Trastornos del movimiento no epilépticos en la infancia / Non-epileptic movement disorders in childhood

Los trastornos paroxísticos son alteraciones episódicas con normalidad intercrítica. Pueden ser epilépticos y no epilépticos y se acompañan habitualmente de movimientos anómalos. Los trastornos paroxísticos (en gran parte el diagnóstico diferencial de la epilepsia), suponen uno de los capítulos más amplios de la Neuropediatría en la frecuencia de la demanda, por la gran variedad de problemas que plantean y por la trascendencia de muchos de ellos. El diagnóstico diferencial de los trastornos paroxísticos incluye reacciones vagales, síndromes suboclusivos intestinales o problemas que plantean riesgo vital, como los síncopes cardíacos. El diagnóstico erróneo de epilepsia supone hacer asumir un diagnóstico y tratamiento equivocados y no apurar la estrategia diagnóstica para descartar otras patologías, algunas de ellas de gran trascendencia, incluso vital. Con frecuencia el diagnóstico se obtiene por una minuciosa anamnesis de los episodios o la visualización en vídeo de los episodios. Debe insistirse en la historia familiar y debe preguntarse por la toma de fármacos. Cuando el diagnóstico es claro, no es necesaria la realización de exámenes complementarios. Pueden estar indicadas analíticas para descartar causas de crisis sintomáticas u otros trastornos del movimiento, como la tetania hipocalcémica. En algunos casos es obligatorio el estudio cardiológico. Es aconsejable en muchos casos la realización de un electroencefalograma (EEG). La neuroimagen, especialmente la resonancia magnética cerebral, está indicada en el estudio de la epilepsia y se debe realizar para descartar otras patologías en algunos TPNE como las disquinesias paroxísticas o el síndrome de hemiplejías alternantes. La mayoría de los TPNE no tienen tratamiento específico (AU)

Paroxysmal disorders are episodic events with normality between crisis. These can be epileptic or non-epileptic, and are usually accompanied by abnormal movements. The study of paroxysmal disorders (and the differential diagnosis of epilepsy) is one of the widest fields of Neuropaediatrics, due to the high number of referrals and the variety of problems and consequences that it brings. The differential diagnosis of paroxysmal disorders include vagal reactions, subocclusive syndrome due to intestinal malrotation or life threatening problems such as cardiac syncope. The erroneous diagnosis of epilepsy means accepting the wrong diagnosis and treatment and failing to rule out other pathologies that could be life threatening..The diagnosis of PNEs is generally based on a careful anamnesis or video images of the episodes. Family history and details of medication used must be queried. When the diagnosis is clear, no complementary examinations are necessary. Specific analysis can be indicated to rule out symptomatic causes or other movement disorders, such as hypocalcaemic tetany. In many cases EEG is well-advised. Brain imaging, particularly MRI is required in the study of epilepsy and to rule out others pathologies in PNEs such as paroxistic dyskinesias or the alternating hemiplegia syndrome. Most of the PNEs have no specific treatment (AU)

[Neuropaediatrics and primary care. Our experience in the 21st century]. / Neuropediatría y atención primaria.Nuestra experiencia en el siglo XXI.

INTRODUCTION: The quality of the health care in a major part of neuropaediatrics benefits from appropriate communication and strategies that have been agreed with primary care (PC) paediatricians. PATIENTS AND METHODS: We analyse the children who were assessed in the Neuropaediatric service at the Hospital Universitario Miguel Servet in Saragossa over a period of eight years and we also discuss the most important courses of action followed in the most prevalent problems. RESULTS: Eight reasons for visiting accounted for 86% of the total number: paroxysmal disorders (33%), headache (27%), psychomotor retardation (11.5%), alterations affecting the shape or size of the head (5.6%), problems at school and/or attention deficit (4.5%), behavioural disorders (4.25%), gait disorders (3.5%) and perinatal distress (3.4%). The most frequent diagnoses are headaches/migraines (26%), non-epileptic paroxysmal disorders (16.5%), prenatal encephalopathy (10.5%), epilepsy (8%), mental retardation (7.5%), infantile cerebral palsy (4.6%), cryptogenic attention deficit hyperactivity disorder (ADHD) (3.8%) and cryptogenic autism (3.6%). CONCLUSIONS: The PC paediatrician working in close relation with the children and their families in all cases is the person mainly responsible for conducting a follow-up on some of the most prevalent problems, such as headaches, many non-epileptic paroxysmal disorders and ADHD. The processes must be established, clearly specified, based on the best evidence, with the participation and within reach of all the professionals involved, in order to favour homogeneity and keep variability in the interventions to a minimum. Channels of communication, including the information and communications technologies, need to be set up to allow health professionals to be permanently up-to-date and capable of controlling their patients in the best possible way.

Neuropediatría y atención primaria. Nuestra experiencia en el siglo XXI / Neuropaediatrix and primary care our experience in the 21ST century

Introducción. La calidad asistencial de gran parte de la neuropediatría se beneficia de una adecuada comunicacióny de estrategias consensuadas con los pediatras de atención primaria (AP). Pacientes y métodos. Se analizan los niños valorados en la consulta de neuropediatría del Hospital Universitario Miguel Servet de Zaragoza en ocho años y se exponen las líneas más importantes de actuación en los problemas más prevalentes. Resultados. El 86% de las primeras visitas se reparteentre ocho motivos de consulta: trastornos paroxísticos (33%), cefalea (27%), retraso psicomotor (11,5%), alteraciones de la forma o tamaño de la cabeza (5,6%), problemas escolares y/o atención deficiente (4,5%), alteraciones del comportamiento (4,25%), trastornos de la marcha (3,5%) y sufrimiento perinatal (3,4%). Los diagnósticos más frecuentes son cefaleas/migrañas(26%), trastornos paroxísticos no epilépticos (16,5%), encefalopatía prenatal (10,5%), epilepsia (8%), retardo mental (7,5%), parálisis cerebral infantil (4,6%), trastorno por déficit de atención con hiperactividad (TDAH) criptogénico (3,8%) y autismo criptogénico (3,6%). Conclusiones. El pediatra de AP cercano a los niños y sus familias en todos los casos es el principalresponsable del seguimiento de algunos de los problemas más prevalentes, como cefaleas, muchos trastornos paroxísticos no epilépticos y TDAH. Los procesos deben estar establecidos, claramente explicitados, basados en las mejores evidencias, con la participación y al alcance de todos los profesionales involucrados, en beneficio de la homogeneidad y reducida variabilidadde las actuaciones. Es necesario establecer vías de comunicación, incluidas las tecnologías de la información y comunicación para una continua actualización y el mejor control de los pacientes

Introduction. The quality of the health care in a major part of neuropaediatrics benefits from appropriatecommunication and strategies that have been agreed with primary care (PC) paediatricians. Patients and methods.We analyse the children who were assessed in the Neuropaediatric service at the Hospital Universitario Miguel Servet in Saragossa over aperiod of eight years and we also discuss the most important courses of action followed in the most prevalent problems. Results. Eight reasons for visiting accounted for 86% of the total number: paroxysmal disorders (33%), headache (27%), psychomotor retardation (11.5%), alterations affecting the shape or size of the head (5.6%), problems at school and/or attention deficit (4.5%), behavioural disorders (4.25%), gait disorders (3.5%) and perinatal distress (3.4%). The most frequent diagnoses areheadaches/migraines (26%), non-epileptic paroxysmal disorders (16.5%), prenatal encephalopathy (10.5%), epilepsy (8%), mental retardation (7.5%), infantile cerebral palsy (4.6%), cryptogenic attention deficit hyperactivity disorder (ADHD) (3.8%) and cryptogenic autism (3.6%). Conclusions. The PC paediatrician working in close relation with the children and theirfamilies in all cases is the person mainly responsible for conducting a follow-up on some of the most prevalent problems, such as headaches, many non-epileptic paroxysmal disorders and ADHD. The processes must be established, clearly specified, based on the best evidence, with the participation and within reach of all the professionals involved, in order to favourhomogeneity and keep variability in the interventions to a minimum. Channels of communication, including the information and communications technologies, need to be set up to allow health professionals to be permanently up-to-date and capable of controlling their patients in the best possible way

[Cases of symptomatic epilepsy at a regional reference neuropediatric unit]. / Casuística de epilepsia sintomática en una unidad de neuropediatría de referencia regional.

OBJECTIVE: The objective of this study was to analyze, from an aetiological angle, the cases seen with symptomatic epilepsy by the Seccion de Neuropaediatria del Hospital Miguel Servet de Zaragoza. PATIENTS AND METHODS: We studied the cases diagnosed as having symptomatic epilepsy between May 1990 and November 1999. RESULTS: Of a total of 4,466 children assessed during the study period, the diagnosis of epilepsy was established in 461 children (10.3% of the total). This included idiopathic epilepsy in 110 cases (23.9%), cryptogenic epilepsy in 119 cases (25.8%) and symptomatic epilepsy in 232 cases (50.3%). The aetiologies of the symptomatic epilepsies were: prenatal encephalopathies in 137 cases (59%), perinatal encephalopathies in 33 (14.3%), postnatal encephalopathies (due to accidents, acquired infections and postnatal cerebrovascular accidents) in 20 (9%), tumours ( including the post-operative period) in 14 (6%), neurocutaneous syndromes in 13 (5.6%), metabolic and degenerative disorders in 13 (5.6%) and one case of vascular malformation. CONCLUSIONS: The symptomatic epilepsies make up half the epilepsies evaluated by the department of neuropaediatrics. In 59% the cause was prenatal. Other causes of symptomatic epilepsy were also represented in the series. A detailed study of these should help us to understand and manage them better. We consider aetiological aspects to be very important in the study of epilepsy, since the aetiology is one of the most important factors in prognosis.

[Lymphoblastic non-Hodgkin's lymphoma: estimation of the survival of patients with the disseminated form of the disease (stages III and IV) treated by the LSA2-L2 protocol]. / Linfoma no Hodgkin de tipo linfoblástico: evaluación de la supervivencia en pacientes con enfermedad diseminada (estadío III y IV) tratados con el protocolo LSA2-L2.

We analyze the survival rate in 17 pediatric patients (twelve males and five females) that were diagnoses of non-Hodgkin's lymphoblastic lymphoma (cases with Burkitt's lymphoma were excluded) during the period from 1982 to 1990. All of them were treated using the LSA2-L2 therapeutic protocol. The follow-up period ranged from 14 to 99 months (follow-up median 43 months). Staging of our newly diagnosed patients, we found 9 patients in stage III and 8 in stage IV disease. Survival was analyzed by the Kaplan-Meier method. The overall survival rate of our patients is 69.5% and the disease free survival is 62.6%. The disease free survival for separated stages is 66.5% for stage III and 58.3% for stage IV. We compare our results with other casuisties published in literature.

[Ultrasonic diagnosis of neuroblastoma]. / Diagnóstico ultrasonográfico en el neuroblastoma.

Neuroblastoma is one of the most frequent tumors in childhood. Suprarenal area is its main abdominal location, appearing in lesser proportion in paravertebral ganglionar chains. Its prediction depends principally on the age of the patient and the pathological stage. US are the best method for its diagnosis. Its most frequent appearance is as an hyperechogenic heterogeneous mass with calcifications inside. In evaluation tumoral extension, other complementary methods such as CT and MRI should be used.

[Eosinophilic cystitis]. / Cistitis eosinofílica.

We report two cases of eosinophilic cystitis in children of 11 months and 11 and a half year of age respectively. The presenting symptoms were the emision of mucosanguinolent filaments in the urine in one case and with lower urinary tract symptoms and macroscopic hematuria in the other. Physical examination revealed in one of them a round, tough and painful mass in the hypogastric area. In one case we found peripheral blood eosinophilia. They both showed in the Urinalysis albuminuria, pyuria and hematuria, with the presence of eosinophils in one of them. Macroscopic examination presented in the two cases a tumoral, mamelonne mass in the wall of the bladded. The diagnosis was anatomopathologic due to the demonstration of an inflammatory infiltration, primarily of eosinophils. The clinical course was unfavourable in the case an hemicystectomy was practicated and self-limited in the one only symptomatic treatment was used.

[Values for the cortical thickness and diameter of the metacarpals of normal Aragonese children in the 1st 4 years of life. Longitudinal study]. / Valores del espesor de la cortical y del diámetro de los metacarpianos de niños aragoneses normales en los cuatro primeros años de vida. Estudio longitudinal.

The measurement of the metacarpal cortical thickness as well of the diameter is an accurate, simple and economic procedure in the clinical diagnosis of the growth deficiency. Our study reports the increasing values of such parameters obtained from a normal Spanish population controlled at ages 0.5, 1, 2, 3 and 4, in the growth studies center "Andrea Prader" of Zaragoza.

[Spontaneous regression of an aneurysmal bone cyst of the vertebrae after partial excision. Bibliographic review]. / Regresión espontánea de un quiste óseo aneurismático de localización vertebral tras su extirpación parcial. Revisión bibliográfica.

A case of a lumbar aneurysmal bone cyst is presented in a 5 year-9 month old girl. The characteristics of this tumor are described, specifically in the spinal area, with particular reference to the diagnostic and therapeutic difficulties referred, taking into account the existing bibliography. The partial resection has been curative, as it has been frequently written about in publication worldwide and which suggest that surgery does not have to be aggressive in unapproachable cases and that radiotherapy is unnecessary.