Epidemiology of hereditary ataxias in Spain: hospital discharge registry and population-based mortality study.

Abstract:

BACKGROUND:Hereditary ataxias (HA) comprise a group of genetically heterogeneous rare diseases. As important public health problems to be monitored, this study analyses the morbimortality of HA in Spain. METHODS:Data were extracted from the national death index (1981-2008), using the International Classification of Diseases (ICD) 9th revision code 334 until 1998, and 10th revision code G11 from 1999 onwards. ICD-9 codes were then selected from the national discharge dataset (1998-2007). Age-adjusted morbidity and mortality rates were obtained by gender and 5-year period. RESULTS:Of the 610 HA deaths from 1981 to 2008, 277 corresponded to Friedreich's ataxia (45.4%) and 333 (54.6%) to other and unspecified ataxias (non-Friedreich group). Both groups showed an increase in mortality trend, which was more pronounced in males from 1985-1989 to 1990-1994. Geographical distribution of mortality revealed higher risk for males, mainly in the north of Spain. A total of 5,341 HA hospitalisations were identified from 1998 to 2007. The average annual age-adjusted hospitalisation rate was 1.19 per 100,000 population, with a rising trend. CONCLUSION:This increase in morbidity and mortality, coupled with the slight interprovincial differences, indicate that more attention should be paid to these rare diseases by public authorities and society alike.

journal_name

Neuroepidemiology

journal_title

Neuroepidemiology

authors

Alonso V,Villaverde-Hueso A,Hens MJ,Morales-Piga A,Abaitua I,Posada de la Paz M

doi

10.1159/000346275

subject

Has Abstract

pub_date

2013-01-01 00:00:00

pages

13-9

issue

1

eissn

0251-5350

issn

1423-0208

pii

000346275

journal_volume

41

pub_type

杂志文章
  • Vascular disease among hospitalized multiple sclerosis patients.

    abstract:BACKGROUND:We examined the prevalence of cardiac and cerebrovascular disease among hospitalized patients with and without multiple sclerosis (MS). METHODS:This study used the Statewide Planning and Research Cooperate System data set of over 15 million hospitalizations in New York City from 1988 through 2002. We identi...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000128103

    authors: Allen NB,Lichtman JH,Cohen HW,Fang J,Brass LM,Alderman MH

    更新日期:2008-01-01 00:00:00

  • The relation of cigarette smoking to incident Alzheimer's disease in a biracial urban community population.

    abstract::The relationship between smoking status and incident Alzheimer's disease (AD) was investigated in a random stratified sample of a biracial community in Chicago. Analyses are based on 1,064 persons (of 1,134 evaluated) who had data on smoking status, disease incidence, and key covariates such as apolipoprotein allele s...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000091654

    authors: Aggarwal NT,Bienias JL,Bennett DA,Wilson RS,Morris MC,Schneider JA,Shah RC,Evans DA

    更新日期:2006-01-01 00:00:00

  • Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis.

    abstract:BACKGROUND:Prevalence and incidence rates of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are required to determine the impact of CIDP on society. We aimed to estimate the prevalence and incidence of CIDP worldwide and to determine the effect of diagnostic criteria on prevalence and incidence. METH...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章,meta分析

    doi:10.1159/000494291

    authors: Broers MC,Bunschoten C,Nieboer D,Lingsma HF,Jacobs BC

    更新日期:2019-01-01 00:00:00

  • Prevalence, Mortality, and Cause of Death in Charcot-Marie-Tooth Disease in Korea: A Nationwide, Population-Based Study.

    abstract:BACKGROUND:Charcot-Marie-Tooth disease (CMT) is a group of clinically and genetically heterogeneous disorders that primarily affect the peripheral nervous system. Epidemiological studies of CMT have not yet been performed in Korea. OBJECTIVES:This study was performed to estimate the prevalence of CMT in Korea and the ...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000505815

    authors: Park HJ,Choi YC,Oh JW,Yi SW

    更新日期:2020-01-01 00:00:00

  • Cerebral haemodynamics in the elderly: the rotterdam study.

    abstract::Transcranial Doppler ultrasonography allows the non-invasive assessment of cerebral haemodynamics. Data on the frequency distribution of these parameters in the oldest old are scarce, which makes a distinction between physiological and pathophysiological ageing difficult. We studied the relation between cerebral haemo...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000078503

    authors: Bakker SL,de Leeuw FE,den Heijer T,Koudstaal PJ,Hofman A,Breteler MM

    更新日期:2004-07-01 00:00:00

  • Assessment of cerebrovascular risk profiles in healthy persons: definition of research goals and the Austrian Stroke Prevention Study (ASPS).

    abstract::The advent of new laboratory methods and noninvasive imaging modalities has extended the diagnostic possibilities in normal individuals. This article elaborates the new options for the assessment of stroke risk offered by these techniques. In this context we present the Austrian Stroke Prevention Study, which is the f...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000110396

    authors: Schmidt R,Lechner H,Fazekas F,Niederkorn K,Reinhart B,Grieshofer P,Horner S,Offenbacher H,Koch M,Eber B

    更新日期:1994-01-01 00:00:00

  • Spatial Assessment of the Association between Long-Term Exposure to Environmental Factors and the Occurrence of Amyotrophic Lateral Sclerosis in Catalonia, Spain: A Population-Based Nested Case-Control Study.

    abstract:BACKGROUND:It is believed that an interaction between genetic and non-genetic factors may be involved in the development of amyotrophic lateral sclerosis (ALS). With the exception of exposure to agricultural chemicals like pesticides, evidence of an association between environmental risk factors and ALS is inconsistent...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000489664

    authors: Povedano M,Saez M,Martínez-Matos JA,Barceló MA

    更新日期:2018-01-01 00:00:00

  • Creutzfeldt-Jakob disease in a recipient of a dura mater graft processed in the US: cause or coincidence?

    abstract::Iatrogenic Creutzfeldt-Jakob disease (CJD) has never been reported among recipients of dura mater grafts processed in the US. We recently investigated a report of such a case in a 72-year-old man with a typical clinical presentation of CJD. We found no evidence of CJD in either the 34-year-old donor or in other, proxi...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000026240

    authors: Dobbins JG,Belay ED,Malecki J,Buck BE,Bell M,Cobb J,Schonberger LB

    更新日期:2000-03-01 00:00:00

  • Trends in risk of stroke in patients with a hospital diagnosis of nonvalvular atrial fibrillation: National Cohort Study in Denmark, 1980-2002.

    abstract:AIM:We examined trends in incidence of stroke of any nature (ischemic and/or hemorrhagic) in subjects with a hospital diagnosis of nonvalvular atrial fibrillation or flutter in Denmark from 1980 to 2002 by sex, age and conditions of comorbidity. METHODS:We identified all individuals, aged 40-89 years, with an incident...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000092795

    authors: Frost L,Vukelic Andersen L,Vestergaard P,Husted S,Mortensen LS

    更新日期:2006-01-01 00:00:00

  • Sociodemographic factors and primary headache syndromes in a Saudi community.

    abstract:BACKGROUND AND PURPOSE:There is increasing evidence that the pathophysiologic mechanisms are different between migraine and tension-type headache. The aim of the study was to determine possible sociodemographic differences in Saudi Arabia subjects with headache. METHODOLOGY:A community-based door-to-door survey with i...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000109670

    authors: Abdul Jabbar M,Ogunniyi A

    更新日期:1997-01-01 00:00:00

  • Does Alzheimer's disease protect against cancers? A nationwide population-based study.

    abstract:BACKGROUND:Previous studies suggested a decreased risk of cancer among patients with Alzheimer's disease (AD). There is still a lack of data on the specific types of cancer, the risk factors, and the impact of cholinesterase inhibitors on developing cancer in AD. METHODS:We performed a nationwide population-based stud...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000341411

    authors: Ou SM,Lee YJ,Hu YW,Liu CJ,Chen TJ,Fuh JL,Wang SJ

    更新日期:2013-01-01 00:00:00

  • Prevalence of Common Types of Compression Neuropathies in Qena Governorate/Egypt: A Population-Based Survey.

    abstract:BACKGROUND:No epidemiological studies on the prevalence of compressive neuropathy have been undertaken in Arab countries. The aim of the study was to estimate the prevalence of the most common types of compressive neuropathies in Qena governorate/Egypt. METHODS:The study was part of a community-based survey carried ou...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000444641

    authors: Khedr EM,Fawi G,Allah Abbas MA,El-Fetoh NA,Zaki AF,Gamea A

    更新日期:2016-01-01 00:00:00

  • The prevalence of cognitive impairment among nursing home residents in Taipei, Taiwan.

    abstract::A random sample of nursing home residents over 65 years of age were interviewed. Two hundred ninety-two participants received the Short Portable Mental Status Questionnaire. Two hundred eleven were unable to answer the questionnaire, but their caregivers provided information about their daily activities and performanc...

    journal_title:Neuroepidemiology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1159/000026166

    authors: Wu SC,Ke D,Su TL

    更新日期:1998-01-01 00:00:00

  • High prevalence of calcified silent neurocysticercosis in a rural village of Mexico.

    abstract::Human neurocysticercosis (NC) is a parasitic disease caused by TAENIA SOLIUM when its larvae lodge in the central nervous system. NC prevalence estimates are obscured by the variable and often asymptomatic clinical picture. While infection depends on exposure, severity is possibly related with various host factors (im...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000068748

    authors: Fleury A,Gomez T,Alvarez I,Meza D,Huerta M,Chavarria A,Carrillo Mezo RA,Lloyd C,Dessein A,Preux PM,Dumas M,Larralde C,Sciutto E,Fragoso G

    更新日期:2003-03-01 00:00:00

  • Short Sitting Height and Low Relative Sitting Height Are Associated with Severe Cognitive Impairment among Older Women in an Urban Community in China.

    abstract:BACKGROUND:Anthropometric indexes are powerful indicators of the environment and the plasticity of the human body. This study aimed at exploring the anthropometric indexes that are associated with late-life cognition impairment among the elderly Chinese in the Shanghai Aging Study. METHODS:The height, weight, and sitt...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000439569

    authors: Liang X,Li X,Ding D,Zhao Q,Luo J,Guo Q,Hong Z

    更新日期:2015-01-01 00:00:00

  • Validity and reliability of the 'Ten Questions' questionnaire for detecting moderate to severe neurological impairment in children aged 6-9 years in rural Kenya.

    abstract:BACKGROUND:The 'Ten Questions' Questionnaire (TQQ) is used to detect severe neurological impairment in children living in resource-poor countries. Its usefulness has been established in Asia and the Caribbean, but there are a few published studies from Africa. We evaluated the TQQ as part of a larger study of neurologi...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000073977

    authors: Mung'ala-Odera V,Meehan R,Njuguna P,Mturi N,Alcock K,Carter JA,Newton CR

    更新日期:2004-01-01 00:00:00

  • Higher Education Is Associated with a Lower Risk of Dementia after a Stroke or TIA. The Rotterdam Study.

    abstract:BACKGROUND:Higher education is associated with a lower risk of dementia, possibly because of a higher tolerance to subclinical neurodegenerative pathology. Whether higher education also protects against dementia after clinical stroke or transient ischemic attack (TIA) remains unknown. METHODS:Within the population-bas...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000443649

    authors: Mirza SS,Portegies ML,Wolters FJ,Hofman A,Koudstaal PJ,Tiemeier H,Ikram MA

    更新日期:2016-01-01 00:00:00

  • Genetic analysis of late-onset diseases using first-degree relatives.

    abstract::We consider the problem of testing that a disease with variable age at onset is an autosomal dominant or recessive genetic trait. Specific reference is made to Alzheimer's disease and recent publications proposing an autosomal dominant inheritance pattern. The pedigree structure allowed is restricted to first-degree r...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000110139

    authors: Sobel E,Davanipour Z,Alter M

    更新日期:1988-01-01 00:00:00

  • Parkinson's and Alzheimer's diseases: epidemiological comparison. 2. Persons at risk.

    abstract::Parkinson's and Alzheimer's diseases are age-related degenerative disorders that share common clinical, pathological and biochemical features. Epidemiological studies demonstrate a similar distribution of the disease in place and time. Causes of death and populations at higher risk are similar. These findings suggest ...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章,评审

    doi:10.1159/000110337

    authors: Treves TA,Chandra V,Korczyn AD

    更新日期:1993-01-01 00:00:00

  • The incidence of dementia and intake of animal products: preliminary findings from the Adventist Health Study.

    abstract::We investigated the relationship between animal product consumption and evidence of dementia in two cohort substudies. The first enrolled 272 California residents matched for age, sex, and zip code (1 vegan, 1 lacto-ovo-vegetarian, and 2 'heavy' meat eaters in each of 68 quartets). This design ensured a wide range of ...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000110296

    authors: Giem P,Beeson WL,Fraser GE

    更新日期:1993-01-01 00:00:00

  • Patterns of mortality from hereditary ataxias in the United States, 1971 and 1973-1978.

    abstract::Mortality rates for deaths 'due to' and 'with' hereditary ataxias are presented for the first time. Age-adjusted mortality rates were higher for whites than for nonwhites of either sex, and for men in both racial groups. Age-specific mortality rates for whites showed a peak for 'hereditary spinal ataxia' in the 20-29 ...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000110182

    authors: Leone M,Chandra V,Schoenberg BS

    更新日期:1989-01-01 00:00:00

  • Stroke etiology among Haitians living in Miami.

    abstract::The black Caribbean population continues to grow in the US and little is known about stroke etiologies in that community. We examined stroke subtypes in 175 consecutive Haitian-born patients living in Miami, admitted for acute stroke. Ischemic stroke was diagnosed in 72%. Small vessel occlusion was the most frequent s...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000087449

    authors: Koch S,Pabon D,Rabinstein AA,Chirinos J,Romano JG,Forteza A

    更新日期:2005-01-01 00:00:00

  • Population-based epidemiological study of neurological diseases in Taiwan: I. Creutzfeldt-Jakob disease and multiple sclerosis.

    abstract:BACKGROUND:The purpose of the study is to investigate the epidemiology of Creutzfeldt-Jakob disease (CJD) and multiple sclerosis (MS) in Taiwan. METHODS:Cases of CJD and MS were identified from the National Health Insurance Research Database from January 2000 to December 2005. Age- and sex-specific incidences of these...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000229779

    authors: Lai CH,Tseng HF

    更新日期:2009-01-01 00:00:00

  • Environmental Factors and the Risk of Brain Tumours in Young People: A Systematic Review.

    abstract:BACKGROUND:Brain tumours (BT) are one of the most frequent tumour types in young people, although little is known about their risk factors. OBJECTIVE:The objective of the current work was to review and summarize the scientific literature concerning exposure to environmental factors and BT risk in young people (<25 yea...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000500601

    authors: Zumel-Marne A,Castano-Vinyals G,Kundi M,Alguacil J,Cardis E

    更新日期:2019-01-01 00:00:00

  • Population-based validation of the iScore for predicting mortality and early functional outcome in ischemic stroke patients.

    abstract:BACKGROUND:We aimed to determine the accuracy of the iScore for predicting mortality and early disability following ischemic stroke in a French population-based study. METHODS:All patients with acute ischemic stroke were identified among residents of the city of Dijon, France, between 2006 and 2011, using a population...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000354634

    authors: Béjot Y,Jacquin A,Daubail B,Durier J,Giroud M

    更新日期:2013-01-01 00:00:00

  • Neuroepidemiologic research initiatives on Guam: past and present.

    abstract::Since the middle of this century, a remarkable concentration of cases of neurodegenerative disease(s), referred to as amyotrophic lateral sclerosis and Parkinson-dementia complex (ALS/PDC), has been recognized among Chamorro natives of Guam. Intense investigations over the last 4 decades have failed to determine the e...

    journal_title:Neuroepidemiology

    pub_type: 历史文章,杂志文章,评审

    doi:10.1159/000026223

    authors: Wiederholt WC

    更新日期:1999-01-01 00:00:00

  • Homocysteine as a risk factor for ischemic stroke: an epidemiological story in evolution.

    abstract::Homocysteine is a metabolite of methionine that may be remethylated by enzymes requiring folate and cobalamin (vitamin B12) to again form methionine or catabolized by the pyridoxine (vitamin B6) dependent enzyme, cystathionine beta synthase (CBS) to form cysteine (fig. 1) [1]. Homocysteine exists as a combination of v...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章,评审

    doi:10.1159/000026169

    authors: Sacco RL,Roberts JK,Jacobs BS

    更新日期:1998-01-01 00:00:00

  • Estimation of short-term effects of air pollution on stroke hospital admissions in southern Sweden.

    abstract:BACKGROUND:Short-term exposure to high levels of air pollution can increase stroke risk. In this study we investigated the short-term effects of air pollution on hospital admissions for stroke in a setting where pollutant levels are rather low. We also addressed methodological issues in evaluating the short-term effect...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000274807

    authors: Oudin A,Strömberg U,Jakobsson K,Stroh E,Björk J

    更新日期:2010-01-01 00:00:00

  • Morphometric analysis of gray matter volume in demented older adults: exploratory analysis of the cardiovascular health study brain MRI database.

    abstract::We tested the feasibility of a fully automated brain MRI voxel count technique--automated labeling pathway (ALP)--in a sample of 15 demented and 13 cognitively normal women (age 75-85 years) participating to the Cardiovascular Health Study (CHS). We hypothesized that ALP would replicate well-established findings of th...

    journal_title:Neuroepidemiology

    pub_type: 临床试验,杂志文章

    doi:10.1159/000085140

    authors: Rosano C,Becker J,Lopez O,Lopez-Garcia P,Carter CS,Newman A,Kuller L,Aizenstein H

    更新日期:2005-01-01 00:00:00

  • Population stratification in a case-control study of brain arteriovenous malformation in Latinos.

    abstract:BACKGROUND:Genetic association studies conducted in admixed populations may be confounded by population stratification resulting in spurious associations. The purpose of this pilot study was to determine the presence and effect of population stratification in a case-control study of brain arteriovenous malformation (BA...

    journal_title:Neuroepidemiology

    pub_type: 杂志文章

    doi:10.1159/000160215

    authors: Kim H,Hysi PG,Pawlikowska L,Choudhry S,González Burchard E,Kwok PY,Sidney S,McCulloch CE,Young WL

    更新日期:2008-01-01 00:00:00