Abstract:
Importance:This study reports the long-term epidemiologic trends of amyotrophic lateral sclerosis (ALS) based on a prospective register. Objective:To examine the 20-year epidemiologic trends of ALS in the Piemonte and Valle d'Aosta regions of Italy. Design, Setting, and Participants:The Piemonte and Valle d'Aosta Register for ALS (PARALS) is an epidemiologic prospective register that covers 2 Italian regions (population of 4 476 931 inhabitants according to the 2011 census) from January 1, 1995, through December 31, 2014. Case ascertainment is based on multiple sources (neurologic departments, hospital discharge archives, and mortality records). Incidence rates are age and sex standardized for the Italian population of the 2011 census. Age-period-cohort (APC) analysis was performed using a Poisson regression model. Main Outcomes and Measures:The primary study outcomes were long-term incidence and prevalence rates of ALS using a prospective design and their determinants. Results:During the study period, a total of 2702 patients (mean [SD] age at onset, 65.7 [11.1] years; 1246 [46.1%] female and 1456 [53.9%] male) received a diagnosis of ALS between 1995 and 2014, corresponding to a crude annual incidence rate of 3.03 per 100 000 population (95% CI, 2.85-3.23) and an adjusted incidence rate of 2.78 per 100 000 population (95% CI, 2.57-2.96). The age-adjusted incidence rate increased in the 2 decades of the study (1995-2004: 2.66; 95% CI, 2.50-2.83; 2005-2014: 2.89; 95% CI, 2.71-3.07; P = .04), mostly in women. The adjusted rate ratio of men to women decreased from 1.27:1 (1995-2004) to 1.17:1 (2005-2014). The analysis of deviance for the APC regression models indicated that the drift variable is relevant in explaining the variation of ALS incidence rates over time in the overall population (change in deviance, 4.6553; P = .03) and in women (change in deviance, 3.8821; P = .05) but not in men (change in deviance, 0.77215; P = .38). A total of 479 patients with ALS were alive and had not undergone tracheostomy at the prevalence day (December 31, 2014), corresponding to a crude prevalence rate of 10.54 per 100 000 population (95% CI, 9.64-11.52). Conclusions and Relevance:During the 1995 to 2014 period, the crude and adjusted incidences of ALS increased in Piemonte and Valle d'Aosta, mostly in women. The APC model revealed that the increase of ALS incidence is attributable to a birth cohort effect in women, with a peak in the 1930 cohort. The different increase of ALS incidence in men and women points to an effect of exogenous factors with a differential effect on the 2 sexes, acting on a genetic background.
journal_name
JAMA Neuroljournal_title
JAMA neurologyauthors
Chiò A,Mora G,Moglia C,Manera U,Canosa A,Cammarosano S,Ilardi A,Bertuzzo D,Bersano E,Cugnasco P,Grassano M,Pisano F,Mazzini L,Calvo A,Piemonte and Valle d’Aosta Register for ALS (PARALS).doi
10.1001/jamaneurol.2017.1387subject
Has Abstractpub_date
2017-09-01 00:00:00pages
1097-1104issue
9eissn
2168-6149issn
2168-6157pii
2643176journal_volume
74pub_type
杂志文章相关文献
JAMA Neurology文献大全abstract:IMPORTANCE:Glioblastoma is the most common primary malignant brain tumor, but despite multimodal treatment with surgery, radiotherapy, and temozolomide chemotherapy, the prognosis is poor, with a median survival of 16 to 19 months and poor quality of life throughout the disease course. New treatments are needed. EVIDE...
journal_title:JAMA neurology
pub_type: 杂志文章,评审
doi:10.1001/jamaneurol.2014.1701
更新日期:2014-11-01 00:00:00
abstract:OBJECTIVE:To assess the reliability of new magnetic resonance imaging (MRI) lesion counts by clinicians in a multiple sclerosis specialty clinic. DESIGN:An observational study. SETTING:A multiple sclerosis specialty clinic. PATIENTS:Eighty-five patients with multiple sclerosis participating in a National Institutes ...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/2013.jamaneurol.211
更新日期:2013-03-01 00:00:00
abstract:Importance:The goal of Alzheimer disease (AD) prevention together with advances in understanding the pathophysiology of AD have led to clinical trials testing drugs in cognitively unimpaired persons who show evidence of AD biomarkers. Data are needed to inform the processes of describing AD biomarkers to cognitively no...
journal_title:JAMA neurology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamaneurol.2017.2954
更新日期:2018-01-01 00:00:00
abstract:IMPORTANCE:Voltage-gated potassium channel complex antibody (VGKCc-Ab) encephalitis is an immunotherapy-responsive syndrome usually associated with causative antibodies that target the leucine-rich, glioma inactivated 1 (LGI1) protein. Although it is expressed throughout the brain, LGI1 is not known to be expressed in ...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2014.1234
更新日期:2014-10-01 00:00:00
abstract:Importance:Mild cognitive impairment (MCI) is a transition state between normal cognitive aging and dementia that increases the risk for progressive cognitive decline. Preventing cognitive decline is a public health priority. Objective:To determine whether behavioral activation prevents cognitive and functional declin...
journal_title:JAMA neurology
pub_type: 杂志文章,随机对照试验
doi:10.1001/jamaneurol.2018.2513
更新日期:2018-12-01 00:00:00
abstract:Importance:Deposition of the pathological α-synuclein (αSynP) in the brain is the hallmark of synucleinopathies, including Parkinson disease (PD), Lewy body dementia (LBD), and multiple system atrophy (MSA). Whether real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA) assays...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.3311
更新日期:2020-09-28 00:00:00
abstract:Importance:Atabecestat, a nonselective oral β-secretase inhibitor, was evaluated in the EARLY trial for slowing cognitive decline in participants with preclinical Alzheimer disease. Preliminary analyses suggested dose-related cognitive worsening and neuropsychiatric adverse events (AEs). Objective:To report efficacy, ...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.4857
更新日期:2021-01-19 00:00:00
abstract:IMPORTANCE:Hematoma volume is the strongest predictor of outcome in intracerebral hemorrhage (ICH). Despite known differences in the underlying biology between deep and lobar ICHs, limited data are available on location specificity of factors reported to affect hematoma volume. OBJECTIVE:To evaluate whether determinan...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2013.98
更新日期:2013-08-01 00:00:00
abstract:Importance:Doses of fingolimod lower than 0.5 mg per day were not investigated during the fingolimod clinical development program. Whether lower doses of fingolimod might retain efficacy with fewer safety risks remains unknown. Objective:To evaluate the efficacy and safety of fingolimod, 0.5 mg, and fingolimod, 0.25 m...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.2950
更新日期:2020-08-24 00:00:00
abstract:BACKGROUND:Therapies designed to decrease the level of SOD1 are currently in a clinical trial for patients with superoxide dismutase (SOD1)-linked familial amyotrophic lateral sclerosis (ALS). OBJECTIVE:To determine whether the SOD1 protein in cerebral spinal fluid (CSF) may be a pharmacodynamic marker for antisense o...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2013.593
更新日期:2013-02-01 00:00:00
abstract:Importance:Although levodopa remains the most effective oral pharmacotherapy for Parkinson disease (PD), its use is often limited by wearing off effect and dyskinesias. Management of such complications continues to be a significant challenge. Objective:To investigate the efficacy and safety of safinamide (an oral amin...
journal_title:JAMA neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1001/jamaneurol.2016.4467
更新日期:2017-02-01 00:00:00
abstract:IMPORTANCE:Magnetic resonance imaging markers of incipient cognitive decline among healthy elderly individuals have become important for both clarifying the biological underpinnings of dementia and clinically identifying healthy individuals at high risk of cognitive decline. Even though the role of hippocampal atrophy ...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2013.3263
更新日期:2013-11-01 00:00:00
abstract:IMPORTANCE:This dose-blinded extension of the phase 2 BOLD (BAF312 on MRI Lesion Given Once Daily) Study in relapsing-remitting multiple sclerosis provides evidence on disease activity and safety of a range of siponimod doses for up to 24 months. OBJECTIVE:To assess the safety and efficacy of siponimod for up to 24 mo...
journal_title:JAMA neurology
pub_type: 杂志文章,随机对照试验
doi:10.1001/jamaneurol.2016.1451
更新日期:2016-09-01 00:00:00
abstract:Importance:Cognitive impairment in multiple sclerosis (MS) can lead to reduced quality of life, social functioning, and employment. Few studies have investigated cognitive outcomes among patients with pediatric-onset MS (POMS) over the long term. Objective:To compare long-term information-processing efficiency between...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2019.1546
更新日期:2019-06-17 00:00:00
abstract:IMPORTANCE:Allogeneic hematopoietic stem cell transplantation (HSCT) has been the only treatment option clinically available during the last 20 years for juvenile metachromatic leukodystrophy (MLD), reported with variable outcome and without comparison with the natural course of the disease. OBJECTIVE:To compare the l...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2016.2067
更新日期:2016-09-01 00:00:00
abstract:Importance:The pathophysiologic mechanisms of Guillain-Barré syndrome (GBS) associated with Zika virus (ZIKV) infection may be indicated by differences in clinical features. Objective:To identify specific clinical features of GBS associated with ZIKV infection. Design, Setting, and Participants:During the ZIKV epidem...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2018.1058
更新日期:2018-09-01 00:00:00
abstract:Importance:Intracerebral hemorrhage (ICH) has the highest mortality of all stroke types and is the most serious complication of anticoagulation. Data regarding trends in ICH incidence and location-specific risk factors on the population level are conflicting. Objective:To assess long-term population-based trends in th...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.1512
更新日期:2020-06-08 00:00:00
abstract:Importance:It is unclear whether relapses and disease-modifying therapies are associated with the rate of disability accumulation in patients with secondary progressive multiple sclerosis (SPMS). Objective:To examine the association of relapses with the rate of disability accumulation in patients with SPMS and to asse...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.2453
更新日期:2020-07-27 00:00:00
abstract:IMPORTANCE:Smoking tobacco is a well-established risk factor for multiple sclerosis (MS), a chronic inflammatory disorder of the central nervous system usually characterized by bouts and remissions and typically followed by a secondary progressive (SP) course. However, it is not clear whether smoking after diagnosis is...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2015.1788
更新日期:2015-10-01 00:00:00
abstract:Importance:A neurophysiologic signature of the melanopsin-mediated persistent constriction phase of the pupillary light reflex may represent a surrogate biomarker for the integrity of the retinohypothalamic tract, with potential utility for investigating alterations in homeostatic mechanisms associated with brain disor...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2016.5131
更新日期:2017-05-01 00:00:00
abstract:IMPORTANCE:Limbic encephalitis that is associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies (VGKCC syndrome) is an autoimmune, usually nonparaneoplastic form of encephalitis that is responsive to immunotherapy. Differentiating this treatable disease from others that have a similar presentatio...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2013.5179
更新日期:2014-01-01 00:00:00
abstract:IMPORTANCE:Understanding the relationships between age-related changes in brain structure and cognitive function has been limited by inconsistent methods for assessing brain imaging, small sample sizes, and racially/ethnically homogeneous cohorts with biased selection based on risk factors. These limitations have preve...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2014.3418
更新日期:2015-02-01 00:00:00
abstract:IMPORTANCE:Symptoms in the genital region, such as pain, discomfort, tingling, and burning sensations, have rarely been reported in Parkinson disease (PD), and the previous cases were attributed to nonmotor off symptoms. We report a patient with PD and severe genital discomfort unrelated to motor fluctuations but compa...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2014.1326
更新日期:2014-12-01 00:00:00
abstract:IMPORTANCE:Focal cortical dysplasia (FCD), hemimegalencephaly, and megalencephaly constitute a spectrum of malformations of cortical development with shared neuropathologic features. These disorders are associated with significant childhood morbidity and mortality. OBJECTIVE:To identify the underlying molecular cause ...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2016.0363
更新日期:2016-07-01 00:00:00
abstract:Importance:Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain. Objective:To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment ou...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2020.4799
更新日期:2021-01-04 00:00:00
abstract:IMPORTANCE:To provide clinical and genetic diagnoses for patients' conditions, it is important to identify and characterize the different subtypes of spinocerebellar ataxia (SCA). OBJECTIVE:To clinically and genetically characterize a Spanish kindred with pure SCA presenting with altered vertical eye movements. DESIGN...
journal_title:JAMA neurology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamaneurol.2013.2311
更新日期:2013-06-01 00:00:00
abstract:IMPORTANCE:Apolipoprotein E (APOE) ε4 is an established risk factor for cognitive decline and the development of dementia, but other factors may help to minimize its effects. OBJECTIVE:Using APOE ε4 as an indicator of high risk, we investigated factors associated with cognitive resilience among black and white older a...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2014.3978
更新日期:2015-03-01 00:00:00
abstract:Importance:Prolonged seizures in super-refractory status epilepticus (SRSE) have been shown to cause neuronal death and reorganization, and visual inspection in individual case studies has demonstrated progressive cortical and subcortical atrophy. At present, magnetic resonance imaging (MRI) studies that evaluate brain...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2016.1572
更新日期:2016-10-01 00:00:00
abstract:IMPORTANCE:Fungal meningitis due to injections of contaminated methylprednisolone acetate can present with vascular sequelae in immunocompetent individuals. This is particularly germane to neurologists because better recognition of the clinical characteristics of patients with fungal meningitis and ischemic stroke will...
journal_title:JAMA neurology
pub_type: 杂志文章
doi:10.1001/jamaneurol.2013.3586
更新日期:2013-09-01 00:00:00
abstract:IMPORTANCE:Cerebrospinal fluid (CSF) is the compartment in closest proximity to the central nervous system (CNS) parenchyma and might reflect immune pathology in inflammatory CNS disorders like multiple sclerosis (MS). Multiparameter flow cytometry is used to characterize immunological alterations in the CSF of patient...
journal_title:JAMA neurology
pub_type: 杂志文章,评审
doi:10.1001/jamaneurol.2014.395
更新日期:2014-07-01 00:00:00