Abstract:
OBJECTIVE:To report the incidence of prion disease in the United States. METHODS:Prion disease decedents were retrospectively identified from the US national multiple cause-of-death data for 2003-2015 and matched with decedents in the National Prion Disease Pathology Surveillance Center (NPDPSC) database through comparison of demographic variables. NPDPSC decedents with neuropathologic or genetic test results positive for prion disease for whom no match was found in the multiple cause-of-death data were added as cases for incidence calculations; those with cause-of-death data indicating prion disease but with negative neuropathology results were removed. Age-specific and age-adjusted average annual incidence rates were then calculated. RESULTS:A total of 5,212 decedents were identified as having prion disease, for an age-adjusted average annual incidence of 1.2 cases per million population (range 1.0 per million [2004 and 2006] to 1.4 per million [2013]). The median age at death was 67 years. Ten decedents were <30 years of age (average annual incidence of 6.2 per billion); only 2 of these very young cases were sporadic forms of prion disease. Average annual incidence among those ≥65 years of age was 5.9 per million. CONCLUSIONS:Prion disease incidence can be estimated by augmenting mortality data with the results of neuropathologic and genetic testing. Cases <30 years of age were extremely rare, and most could be attributed to exogenous factors or the presence of a genetic mutation. Continued vigilance for prion diseases in all age groups remains prudent.
journal_name
Neurologyjournal_title
Neurologyauthors
Maddox RA,Person MK,Blevins JE,Abrams JY,Appleby BS,Schonberger LB,Belay EDdoi
10.1212/WNL.0000000000008680subject
Has Abstractpub_date
2020-01-14 00:00:00pages
e153-e157issue
2eissn
0028-3878issn
1526-632Xpii
WNL.0000000000008680journal_volume
94pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::The authors report seven patients from six neuro-ophthalmology referral centers who developed pseudo-tumor cerebri during treatment with doxycycline. All four female patients and one of three male patients were obese. Vision was minimally affected in most patients, but two had substantial visual acuity or visual field...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.62.12.2297
更新日期:2004-06-22 00:00:00
abstract:OBJECTIVE:Bevacizumab has been shown to be effective in the treatment of recurrent glioblastoma in combination with chemotherapy compared with historic controls but not in randomized trials. METHODS:We conducted a retrospective analysis of patients treated for recurrent glioblastoma with bevacizumab vs a control group...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000345668.03039.90
更新日期:2009-04-07 00:00:00
abstract::Motivationally responsive motor deficits may occur in isolation or as part of more general neglect syndromes. We describe three patients with two discrete syndromes of isolated motor neglect, differentiated primarily by their performance in motor tasks enacted within or toward the contralesional hemispace. The lesions...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.45.3.525
更新日期:1995-03-01 00:00:00
abstract:OBJECTIVE:Clinical trials of fetal neural tissue transplantation for Huntington disease (HD) have been conducted with variable clinical results. However, no long-term analysis of graft survival and integration has been published. Here, we report the pathologic findings in two patients with HD who died 74 and 79 months ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000264504.14301.f5
更新日期:2007-06-12 00:00:00
abstract::The mitochondrial genome has an underdeveloped "DNA repair repertoire" compared with the nuclear genome, making the mitochondrial DNA more susceptible to mutations by endogenous factors such as defects of the mitochondrial polymerase itself, and by exogenous factors such as radiation and UV light. Increased sensitivit...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.43.2.280
更新日期:1993-02-01 00:00:00
abstract:OBJECTIVE:To analyze the effect of age-related DNA methylation changes in multiple cytosine-phosphate-guanine (CpG) sites (biological age [b-age]) on patient outcomes at 3 months after an ischemic stroke. METHODS:We included 511 patients with first-ever acute ischemic stroke assessed at Hospital del Mar (Barcelona, Sp...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004261
更新日期:2017-08-22 00:00:00
abstract:BACKGROUND:Imaging determinations of the spatial extent of diffuse low-grade gliomas (DLGGs) are of paramount importance in evaluating the risk-to-benefit ratio of surgical resection. However, it is not clear how accurately preoperative conventional MRI can delineate DLGGs. METHODS:We report a retrospective histologic...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181e04264
更新日期:2010-05-25 00:00:00
abstract:OBJECTIVE:To perform a meta-analysis of 4 core CSF biomarkers (β-amyloid [Aβ]42, Aβ40, total tau [t-tau], and phosphorylated tau [p-tau]) to assess which of these are most altered in sporadic cerebral amyloid angiopathy (CAA). METHODS:We systematically searched PubMed for eligible studies reporting data on CSF biomark...
journal_title:Neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1212/WNL.0000000000005030
更新日期:2018-02-27 00:00:00
abstract::The authors conducted a randomized, double-blind, placebo-controlled, twofold crossover study in 16 patients with MS who presented with severe spasticity to investigate safety, tolerability, and efficacy of oral Delta(9)-Tetrahydrocannabinol (THC) and Cannabis sativa plant extract. Both drugs were safe, but adverse ev...
journal_title:Neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1212/wnl.58.9.1404
更新日期:2002-05-14 00:00:00
abstract::Generalized epilepsy with febrile seizures plus (GEFS+) is a clinically and genetically heterogeneous syndrome with childhood onset, characterized by febrile seizures (FS) and a variety of afebrile epileptic seizure types. The authors performed a mutational analysis of SCN1B on 74 unrelated probands with GEFS+, FS, or...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000080362.55784.1c
更新日期:2003-09-23 00:00:00
abstract::A cross-sectional survey and subsequent longitudinal study among diabetic residents of Rochester, MN--The Rochester Diabetic Neuropathy Study (RDNS)--is population-based and uses quantitative, validated, and unique end points to detect, classify, and stage neuropathy. Nondiabetic persons, drawn from the same populatio...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.41.6.799
更新日期:1991-06-01 00:00:00
abstract:BACKGROUND:Ullrich congenital muscular dystrophy (UCMD) is a form of merosin-positive congenital muscular dystrophy characterized by proximal contractures, distal laxity, rigidity of the spine, and respiratory complications. Recently, a deficiency of collagen VI on muscle and skin biopsy together with recessive mutatio...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.58.9.1354
更新日期:2002-05-14 00:00:00
abstract::An emerging issue in stroke prevention and stroke treatment is that therapies shown to be effective in clinical trials are not being used or are being used suboptimally. One of many explanations relates to cost, ranging from concerns about inappropriate use of societal resources to more immediate concerns of organizat...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.51.3_suppl_3.s31
更新日期:1998-09-01 00:00:00
abstract:OBJECTIVE:To determine the diagnostic accuracy of confrontation visual field testing and to compare the accuracy of confrontation tests both individually and in combination. METHODS:Patients were prospectively recruited from ophthalmology clinics over a 6-month period. All patients underwent SITA-standard 24-2 Humphre...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181d90017
更新日期:2010-04-13 00:00:00
abstract::In the Clinical/Scientific Note “CNS neutrophilic vasculitis in neuro-Sweet disease” by R. Charlson et al. (Neurology® 2015;85:829–830), there is an error in the discussion and the references. The last name of the first author of reference 2 should be Hisanaga, not Hisanga as originally published. The authors regret t...
journal_title:Neurology
pub_type: 已发布勘误
doi:10.1212/WNL.0000000000002139
更新日期:2015-11-17 00:00:00
abstract:BACKGROUND:Clinical trials involving patients with glioblastoma (GBM) distinguish cohorts who are treated with enzyme-inducing anticonvulsants (EIAC). Such anticonvulsants induce hepatic P450 microsomal enzymes, which accelerate the metabolism of certain chemotherapy and molecular targeted agents. However, the resultan...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181bbfeca
更新日期:2009-10-13 00:00:00
abstract::We studied a family in which the father and 7 of 10 children had episodes of Bell's palsy. Five of the eight affected family members also had ocular motor palsies. Facial nerve and blink reflex studies in four affected siblings demonstrated asymmetrically reduced amplitude of evoked responses without delayed conductio...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.37.8.1369
更新日期:1987-08-01 00:00:00
abstract::In Icelandic pedigrees a cystatin C mutation, glutamine 68 (L68Q), causes autosomal dominant cerebral amyloid angiopathy-related hemorrhage (CAAH). We examined 33 patients with sporadic CAAH for this mutation. None carried L68Q and, including this report, only one of 52 published cases of sporadic CAAH has had the cys...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.54.1.242
更新日期:2000-01-11 00:00:00
abstract:OBJECTIVE:To examine associations of orthostatic hypotension (OH) with dementia and long-term cognitive decline and to update previously published results in the same cohort for stroke with an additional 16 years of follow-up. METHODS:We analyzed data from 11,709 participants without a history of coronary heart diseas...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000006027
更新日期:2018-08-21 00:00:00
abstract:OBJECTIVE:To evaluate interictal, circulating sphingolipids in women migraineurs. METHODS:In the fasting state, serum samples were obtained pain-free from 88 women with episodic migraine (EM; n=52) and from controls (n=36). Sphingolipids were detected and quantified by high-performance liquid chromatography coupled wi...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000002004
更新日期:2015-10-06 00:00:00
abstract:OBJECTIVES:To identify gene mutations in patients with dystroglycanopathy and prove pathogenicity of those mutations using an in vitro cell assay. METHODS:We performed whole-exome sequencing on 20 patients, who were previously diagnosed with dystroglycanopathy by immunohistochemistry and/or Western blot analysis. We a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001162
更新日期:2015-01-20 00:00:00
abstract:OBJECTIVE:The aim of the study was to perform a third cognitive assessment in our pediatric-onset multiple sclerosis (MS) patient cohort and determine predictors of the individual cognitive outcome. METHODS:After 4.7 ± 0.7 years from baseline evaluation, 48 of 63 patients in the original cohort were reassessed on an e...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000000885
更新日期:2014-10-14 00:00:00
abstract::We report a longitudinal follow-up study on six patients with chronic manganese-induced parkinsonism following cessation of manganese exposure. Compared with the 1987 study, their parkinsonian symptoms showed a slow progression, particularly in gait disturbances such as freezing during turning and walking backward wit...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.8.1479
更新日期:1993-08-01 00:00:00
abstract::Subarachnoid neurolysis using ethanol to destroy selectively the posterior roots of the spinal cord is a method for providing pain relief in patients with advanced cancer. Weakness of the extremities is a complication of the procedure that has been attributed to spread of the neurolytic agent to the anterior roots. Th...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.54.7.1522
更新日期:2000-04-11 00:00:00
abstract::The association between migraine and cervical artery dissection (CAD) was explored in a hospital-based case-control study. Migraine was present in 49.1% (23/47) of patients with CAD and in 21% (11/52) of patients hospitalized for a cerebral ischemic event not related to a CAD (adjusted odds ratio = 3.6; 1.5 to 8.6, p ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.59.3.435
更新日期:2002-08-13 00:00:00
abstract::We retrospectively studied the effects of changing antiepileptic drug levels on patterns of seizure discharge in 8 patients investigated with intracerebral electrodes during presurgical evaluation. We compared seizures recorded at high levels of medication to seizures recorded at low levels for changes in seizure dura...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.40.3_part_1.407
更新日期:1990-03-01 00:00:00
abstract::In the current medical climate, medical education is at risk of being de-emphasized, leading to less financial support and compensation for faculty. A rise in compensation plans that reward clinical or research productivity fails to incentivize and threatens to erode the educational missions of our academic institutio...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000007664
更新日期:2019-07-02 00:00:00
abstract::This manuscript provides a practical summary of guidelines for institution of apomorphine subcutaneous injectable therapy, including patient education, pre-treatment issues, dosage titration and side-effect care. The timing of each injection is crucial if an impending "off" period is to be averted. Patients need to be...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.62.6_suppl_4.s32
更新日期:2004-03-23 00:00:00
abstract:OBJECTIVE:To determine whether brain function is altered in cognitively normal individuals at high risk for AD several years before the typical age at onset for this illness. BACKGROUND:Neuropathologic alterations in AD precede cognitive impairment by several years. It is unknown whether functional alterations in neur...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.53.7.1391
更新日期:1999-10-22 00:00:00
abstract:OBJECTIVE:Gingival overgrowth is an important adverse effect of phenytoin (PHT) therapy, occurring in about half of the patients. This study aimed to evaluate the effect of oral folic acid supplementation (0.5 mg/day) for the prevention of PHT-induced gingival overgrowth (PIGO) in children with epilepsy aged 6-15 years...
journal_title:Neurology
pub_type: 杂志文章,随机对照试验
doi:10.1212/WNL.0b013e3182152844
更新日期:2011-04-12 00:00:00