Abstract:
INTRODUCTION:Valproic acid (VPA) is a drug mainly used to treat epilepsy. Hyperammonaemic encephalopathy due to VPA is a rare but serious complication. The mechanism by which VPA influences the increase in ammonia consists in blocking the urea cycle, thereby inhibiting N-acetylglutamate synthase and diminishing acetyl coenzyme A. Generally, the treatment employed has been to withdraw VPA and to administer arginine, carnitine, antibiotics, glucose and protein restriction. Previous experience with carglumic acid is limited to reports of isolated cases of paediatric patients. CASE REPORTS:We report the cases of two adult patients with encephalopathy due to VPA who were treated with carglumic acid, in addition to the conventional measures. Following treatment with the drug, ammonia levels can be seen to return to normal values. In one of the two cases, owing to the existence of another cause of encephalopathy, no clinical improvement was observed. CONCLUSIONS:From the biochemical point of view, treating encephalopathy due to VPA with carglumic acid is a logical step, as it reverses the blockage of the urea cycle conditioned by VPA. The mechanism proposed as being the one by which brain toxicity and, therefore, encephalopathy are produced is the passage of ammonia in the form of glutamine to the inside of the cell, which then returns to ammonia and glutamate in the mitochondria and leads to oxidative stress. Carglumic acid must be considered an important part of the treatment in adult patients with hyperammonaemic encephalopathy due to VPA, although a randomised clinical trial needs to be conducted with the drug in order to test its efficacy. TITLE:Tratamiento de la encefalopatia por acido valproico con acido carbaglumico: descripcion de dos casos y revision de la bibliografia. :Introduccion. El acido valproico (VPA) es un farmaco principalmente usado en la epilepsia. La encefalopatia hiperamoniemica por VPA es una complicacion grave e infrecuente. El mecanismo por el que el VPA influye en la elevacion del amonio es bloqueando el ciclo de la urea, inhibiendo la N-acetilglutamato sintasa y disminuyendo la acetil coenzima A. De forma general, el tratamiento que se ha empleado ha sido la suspension del VPA y la administracion de arginina, carnitina, antibioticos, glucosa y restriccion proteica. La experiencia con acido carbaglumico se limita a comunicaciones de casos aislados de pacientes pediatricos. Casos clinicos. Descripcion de dos casos de pacientes adultos con encefalopatia por VPA tratados, ademas de las medidas convencionales, con acido carbaglumico. Tras el tratamiento con el farmaco se aprecia una normalizacion de los niveles de amonio. En uno de los casos, al existir otra causa de encefalopatia, no se aprecio mejora clinica. Conclusiones. Desde el punto de vista bioquimico es logico tratar la encefalopatia por VPA con acido carbaglumico, ya que revierte el bloqueo del ciclo de la urea condicionado por el VPA. El mecanismo propuesto por el que se produce la toxicidad cerebral y, por tanto, la encefalopatia, es el paso de amonio en forma de glutamina al interior celular, que retorna a amonio y glutamato en la mitocondria y genera estres oxidativo. El acido carbaglumico ha de considerarse como parte importante del tratamiento en pacientes adultos con encefalopatia hiperamoniemica por VPA, aunque es necesario realizar un ensayo clinico aleatorizado con el farmaco para comprobar su eficacia.
journal_name
Rev Neuroljournal_title
Revista de neurologiaauthors
Nava-Mateos JJ,Roiz-Rey P,Diaz Alvarez-Mediavilla J,Cebrian-Novella D,Gomez-Del Olmo V,Ceberio-Hualde Lsubject
Has Abstractpub_date
2017-11-01 00:00:00pages
409-414issue
9eissn
0210-0010issn
1576-6578pii
rn2017226journal_volume
65pub_type
杂志文章,评审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:TITLE:Signo del cerebelo blanco (reversal sign). : ...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2014-01-01 00:00:00
abstract:INTRODUCTION:Aseptic meningitis is a process that is characterised by an inflammatory reaction of the meninges that is not due to any infectious agent. Its aetiology is varied and is most frequently caused by rheumatologic and/or autoimmune processes, chemical or medication-induced meningitis, the most notable drugs in...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2006-02-16 00:00:00
abstract:OBJECTIVE:To carry out a bibliographic review of articles indexed in MEDLINE over the past 20 years concerning the pharmacological treatment of agitation in head injury. DEVELOPMENT:Head injury may cause different behaviour changes, of which agitation is the most dramatic. The incidence of agitation after severe head ...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2000-05-01 00:00:00
abstract::By means of the modified trichromic method of Gomori, introduced by Engel and Cunningham in 1963 [1], it has been possible to demonstrate the presence of ragged-red fibers (RRF) in the mitochondrial myopathies. At present, to study these disorders in muscle biopsies, one may also use morphological, ultrastructural, hi...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:1998-04-01 00:00:00
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journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2005-01-15 00:00:00
abstract:TITLE:Epilepsia dependiente de piridoxina por deficiencia en el gen PNPO. ...
journal_title:Revista de neurologia
pub_type: 信件
doi:10.33588/rn.6907.2019084
更新日期:2019-10-01 00:00:00
abstract:INTRODUCTION:Stroke is a leading cause of mortality and disability. Policosanol has been effective in brain ischemia models. The aim of this study is to investigate whether policosanol, added to aspirin therapy within 30 days of stroke onset, is better than placebo + aspirine for the long-term recovery of non-cardioemb...
journal_title:Revista de neurologia
pub_type: 杂志文章,随机对照试验
doi:
更新日期:2017-02-16 00:00:00
abstract:INTRODUCTION:Synthesis of IgG is useful data for the diagnosis of multiple sclerosis, and different formulas, both direct and indirect, are used to quantify this. We analyze these formulas with the objective of finding whether certain combinations of them would give better results than the individual formulas on their ...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2000-11-01 00:00:00
abstract:INTRODUCTION:The surgery of epilepsy is expanding considerably all over the world. It is becoming a standard treatment in patients who are resistant to conventional medical treatment. DEVELOPMENT:In this article we explain the methodology used by the authors and suggest concepts regarding the epileptogenous area. We a...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2000-06-16 00:00:00
abstract:OBJECTIVES:To evaluate the diagnostic efficacy of an intra-arterial electroencephalographic recording and determine which patients obtain most benefit from this technique, to compare the results obtained using other recording techniques and to establish a standard for recording. PATIENTS AND METHODS:We made 64 intra-a...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2000-04-01 00:00:00
abstract:INTRODUCTION:Transcranial Doppler (TCD) is a new technique which is becoming increasingly used in neurology and is highly dependent on the user. OBJECTIVE:To evaluate the reliability of TCD in our hospital. PATIENTS AND METHODS:During a five month period, all patients who had a cerebral angiogram done, also had DTC w...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2001-09-16 00:00:00
abstract:INTRODUCTION:CLIPPERS syndrome (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory process of the central nervous system whose distinguishing features are the enhancing punctiform lesions in the brainstem that appear in the magnetic resonance images. Clinic...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2013-10-16 00:00:00
abstract::The term 'acute myelopathies'--referred to a spinal cord dysfunction--represent a heterogeneous group of disorders with distinct etiologies, clinical and radiologic features, and prognoses. The objective of this review is to discuss the non-traumatic acute myelopathies. Acute myelopathy can be due to several causes as...
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:In partial seizures, from a clinical point of view or even from electroencephalography characterization of post-ictal period can be difficult. The spectral and complex network analysis could lead to a more accurate definition of its limits, as well as to a great understanding of the seizures. PATIENTS AND...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:2017-04-16 00:00:00
abstract:INTRODUCTION:Chronic daily headache (CDH) includes primary headaches that last more than four hours with a frequency equal or superior to 15 days a month over the last three months. It has a prevalence of 4-5% in the general population and is a frequent reason for visiting the physician in headache units. AIM:To evalu...
journal_title:Revista de neurologia
pub_type: 临床试验,杂志文章
doi:
更新日期:2007-10-16 00:00:00
abstract:INTRODUCTION:Late preterm infants currently constitute 70% of preterm infant births. They present greater comorbidity, including neurodevelopment disorders, which may not manifest until the school age. AIM:To identify the existence of difficulties in the neurodevelopment at the age of two years. SUBJECTS AND METHODS:...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:10.33588/rn.6812.2018360
更新日期:2019-06-16 00:00:00
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journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2012-06-01 00:00:00
abstract:INTRODUCTION:The hyperperfusion syndrome (HS) is described as an infrequent complication of carotid endarterectomy. It may also occur in percutaneous transluminal carotid angioplasty with a similar causal mechanism and clinical features to those seen in HS related to carotid endarterectomy. CLINICAL CASE:We describe t...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:1999-11-16 00:00:00
abstract:INTRODUCTION AND DEVELOPMENT:Reported observations of acquired aphasia in children contain all the symptoms and syndromes that have been described in adult aphasiology. Recovery of colloquial language is good even if anomia and verbal memory deficits persist as the most frequent defects. At long term outcome there are ...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2008-01-01 00:00:00
abstract:INTRODUCTION:The value of the P300 cognitive event-related potential in the diagnosis of Alzheimer subtype neurocognitive disorders is still incipient. Recent studies suggest that combining it with neuropsychological tests by cognitive domains would allow an objective and early characterisation of the cognitive impairm...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:10.33588/rn.7101.2019341
更新日期:2020-07-01 00:00:00
abstract:INTRODUCTION:Face perception involves a broad network of connections between cortical and subcortical regions for the exchange and synchronization of information using white matter fibers. This precise communication system can be affected by the structures and the pathways that connect them. AIMS:To establish the neur...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2017-02-01 00:00:00
abstract:INTRODUCTION AND DEVELOPMENT:The history of prion diseases is one of the most exciting chapters of the story of medicine in the twentieth century. It began in the nineteen twenties when Creutzfeldt (in one patient) and Jakob (in five patients) described a clinically polymorphic cerebral syndrome with a subacute or chro...
journal_title:Revista de neurologia
pub_type: 历史文章,杂志文章
doi:
更新日期:2000-07-16 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
abstract:INTRODUCTION:Sleep and epilepsy have strong reciprocal influences and this is true for all kinds of epilepsies. The interictal paroxysmal activity increases in slow wave sleep and the localization of the primary epileptogenic area is more reliable in paradoxical sleep. DEVELOPMENT:From a practical point of view, an al...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2004-01-16 00:00:00
abstract:INTRODUCTION:Over the last twenty years much work has been done to study the P300 in alcoholism. A systematically reduced amplitude, both in patients and in populations at risk (children of alcoholics) was found. Besides, studies on twins indicate that the waves of the evoked potentials are under genetic control. Furth...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2000-04-16 00:00:00
abstract:INTRODUCTION:The research group from Willmes and colleagues is one of the most advanced research groups in cognitive neuroscience. The use and nature of the numeric magnitude representation constitutes one of the study objects of the mentioned research group. This mental representation provokes a great controversy amon...
journal_title:Revista de neurologia
pub_type: 杂志文章,评审
doi:
更新日期:2008-03-01 00:00:00
abstract::The new communication techniques and, particularly, Internet, are changing our way of collaborating, relating to each other and searching for information, paving the way for telemedicine. Such initiatives are described in the Revista de Neurologia, as well as the methodology followed to prepare the first Electronic Fo...
journal_title:Revista de neurologia
pub_type: 社论
doi:
更新日期:1998-04-01 00:00:00
abstract:TITLE:GNAO1: un nuevo gen a considerar en la distonia temprana de la infancia. : ...
journal_title:Revista de neurologia
pub_type: 信件,评审
doi:
更新日期:2018-05-01 00:00:00
abstract:INTRODUCTION:Infection of the Central Nervous System by free living amebas is an unusual event, 344 cases have been reported to date. The disease becomes evident in two different clinical fashions: Primary Amebic Meningoencephalitis (PAM) caused by Naegleria fowleri and Granulomatous Amebic Encephalitis (GAE) induced b...
journal_title:Revista de neurologia
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00