q) and one was homozygous for P369S. No statistically significant differences were found among patients with MS and healthy individuals with respect to existing mutations. In addition, we did not observe a statistically significant relationship between MEFV mutations and the gender of the patients, oligoclonal band (OCB) positivity, Expanded Disability Status Scale (EDSS), disease onset age, clinical presentation, affected neurologic systems, existence of spinal lesions, response to immunomodulatory treatment, time to reach EDSS scores of 3 and 6, the number of attacks and the average number of lesions on a brain MRI.Our results indicate that MEFV gene mutations do not affect the neurologic prognosis in patients with MS. However, additional research studies involving more patients with MS and clinical forms are warranted to confirm our results.
Multifokal motor noropati (MMN), kalici iletim bloklarinin eslik ettigi, ilerleyici, asimetrik ekstremite gucsuzluguyle karakterizedir. Duysal etkilenim cok seyrek olarak gorulur. MMN’nin kranial sinir tutulumu ve refleks artisi ile birlikteligi nadiren bildirilmistir. Burada, el kaslarindaki ilerleyici gucsuzluge, orbikularis okuli kaslarinda gucsuzluk ve dilde fasikulasyonun eslik ettigi 32 yasinda MMN’li bir kadin hasta sunulmustur. Elektrofizyolojik incelemede her iki ulnar, sag median ve sol posterior tibial sinirlerde iletim bloklari ve F dalgalarinda persistans anormalligi saptandi. Bu bulgularla birlikte, ileti bloklarinin persistansi ve tablonun IVIG’e yanitli olmasi bize multifokal motor noropati tanisini dusundurttu. Hasta, klinigindeki seyrek rastlanir ogeler nedeniyle rapor edilmeye deger bulundu. Anahtar Kelimeler: Multifokal motor noropati, kranial sinir tutulumu, refleks artisi
Venoz tromboembolizm kanserli hastalarda gorulebilen bir komplikasyondur. Literaturde lenfoma’yi iceren hematolojik malinitelerde gorulen venoz tromboembolizm konusu yeterince bilinmemektedir. Hiperkalsemi lenfoma hastalarinda gorulebilen bir elektrolit bozuklugudur. Bu yazida kavernoz sinus trombozu ve hiperkalsemi ile prezente olan diffuz buyuk B hucreli lenfoma olgusunu sunuyoruz
Multiple sclerosis (MS) is defined as a chronic, inflammatory, and demyelinating disease of the central nervous system.MS is more frequently diagnosed in women and in individuals aged between 20 and 40 years (1).Its prevalence is 30-80/100,000 in the population in high-risk areas including Canada, North America, and North Europe (2,3).The number of studies on the prevalence of MS in Turkey is insufficient; however, studies conducted in Maltepe district of İstanbul (4) and in Erbaa, a rural area in the coastal Black Sea region of Turkey (5), have reported the a moderate-to-high prevalence of MS.Alp et al. (6) have demonstrated that the prevalence of MS in the North Caucasus Region of Turkey is higher than expected (68.97/100,000).The prevalence of MS, which is known to be affected by genetic and environmental factors, varies in different regions.It is considered that this variation is caused by different seasonal characteristics, geographic locations, and ethnic profiles of the population (1,3).Turkey is located between Asia and Europe and constitutes a link between these two continents.Along with the high rate of migration from both inland regions and foreign countries, this migration may affect the prevalence of MS in Turkey.The present study investigated the prevalence of MS in the Middle Black Sea Region of Turkey as well as the demographic characteristics of patients. METHODSPatients who were diagnosed with MS and who were living in the Middle Black Sea Region were included.The study was conducted between August 2010 and May 2011.All patients were informed about the study, and their consent was obtained.Approval of the Ethical Committee of Ondokuz Mayıs University was obtained for the study.Patient admission records between January 2001 and February 2011 were obtained from secondary and tertiary hospitals in Samsun, Sinop, Ordu, Amasya, Tokat, and Çorum provinces and in the districts of these provinces in the Middle Black Sea Region.Neurology specialists working in these centers were informed about the study.In district hospitals without neurology specialists, chief physicians were informed.Information was obtained from data processing services after receiving permission from the chief physicians.All hospitals accepted to participate in the study and shared patient information on admission.All clinical and radiological characteristics of patients were evaluated in the MS unit at Ondokuz Mayıs University.Patients who did not meet the clinical and radiological criteria for MS or who met the radiological criteria for MS but did not have any clinical history of MS
'%3e%3cpath fill='%23012169' d='M0 0v30h60V0z'/%3e%3cpath stroke='%23fff' stroke-width='6' d='m0 0 60 30m0-30L0 30'/%3e%3cpath stroke='%23C8102E' stroke-width='4' d='m0 0 60 30m0-30L0 30' clip-path='url(%23b)'/%3e%3cpath stroke='%23fff' stroke-width='10' d='M30 0v30M0 15h60'/%3e%3cpath stroke='%23C8102E' stroke-width='6' d='M30 0v30M0 15h60'/%3e%3c/g%3e%3c/svg%3e)
