Abstract：
INTRODUCTION:Type 1 myotonic dystrophy (MD-1) or Steinert disease is a multisystemic progressive disorder. Studies have shown cognitive deficits, depressive symptoms and a high incidence of anxiety personality traits that compromise both the functionality and the quality of life of these patients. AIM:To describe the cognitive and psychopathological profile of a sample of patients with the adult variant of MD-1. PATIENTS AND METHODS:A sample of 27 patients diagnosed with MD-1 was selected from those being followed up in the neurology service of the Complejo Hospitalario de Navarra. Eligibility criteria were age under 50 years and the absence of any other pathology or physical condition that prevented them from performing the psychological evaluation. A neuropsychological evaluation battery specifically designed for this kind of pathology was used, together with psychopathological and functionality measures. RESULTS:The neuropsychological evaluation revealed mainly deficits in visual-constructional and visuospatial skills, alternating attention and in other-reported dysexecutive signs and symptoms. The group of patients did not present any clinically significant symptoms of depression or anxiety, but did score high on obsession-compulsion, interpersonal sensitivity, paranoid ideation and psychoticism. The results point towards a deterioration in functionality. CONCLUSIONS:In the integral approach to MD-1, the characterisation and developmental monitoring of the cognitive, psychopathological and personality profile, as well as the level of functionality, all contribute to an improvement in the quality of life of these patients. TITLE:Estudio descriptivo del perfil neuropsicologico y psicopatologico en pacientes con distrofia miotonica tipo 1. :Introduccion. La distrofia miotonica tipo 1 (DM-1) o enfermedad de Steinert es un trastorno multisistemico y progresivo. Se han encontrado deficits cognitivos, clinica depresiva y alta incidencia de rasgos de personalidad ansiosos con afectacion tanto en la funcionalidad como en la calidad de vida de estos pacientes. Objetivo. Describir el perfil cognitivo y psicopatologico de una muestra de pacientes con la variante adulta de DM-1. Pacientes y metodos. Se selecciono una muestra de 27 pacientes con diagnostico de DM-1 en seguimiento en el Servicio de Neurologia del Complejo Hospitalario de Navarra. Los criterios de inclusion fueron tener menos de 50 anos y descartar cualquier otra patologia o condicion fisica que impidiese realizar la evaluacion psicologica. Se utilizo una bateria de evaluacion neuropsicologica especificamente disenada para este tipo de patologia, ademas de medidas de psicopatologia y funcionalidad. Resultados. La evaluacion neuropsicologica reflejo, principalmente, deficits en habilidades visuoconstructivas, visuoespaciales, atencion alternante y en sintomatologia disejecutiva heteroinformada. El grupo de pacientes no presento sintomatologia depresiva ni ansiosa clinicamente significativa, pero si puntuaciones elevadas en obsesion-compulsion, sensibilidad interpersonal, ideacion paranoide y psicoticismo. Los resultados orientaron hacia un deterioro en la funcionalidad. Conclusiones. En el abordaje integral de la DM-1, la caracterizacion y el seguimiento evolutivo del perfil cognitivo, psicopatologico y de personalidad, asi como del nivel de funcionalidad, contribuyen a la mejora de la calidad de vida de estos pacientes.
journal_name
Rev Neuroljournal_title
Revista de neurologiaauthors
Seijas-Gomez R,Basterra-Jimenez I,Luna-Lario P,Tirapu-Ustarroz J,Cabada-Giadas T,Iridoy-Zulet M,Jerico-Pascual I,Gargallo-Vaamonde Á,Lopez-Goni JJsubject
Has Abstractpub_date
2015-12-16 00:00:00pages
529-35issue
12eissn
0210-0010issn
1576-6578pii
rn2015299journal_volume
61pub_type
杂志文章abstract：AIM:To evaluate the performance of patients with Alzheimer's disease (AD) in the mild-moderate stage in a verbal material abstraction task that involves interpreting the implicit meaning of proverbs and sayings. SUBJECTS AND METHODS:A qualitative-quantitative analysis was carried out of the performance of 30 patients ...
journal_title：Revista de neurologia
pub_type： 杂志文章
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更新日期：2009-12-01 00:00:00
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abstract：INTRODUCTION:Piribedil is a D2 D3 dopamine agonist, which has been shown to be well tolerated and to improve Parkinsonian symptoms, particularly tremor. However, few studies have been published about this Dopamine Agonist as an adjunct to levodopa therapy in patients with Parkinson's disease (PD). This placebo controll...
journal_title：Revista de neurologia
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：2004-04-16 00:00:00
abstract：INTRODUCTION:In all cases of young persons with clinical Parkinson s disease it should be suspected that it is secondary to some primary disorder. Therefore a battery of diagnostic tests should be done before classification as idiopathic Parkinson s disease. CLINICAL CASE:A 31 year old woman whose only previous illnes...
journal_title：Revista de neurologia
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abstract：:Neuroinflammation is a key process in the neuropathogenesis of AIDS virus since as a result of the aberrant activation of the chemokine receptors (CXCR4, CX3CR1 and CR5) produces proinflammatory cytokine release by infected cells, increases microglial neurotoxicity and generates lipoperoxides and reactive oxygen speci...
journal_title：Revista de neurologia
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更新日期：2011-01-16 00:00:00
abstract：AIMS:The principal aims of this review are: (a) to offer a perspective of the principal challenges that forensic neuropsychology handles in its scientific and professional development, and the empirical advances that have been developed to deal with these challenges; and (b) analyze the potential effects of these advan...
journal_title：Revista de neurologia
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更新日期：2004-07-01 00:00:00
abstract：INTRODUCTION:Oxidative stress increases brain lipid peroxidation, memory and motor deficits and progressive neurodegeneration. Tibolone, a treatment for menopausal symptoms, decreases lipid peroxidation levels and improves memory and learning. AIM:To study the effect of chronic administration of tibolone on lipid pero...
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更新日期：2014-05-16 00:00:00
abstract：TITLE:Equivalente migrañoso de inicio tardío con aura prolongada y alteración de la perfusión cerebral. : ...
journal_title：Revista de neurologia
pub_type： 信件
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abstract：:In recent years, image techniques have transformed the diagnosis in Medicine. Nevertheless, they are, by now, far from a widespread clinic and surgical use. Beyond the simple picture inspection, DICOM digital files can be explored by using image analysis/processing techniques in a better way. In practice, clinicians a...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2011-10-16 00:00:00
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journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2000-12-01 00:00:00
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更新日期：2006-12-16 00:00:00
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journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2004-09-01 00:00:00
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journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2009-04-16 00:00:00
abstract：INTRODUCTION:Only the organic and cryptogenic forms of frontal lobe epilepsy have been admitted by the ILAE, but according to several reports in the literature, it probably exists also an idiopathic form, at present not well recognized. OBJECTIVE:To study the differences between the organic and the cryptogenic forms o...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：1998-06-01 00:00:00
abstract：:Autism is a developmental disorder affecting social relationships, communication and flexibility of thought. These three basic aspects of autism may present in many different forms and degrees. Therefore autism should be considered to be a spectrum of autistic disorders rather than a single strictly defined condition....
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1999-02-01 00:00:00
abstract：INTRODUCTION:Severe myoclonic epilepsy in infancy (SMEI), or Dravet s syndrome, is one of the most serious forms of epilepsy in infancy. In this study we analyse the clinical characteristics of the process. PATIENTS AND METHODS:The cases reported in the literature are surveyed, together with a personal casuistic, from...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2003-07-01 00:00:00
abstract：INTRODUCTION:There is increasing evidence that B cells and humoral immunity play key roles in the pathogenesis of multiple sclerosis (MS). Ocrelizumab, an anti-CD20 monoclonal antibody, has been shown to be effective in controlling the disease and has recently been aproved by the Food and Drug Administration for the tr...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2018-01-01 00:00:00
abstract：INTRODUCTION:Behavior disorders are frequently seen in children attending a neuropaediatric clinic. The classical neuroleptic drugs are usually used for treatment. However response tends to be irregular with adverse effects at a cognitive level and extrapyramidal symptoms. PATIENTS AND METHODS:We started to use risper...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2001-08-01 00:00:00
abstract：INTRODUCTION:The early years of life, more especially the first three, are dominated by a series of anatomical-functional facts that express the progressive enrichment of behaviour through the process of neurological maturation, which is expressed as reaching the maximum level of functioning at each stage of developmen...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2013-09-06 00:00:00
abstract：INTRODUCTION:The rapid-onset dystonia-parkinsonism is a movement disorder which associates dystonic symptoms, especially those affecting orofacial muscles, and parkinsonian symptoms. All these symptoms start suddenly and then they stabilize along the process. This disorder usually occurs to young adults and is an autos...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2008-12-16 00:00:00
abstract：INTRODUCTION:SPECT, EEG AND CT scan offer information with several pathophysiologic meanings. Their results vary with time and according to the vascular affected territory. OBJECTIVE:We wanted to study how the sensibility varies and the relationship with the clinic of SPECT, qEEG and CT scan in the acute, subacute and...
journal_title：Revista de neurologia
pub_type： 临床试验,杂志文章
doi：
更新日期：1998-08-01 00:00:00
abstract：INTRODUCTION:Congenital insensitivity to pain with anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV (HSAN IV) is a rare autosomal recessive disorder featuring recurrent fever episodes, inability to sweat, absent response to noxious stimuli, self mutilating behavior and mental retardation. It has...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2005-08-16 00:00:00
abstract：INTRODUCTION AND DEVELOPMENT:Attention deficit hyperactivity disorder (ADHD) is one of the most frequently observed processes in our Neuropaediatric unit, and in recent years has become one of the leading reason for visits, together with headaches. On occasions, the diagnosis that should be made, in accordance with the...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2004-02-01 00:00:00
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journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2000-05-01 00:00:00
abstract：INTRODUCTION:Mental retardation (MR) is a clinical condition that may be due to a large variety of causes, the most important of which are those of a genetic origin, owing to the repercussions they can have on the family. There are more than one thousand types of MR with a genetic origin, and they are not usually found...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2006-01-07 00:00:00
abstract：INTRODUCTION:In the early stages of their development, children acquire the skills and knowledge that provide them with the foundations on which they will later add what they learn at school, including skills such as reading, writing and mathematics. The presence of learning difficulties at the infantile stage can affe...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2006-02-13 00:00:00
abstract：INTRODUCTION:Congenital malformations of the brain often are complex disorders which display a wide spectrum of neurological manifestations. The presence of this diversity makes it difficult to correlate clinical findings with neuropathological abnormalities or through neuroimaging studies. DEVELOPMENT:Furthermore, th...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：1999-01-16 00:00:00
abstract：INTRODUCTION:Latin American medicine in the 16th century produced the first collections of herbs with native plants from the New World. Treatises on popular therapies appeared, which included gallenic remedies as well as incorporating autochthonous plants. We review the main neurological diseases and their treatments d...
journal_title：Revista de neurologia
pub_type： 传,历史文章,杂志文章
doi：
更新日期：2009-08-01 00:00:00
abstract：INTRODUCTION:The epidemiology of multiple sclerosis (ME) in the Alcoi area has been studied by our groups for many years. When studying the factors linked to ME in this area initially we observed that migration, contact with dogs and the textile industry were associated with the disorder. A later study, in which the mi...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2002-05-01 00:00:00
abstract：OBJECTIVE:To evaluate the clinical, electro-encephalographic and evolutionary characteristics of a series of patients diagnosed as having benign neonatal sleep myoclonus (BNSM). MATERIAL AND METHODS:The clinical histories of 21 patients with BNSM were analyzed. Criteria for inclusion in the study were: neonates who ha...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
