bluuebunny/crossref_metadata_2025 huggingface dataset:
BAEPs IN INFANTS WITH MYELOMENINGOCELE AND LATER DEVELOPMENT OF CHIARI II MALFORMATION‐RELATED BRAINSTEM DYSFUNCTION
Authors: Worley Gordon , Erwin Charles W. , Schuster James M. , Park Young , Boyko Orest B. , Griebel May L. , Weidman Eric R. , Radtke Rodney A. , Oakes W. Jerry
Published: July 2010

Thirty‐seven infants with myelomeningocele received brainstem auditory evoked potentials (BAEPs) at a median age of eight days. No infant had brainstem dysfunction at the time of testing. Median follow‐up was at 30 months. Of 12 infants who subsequently developed brainstem dysfunction at a median age of three months, 11 had had abnormal neonatal BAEPs. In contrast, only 10 of 25 infants who did not develop brainstem dysfunction had abnormal BAEPs. The mean average I‐V interpeak latencies was greater among those who developed symptoms than among those who did not. Neonatal BAEPs can identify a group of asymptomatic infants with myelomeningocele who need close follow‐up for the subsequent development of brainstem dysfunction.

BAEP néonataux chez des nourrissons spina bifida et apparition ultérieure ďune dysfonction du tronc cérébral en rapport avec une malformation de Chiari de type II

A un âge moyen de sept jours, les potentiels évoqués auditifs du tronc cérébral (BAEP) ont été recueillis chez des nourrissons spina bifida. Aucun nourrisson ne présentait de dysfonction du tronc cérébral au moment des enregistrements. Le suivi moyen fut de 30 mois., Parmi les 12 nourrissons ayant présenté ultérieurement une dysfonction du tronc cérébral, 11 avaient eu des BAEP néonataux anormaux. En revanche, 10 enfants seulement parmi les 25 nourrissons n'ayant pas présenté ultérieurement de dysfonction du tronc cérébral, avaient des BAEP anormaux. La durée moyenne de latence entre les pointes I‐V étaient plus longue chez les enfants devant présenter des symptômes que chez les autres. Les BAEP néonataux ont done pu identifier un groupe de nourrissons spina bifida asymptomatiques, exigeant un suivi soigneux en raison du développement ultérieur ďune dysfonction du tronc cérébral.

PEA en recién nacidos con mielomeningocele y desarrollo subsiguiente de malformación de Chiari II en relación condisfunción del tronc cerebral

Se estudiaron los potenciales evocados acústicos (PEA) del tronco cerebral en 37 Iactantes con mielomeningocele a la edad media de ocho días. En el momento de la exploración ningiin niño presentaba disfunción del tronco cerebral. El seguimiento promedio fue de 30 meses. De 12 niños que posteriormente desarrollaron una disfunción de tronco a la edad media de tres meses, 11 tenfan PEA anormales. Por el contrario sólo 10 de 25 niños que no sufrieron disfunción de tronco tenian un PEA anormal. El promedio de las latencias entre los picos I‐V era mayor en los que desarrollaron sintomas, que en los que no los sufrieron. Los PEA pueden identificar un grupo de Iactantes asintomáticos con mielomeningocele, que requerirán un seguimiento cuidadoso por el posible desarrollo posterior de una disfunción de tronco cerebral.
Bilateral Painful Epileptic Seizures of the Hands
Authors: Otani Kazumasa , Imai Katsumi , F'utagi Yasuyuki , Yanagihara Keiko
Published: July 2010

Secondary sensory seizures (SSS) are very rare epileptic seizures. A 14‐year‐old male patient with habitual painful seizures of the backs of both hands since the age of four is reported. The painful seizures were rarely followed by impairment of consciousness, Ictal EEG showed 4 to 6Hz rhythmic waves over the right anterior and mid‐temporal area following complaints of pain. The absence of tumorous lesions on brain CT or MRI, childhood onset and the absence of combined seizures were characteristic features of this case compared with other patients reported to have SSS.

Crises épileptiques bilatérales douloureuses des deux mains

Les crises scnsorielles sccondaircs (SSS) constituent une forme très rare d'épilepsie. L'article décrit le cas d'un aolescent de 14 ans présentant fréquemment depuis l'âge de quatre ans des crisesdouloureuses du dos des deux mains. Les crises douloureuses étaient rarement suivies de perte de conscience. Les LEG ont montré des ondes rythmiques de 4 à 6Hz sur les parties antérieures et moyennes de l'aire temporale droite à la suite de la perception de douleur. L'absence de lésions tumorales au sanner cérébral ou à l'FRM, le début durant durant l'enfance et l'absence de crises combinées différenciaient ce patient des autres patients avec SSS.

Bilaterale schmerzhafie epileptische Anfälle der Hände

Sekundäre sensorischc Anfälle (SSS) sind sehr seltene epileptische Anfälle. Es wird von einem 14‐jährigen Jungen berichtet, der seit dem viertcn Lebensjahr schmerzhafte Anfälle in beiden Handrücken hatte. Auf diese schmerzhaften Anfälle folgte selten eine Bewußtseinsstörung. Nach Eintrilt des Schmerzes zeige das ictale EEG rhythmische Wellen von 4 bis 6Hz über der rechten vorderen und midtemporalen Region. Im Verglcich zu den Berichten von anderen Patienten mit SSS waren die charakteristischen Merkmale bei diesem Fall das Fehlen von Tumorläsionen im Hirn CT oder MRI, der Beginn im Kindesalter und das Fehlen kombinierter Anfälle.

Crisis epilépticas bilaterales dolorosas de las manos

Las crisis sensorials secundarias (CSS) son unas crisis epidlepticas muy raras. Sc aporta un caso de un varón de 14 años de edad con crisis habituales de dolor en el dorso de ambas manos desde la edad de cuatro años. Los ataques de dolor se seguian muy raramente de alteración de la conciencia. El EEG ictal mostró ondas rítmicas de 4 a 6Hz en las áreas anterior y medio temporal del lado derecho, después de quejarse del dolor. La ausencia dc lumores eerebrales tanto a la TAC como a la IRM. el inicio en la infancia y la ausencia de crisis combinadas eran las características de este caso en comparacion con otros pacientes referidos con CSS.
Immunoglobulin Therapy in Guillain‐Barré Syndrome in Children
Authors: Nicolaides P. , Appleton R. E.
Published: July 2010

Four children with an evolving Guillain‐Barré syndrome were treated with a five‐day course of intravenous immunoglobulin. No patient showed further progression of the condition and all made a rapid and complete recovery with no evidence of relapse over a six‐ to 24‐month follow‐up period. The early use of immunoglobulin in this disorder may prevent further progression of the disease and accelerate short‐term recovery, with resulting medical, social and financial implications.

Thérapeutique immunoglobulinique dans le syndrome dc Guillain‐Barré chez l'enfant

Un traitement par immunoglobuline intraveineuse a été appliqué pendant cinq jours à quatre enfants atteints d'un syndrome de Guillain‐Barré en évolution. Aucune progression de la maladie ne fut observée et une guérison rapide et complète fut notée, sans manifestation de rechute durant un suivi de six à 24 mois. L'appel précoce aux immuniglobulines dans ce trouble peut suspender l'évolution et accélérer la guérison á court terme, avec toutes les implications sur les plans médicaux, sociaux et financiers.

Immunglobulintherapie beim Guillain‐Barré Syndrom bei Kindem

Vier Kinder mit beginnendem Guillain‐Barré Syndrom wurden über fünf Tage intravenös mit Immunglobulinen behandelt. Keiner der Patienten zeigte eine Progression der Erkrangkung und alle erholten sich schnell und vollständing und über einen Kontrollzeitraum von 6–24 Monate wurden keine Anzeichen für ein Rezidiv beobachtet. Die frühzeitige Anwendung von Immunglobulinen bei dieser Erkrankung kann eine Progression verhindern und zu einer schnellen Gesundung beitragen, woraus sich medizinische, soziale und finanzielle Vorteile ergeben.

Terapia con inmunoglobulina en el síndrome de Guillain‐Barré en niños

Cuatro niños con un síndrome de Guillain‐Barré activo fueron tratados con inmunoglobulina endovcnosa durante cinco dias. Ningún paciente mostró una sucesiva progresión de la enfermedad y de forma completa, sin evidencia de ŕecaida, durante un seguimiento de seis a 24 meses. El uso precoz de la inmunoglobulina en esta enfermedad puede prevenir una progresion de la enfermedad y acelerar una recuperación a corto plazo, con las adecuadas implicaciones médicas, sociales y financieras.
THE MATURATION OF LINEAR ACUITY AND COMPLIANCE WITH THE SONKSEN‐SILVER ACUITY SYSTEM IN YOUNG CHILDREN
Authors: Salt A. T. , Sonksen P. M. , Wade A. , Jayatunga R.
Published: July 2010

The compliance and maturation of visual acuity of 1245 ½‐ to nine year olds was investigated. Testing was undertaken as part of routine surveillance. Ability to letter match, to achieve a binocular measure and to achieve monocular measures for children under three years was 89, 78 and 37 per cent, for children aged three to four years 97, 91 and 66 per cent, and for children over four years 99.9. 99.9 and 99. respectively A statistical model was developed to give acuity norms by age for use by surveillance and diagnostic teams.

La maturation tie lcar;acuitc linéaire et la participation au systéme de mesuie dcar;acuité de Sonksen ‐Silver che: les jeunes enfanls

La participation et la maturation de lcar;acuilc visttelle out été recherchees chez 1245 enfants ages de 27‐ à neuf ans. Le test fut entreprts dans le cadre dcar;une surveillance de routine, l.a capacité dcar;appaner des leures. dcar;elfecture des mesures binoculaires et monoculaires fut respectivement de 89. 78 et 37 pour cent des enfants en dessous de trois ans. respectivement de 97. 9] et 66 pour cent chez les enfants ágés de trois a quatre ans et respectivement de 99.9, 99.9 et 99 pour tent chez les enfants de plus de quartre ans. Un modèle statisque a pu étre effectué. dormant les noniics dcar;acuité par âge en vue de'emploi par les équipes de surveillance et de diagnostic.

de tres a cuatro años. y de 99,9, 99,9 y 99 por ciento respectivamente en niños de más de cuatro anos. Se desarrolló un inodelo estadistico para dar unas nornias de agutdeza según la edad. para uso de equipos de vigilancia y diagnóstics.

Compliance und Reifung der Sehschärfe wurden bci 1245 Kinderen im Alter von 2½‐ bis neun Jahren untersucht. Die Kinder wurden im Rahmen von Routineuntersuchungen getestet. Die fähigkeit Buchstaben zu vergleichen. einen binokulären Befund und monokuläre Befunde zu erlangen. betrug für Kinder unler drei Jahren 89 b/w. 78 b/w. 37 Prozent, für Kinder zwischcn drei und vier Jahren 97, 91 und 66 Prozent und für Kinder über vier Jahren 99.9, 99.9 und 99 Prozent. I‐s wurde em statistisches Modell entwickelt. altersentsprechende Normwerte der Sehschärfe für Vorsorge‐ und diagostische lintersuchungen herauszugeben.

Maduración de la axtideza linear y conipliunza usando el sistenia de agudeza Sonksen‐Sitver en nihos pe que lias

Se investigó la complianza y la maduración de la agudcza visual de 1245 niños de 27½.‐ a neueve años. La exploraeion se realize formando parte de una vigilancia rutinaria. l.a habilidad de emparejar leirasy hacer mediciones binoculares y monoculares fue de 89. 78 y 37 por ciento respectivamente en niños de tres a cuatro añose de 97, 91 y 66 por ciento respectivamente en niños
THE LIFE‐EXPECTANCY OF PERSONS WITH CEREBRAL PALSY
Authors: Crichlan J. U. , Mackinnon M. , White C. P.
Published: July 2010

Survival rates for persons with cerebral palsy were calculated using information front a population‐based registry which has been collecting data since 1952. The 30‐year survival rate approximates 87 per cent. Adverse factors affecting survival are the type of cerebral palsy (spastic quadriplegia having the worst prognosis), epilepsy and severe or profound menial retardation. The length of time over which the data were collected precludes estimating survival time beyond 30 years.

Espérame de vie chez, les IMC/1MOC

Les taux de survie chez les IMC/IMOC ont été calculés à partir des informations de registres dcar;état‐civil recueillies depuis 1952. Le taux de survie à ans est approximativement de 87 pour cent. Les facleurs négatifs affectanl la survie sont le type dcar;alleinte (les quadriplégic's spastiques ont le plus mauvais pronostic). lcar;épilepsie et lcar;arriération mentale sévére ou profonde. La période durant laquelle les données ont été recueillies ne perniet pas de calculer une espérance de vie au delà de 30 ans.

Lebenserewartung; von Personal mit Cerebralparese

Die Überlebensratcn von Personen mit Cerebralparese wurden anhand eines Registers, in dem seit 1952 Daten gesammelt worden waren, berechnet. Die 30‐Jahre‐Überlebensrate entspricht etwa 87 prozent. Fakiorert, die das Überleben negativ beeinflussen, sind bestimmte A.nen der Cerebralparese (wobei die spastische Tetraplegie die schechtestc Prognose hat). Epilepsic und schwere geistige Relardierung. Die Zeitspanne, in der die Daten gcsammelt wurden. Schlietßt die Beurteilung einer Überlebenszeit von mehr als 30 Jahren aus.

Expeclativas de villa de persomts con paralisis cerebral

Se calculó el porcentaje de supervivencias de personas con paralisis cerebral utilizando la information suministrada por un registro de una población base, cuyos datos se habia recogido a partir tie 1952. El porcentaje de supervivencia a los 30 años de edad era aproximadamente del 87 por ciento. Los factores que afectan negalivamente la supervivencia son ciertos tipos de paralysis cerebral (la que tiene peor pronoslieo es la cudriplejia espàstica). la epilepsis y un retraso mental profundo o grave. El largo tiempo durante el cual se recogieron los datos. impide hacer la estimación más allá de los 30 años.
A LONG PERSPECTIVE ON CHILDHOOD MULTIPLE SCLEROSIS
Authors: Cole G. F. , Smart C. A.
Published: July 2010

Twenty‐eight patients, all of whom developed multiple sclerosis before 16 years of age and all from a well‐defined geographical population (Grampian, north‐eastern Scotland) were retrospectively studied. Age at presentation, presenting features and interval between first and second attacks are discussed. Follow‐up ranged from three to 47 years and adult categorisation was applied to outcome. This suggested that on the whole, childhood‐onset multiple sclerosis is a less aggressive disease than its adult counterpart.

Perspectiva a largo plaza en niños con esclerosis múltiple

Veintiocho pacientes que dcsarrollaron una esclerosis multiple antes de los 16 años de edad. Y provinentes todos de una poblacion geografica bien definida (Grampian, Norcste de Eseocia) fueron estudiados retrospectivamente. Se discute la edad de inicio, la clínica inicial y los intervalos entre el primer y segundo ataques. El seguimiento varió cntre los tres y 47 años y en el curso se aplicó la categoriación adulta. Ello sugiere que en conjunto la esclerosis múltiple de inicio infantile es menos agresiva que la forma adulta.

Eine Langzeitprognose der Multiplen Sklerose tin Kindesalter

21 Patienten, von denen alle vor dem 16. Lebensjahr eine Multiple Sklerose entwickelten und die alle aus einer genau definierten gcorgraphischen Population stammten (Grampian, Nord‐Ost‐Schottland) wurden retrospektiv untersucht. Alter bei Beginn, Anfangssymptome und Interval zwicshen dem ersten und zweiten Schub wurden diskutiert. Der Konlrollzeitraum betrug zwicshen drei und 47 Jahre und die Erwachseneneinteilung wurde auf den Outcome angewendet. Dies ließ vermuten. Daß die im Kindesalter beginnende Multiple Sklerose weniger aggressiv ist als die Form im Erwachsenenalter.

Une tongue approche de la sclérose en plaque de ľenfance

Une étude rétrospective a porté sur 28 patients ayant tous présenté une sclérose en plaque avant ľâge de 16 ans et provenant de la population géographiquement bien définie de Grampian, au nordest de ľEcosse. ľâge àľ examcn, les symptómes préséntes et ľintervalle entrc la premiére et la seconde poussé sont discutés. Ue suivi était de trois à 47 ans et la classification adulte fut appliquee au devenir. Ces donnes suggèrent que globalement, un début de sclérose en plaque duraut ľenfance traduit une affection moins agressive que son ëquivalent adulte.
EARLY INFANT GRASPING INVOLVES RADIAL FINGERS
Authors: Lantz Cecilia , Melén Karin , Forssberg Hans
Published: July 2010

Halverson's hypothesis states that prehension gradually develops from ulnar to forefinger holding. This hypothesis was tested by measuring the grip force of individual Fingers (digits 11 to V) in 36 healthy children from 2.5 to 7 months of age. The results dismissed Halverson's hypothesis, since it was shown that from an early age the index finger played a leading role in grasping, usually contacting and initiating the grasp prior to the other fingers and producing a stronger grip force.

La préhension infantile précoce implique les doigts radiaux

Scion ľhypothèse ďHalverson, la préhension se développe progressivement des doigts cubitaux aux premiers doigts. Cette hypothèse a étéévaluée en mesurant la force ďagrippement (doigts II à V) chez 36 enfants en bonne santéâgés de 2.5 à 7 mois. Les: résultats ont invalidéľhypothèse ďHalverson, puisqu'il a pu être montré que ľindex joue un rôle prépondérant dans la préhension dès un âge précoce, assurant habituellement le contact et ľinitiation de la préhension avant même les autres doigts, et assurant une force ďagrippement plus élevée.

Frühkindliches Greifen involviert die radialen Finger

Halvcrson's Hypothese besagt, daß sich das Greifen schrittweise vön ulnar zum Zeigefinger hin entwickelt. Diese Hypothese wurde getestet, indem die Kraft beim Greifen für jeden einzelnen Finger (Finger II bis V) bei 36 gesunden Kindern im Alter zwischen 2.5 und 7 Monaten gemessen wurde. Die Ergebnisse widerlegten Halverson's Hypothese, da gezeigt wurde, daß vom frühcn Kindcsalter an der Zeigefinger beim Greifen cine führende Rolle spiclt und im allgemeinen vor den anderen Fingern Kontakt hat, das Greifen initiiert und eine stärkcre Kraft beim Greifen entwickelt.

El ‘grasping’ precoz del lactante incluye los dedos radiates

La hipótesis de Halverson cstablcce que la prensión se desarrolla gradualmente dc la zona cubital al extremo de los dedos. Esta hipótesis fue examinada midiendo la fuerza de agarre de cada dedo por separado (del índice al meñique) en 36 niños sanos de 2.5 a 7 meses de edad. Los resultados rechazan la hipótesis de Halverson, ya que mostró que ya en una cdad precoz el dedo índice jugaba un papel director en el ‘grasping’, habitualmente contactando e iniciando el agarre antes que los otros dedos y produciendo una fuerza dc agarre más fuerte.
ATAXIA‐TELANGIECTASIA: SOMATOSENSORY,‘BRAINSTEM AUDITORY. AND MOTOR EVOKED POTENTIALS IN SIX PATIENTS
Authors: Scarpini C. , Mondelli M. , Guazzi G. C. , Federico A.
Published: July 2010

Six patients with ataxia‐telangiectasia were given neurophysiological examinations. The patients had progressive sensorimotor axonal neuropathy that had begun at about age eight years. Brainstem auditory evoked potentials (BAEPs) showed central alterations in two of four cases. Somatosensory (BAEPs) and motor evoked potentials (MEPs) were altered in four of five cases. In the advanced stage of the disease the neurophysiological findings, except BAEPs and MEPs, resembled those in Friedreich's ataxia. MEP and BAEP results may therefore be useful for differential diagnosis.

Ataxie‐télangiectasie: polentiels évoqués somatosensoriels, auditifs du tronc cérébral et moteurs chez six patients

Des examens neurophysiologiques ont été pratiqués chez six patients avec ataxie‐télangiectasie. Les patients présentaient une neuropathie axonale sensorimotrice, ayant commencé vers l'âge de huit ans. Les potentiels évoqués auditifs du tronc cérébral (BAEPs) révélaient une atteinte centrale dans deux de quatre cas. Les potentiels évoqués somatosensoriels (BAEPs) et moteurs (MEPs) étaient altérés dans quatre de cinq cas. A un stade avancé de l'affection, les données neurophysiologiques, exceptés les BAEPs et les MEPs, resemblaient à celles d'une ataxie de Friedreich. Les résultats MEP et BAEP peuvent ainsi être utiles pour un diagnostic différentiel.

Ataxia‐Telangiectasia: somatosensorisch‐, akiistisch‐ und inotorisch evozierte Potentiate bei seclis Patienten

Sechs Patienten mit Ataxia‐Telangiectasia wurden neurophysiologisch untersucht. Die Patienten hatten eine progressive sensomotorische axonale Neuropathie, die im Alter von etwa acht Jahrcn begann. Bei zwei von vier Fällen zeigten die akustisch evozierten Hirnstammpotentiale (BAEPs) zentrale Veränderungen. Die somatosensorisch (BAEPs) und motorisch evozierten Potentiale (MEPs) waren in vier von fünf Fällen verändert. Im fortgeschrittenen Stadium der Erkrankung ähnelten die neurophysiologischen Befunde–außer BAEPs und MEPs–denen bei der Friedreich schen Ataxic MEP und BAEP Ergebnisse können daher für die Differentialdiagnose von Bedeutung sein.

Ataxia‐telangiectasia: potenciales evocados somato‐sensoriales, audilivos del tronco cerebral motores, en seis pacientes

Se exploraron neurofisiológicamente seis pacientes con ataxia telangiectasia. Los paciente tenían una neuropatía axonal sensorio‐motora progresiva que se inició hacia los ocho años de edad. Los potenciales evocados auditivos del tronco cerebral (BAEP) mostraron alteracioncs centrales en dos de los cuatro casos. Los potenciales evocados somatosensoriales (BAEP) y los motores (MEP) estaban alterados en cuatro de los seis casos. En la fase avanzada de la ehfermedad, los hallazgos neurofisiológicos, excepto los BAEP y los MEP, se asemejaban a los de la ataxia de Friedreich. Asi pues, los MEP y los BAEP peuden ser útiles para un diagnóstico diferencial.
Arthrogryposis Multiplex Congenita, AD 1156
Authors: Gordon Eleanora C.
Published: July 2010

A case of arthrogryposis multiplex congenita in an eight‐year‐old girl was recounted by Thomas of Monmouth in a mid twelfth‐century English hagiographic narrative. The Life and Miracles of St William of Norwich. The child had deformities of both hands and both feet at birth, and she developed torticollis and probably had some degree of hypotonia. She needed total care, her family took her to the tomb of St William in the cathedral at Norwich. This visit produced some sort of improvement in her health. Her parents, seeking a miracle, were satisfied that one had occurred.

Arthrogrypose multiple congenitale en 1156

Un cas d'arthrogrypose multiple congénitale chez une fillelte de huit ans est rapporté par Thomas of Monmouth dans une hagiographie narrative anglaise du milieu du XIIème siècle, “La vie et les Miracles de Saint Guillaume de Norwich”. L'enfant présentait à la naissance des déformations des deux pieds et des deux mains, et elle présents par la suite un torticolis et probablement quelques degrés d'hypotonie. Elle demandait une lourde prise en charge. Sa familie la conduisit à la tombe de Saint Guillaume à la cathédrale de Norwich. La visite obtint quelques degrés dans l'amélioration de la santé. Ses parents, cherchant le miracle, estimèrent qu'il y en avait eu un.

Arthrogryposis Multiplex Congenita, AD 1156

Thomas von Monmouth hat Mitte des 12.Jahrhunderts in einer Englischen Heiligenlegende, das Leben und die Wunder des Sankt William von Norwich, einen Fall von Arthrogryposis multiplex congenita bei einem achtjährigen Mädchen beschrieben. Das Kind hatte bei Geburt

Deformierungen beider Hände und Füße und entwickelte einen Torticollis und hatte wahrscheinlich eine Hypotonie. Es war ein Pflegefall. Seine Familie brachte es zu dem Grab von Sankt William in der Kathedrale von Norwich. Dieser Besuch brachte eine gewisse Besserung des

Gesundheitszustandes. Seine Eltern, die auf ein Wunder hofften, waren befriedigt, daß ein solches geschah.

Artrogriposis multiple congenita, AD 1156

Una caso de artrogriposis múltiple congénita en una niña de ocho años de edad fue aportada por Thomas de Monmouth a mediados del siglo doce en la narrativa hagiográfica inglesa “Vida y Milagros de San Guillermo de Norwich”. El niño tenia deformidades de manos y pies al nacer y desarrolló una tortícolis y probablemente tenia cierto grado de hipotonia. Necesitaba cuidados totales. Su familia la llevó a la tumba de San Guillermo en la catedral de Norwich. La visita produjo cierta mejoría en su salud. Sus padres, que buscaban un milagro, quedaron satisfechos del que habia tenido lugar.
Oxygen cost, walking speed, and perceived exertion in children with cerebral palsy when walking with anterior and posterior walkers
Authors: Mattsson Eva , Andersson Christina
Published: July 2010

Walkers with the frame positioned behind the child have been advocated recently, mainly because they may allow a more upright and therefore more normal ambulation and perhaps encourage favourable neuromuscular development. The purpose of this study was to estimate and compare speed, energy cost, and perceived exertion during walking with an anterior walker and a posterior walker. Ten children with spastic diplegia, average age 11 years, who were familiar with both types of walker participated in the study. Spasticity was measured according to the modified Ashworth scale. Oxygen cost was determined by the argon‐diluted method using a mixing box mounted on a backpack, and the perceived exertion was graded. The results of the study showed that there are no differences in the measured variables in walking between the anterior and the posterior walker in children familiar with both walkers and also that most of the children preferred the posterior walker.
Determinants of nocturnal enuresis in England and Scotland in the'90s
Authors: Rona R J. , Li L. , Chinn S.
Published: July 2010

The aim of this study was to assess whether changes have occurred in the determinants of nocturnal enuresis in Scotland and England in comparison with previous studies. The study was based on 22 study areas from a representative English sample, 14 areas from a representative Scottish sample, and 20 areas from an English inner‐city sample. A total of 14 674 subjects was included in the analysis from 16 835 eligible children in the age range 5 to 11 years. For the main analysis, an enuretic child was one who wet the bed at least once a week. As expected, the frequency of enuresis was higher in boys and decreased markedly with age in both sexes. Bedwetting was more frequent in: Afro‐Caribbean children compared with white children in the representative samples (OR 1.72 95% CI 1.22 to 2.42); those whose mothers smoked at least 10 cigarettes at home compared with non‐smokers (OR 1.58 95% CI 1.26 to 1.98); children who had disturbed sleep compared with those who slept well (OR 1.96 95% CI 1.53 to 2.51); those with mothers aged less than 20 years at the child's birth compared with mothers in the age range 25 to 34 (OR 1.63 95% CI 1.20 to 2.22); and in the second‐ or third‐born in the family in comparison with the first‐born (OR 1.42 95% CI 1.17 to 1.72). Father's social class was associated with enuresis only in girls. Only 50% of the parents consulted a doctor for enuresis in their child. The percentage was even lower in Afro‐Caribbean families (33%). Enuresis continues to be a highly prevalent problem and has not decreased over the last 45 years. We confirm that environmental factors are still important in the a etiology of enuresis. It is surprising that despite the availability of effective treatment only half of parents consult a doctor about the problem.
Complex orofacial movements and the disappearance of cerebellar mutism: report of five cases
Authors: van Mourik Marijke , Catsman‐Berrevoets Coriene E. , van Dongen Hugo R. , Neville Brian GR
Published: July 2010

A syndrome of mutism and subsequent dysarthria occurs frequently in children after resection of a cerebellar tumour. The role of orofacial and speech motor control in this syndrome has not been studied systematically. We examined simple and complex orofacial movements during the mute phase and shortly after the resumption of speech in five children with mutism and subsequent dysarthria. The recovery of complex orofacial movements coincided with the disappearance of the mutism
Boys with Asperger's disorder, exceptional verbal intelligence, tics, and clumsiness
Authors: Nass Ruth , Gutman Renee
Published: July 2010

Five boys with both Asperger's disorder and Tourette syndrome, exceptional verbal intelligence, and clumsiness are reported. Each presented at early elementary school age with a prominent complaint of social difficulties with peers. History was notable for a flapping stereotypy and the neurological examination revealed motor and/or vocal tics and numerous motor soft signs. Highly specialized interests were characteristic. Language prosody and/or pragmatics was impaired. Despite exceptional verbal intelligence, the children were not, according to their teachers and parents, faring well either socially or academically. Motor difficulties, manifested psychometrically as a significant performance IQ disadvantage, interfered with school performance and social adjustment. Tics, although not noted by parents in the clinical history, compounded their social difficulties. Asperger's disorder in these highly verbal children overlaps with pervasive developmental disorder (PDD) on account of the socioemotional difficulties and stereotypies seen in both. Asperger's disorder and Tourette syndrome overlap in these children on account of the tics. Finally, Asperger's disorder and the right‐hemisphere‐based learning disorders overlap on account of the visuoperceptual and attentional deficits that can occur in both.
Ischaemic cerebral injury, intrauterine growth retardation, and placental infarction
Authors: Burke Christopher J. , Tannenberg Anthony E. , Payton Diane J.
Published: July 2010

Two hundred and twenty‐five consecutive autopsies performed on fetuses >20 weeks' gestation were reviewed, and 37 growth‐retarded stillborn fetuses without multiple congenital abnormalities or evidence of intrauterine infection were identified. Histological evidence of ischaemic cerebral injury was found in 31 of the 37 cases and placental infarction was seen in 26 of 36 placentas. Of the 31 cases with cerebral ischaemia, 24 had placental infarcts. Twenty‐six of 27 stillborn fetuses >26 weeks' gestation showed histological evidence of cerebral ischaemia. It was concluded that in the group of growth‐retarded fetuses studied, there was a high. incidence of both cerebral and placental ischaemic abnormality.
Fast‐wave periodic complexes in a mentally retarded child who later developed subacute sclerosing panencephalitis: a modification of a classic EEG by preexisting brain damage?
Authors: Narayan Sunil K. , Kuruvilla Abraham , Shanmugam J. , Radhakrishnan V V.
Published: July 2010

The EEG of a 12‐year‐old girl with stage H subacute sclerosing panencephalitis (SSPE), who had also suffered from a non‐progressive mental retardation of unknown aetiology since early childhood, revealed periodic generalised stereotyped fast wave bursts synchronous with myoclonic jerks. The background activity was nearly normal. The diagnosis of SSPE was established by raised serum and measles antibody titres, raised CSF IgO, and brain biopsy. This rare type of periodic complex has only once been described in the literature, again in a mentally retarded child who had developed SSPE. We suggest a mechanism of origin of this type of periodic complex drawn from observations in these two cases, and discuss its significance.
Absence of alpha‐sarcoglycan and novel missense mutations in the alpha‐sarcoglycan gene in a young British girl with muscular dystrophy
Authors: Quinlivan RM , Robb SA , Sewry C. , Dubowitz V. , Piccolo F. , Kaplan JC
Published: July 2010

An 11‐year‐old white female presented with progressive proximal muscle weakness and marked calf hypertrophy. Muscle biopsy showed severe dystrophy with normal expression of dystrophin. There was complete absence of the 50kDa dystrophin‐associated glycoprotein (alpha‐sarcoglycan). DNA analysis showed novel point mutations (one missense and one splicing) in the alpha‐sarcoglycan gene at chromosomal location 17q21, confirming the diagnosis of limb‐girdle muscular dystrophy type 2D (LGMD‐2D). We believe this is one of the first confirmed white cases of primary alpha‐sarcoglycanopathy identified in the UK. This case supports the assumption of a wide geographic prevalence of severe childhood onset autosomal recessive muscular dystrophy and genetic heterogeneity. In the future, with improved diagnostic accuracy it is likely that more cases demonstrating primary or secondary deficiency of alpha‐sarcoglycan will be identified. We would recommend staining for dystrophin‐associated glycoproteins (sarcoglycans) in all new cases of muscular dystrophy with normal dystrophin, and confirmation with DNA analysis where possible.
Myositis ossificans complicating severe Guillain‐Barre syndrome
Authors: Hung J CC
Published: July 2010

We report myositis ossificans occurring in a 13‐year‐old boy with severe and rapidly progressive Guillain‐Barre syndrome. This complication should be considered when severe musculoskeletal pain is experienced by such patients. Disodium etidronate may be of benefit in this condition.
Hidden dysfunction in childhood epilepsy
Authors: Beckung Eat , Urebrant Paul
Published: July 2010

Sepsory and motor function were evaluated in 84 children and adolescents with epilepsy but not additional neuroimpairments such as mental retardation or cerebral palsy. The influence of sex, age, age at onset of epilepsy, duration, seizure type and frequency and medication was analysed. The Bruininks‐Oseretsky Test of Motor Proficiency and the EB (Eva Beckung) test were used. We found significant dysfunction in gross as well, as in fine motor functions. The most obvious deviation from normal was noted in gross motor function, mainly in the areas of running speed, balance, response speed and bilateral coordination, but also in upper‐limb speed, dexterity and coordination. Boys performed better than girls. Motor function scores improved with age but sensory and motor problems persisted in the teenage years. Early onset and a long duration of the epilepsy were associated with significantly more sensory and motor difficulties. The children on monotherapy performed significantly better than the children on polytherapy and, surprisingly, also better than those without antiepileptic medication. Sensory and motor function should be carefully investigated in the context of a comprehensive rehabilitation programme for children.with epilepsy.
Febrile convulsions in selected large families: a single‐major‐locus mode of inheritance?
Authors: Maker John , McLachlan Richard S.
Published: July 2010

As part of a genetic linkage study of febrile convulsions, families with at least four members with the disorder were identified. By applying known principles of human inheritance, possible modes of transmission of febrile convulsions and factors which might impede progress in understanding their inheritance were analyzed. In eight families, 75 of 333 members had febrile convulsions. Inheritance of the disorder occurred in 43,51, and 46% of children, siblings and all first‐degree relatives of probands respectively. Vertical transmission occurred through three to four generations, including from male to male. In these families with multiple affected members, the findings support an autosomal dominant mode of inheritance of febrile convulsions.
Visual evoked potentials: a diagnostic test for migraine headache in children
Authors: Lahat Eli , Nadir Erez , Barr Joseph , Eshel Gideon , Aladjem Mordecliai , Biatrilze Tzeti
Published: July 2010

Visual evoked potentials (VEPs) were performed in a blind fashion in 114 children aged 3 to 17 years with different types of headaches, in order to investigate whether this could be helpful in the diagnosis of migraine versus other types of headaches in children. The study showed that the amplitude between P100 and N2 was significantly larger in children with migraine headaches (mean 19.8 nvol, SD 7.75) compared with other types of headaches (mean 13.1 nvol, SD 7.45). These results suggest that the sensitivity of VEP P100 amplitude to distinguish migraine headaches in children is 67%, the specificity of VEP is 83%, and the predictive positive value is 83%. VEP study might be helpful in workup of a child with headache, particularly a young child, when signs and symptoms may not be characteristic.
Measuring anteversion and femoral neck‐shaft angle in cerebral palsy
Authors: Miller Freeman , Liang Youhui , Merlo Marco , Harcke H Theodore
Published: July 2010

Femoral anteversion was measured by computed tomography (CT) in 40 children with cerebral palsy, however an accurate measurement was possible in only 59 of the 80 hips. Flat‐surface measurement of anteversion was possible in 72 of 80 hips by CT, and in 36 of 40 hips by ultrasound. A high neck‐shaft angle correlated with an inability to measure anteversion by CT. Measuring by ultrasound allowed subjects to be positioned in internal rotation and in this way accurate measurements of neck‐shaft angles were obtained.
The changing prevalence of autistic disorder in a Welsh health district
Authors: Webb EVJ , Lobo S. , Hercas A. , Scourfield J. , Fraser WI
Published: July 2010

A prevalence study of autistic disorder(AD) in childhood was undertaken in a Welsh health district as part of a needs' assessment exercise. The study population were all children resident in the district with dates of birth between 1977 and 1988. Children were identified after a trawl of service providers in health and education and diagnosis was confirmed at a multidisciplinary assessment. An increase in prevalence was found, an observation that has been made in other parts of the world. The increase in prevalence, whether real or administratively apparent, has major implications for service providers in health, education, and social care, in that a larger number of children are being recognised as having a triad of impairments which requires very specific interventions and support from all three agencies.
Long‐term grating acuity and visual‐field development in preterm children who experienced bronchopulmonary dysplasia
Authors: Harvey Erin M. , Dobson Velma , Luna Beatriz
Published: July 2010

Grating acuity, visual‐field extent, and recognition acuity were examined in 77 children who had bronchopulmonary dysplasia (BPD), 101 children who had hyaline membrane disease (HMD), and 77 healthy preterm (HPT) children. None of the subjects had significant retinopathy of prematurity (ROP) or neurological abnormalities. Grating acuity and field extent were tested at 0 to 1,4,9,12,17,24, 30,36, and 48 months corrected age and recognition acuity was tested at 36 and 48 months corrected age. Ophthalmological examinations indicated that a greater proportion of subjects in the BPD group than in the HMD or in the HPT group had strabismus or high refractive error. However, grating acuity and field extent in the BPD group did not significantly differ from the HMD group or from the HPT group at any test age. Recognition acuity was poorer in the BPD and HMD groups than in the HPT group at both 36 and 48 months, but the difference was significant only at 36 months. These results suggest that children who experienced BPD with no significant ROP and no neurological complications show grating acuity and visual‐field development comparable to that of healthy preterm children, but that recognition acuity, or the skills required to perform a recognition‐acuity task, may be delayed in this BPD population.
Could L‐carnitine be an acute energy inducer in catabolic conditions?
Authors: Keskin S. , Seren A. , Mert M. , Akalp F. , Yardakul F. , Candan G.
Published: July 2010

Serum free carnitine levels in five children (aged between 2.5 months and 4 years) with the findings of septic shock without disseminated intravascular coagulopathy and seven children (aged between 1,5 and 6.5 years) with the first attack of idiopathic status epilepticus were compared with those of eight healthy children (aged between 2.5 months and 5 years). Serum free carnitine levels showed a statistically significant decrease in the sepsis (mean 51.5±19mg/L) and status epilepticus groups (mean 4.1±12.4mg/L) (P=0.006 and P=0.001, respectively)when compared with the controls (mean 90.8±17.2mg/L).
Gait analysis by measuring ground reaction forces in children: changes to an adaptive gait pattern between the ages of one and five years
Authors: Preis Sabine , Klemms Anja , Müller Kristiina
Published: July 2010

The aim of this study was to look at the maturational profile of gait parameters by measuring ground reaction forces during independent walking in children. Fifty‐four normal children aged 1 to 5 years were examined. The children walked with eight force transducers under each sole. Gait velocity and step length increased with age, whereas step frequency remained relatively constant. Phases of double ground contact expressed as percentages of the total gait cycle decreased significantly from age 1 to 5 with the steepest decrease occuring in the first year of independent walking. No asymmetry between left and right could be detected for any of these parameters. The pattern of ground reaction forces with a significant heel strike and obvious enrollment process resembling that in adults was achieved between the age of 2 and 3 years. Measuring ground reaction forces is a fast and easily manageable method of analysing gait pattern in children and is also a promising tool for detection of gait abnormalities in children with neurological disease.
Energy consumption in children with myelomeningocele: a comparison between reciprocating gait orthosis and hip–knee–ankle–foot orthosis ambulators
Authors: Cuddeford Tyler J. , Freeling Renée P. , Thomas Susan Sienko , Aiona Michael D. , Rex Dorothea , Sirolli Henry , Elliott Joanne , Magnusson Mark
Published: July 2010

This study compared the differences in energy efficiency (energy cost) in children with myelomeningocele ambulating with either reciprocating gait orthoses (RGOs) or hip‐knee‐ankle‐foot orthoses (HKAFOs). There were 15 children who ambulated with RGOs and 11 children braced and ambulating in HKAFOs. Velocity was measured in m/s, energy consumption was measured in mL/kg/min, and energy cost (energy consumption/velocity) was measured in mL/kg/m. Children in HKAFOs had a significantly higher energy consumption rate than children in RGOs. However, children who swing through in a HKAFO have a significantly faster velocity than children who ambulate with the RGO using a reciprocating pattern. The increased energy cost in the RGO group is influenced by their slower velocity, just as the decreased energy cost in the HKAFO group is influenced by their increased velocity. Therefore it appears that children in HKAFOs are more energy efficient than children in RGOs.
A preliminary evaluation of ankle orthoses in the management of children with cerebral palsy
Authors: Hninsworth F. , Harrison M J. , Sheldon TA , Rousnounis S H.
Published: July 2010

The effectiveness of ankle‐foot orthoses (AFOs) on walking pattern was studied in 12 children with cerebral palsy between the ages of 3 and 7 years. Over a 2‐year period two trials of fortnightly periods without AFOs were carried out. The range of ankle dorsiflexion, video analysis looking specifically at footfall, and rank scoring of the mediolateral shear force obtained using an oscilloscope printout from a Kistler force platform were compared with measurements obtained during periods when the child was wearing AFOs. The results using different outcomes were consistent, and indicated that the range of movement and gait deteriorated during the two trial periods during which the splints were not worn compared with periods during which the splints were worn. This finding needs to be confirmed in a large randomised controlled trial.
Resolution of subependymal cysts in neonatal holocarboxylase synthetase deficiency
Authors: Squires Liza , Betz Bradford , Umfleet Jason , Kelley Richard
Published: July 2010

Holocarboxylase synthetase deficiency is typically a biotin responsive disorder that presents with lactic acidosis, tachypnea, temperature instability, and shock in neonates (Briones et al. 1989 and Fuchshuber et al. 1992). The primary defect in cases studied to date appears to be the decreased affinity of HCS for its substrate, biotin (Gompertz et al. 1971). Supplemental biotin can provide sufficient substrate to increase HCS enzymatic function and thereby permit biotinylation of the four carboxylase apoenzymes (Briones et al. 1989). We report an infant with HCS deficiency who presented with lactic acidosis, shock, and hypertonia. Subependymal cysts were identified on cranial ultrasound and subsequently confirmed by MRI. Six months following biotin supplementation, she is developmentally normal and MRI of the brain shows complete resolution of the cysts.
A hypothesis for the aetiology of spastic cerebral palsy ‐ the vanishing twin
Authors: Pharoah P O D. , Cooke R W I.
Published: July 2010

The aetiology of spastic cerebral palsy (CP), in the majority of cases, is not known but the general consensus is that cerebral impairment occurs prepartum. In monochorionic twin pregnancies, death of one twin late in gestation is recognised as being an important risk factor for the surviving cotwin to have CP. It has been suggested that a significant proportion of singletons with spastic CP may be the result of death of a cotwin in the second half of gestation. In this paper it is hypothesised that spastic CP of unknown aetiology is the result of the death of a monochorionic cotwin and that the death of the cotwin may impair the neurological development of the survivor throughout gestation. If so, vanishing‐twin syndrome, which is now a recognised phenomenon revealed by ultrasound examination in early pregnancy, is important in the aetiology of spastic CP.
Possible effects of tetrahydrobiopterin treatment in six children with autism ‐ clinical and positron emission tomography data: a pilot study
Authors: Fernell Elisabeth , Watanabe Yasuyoshi , Adolfsson Ingrid , Tani Yoshihiro , Bergström Mats , PhD Per Hartvig , MD Anders Lilja , PhD. Anne‐Liis von Knorring MD. , PhD. Christopher Gillberg MD. , PhD. Bengt Lángström
Published: July 2010

Six children, between 3 and 5 years of age, having infantile autism according to DSM‐III‐R, were treated for 3 months with 6R‐L‐erythro‐5,6,7,8‐tetrahydrobiopterin (R‐BH4), a cofactor for tyrosine hydroxylases in the biosynthetic pathway of catecholamines and serotonin. A criterion for inclusion in the study was a relatively low level of R‐BH4 in the cerebrospinal fluid. For clinical evaluation, the Parental Satisfaction Survey (PASS) was used every fourth week and the Griffiths Developmental Scales were used before starting and 3 months after completing the treatment. During the treatment period, all parents reported improvements in the child's social functioning ‐ mainly eye contact and desire to interact ‐ and in the number of words or sounds which the child used. Small positive changes were noted on the Griffiths Developmental Scales between the two testing occasions. R‐BH4 levels in CSF increased significantly after treatment. The positron emission tomography (PET) study showed that the high value of dopamine D2 receptor binding in the caudate and putamen decreased by about 10% towards the normal level after treatment with R‐BH4. The observations in this open study indicate that the drug might be useful for a subgroup of children with autism, but there is a need for a larger double‐blind study with a longer treatment period.
Cerebral palsy ‐the transition from youth to adulthood
Authors: Stevenson Clarissa J. , Pharoah Peter O D. , Stevenson Richard
Published: July 2010

Service provision for those with cerebral palsy (CP) becomes fragmented after adolescence. Young people with CP in two age groups, their carers and associates, were interviewed, and use of health and social services was investigated. Leisure activities were also compared with a matched control group of able‐bodied young people. Use of health and social services decreased when the children with CP left school, thereby increasing the demands upon carers. Carers expressed anxieties about the provision of services, and frustration in obtaining information about help. Findings from the questionnaires showed the young people with CP in both age groups to be socially less active than the controls. Among the subjects, the older age group was socially less active than the younger age group. Communication is poor both between the agencies providing care and between the agencies and the young person or carer. There is a need to identify those with responsibility for coordinated‐care planning for adolescents with CP.