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V López-Martín

Publications and source records attributed to V López-Martín.

At least 19 recordsLinked to original sources
PubMed2001

Hypohidrosis related to the administration of topiramate to children.

PURPOSE: Topiramate (TPM) is an antiepileptic agent, first licensed in the United Kingdom in 1994, that is used in the treatment of patients with refractory seizure disorders. TPM is a monosaccharide d-fructose derivate, with sulfamate function, and so far, few adverse side effects have been reported. METHODS: We describe three patients with epilepsy who were treated with TPM and developed hypohidrosis, heat and exercise intolerance, as well as fever. The sudomotor function was assessed after peripheral stimulation with pilocarpine iontophoresis. RESULTS: Sweat response was reduced in all three patients. Signs and symptoms ceased after drug suppression. CONCLUSIONS: This side effect associated with TPM, which has not been described previously, can be clinically significant during heat stress and exercise challenge.

Adolescent↗
PubMed1998

[Diagnosis in severe myoclonic epilepsy in childhood: study of 13 cases].

OBJECTIVE: The purpose of this report is to describe clinical pattern, EEG, outcome and differential diagnosis in severe myoclonic epilepsy in infancy (SMEI). MATERIAL AND METHODS: We report 13 cases initially diagnosed of SMEI and selected according to the following criteria: first seizure between 1 and 12 months of life, frequent seizures resistant to antiepileptic drugs, no previous personal history of disease, normal psychomotor development before the first seizure and normal EEG, CT scanning and laboratory analyses at the beginning. CT and/or MRI were performed in 13 cases, arteriography in 2 patients, MR spectroscopic imaging in 1 child and SPECT in 3 cases. Quantification of enzymatic activities of the mitochondrial respiratory chain was made in 5 patients. RESULTS: Only 8 cases were finally diagnosed of SMEI according to ILAE definition. In two cases, seizures were finally controlled with antiepileptic treatment and EEG abnormalities disappeared. Three patients showed other findings: mesiotemporal sclerosis, angiitis diffusely involving CNS and mitochondrial cytopathy with deficiency of the complex IV. CONCLUSIONS: Although diagnosis of SMEI, based on clinical manifestations, is suspected in most cases from the first year of life, final diagnosis should not be confirmed until steady state, when polymorphous seizures occur. Even then, differential diagnosis should be made with other disorders. Perhaps, further studies should be performed in order to identify and eliminate another etiology.

Anticonvulsants↗
PubMed1998

[Munchausen syndrome by proxy: report of one case with epilepsy].

INTRODUCTION: Munchausen syndrome by proxy (MSBP) is a rare form of child abuse in which a parent, usually the mother, fabricates or produces illness in a child, so causing them unnecessary medical investigations, treatments and hospitalizations. One of the commonest false presenting symptom is 'seizures'. CLINICAL CASE: An eight years old boy with Munchausen syndrome by proxy is reported. This child had had genuine seizures well controlled by standard anticonvulsant treatment at the start of the false illness. At the age of seven years, the patient showed very frequent seizures. The child was treated with antiepileptic drugs, but treatments were ineffective and seizures continued. Results of multiple tests, including an extensive blood chemistry analyses, CT, MRI, SPECT, were normal. Electroencephalogram showed posterior slow waves. Acute neurological deterioration was observed six weeks after hospitalization and it was finally proved that seizures were caused or triggered by clomipramine poisoning given by her mother. CONCLUSIONS: MSBP frequently presents as epileptic seizures in these abused children. MSBP diagnosis is more difficult to be made if true seizures exists with multiple fictitious seizures. Pediatrician should be alerted to the possibility of MSBP when seizures are poorly controlled, treatments are ineffective and there is no neurophysiologic dysfunction. Early diagnosis and intervention are essential because high mortality and psychologic morbidity are associated with this syndrome.

Antidepressive Agents, Tricyclic↗
PubMed1998

[Brain stem encephalitis in childhood].

OBJECTIVE: To present two cases of post-infectious encephalitis of the brain stem (ETC) in infancy, which is very infrequent at this age. CLINICAL CASES: Two patients aged 4 months and 9 months respectively had a previous history of a catarrhal illness a few days before the onset of encephalitis. The clinical condition was of subacute onset and torpid course, characterized by ataxia, reduced level of consciousness, involvement of the pyramidal tracts and paralysis of the cranial nerves. No significant information for the diagnosis of either case was obtained from CT. MR showed lesions at the level of the pons. However, the MR image did not correspond in seventy to the clinical condition. The clinical courses of the two patients were different. One case recovered with no sequelae. In the other case the cranial nerves and gait did not return to normal. CONCLUSIONS: In our experience, ETC is rarely seen in infancy. A high degree of suspicion and early treatment of ETC caused by the herpes simplex virus is necessary, since there is usually a high mortality or serious neurological sequelae.

Ataxia↗
PubMed1998

[The value of images in diagnosis of neuron migration disorders].

OBJECTIVE: To present the fitest classification and the imaging peculiarities of the malformations of cortical development, most of which have been related with the epilepsy origin. METHODS: The study is based on an anatomical-histological classification scheme that shows three great groups of malformations of cortical development: 1. Malformations due to abnormal neuronal and glial proliferation. 2. Malformations due to abnormal neuronal migration. 3. Malformations due to abnormal cortical organization. RESULTS: The result of these abnormalities of the cortical development is the presence of several anatomical histological entities, actually perfectly identified by the magnetic resonance (MR), especially with the new high resolution methods. The most frequent entities, such as polymicrogyria, lissencephaly, pachygyria, schizencephaly, cerebral heterotopia (cortical, subcortical or subependymal), and other rarer types are reviewed according with the numerous references of the literature and the findings observed in the cases of our series of about one hundred patients which includes cases of every type of malformation. CONCLUSION: MR is a conclusive study in order to identify and classify the malformations of cortical development, most of which are associated with neurological disturbances: epilepsy, mental retardation, language and/or behavioral problems or motor dysfunction.

Brain↗
PubMed1997

[Clinical significance of episodes of apnea in babies].

INTRODUCTION: The presence of episodes of apnea (EA) in infants is very alarming and requires rapid, precise determination of the etiology in view of the relationship with the syndrome of sudden death of infants. MATERIAL AND METHODS: We therefore studied 16 patients, aged between 1 and 12 months, admitted to to hospital after having episodes where they stopped breathing for more than 15 seconds, with or without associated bradycardia. Patients with previous or associated convulsions, premature birth or low birth weight for gestational age were excluded from the study. In all cases a prolonged EEG was recorded. RESULTS: The commonest age of presentation of EA was 3-4 months. In one case there was a history of a sudden infant death occurring in a brother. The etiology was determined in 12 cases: gastroesophageal reflex in 5, epilepsy in 3, hyponatremia in 2, drug reaction in 1, bronchiolitis in 1 and hypoglycemia in another. In three cases where no cause was found the EA was not repeated, although apnea monitorization was necessary for several months because of the anxiety of the family. CONCLUSIONS: Of the 3 cases in which EA was a critical epileptic sign, only 2 could be demonstrated on a Holter recording. In one of these, progress was poor and resistant crises of different morphology later occurred. We consider that EA in infants requires a thorough etiological study. Cases of unknown etiology require prolonged EEG recording to determine whether the apnea is cerebral in origin, followed by prolonged monitorization of apneas to avoid possible sudden death.

Apnea↗
PubMed1997

[Unilateral agenesis of the internal carotid artery in childhood: description of a case].

INTRODUCTION: Unilateral or bilateral agenesis of the internal carotid artery has not often been described in children since it is usually asymptomatic. CLINICAL CASE: We describe a case of isolated unilateral agenesis of the internal carotid artery in a child of 20 months old who presented with two episodes of status convulsives, the first after a head injury and the second when he was febrile. The anomaly was a casual finding, diagnosed on cerebral magnetic resonance when there was no signal corresponding to carotid blood flow. It was confirmed by echography-doppler and angiographic magnetic resonance. We also describe the neuro-radiological findings which led to the diagnosis of this anomaly. CONCLUSIONS: We consider that unilateral agenesis of the carotid artery was a casual finding in this case. The provisional diagnosis may be made when there is absence of vascular signals on conventional magnetic resonance.

Carotid Artery, Internal↗
PubMed1996

[Spectroscopic magnetic resonance in hemimegalencephalus].

INTRODUCTION: Hemimegalencephalus (HM) is a disorder of cerebral migration characterized by the overdevelopment of one cerebral hemisphere. It is usually associated with pachygyria, gliosis and neurone loss. We present a study using stereoscopic magnetic resonance (SMR) in a case of HM confirmed by the pathologist. CLINICAL CASE: A girl with right HM had hemigeneralized crisis since birth. A selective right temporoccipital cortectomy was done when she was two and a half years old. The resected piece showed thickening and absence of cortical striation, neurone loss, gliosis, giant neurones and heterotopias. After a symptom-free period the crises reappeared as right fronto-parietal epileptiform anomalies. When she was four years old SMR was done to compare this area with the corresponding area of the radiologically normal left hemisphere. Comparative study showed a marked drop in N-acetyl-aspartate (NAA), glutamate (Glu) and Gaba, and increased choline (Col) and inositol (Ino). We found no difference in the creatinine levels. CONCLUSIONS: The histological findings are in concordance with the levels of metabolites found in the affected hemisphere. The drop in NAA and Glu is related to neurone loss and the increase in glial cells, and the increase in Col and Ino with increase in membranes metabolism, as is observed in the gliosis. SMR is an advance in the identification and grading of changes seen on conventional MR, when establishing the prognosis and choice of treatment in HM.

Brain↗
PubMed1981

Ataxia-telangiectasia (A-T). Contribution with eighteen personal cases.

Eighteen patients with ataxia-telangiectasia are presented. They belong to only one series. They are 7 males and 11 females aged between 17 months and 12 years. All of them presented the usual signs of the illness. There was more than one sibling affected in three families, in one of them three brothers and one second cousin of theirs. The immunological studies showed a deficit of IgA in 13 cases and an absence of IgG in one case. Six children died, one of which suffered from lymphosarcoma. The other appear to have died of infectious respiratory problems. The clinical picture deteriorated progressively in the majority of the cases, although in some of them a halt in the progression of the illness was observed for some time.

Antibody Formation↗
PubMed1978

[Prognostic value of neonatal paroxyistic electroencephalogram (author's transl)].

Twenty full-term newborns showing acute neurologic symptoms of different etiologies, with neonatal paroxistic EEG tracings are reported. Clinical and electroencephalographic follow-up at three and twelve months confirmed in every case the presence of severe neurologic sequelae. A review of the literature is made, comparing the results of other investigators with those obtained in the present study specially from an ethiologic, clinical, electroencephalographic and evolutionary points of view.

Brain Diseases↗
PubMed1978

[Flat and hypoactive neonatal E.E.G. evolution in eighteen cases (author's transl)].

Eighteen new-borns were studied, four of them were pre-term with a pregnancy of 35 weeks. They had an acute neurological symptomatology and also showed a E.E.G. with flat or hypoactive aspect in the neonatal period. The evolution of these patients is analyzed clinically and electroencephalographically of flat E.E.G. and an uncertain future in those cases with hypoactive E.E.G. A review of the literature on the problem was performed in order to compare the results of other series with those obtained in the present study, attending specially etiological, clinical, electroencephalographic and evolutive aspects.

Electroencephalography↗
PubMedDate not supplied

[A study of Gobbi's syndrome in Spanish population].

INTRODUCTION: The association of epilepsy, occipital cerebral calcifications and coeliac disease was recognized for the first time in 1992 by Gobbi as an independent syndrome. He emphasized that it occurred almost exclusively in persons of Italian origin. OBJECTIVE: To define the prevalence of this syndrome in the Spanish population with a view to confirming its probably genetic etiopathogenesis. PATIENTS AND METHODS: Neurological studies were done in 44 coeliac patients, as were cranial CT scans. All cases of Spanish origin described in the literature were noted. RESULTS: In the patients with coeliac disease there was an increased incidence of crises and generalized epilepsy, but no patient was found to have occipital calcifications. Only 12 cases of Spanish origin were found, mainly in the Canary Isles and Mediterranean littoral, in spite of the high incidence of coeliac disease in Spain. No familial cases were seen. CONCLUSIONS: We suspect a genetic etiopathogenesis, associated with different environmental factors, to be the origin of the syndrome. This is supported by the common geographical origin of most cases and the anatomopathological findings described in those cases studied.

Adolescent↗
PubMedDate not supplied

[Occipital leptomeningeal angiomatosis without facial angioma. Could it be considered a variant of Sturge-Weber syndrome?].

INTRODUCTION: The association of cerebral leptomeningeal angioma and facial nevus flameus in the territory of the first branch of the trigeminal nerve ipsilateral to the angioma is known as the Sturge-Weber syndrome. The cases with absence of a facial angioma are usually considered to be variants of the syndrome. OBJECTIVE: To present four cases with occipital leptomeningeal angioma without facial angioma and describe the characteristics which differentiate them from or permit their inclusion within the group of Sturge-Weber syndrome, and also to establish the differences between this and the Gobbi syndrome (occipital cerebral calcifications, epilepsy and coeliac disease. CLINICAL CASES: We selected four cases in whom cranial magnetic resonance was done with intravenous gadolinium and three cases studied to rule out coeliac disease. The cerebral calcifications, unilateral in all four cases, were similar to those observed in the Sturge-Weber syndrome. All cases had leptomeningeal angiomas at the level of the cerebral calcification shown by the uptake of contrast material on magnetic resonance. Three patients had epilepsy but none had facial angiomas, hemiparesis or glaucoma. Coeliac disease was also ruled out, both on laboratory investigations and on intestinal biopsy. CONCLUSIONS: The cases described coincide with the Sturge-Weber syndrome in all having cerebral leptomeningeal angiomas. This differentiated them from the Gobbi syndrome which does not include meningeal angiomata. Another characteristic of the Sturge-Weber syndrome is the occurrence of epilepsy and mental deficiency. Whilst awaiting molecular genetic studies, our cases may be included semantically as a variant of the Sturge-Weber syndrome without the characteristic facial angioma, although they may possibly correspond to genetically different conditions.

Adolescent↗
PubMedDate not supplied

[Schilder's diffuse myelinoclastic sclerosis].

OBJECTIVE: The aim of this study is to analyze clinical features, neuroradiological findings and evolution associated with Schilder s disease (SD). PATIENTS AND METHODS: We describe 5 cases (4 female/1 male) diagnosed of SD. Clinical characteristics, neuroimaging (CT and MRI), EEG, evoked potential analysis (4/5) and laboratory tests are provided, including the level of serum very long chain fatty acid of plasma cholesterol esters (3/5). RESULTS: Patients were aged between 7 and 12 years. The first clinical manifestations were: hemiparesis (3/5), quadriparesis dysarthria (1/5), and seizures cerebellar dysfunction (1/5). Other clinical features were: partial seizures (3/5), cerebellar dysfunction (2/5), loss of sensibility (3/5), visual loss (1/5), and dysarthria (2/5). CT scan and MRI showed large zones of hypodensity in the hemispheric white matter (4/5) with enhancement in T2 weighted MRI images. This finding was also observed in medulla (1/5) and cerebellum (1/5). Laboratory data were normal. EEGs showed general slow background patterns in all cases. Abnormal evoked potential analysis were recorded in 3 children. Clinical improvement followed the steroid therapy in all cases. Clinical evolution was: minimal motor disabilities (5/5), recurrences (3/5), controlled seizures (3/3), and psychomotor retardation (1/5). CONCLUSIONS: SD is a rare demyelinating disorder, with a probable relationship to multiple sclerosis. The course of this disease is unpredictable; recurrences may appear and sequelae are frequently observed. Diagnosis should be based on clinical features, neuroradiological findings and evolution.

Child↗
PubMedDate not supplied

[Bilateral hypodensity of the basal ganglia. Clinico-evolutionary correlation in children].

INTRODUCTION: The presence in neuroimaging of areas of symmetrical bilateral hypodensity in the basal ganglia (SBHBG) is a striking and unusual finding. OBJECTIVE: To determine the aetiology, clinical significance and evolution of a group of paediatric patients with SBHBG. PATIENTS AND METHODS: We made a study of 21 patients with neuroimaging studies (CT or MR) showing SBHBG. The affected area was related to the aetiology, clinical features and evolution. RESULTS: The ages varied between 4 months and 16 years. In 7 cases Leigh s disease was diagnosed, 5 had had acute hypoxia, 4 type I glutaric aciduria, and 1 case each of methylmalonic aciduria, Ia gluconeogenesis, CO intoxication, acute striatal necrosis and bacterial meningitis. The putamen was affected in 6 cases, globus pallidus in 4 cases and the lenticular nucleus was damaged in the rest. Three cases also had lesions in the caudate nucleus. MR was better than CT for localization of the precise area involved. Clinically, 13 cases had extrapyramidal signs. We found no relation between the size, localization of the lesion and the prognosis, which was more dependent on the aetiology, only one patient (CO intoxication) recovered and eight died (Leigh s disease and 1 case of hypoxia). CONCLUSIONS: The presence of SBHBG in a patient makes extensive study necessary to find the aetiology. It is a nonspecific finding, usually of metabolic origin and with little correlation with the clinical condition. Its presence implies a poor prognosis and raises suspicion of the presence of certain neurological disorders.

Adolescent↗
PubMedDate not supplied

[Neonatal EEG trace of burst suppression. Etiological and evolutionary factors].

INTRODUCTION: The electroencephalographic (EEG) trace seen during the neonatal period which shows so called discharges of burst suppression, is caused by a severe disorder of cerebral electrogenesis occurring at this time. OBJECTIVE: To determine the aetiology, clinical significance and evolution of a group of newborn babies with this type of EEG trace. PATIENTS AND METHODS: We made a retrospective study of fullterm babies in whom burst suppression EEG recordings had been obtained during the neonatal period. RESULTS: We studied 34 patients. In 14 cases the trace was associated with hypoxic ischemic encephalopathy; 4 with meningitis; another 4 with early infantile epileptic encephalopathy (Ohtahara syndrome); 4 cases were attributed to drugs (4 with fentanyl associated in one case with phenobarbitone and in another with midazolam); 2 cases were due to early myoclonic epilepsy; 3 to multiple organ failure; one to non ketotic hyperglycinemia and another to leucinosis. In one patient the aetiology could not be determined. Seven patients died before the age of 6 months. Severe neurological sequelae were seen in all the others except for four cases (3 treated with fentanyl and one case with hypoxic ischemic encephalopathy). CONCLUSIONS: The presence of a burst suppression EEG trace in a neonate makes extensive study to determine the aetiology necessary. Although associated with a worse prognosis, those not treated with piperidine derivatives should be classified separately. Those treated with piperidine derivatives have a good prognosis.

Calcium Channel Blockers↗
PubMedDate not supplied

[Alpha coma: clinical, electroencephalographic and aetiological correlation in childhood].

INTRODUCTION: Alpha coma is defined as the appearance, in an unconscious patient, of EEG activity in the alpha frequency band (8-13Hz). OBJECTIVE: To determine, in our setting, the incidence, aetiology, clinical significance and course of alpha coma in paediatric patients and review the literature. PATIENTS AND METHODS: We did a retrospective study of the patients admitted to a paediatric intensive care unit who had alpha activity recorded whilst in coma. RESULTS: Only two patients, aged 5 and 12 years, were found. Both had complex congenital cardiopathy and cardio-respiratory arrest one and twelve days postoperatively, respectively. Several EEGs were done whilst they were in coma. Barbiturate coma was not used. Both had clinical mid-brain involvement during alpha recording. The pattern was bilateral, arreactive and preceded by a theta pattern, appeared two days after the arrest, was maintained for two and three days in the two cases and was followed by delta pattern. The second case finally showed a 'burst-suppression'. The CAT was normal. The patients died 4 and 11 days after the appearance of alpha coma. CONCLUSIONS: Alpha coma is rare in childhood. Both the aetiology and the bad prognosis are similar to that of adults. Alpha coma is a transient EEG pattern occurring during the evolution of coma. We consider it to be due probably to mid-brain involvement.

Adult↗