Abstract：
INTRODUCTION:Progressive cerebral polidystrophy or Alpers syndrome is a clinico-pathological picture, with no specific biological marker, characterized by involvement mainly of the cerebral grey matter and which shows clinically as a rapidly progressive encephalopathy with intractable seizures, usually myoclonic. The typical picture starts, after a normal neonatal period, during the first two years of life. CLINICAL CASE:A boy who after some previous difficulty with school-work presented with epilepsy at the age of 10 years and when he was 11 years old had a sudden illness with intractable seizures and severe neurological deterioration with spastic-dystonic tetraparesia, absence of visual function and minimal social contact to vocal or tactile stimuli. He had had a previously normal brother who died at the age of seven years during status epilepticus. Skin and muscle biopsies showed increase in the number and size of the mitochondria. Study of the respiratory chain in muscle showed a partial deficit in the activity of cytochrome C oxidase. CAT scanning showed marked generalized atrophy after four years. CONCLUSIONS:This case fulfils the criteria for Alpers syndrome established by Adams and Lyon in 1996. We consider that in the context of Alpers syndrome ultrastructural changes in the mitochondria of skin and muscle and partial deficit of enzyme activity of the IV complex of the respiratory chain should be evaluated. We emphasize the late presentation of Alpers syndrome, which has rarely been reported in the literature.
journal_name
Rev Neuroljournal_title
Revista de neurologiaauthors
Galván-Manso M,Tabuenca Y,Medrano P,Ruiz-Escusol S,Giménez-Mas JA,López-Pisón Jsubject
Has Abstractpub_date
2000-12-01 00:00:00pages
1036-9issue
11eissn
0210-0010issn
1576-6578journal_volume
31pub_type
杂志文章abstract：INTRODUCTION:Overweight and obesity present significant public health concerns because of the link with numerous chronic health conditions. During the last ten years, since the discovery of leptin, great advances were obtained in the characterization oh the hypothalamic mechanisms involved in the control of food intake...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2007-12-01 00:00:00
abstract：INTRODUCTION:The corpus callosum is the major neopallial connection between the two cerebral hemispheres. The corpus callosum agenesis (CCA) is found in 14% of CNS malformations. The diagnosis is based on neuroimaging procedures (ultrasonography, CT, MRI). The CCA is usually associated with facial dysmorphia, developme...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1999-01-01 00:00:00
abstract：INTRODUCTION:Fibrinolytic agents are effective in the treatment of acute ischemic stroke. However, logistic and clinical factors limit their use. Neuroprotective drugs pose less risks and can be used even before performance of computed tomography of the brain as they are not detrimental in hemorrhagic stroke. These asp...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2001-05-01 00:00:00
abstract：:Different therapeutic models have been presented with which to treat the condition of attention deficit hyperactivity disorder (ADHD) and the scientific literature speaks of the benefits linked to multimodal approaches. The National Institute of Mental Health report grants combined treatments an essential role. Throug...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2004-02-01 00:00:00
abstract：INTRODUCTION:Hypnosis is a well known and efficient psychotherapeutic treatment. It has been stated as useful in memory improvement, however, there are only a few reports of this method in teaching, and its neurophysiological aspects. OBJECTIVE:In this paper, the authors were intended to deal with some students' acade...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1997-12-01 00:00:00
abstract：INTRODUCTION:The cognitive effects of long-term exposure to organic solvents could be similar to those triggered by certain neurodegenerative diseases. AIMS:The purpose of this study was to evaluate the effects exerted by accumulated exposure on the cognitive functions. PATIENTS AND METHODS:105 subjects with an avera...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2003-12-01 00:00:00
abstract：OBJECTIVE:In this paper we review the main magnetic resonance studies to show a possible relationship between changes in the white matter of the brain or leukoaraiosis, and the neuropsychological profile of elderly persons without dementia. DEVELOPMENT:The articles published to date show contradictory data, and in nea...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2001-08-16 00:00:00
abstract：INTRODUCTION:Language widely varies in children with autism spectrum disorders (ASD). Evidence, however, suggests that these children understand language worse than their peers with typical development, showing a delay in acquisition of receptive vocabulary. Research relating quality of life (QOL) and language is limit...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2015-03-01 00:00:00
abstract：INTRODUCTION:Lymphomas of the central nervous system include primary and secondary lymphomas of Hodgkin and non-Hodgkin types. For a long time central nervous system involvement during the course of systemic non-Hodgkin lymphoma has been considered unusual, to occur late on in the disease process and usually to be loca...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1999-07-01 00:00:00
abstract：INTRODUCTION:Unilateral isolated paralysis of the soft palate is a rare clinical entity. CASE REPORT:We describe the case of a 12 year old girl who presented acute dysphagia, a nasal voice and regurgitation of liquids into the nose. Exploration revealed right velopalatine insufficiency with normal gag reflex and phary...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2003-02-15 00:00:00
abstract：:It has been stated that findings in neonatal neurological examinations can play a role as a pointer to adverse developmental outcome. The description of the examinations, and their presence or absence differed among authors without clear reference to their physiological variability ranges. We approached the study of s...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2002-03-01 00:00:00
abstract：INTRODUCTION:Brain creatine deficiencies are a group of inborn errors of metabolism recently recognized which are caused by arginine: glycine amidinotransferase (AGAT) deficiency, guanidinoacetate metiltransferase (GAMT) deficiency and defects in creatine transporter (CRTR). Although all of them are characterized by a ...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2007-03-16 00:00:00
abstract：AIM AND DEVELOPMENT:To debate about the application of on-going 'revolutions' in medical knowledge to Spanish neurological journals in the 21st century. This article reviews the current status of five revolutions in the field of health sciences, in general, and in neurological sciences, in particular: 1) the knowledge ...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2007-01-16 00:00:00
abstract：AIM:To identify the clinical features that predict a favourable response to onabotulinumtoxinA (OnabotA) treatment in patients with refractory migraine. PATIENTS AND METHODS:Retrospective analysis of patients with refractory migraine who underwent at least two pericranial injections of OnabotA between 2008 and 2012. P...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2014-03-16 00:00:00
abstract：:INTRODUCTION. Since James Parkinson published what can be considered the first treaty on the disease that bears his name in 1817, the scientific literature on this pathology has not ceased to grow. But the illness has also been represented in literature, the cinema and on television, where the symptoms, treatment and ...
journal_title：Revista de neurologia
pub_type： 历史文章,杂志文章
doi：
更新日期：2014-02-01 00:00:00
abstract：INTRODUCTION:The combination of subarachnoid hemorrhage (SAH) and subhyaloid hemorrhage is known as 'Terson syndrome'. Retinal hemorrhage is commonly observed clinically in the optic fundi of patients with SAH, however, subhyaloid hemorrhage of the globe in the setting of SAH has been rarely on CT of the brain. Several...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：1997-07-01 00:00:00
abstract：AIMS:In the search for new, potentially treatable, vascular risk factors, one of the most recent to be put forward is the presence of increased total homocysteine (tHc) levels in blood plasma and this has also given rise to a large amount of literature and controversy. The origin of this hypothesis lies in the observat...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2004-02-16 00:00:00
abstract：INTRODUCTION:Cystinosis is a hereditary disease with clinical symptoms that are caused by the accumulation of cystine crystals in different tissues. Distal vacuolar myopathy has been reported as one of its later complications. CASE REPORT:Here, we present the case of a 20-year-old male diagnosed with cystinosis at the...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2005-02-01 00:00:00
abstract：: TITLE:Sindrome de Guillain-Barre en tiempos de arbovirosis. ...
journal_title：Revista de neurologia
pub_type： 评论,信件
doi：
更新日期：2018-12-01 00:00:00
abstract：TITLE:Actos reiterativos y ensayo-error. : ...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2016-01-01 00:00:00
abstract：:From studies of the incidence of epilepsy, figures of between 40 and 70 per 100,000 inhabitants are obtained whilst in studies of prevalence, 5 to 7 cases per 1,000 are found. Cerebrovascular disease is the commonest cause of epileptic crises in the developed world, being more frequent in patients aged over 60, who ma...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：1999-02-01 00:00:00
abstract：INTRODUCTION:Akinetic mutism is considered as an alteration of the motivational state of the person, which the patient is unable to initiate verbal or motor responses voluntary, even with preserved sensorimotor and surveillance functions. CASE REPORT:A 43 year-old male involved in a cerebellum arteriovenous fistula co...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2017-01-16 00:00:00
abstract：INTRODUCTION:The damage caused to the central nervous system by lightning can be immediate or delayed. Cerebrovascular accidents are usually an infrequent complication of lightning strikes. CASE REPORT:We report the case of a patient who was hit by lightning and then developed an acute bilateral intraparenchymatous ha...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2004-09-16 00:00:00
abstract：INTRODUCTION:Many neuropsychological tests used in Spain have been normed on Spanish-speaking persons in the USA and South America. In addition, the normative samples often span limited age or educational ranges, potentially decreasing their representativeness of the population, the interpretation of test results, and ...
journal_title：Revista de neurologia
pub_type： 杂志文章,多中心研究
doi：
更新日期：2015-07-16 00:00:00
abstract：:Disorders of gait are a common problem in elderly people. It is estimated that over 15% of people aged over 65 years have disorders of gait. Disorders of balance are also common in old age, with increased risk of falling and lesions due to falls. Every year 20-30% of the elderly people living alone have falls. The cau...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2000-07-01 00:00:00
abstract：INTRODUCTION:Since the 1980s, in relation to the spread of AIDS, there has been an enormous increase in cryptococcosis, a mycotic disorder which usually affects the central nervous system (CNS). This disease is caused by Cryptococcus neoformans, a microorganism acquired by inhalation of bird excrement. This germ produc...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2000-05-16 00:00:00
abstract：OBJECTIVE:To review the current treatment and usefulness of lamotrigine in absence seizures. DEVELOPMENT:Absence seizures are classified amongst the generalized epilepsies. They are defined as a transient loss of consciousness of sudden onset and recovery characteristically associated with generalized spike-and-wave d...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2001-02-01 00:00:00
abstract：INTRODUCTION AND OBJECTIVES:Advances in genetics, techniques for diagnosis and treatment, and increasing concern for the quality of life and neuropsychological aspects of epileptic children and the possible repercussions of treatment lead to changes in the approach to epilepsy and require continual adaptation. In this ...
journal_title：Revista de neurologia
pub_type： 杂志文章
doi：
更新日期：2000-10-16 00:00:00
abstract：AIM:To describe the main physiopathological mechanisms of obstructive sleep apnea syndrome (OSAS) associated with the development of stroke. DEVELOPMENT:Sleep breathing disorders have a high prevalence in the healthy population, among them, the OSAS is the most recognized. This syndrome has been associated with vascul...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：10.33588/rn.6906.2019061
更新日期：2019-09-16 00:00:00
abstract：INTRODUCTION:Headaches are a frequent cause of children's visits to different health care services but the epidemiological profile varies widely according to whether the sample is taken from among visits to hospital clinics, neuropaediatric units or emergency services. AIMS:The aim of this study was to analyse the aet...
journal_title：Revista de neurologia
pub_type： 杂志文章,评审
doi：
更新日期：2004-08-01 00:00:00
