Meta-analytic study on the frequency and treatment of oral antral communications.

OBJECTIVE: To determine the optimum surgical treatment for oral antral communications (OAC) and to understand the main post-operative complications. STUDY DESIGN: Meta-analytical, observational and retrospective study of 1.072 cases of OAC obtained from a literature review of 15 articles. RESULTS: OAC occur slightly more often in men and during the fourth decade of life. Its primary etiological factor is dental extraction, most often affecting the third molar. The most common treatment has been the use of Bichat's fat pad grafts, whereas the technique with the highest percentage of complications has been the use of the palatal rotation flap. The most frequent complication has been the fistulization of the OAC. CONCLUSIONS: Early diagnosis of OAC and its treatment within 48 hours of evolution are fundamental in order to properly resolve this pathology. The use of Bichat's fat pad grafts is a simple technique that offers excellent vascularization and results.

Enfermedades metabólicas de aparición en la edad adulta / Adult-onset metabolic diseases

Los errores congénitos del metabolismo (ECM)pueden debutar en la adolescencia y en la edad adulta.Aunque es difícil aportar datos exactos de prevalenciaya que existen escasos estudios al respecto, inclusoconsiderándolos poco frecuentes, la importancia de sudetección radica en las posibilidades terapéuticas y deconsejo genético familiar.La principal sintomatología de los ECMdel adulto esla neurológica, seguida de la hepática. Se puede establecerdos modos básicos de debut. Uno es el agudo, normalmenteen forma de alteración del nivel de conciencia,letargia, coma de etiología desconocida en un pacientepreviamente sano (déficits del ciclo de la urea, trastornosde la remetilación de la homocisteína y porfirias sonaquí las causas más frecuentes). Por otra parte está lasintomatología crónica, insidiosa, a menudo progresiva,en la que suele haber cuadros clínicos complejos, y másraramente un síntoma aislado de manera persistente (laenfermedad de Wilson, enfermedades mitocondriales,lisosomales, la enfermedad de Refsumy las glucogenosisson algunos ejemplos en este grupo).Es de especial importancia conocer las formas dedebut agudo, que suelen ser situaciones de extremaurgencia, en las que una conducta adecuada puede evitarel fallecimiento del paciente. En este caso, exámenessencillos de laboratorio como la determinación del amonio,homocisteína, lactato, acilcarnitinas, aminoácidos,ácidos orgánicos y porfirinas, pueden orientar el diagnósticoy permiten iniciar un tratamiento intensivo.En este capítulo se pretende realizar un enfoquepráctico, abordando las características generales y clavesclínicas de sospecha de los ECM más habituales enel adulto

Inborn errors of metabolism (IEM) can have theironset in adolescence or in adulthood. Although it isdifficult to contribute exact data on prevalence–because there are few studies in this respect, and IEMare regarded as infrequent– their detection is importantdue to the possibilities for therapy and family geneticcounselling.The main symptoms of IEM in the adult areneurological, followed by hepatic. Two basic modes ofonset can be established. One is acute, normally takingthe form of consciousness alteration, lethargy, coma ofunknown etiology in a previously healthy patient (ureacycle deficits, homocysteine remethylation disordersand porphyries are the most frequent causes). Theother is an insidious, often progressive, chronicsymptomathology that can involve complex clinicalfeatures, and more rarely a symptom that is isolated ina persistent way (Wilson’s disease, mitochondrialdiseases, lysosomal storage disorders, Refsum’sdisease and glycogenosis are some examples of thisgroup).It is especially important to determine the forms ofacute onset as these can present situations of extremeemergency where appropriate conduct can prevent thedeath of the patient. In this case, simple laboratoryexaminations, such as determination of ammonia,homocysteine, lactate, acylcarnitines, amino acids,organic and porfirines, can guide the diagnosis andenable the start of intensive treatment.This article provides a practical approach thatdeals with the general characteristics and the clinicalkeys for suspecting the most usual IEMs in the adult

[Acute onset ataxia in infancy: its aetiology, treatment and follow-up]. / Ataxia de aparición aguda en la infancia: etiología, tratamiento y seguimiento.

INTRODUCTION: Acute childhood ataxia is a cause of referency to the pediatric emergency room. AIM. To characterize the etiology, clinical picture, management, and outcome of acute ataxia in our hospital. PATIENTS AND METHODS: A prospective study was undertaken including 39 children with acute ataxia who were admitted between January 1, 2001 and December 31, 2003. RESULTS: During the study period 159,002 episodes were evaluated, 39 children (0.024%) with acute ataxia. The most common diagnoses were post-infectious ataxia (51.2%) and toxic exposure (25.6%). The mean age at presentation in post-infectious ataxia was 55 +/- 27.61 months, 60% females. A prodromal febrile illness was noted in 95%: varicella (10), nonspecific viral infection (6), mycoplasma, enterovirus, and Epstein-Barr virus. The latency from the prodromal illness to the onset of ataxia was 5.86 +/- 3.78 days. Lumbar punctures were altered in 11/17. All computed tomography scans performed were normal. At follow up, one boy presented asymmetric signs of cerebellar dysfunction secondary to hemicerebellitis. The media of the patient who showed full-gait recovery was 18 days, and was complete in all children, except one boy who presented hemophagocytic lymphohistiocytosis. Toxic ingestion was the second most common cause. Boys less than 6 years were more commonly affected. CONCLUSIONS: Acute childhood ataxia are an uncommon cause of presentation to our pediatric emergency room. Postinfectious ataxia and drug ingestion are the most common diagnosis, with a usually benign and self-limited process. A thorough history and neurology examination should be guided to etiology. Neuroimaging studies and hospitalization are needed only if atypical presentation, asymmetric neurologic examination and prolonged ataxia.

Tricobezoar gástrico en una niña diagnosticada de anorexia nerviosa purgativa / No disponible

El bezoar es una concreción de carácter variado, que puede encontrase en el estómago o intestino de hombre u otros animales. Los que se componen fundamentalmente por retos de pelos son los denominados tricobezoares. Esta entidad se observa fundamentalmente en menores de 30 años de edad y es más frecuente en las mujeres (9:1) y en personas con alteraciones del comportamiento alimentario. El propósito de este trabajo es documentar un caso de tricobezoar en una niña de 9 años que se presentó con síntomas inespecíficos que se confundieron con los de la anorexia nerviosa de la que previamente había sido diagnosticada (AU)

A bezoar is a collection of diverse aetiology that can be found in stomach or intestine of men or other animals. Those made basically of hair are called trichobezoar. This rare pathology is more usual in the group of age under 30 years old and also in women (9:1), basically if they associate eating disorders. Our objective was to relate a case of trichobezoar in a 9 year-old-girl, with non-specific sintomatology that was confounded with the clinic of anorexia nervosa, which she had been previously diagnosed (AU)

[Apraxia of gait: an acquired sequela with a poor prognosis]. / Apraxia de la marcha: una secuela adquirida de evolución desfavorable.

INTRODUCTION: Gait apraxia is not used to be considered as a diagnostic entity in Pediatric Neurology. CASE REPORTS: We present two pediatric patients that, after to have acquired normal gait and in consequence of a acute process, they lost the capacity to walk. In spite of intensive rehabilitation treatment hold along various years, they had not been able to help them. Both injury were very dissimilar; in one of them was affected the precentral and paracentral cortex in consequence of an encephalitic process. In the other, the basal ganglia and the hippocampus after a situation of near-drowning at the age of 15 months. CONCLUSION: The mechanism of this disorder is discussed and emphasis is done in its badly long-term prognosis.

[Idiopathic catastrophic epileptic encephalopathy: an untreatable convulsive malady in infancy]. / Encefalopatía epiléptica catastrófica idiopática: una forma intratable de estado de mal convulsivo en la infancia.

AIM: To present a case of catastrophic childhood epileptic syndrome with multifocal status epilepticus. CASE REPORT: A 4 years old boy with a multifocal status epilepticus of unknown origin which could only be controlled along some days with thiopentone enough to cause electrical suppression, and relapsed again after having stopped it. CONCLUSION: But for very high doses of barbiturates, any antiepileptic drug could control or improve the convulsions. MRI, initially normal, was followed by a progressive cerebral and cerebellar atrophy and the boy survived with heavy neurological secuelae.

[Paracetamol poisoning in infants aged less than six months: dosage errors]. / Intoxicación por paracetamol en menores de 6 meses: error de dosificación.

In infants under 6 months of age, paracetamol overdose is usually due to dose confusion by caretakers. Recently, liquid formulations of this drug have been commercialized in larger,60-ml bottles. The syringe to measure the syrup in these new formulations is also bigger (5 cc versus 1.2-2 cc). We present six cases of 2-4-month-old infants mistakenly given an overdose of paracetamol, each from this new 60-ml formulation. These patients are especially susceptible to poisoning because of liver immaturity and require more aggressive management. To prevent this kind of poisoning, correct and clear information must be given to caregivers about drug dosage.

Intoxicación por paracetamol en menores de 6 meses: error de dosificación / Paracetamol poisoning in infants aged less than six months: dosage errors

En lactantes menores de 6 meses la intoxicación por paracetamol suele ser secundaria a un error de dosificación por parte de los cuidadores. Recientemente se han comercializado presentaciones líquidas de paracetamol en envases mayores (60 ml). Adjuntan una jeringa administradora también mayor (5 ml frente a 1,2-2 ml). Presentamos 6 casos de lactantes de 2-4 meses que recibieron por equivocación dosis superiores a las terapéuticas, en todos ellos se trataba de un frasco de 60 ml. Estos pacientes son especialmente susceptibles por su inmadurez hepática a la intoxicación, requiriendo un tratamiento más agresivo. Para prevenir estas intoxicaciones, es fundamental una correcta información a los cuidadores sobre la posología de los fármacos (AU)

[Factors related to the short term remission of tics in children with Tourette syndrome]. / Factores que influyen en la remisión a corto plazo de los tics en niños con síndrome de Gilles de la Tourette.

INTRODUCTION: Tourette syndrome shows a fluctuating evolution, often masked by its comorbidity. OBJECTIVE: To study the clinical factors predicting the initial remission of tics in children with Tourette syndrome. Patients and methods. All patients attended during the last 5 years at a Child Neurology hospital based out patient clinic, with the diagnosis of Tourette syndrome according to DSM IV criteria, were selected. OUTCOME MEASURE: total remission of tics during at least 3 months, evaluated during the patient s second visit to our clinic. Demographic, clinic and therapeutic variables were studied. Statistical analysis was based on the Student t test or non parametric tests, as necessary. RESULTS: 53 patients, 44 males and 9 females. Age at starting tics: 6.9 2.2 years, time of evolution: 2 years (range: 1 9.4). Comorbidity in 51%: 34% with attention deficit hyperactivity disorder (ADHD), 17% with obsessive compulsive disorder (OCD) and school underachievement: 26%. Familial antecedents of tics, OCD, or ADHD: 49%. Tics remission at second visit to our clinic: 41.5%. Patients without remission were those with an earlier onset of tics (p=0.085), longer time of evolution (p< 0.05), or school underachievement (p= 0.024). Remission was not statistically associated with treatment. OCD and ADHD were associated with school failure but were not related to the tics evolution. CONCLUSION: The short term (at second visit), temporal (minimum 3 months) total remission of Tourette syndrome was not related to treatment but to previous duration of the syndrome and to factors (other than OCD and ADHD) that lead to school failure.

[Disturbances of neuroblast migration and amniocentesis. An under diagnosed problem?]. / Trastornos de la migración neuroblástica y amniocentesis. Un problema infradiagnosticado?

INTRODUCTION: Performing amniocentesis between the fourth and sixth months of gestation is a widespread practice. However, it can entail serious consequences, apart from the loss of the foetus, such as injuries to the developing central nervous system. CASE REPORTS: Over the past few years we have dealt with four patients who were carriers of a focal disturbance of neuroblast migration, which could have its origins in amniocentesis. Two patients displayed mental retardation and difficult to treat epilepsy, while the other two only presented hemiparesis. In two cases the right hemisphere of the brain was affected. One of them also presented extracranial brain tissue, which was possibly extruded during the accidental puncture of the skull. CONCLUSIONS: Despite its being rarely mentioned in the literature, a direct or indirect attack on the brain during diagnostic amniocentesis is a possibility that must be borne in mind when considering the use of this technique

[The efficacy of lamotrigine in refractory infantile epilepsy]. / Eficacia de la lamotrigina en la epilepsia infantil rebelde.

INTRODUCTION: The new antiepileptic drugs should also be evaluated outside clinical trials. OBJECTIVE: To study the efficacy of treatment with lamotrigine in everyday neuropaediatric clinical practice. PATIENTS AND METHODS: We made a longitudinal study of all patients treated with lamotrigine in a hospital outpatients department. The efficacy was evaluated by: 1. The number of patients showing partial response (>/=50% reduction in seizures as compared to the basal rate), and total response maintained throughout the follow up period. 2. Duration of treatment, studied using the Kaplan Meier method. RESULTS: 80 patients treated with LTG had as the aetiology of their seizures: idiopathic 3.7%, cryptogenic (51.3%) and symptomatic (45%). We found there to be partial control during the first three months of treatment in 60.8% of the patients, which was maintained after 12 months in 43.1%, and after three years in 30.7%. Total control of the seizures after three months was found in 14.8%, after twelve months in 9.8% and after three years in 3.8%. The probability of treatment with lamotrigine being maintained for six months was 86%, for twelve months 61% and for three years 31%. Lamotrigine was suspended in 38 patients (47.5%). Side effects were reported in five cases. CONCLUSIONS: In clinical practice, the therapeutic effect not only consists of the percentage of patients who respond to treatment, but also the length of time this response is maintained. In our study, both measurements of effect showed results similar to those with classical antiepileptic drugs.

[Course of drug-resistant childhood epilepsy treated with topiramate]. / Evolución de la epilepsia infantil rebelde tratada con topiramato.

INTRODUCTION: The variable proportion of cases with childhood epilepsy resistant to treatment, has led to the development of different new drugs. OBJECTIVE: To study the efficacy of topiramate as add-on therapy in the everyday practice of a neuropaediatric clinic. PATIENTS AND METHODS: A retrospective study of all the patients treated with topiramate in a hospital outpatient clinic was performed. OUTCOME MEASUREMENT: 1. Reduction in >/= 50% as compared to basal frequency (partial control) and total control of seizures, and 2. Duration of drug treatment, using the Kaplan-Meier method. RESULTS: We studied 31 courses of treatment in 29 children. The aetiology of the epileptic syndromes was: idiopathic epilepsy (3.3%), cryptogenic epilepsy (58%) and symptomatic epilepsy (38.7%). We found a >/= 50% reduction in the basal monthly frequency of seizures after 3 months of treatment in 52% of the patients, which persisted 18 months later in 25%. Total control of seizures was obtained in 18.5% of the patients after 3 months of treatment, and this response was maintained 12 months later in 12.5%. The probability of maintaining treatment with topiramate for 6 months was 80%, and for 12 months was 49%. The average duration of treatment was 7.9 months (interval 1-29 months). Topiramate was suspended in 9 patients (29%). CONCLUSION: In difficult to treat childhood epilepsy topiramate as add-on therapy provides a partial and total responses which are similar to those reported with classical drugs, and depend on the duration of follow-up.

[Hereditary spherocytosis in neonates. Review of our caseload]. / Esferocitosis hereditaria neonatal: revisión casuística.

AIM: Review of hereditary spherocytosis diagnosed in infants younger than two months and their follow up. PATIENTS AND METHODS: Retrospective study of 18 infants younger than two months diagnosed from 1973 to 1995. RESULTS: Diagnosis was established in the first week of life in 50% of the patients. Hereditary pattern was autosomic dominant in 94% of the cases. Anaemia was observed in all the patients and hyperbilirubinemia in only 44%, although the latter was the clinical presentation in patients diagnosed at younger age. Exchange transfusion was performed in 3 children (1 with the severe form and 2 with the typical form of the disease). During the first 6 months of age, 55% of infants presented hemolytic crises that required transfusion in 91% of them. Both periodicity of crises and transfusions decreased to 38 and 44% respectively after the first year. Splenectomy was performed in the 3 children with severe forms and in 6 with typical forms (mean age 8 years and 3 months). No cholecystectomy was required so far. CONCLUSIONS: The authors believe that neonatal spherocytosis does not implicate worse prognosis at follow up. Blood support is higher during the first year of life. Elective splenectomy depends on age and transfusional requirements.

[Telephone consultation in child neurology practice: quantification and contents]. / Labor asistencial telefónica en la consulta neuropediátrica: cuantificación y contenido.

INTRODUCTION: The workload of telephone consultations has been scarcely studied except at the Emergency Units, in spite of the considerable amount of time consumed by the task in normal practice. OBJECTIVE: To asses the workload of telephone consultations in a Neuropediatric Unit, during normal working hours. METHODS: A prospective analysis of all calls received during a year, at the out-patient hospital-based neuropediatric clinic. RESULTS: 150-200 telephone consultations were received every month, decreasing during the holiday seasons. They amount to two thirds of the face-to-face contacts at the clinic, during the same period of time, and require about 10% of our day-time working hours. Disorders more likely to produce the calls: epilepsy (40%), mental retardation (19%), cerebral palsy (11%), all others with a frequency lower than 5%. These percentages, and those of hydrocephalus, autism and spina bifida, are similar to those encountered in normal practice, while headaches, speech delay, hyperactivity, syncopes, etc. produced half the number of calls expected; myopathies doubled the number of calls expected. The monthly frequency of calls varied significatively, along the year, for epilepsy, mental retardation, hyperactivity, migraine and autism. Calls were made especially by the family (49%), but it depends of the purpose and the patient's diagnosis. The purpose was most frequently to consult about the symptomatology of the illness; treatments produced 7% of calls; and discussion about citations, 19.5%, regardless of the diagnosis. CONCLUSION: In neuropediatric practice, telephone consultations should be recognized and provided for in order to manage effectively the clinical demand.

[Neuropediatric clinical practice in a tertiary hospital of Basque Country]. / La práctica clínica neuropediátrica en un hospital terciario del País Vasco.

INTRODUCTION: In our country, studies on the neuropediatric practice are scarce, in spite of their importance for planning of spending and resources, and definition of quality criteria. OBJECTIVE: To study the clinical workload of the main neuropediatric clinical problems. METHODS: Prospective, longitudinal, descriptive study of doctor-patient encounters, according to diagnosis, in the Child Neurology Division of a tertiaty hospital. RESULTS: 3,200 visits, of which 83% were ambulatory. 24 ambulatory visits per 1,000 inhabitants younger than 15 years, and year. Diagnosis demanding larger clinical activity for out-patients were: epilepsy/seizures (38%), mental retardation (17%), headaches (17%), hyperactivity/conduct disorders (12%), and cerebral palsy/permanent motor sequela (10%). Among in-patients: epilepsy/seizures (47%), mental retardation (10%), brain tumors/neurological complications of oncology disorders (8%), cerebral palsy/motor sequela (8%), and neurological complications of prematurity (7%). Among new out-patients, headaches is the most frequent diagnosis, followed by epilepsy; among new in-patients, epilepsy holds the first place, followed by neurological complications of prematurity and brain trauma. The index follow-up/first visit is much larger for the real neurologic patient than for functional disorders so frequent among new referrals. CONCLUSIONS: The neuropediatric practice comprises essentially epilepsy, cognitive and conduct disorders, and headaches. Unfortunately, our practice is in part being defined by the pressure exerted by the primary medical care and by hospital management practices.