bluuebunny/crossref_metadata_2025 huggingface dataset:
CONVULSIVE STATUS EPILEPTICUS IN CHILDREN TREATED FOR EPILEPSY: AN ASSESSMENT OF MANAGEMENT
Authors: Matthes J. W. A. , Wallace S. J.
Published: July 2010

The acute care and further management plans accorded to eight children, all already on treatment for epilepsy, admitted to hospital with a total of 17 episodes of convulsive status epilepticus were examined. Emergency treatment was appropriate, but longer‐term management tended to be less than adequate. The causes of status epilepticus were not considered before discharge on 10 occasions. Advice on simple preventative measures was rarely given to the parents. Junior staff require further training in these areas.

Etat de mal épileptique chez l'enfant suivi pour épilepsie: évaluation de la prise en charge

Le traitement aigu et la prise en charge ultérieure portés à huit enfants, tous déjà traités pour épilepsie, admis à l'hôpital pour un total de 17 épisodes d'état de mal, ont été analysés. Le traitement d'urgence fut approprié mais la prise charge ultérieure fut loin d'étre adéquate. La cause de l'état de mal ne fut même pas recherchée avant la sortie pour 10 épisodes. Des simples conseils pour la prévention furent rarement données aux parents. Les services non spécialises devraient être mieux formés dans le domaine.

Status epilepticus bei Kindern unter Epilepsietherapie: Beurteilung der Behandlung

Es wurden Notfallversorgung und weitere Behandlung von acht Kindern untersucht, die alle schon wegen einer Epilepsie behandelt wurden und die wegen insgesamt 17 Episoden eines Status epilepticus im Krankenhaus aufgenommen wurden. Die Notfallversorgung war in Ordnung, aber die langfristige Betreuung war eher unzureichend. Bei 10 Fällen wurde über die Ursache des Status epilepticus erst bei der Entlassung nachgedacht. Die Eltern bekamen selten Ratschläge für einfache vorbeugende Maßnahmen. Die jungen Assistenten müssen in diesem Bereich besser ausgebildet werden.

Estado epiléptico convulsivo en niños tratados por epilepsia: evaluateón de su manejo

Se examinaron el cuidad en fase aguda y los planes de manejo posterior aplicados a ocho niños, todos con tratamiento antiepiléptico en curso, ingresados en el hospital con un total de 17 episodios de estado epiléptico convulsivo. El tratamiento de urgcncia era el apropiado, pero el manejo a largo plazo tendia a ser insuficiente. Las causas del estado epiléptico no fueron consideradas antes de ser dados de alta en 10 casos. Raramente se dieron a los padres consejos sobre medidas preventivas. El personal más joven necesita un entrenamiento adecuado en este campo.
CHANGES IN WALKING PATTERN BETWEEN FIVE AND SIX YEARS OF AGE
Authors: Pellico L. Gómez , Torres R. Rodriguez , Mora C. Dankloff
Published: July 2010

A study was carried out of the parameters of the ground reaction forces and temporal and spatial walking parameters of 62 children aged between five and six years. The children were evaluated barefoot, walking freely at normal speed over the surface of two platforms. The aims were to establish normal parameters for children of this age and to identify any differences existing between these ages. Differences were noticeable in the kinetic and temporal and spatial walking parameters, indicating that maturation has not yet been completed at these ages.

Modifications dans les coniposanies dc tnarche enire les âges dc cinq el six ans

Les auteurs ont entrepris une étude sur les parametres temporaux et spatiaux de 62 enfants âges de cinq à six ans. Les enfants étaicnt examinés nus‐pieds. marchant librement à vitesse normale sur la surface de deux plate‐formes. Le but de l'étude était d'etablir les paramétres normaux pour les enfants de ce âge el identifier toute différence existant entre les deux âges. De différences notables furent relevées dans les paramétres de marche. einétiques, temporaux et spatiaux, indiquant que le maturation n'est pas compléte a ces âges.

Veräanderungen des Gangnntsiers im Alter zwischen fünf und sechs Jahren

Bei 62 Kindern im Alter zwischen fünf und sechs Jahren wurden Parameter der Ground Reaction Forces sowie zeitliche und räumliche Parameter des Gehens untersucht. Die Kinder wurden beurteilt während sie barfuß, frei mit normaler Geschwindigkeit über zwei Platformen gingen. Es war das Ziel dieser Studie. normale Parameter für Kinder diesen Alters festzustellen und irgendwelche bestehenden Unterschiede in diesem Altersabschnilt hcrauszufinden. Es fanden sich Unterschiede bei den kinetischen, zeitliehen und rämliehen Parametern des Gehens, wazs darauf hinweist, daß der ReifungsprozeB in diesem Alter noch nicht abgcschlossen ist.

Cumbios en los put rones de marcha entre los cinco y sets años de edad

Se llevó a cabo un estudio de los parámetros de las fuerzas de reacción del suelo y los parámetros temporales y espaciales de la marcha en 62 niños de cinco a seis años de edad. Los niños fueron examinados descalzos andando libremente a velocidad normal sobre una superficie de dos plataformas. La finalidad era el establecer parámetros normales para niños de esta edad e identificar cualquier diferencia existente entre las dos edades. Sc observaron diferencias en los parámetros cinéticos y temporo‐espacial, lo que indica que en estas edades todavía no se ha complctado la maduración.
VERY EARLY ONSET MULTIPLE SCLEROSIS
Authors: Cole G. F. , Auchterlonie L A. , Best P. V.
Published: July 2010

The authors report two cases of multiple sclerosis occurring in very young children. Age at onset in one child was 13 months; he had the primary progressive form of the condition and died at nine years. Age at onset of the other child was two years; he has the classical relapsing‐remitting form and is well at 15 years. These children illustrate the broad clinical spectrum of the condition even in very‐early‐onset disease.

Début trés précoce de sclérose en plaque.

Les auteurs rapportent deux cas de sclérose en plaque apparus chez de trés jeuncs enfants. Lâge de début pour L'un des enfants était de 13 mois; il présentait une forme ďemblée progressive et mourut á lâge de neuf ans. ľâge de début pour ľautre enfant fut de deux ans; il présenta la forme classique avec des rémissions et rechute, et il est en bon état à 15 ans. Ces deux cas illustrent le large spectre clinique de ľaffection, même en cas de début très précoce.

Multiple Sklerose mil sehr frühem Beginn

Die Autoren beriehten über zwei Fälle, bei denen die Multiple Sklerose sehr früh auftrat. Das Alter des einen Kindes war 13 Monate; es hatte die ehronisch progrediente Form und starb mit neun Jahren. Das Alter des anderen Kindes betrug zwei Jahre; dieses hat die klassische in Schüben verlaufende form mit Remissionen und ist mit 15 Jahren wohlauf. Diese Kinder zeigen das breite klinische Spektrum der Erkrankung, selbst wenn sie sehr früh beginnt.

Esclerosois múltiple de inicio muy precoz: dos casos que ilustran el amplio espectro de la gravedad clínica

Los autores aportan dos casos de esclerosis múltiple aparecida en niños muy pequeños. La edad de inicio en uno era de 13 meses; tovo la primera forma progresiva de la enfermedad y murió a los nueve años. La edad de inicio en el otro niño fue de dos años. Sufrió la forma clásica de broteremisión y se encuentra bien a los 15 años. Estos casos ilustran el amplio espectro clínico de la enfermedad. incluyendo un inicio muy precoz de la misma.
IQ AND ITS PREDICTORS IN CHILDHOOD HEMIPLEGIA
Authors: Goodman Robert , Yude Carole
Published: July 2010

In a population‐based sample of 149 children with hemiplegia. IQ was highly correlated with a simple‐to‐calculate index of neurological severity. Performance IQ was an averâge of 13 points lower then verbal IQ ‐ a difference that seemed to reflect specific visuospatial difficulties rather than an effect of the motor disability itself. Side of lesion was not significantly related to either IQ or verbal‐performance discrepancy. Neurological factors and social class had additive effects on IQ, suggesting that neurological damâge neither amplified nor obliterated the impact of ordinary psychosocial and genetic factors on IQ.

Le QI et sa valeur predictive dans I'hemiplegie infantile

Sur un échantillon de population de 149 enfants hémiplégiques, le QI était hautement corrélé avee un index de simple calcul de la gravite neurologiquc. Le QI de performance etait en moyenne de 13 points inferieur au QI verbal, une difference qui semblait traduire des diffcultes visuo‐spatiales specifiques plutot que l'incapacitd motrice par ellc‐meme. Le cote de la lesion n'etait relie significativement, ni aux QI verbal ou performance, ni a la difference verbal‐performance. Les facteurs ncurologiqucs et la classe sociale avaient des effcts additionnels sur le QI, suggerant que le dommâge neurologique n'amplifie ni masque l'impact des facteurs psychosociaux et genetiques habitucls sur le QL

IQ und seine prognostischen Faktdren bei Kindern mil Hemiplegie

Bei einer Gruppe von 149 Kindern mit Hemiplegie bestand eine deutliche (Correlation zwischen IQ und einem emfach zu berechnenden Index für den neurologischen Schweregrad. Der Handlungs‐IQ war im Durchschnitt 13 Punkte niedriger als der verbale IQ ‐ ein Unterschied, der cher Ausdruck speziellcr visuell‐riiumlicher Storungen als der motorischen Stoning selbst war. Die Seite der Lasion spielte keine signifikante Rolle, weder für den IQ noch für die Diskrcpanz zwischen verbalem ‐ und Handlungs‐IQ. Neurologische Faktoren und sozialer Status hatten einen additiven Effeckt auf den IQ. was vcrmuten laBt, daß die neurologische Schadigung das Gewicht der allgemeinen psychosozialen und genetischen Faktoren auf den IQ weder verstarkt noch vermindert.

CI y sus predictores en la hemiplegia infantil

En una poblacion de 149 niños con hemiplegia, el CI estaba altamente correlacionado con el indice de gravedad neurologica, facilmente calculable. El CI de realizacion era de un promedio de 13 puntos por debajo del verbal. Esta diferencia parecc reflejar dificultades visuo‐espaciales espeefficas, mas que un efecto de la disfuncion motora en si. El lado de la lesi6n no estaba significativamente en relacion con el CI o con la discrepancia cntre el CI verbal y el de realizacion. Los factores neurologicos y la clase social tenian efectos aditivos sobre cl CI, lo que sugiere que el dafio neurologico ni amplificaba ni obliteraba el impacto de los factores ordinaries psicosociales y geneticos sobre el Cl.
Cortical Dysgenesis in a Patient with Turner Mosaicism
Authors: Terao Yasuo , Hashimoto Kouichi , Nukina Nobuyuki , Mannen Toru , Shinohara Tamiko
Published: July 2010

In a patient with Turner mosaicism who had mental retardation, epilepsy and cerebellar ataxia, MRI showed cerebellar atrophy and a bizarre cortical dysgenesis of the cerebrum, which was considered to comprise a mixture of relatively normal gyri and structures resembling pachygyria and Iissencephaly. The karyotype of the patient was 45.X/47.XXX, but the brain dysgenesis could not be explained solely on the basis of this mosaicism, which is rarely associated with a gross abnormality in brain pathology. Abnormality of the X chromosome seems to have some potential for inducing cortical dysgenesis, and this case may be partially attributable to an abnormal locus on the X chromosome.

Dysgénésie corticale chez un sujei avec un Turner mosaïque

Chez un patient avec Turner mosaïque présentant un retard mental, une épilepsie et une ataxie cérébelleuse, l'IRM révéla une atrophie cérébelleuse et une dysgénésie corticale bizarre du cerveau, qui fut considérée comme un mélange d'un gyrus relativement normal et de structures ressemblant à une pachygyrie et une lissencéphalie. Le caryotype du patient était 45,X/47,XXX, mais la dysgénésie cérébrale ne pouvait pas étre expliquée seulement par la mosaïque, rarement associée à une anomalie sévère dans la pathologie cérébrale. L'anomalie du chromosome X semblc présenter un potentiel d'induction de la dysgénésie corticale, et ce cas peut étre partiellement attribuable à un locus anormal du chromosome X.

Kortikale Dysgenesie bei einem Patienten mil Turner Mosaik

Bei einem Patienten mit Turner Mosaik mit geistiger Retardierung, Epilepsie und cerebellärer Ataxie fand sich im MRI eine cerebelläre Atrophie und cine seltsame kortikale Dysgenesie des Gehirns, was als eine Mischung aus relativ normalen Gyri und Strukturen wie bei der Pachgyrie und Lissenzephalie angesehen wurde. Der Karyotyp des Patienten war 45, X / 47, XXX, aber die Dysgenesie ließ sich nicht nur auf der Basis dieses Mosaiks erklären, da bei diesem selten auffällige Anomalien in der Hirnpathologie auftreten. Durch eine Anomalie des X Chromosoms könnte eine kortikale Dysgenesie hervorgerufen werden, und dieser Fall könnte einem abnormen Locus auf dem X Chromosom zugeschrieben werden.

Dysgenesia conical en un paciente con mosaicismo de Turner

En un caso de mosaicismo de Turner con retraso mental, epilepsia y ataxia cerebclosa. la IRM mostró una atrofia cercbelosa y una disgenesia cortical rara que comprendía una mezcla de circulvoluciones relativamerite normales y estructural que semejaban paquigiria y lisencefalia. El cariotipo del caso era 45.X/47.XXX, pero la disgenesia cerebral no podía ser expresada nicamente sobre la base del mosaicismo, que raramente va asociado a anomalias groseras del cerebro. La anomalia del cromosoma X parece tener una capacidad para inducir una disgenesia cortical y este caso puede ser parcialmente atribuible a un locus anomalo en el cromosoma X.
Neuropsychological, intellectual, and behavioral findings in patients with centrotemporal spikes with and without seizures
Authors: Weglage Josef , Demsky Alike , Pietsch Michael , Kurlemann Gerd
Published: July 2010

Forty children (23 boys, 17 girls) with centrotemporal spikes (rolandic focus) with and without seizures (mean age 8.4 years ±4.8 SD), and 40 healthy controls matched for age, sex, and socioeconomic status were assessed for their neuropsychological, intellectual, and behavioral outcome. Compared with the controls, patients were significantly impaired in their IQ, visual perception, short‐term memory, in their psychiatric status and in some subtests in a fine motor performance task. No significant differences could be computed for a simple finger‐motor speed exercise or a linguistic performance test. In patients, deficits in IQ were significantly correlated with frequency of spikes in the EEG, but not with frequency of seizures, lateralization of the rolandic focus, or time since rolandic focus was diagnosed. It was concluded that a rolandic focus is not as benign as once thought.
Status epilepticus in children: aetiology, treatment, and outcome
Authors: Eriksson KJ , Koivikko MJ
Published: July 2010

This retrospective study includes 65 children treated for status epilepticus at Tampere University Hospital in Finland. Aetiology of the condition, effectiveness of the treatment protocol, including short barbiturate anaesthesia to prevent prolonged status epilepticus episodes, and neurological outcome were evaluated. Symptomatic aetiology was present in 40% of status epilepticus episodes, and 37% of episodes were induced by fever. Neurological sequelae secondary to status epilepticus were identified in 15% of the cases and subsequent epilepsy in 23% during the mean follow‐up time of 3.6 years. There were no status epilepticus‐related deaths. The cut‐off point of status epilepticus duration for significant risk for permanent neurological sequelae was 2 hours. Our treatment protocol, including short barbiturate anaesthesia in refractory cases, was able to abort status epilepticus in less than 2 hours in 75% of cases. We conclude that early and prompt use of barbiturate anaesthesia should be encouraged, and may explain our low morbidity figures.
A comparison of intensive neurodevelopmental therapy plus casting and a regular occupational therapy program for children with cerebral palsy
Authors: Law Mary , Russell Dianne , Pollock Nancy , Rosenbaum Peter , Walter Stephen , King Gillian
Published: July 2010

The purpose of this research was to evaluate the combined effect of intensive neurodevelopmental therapy (NDT) and casting in improving hand function, quality of upper‐extremity movement and range of motion in children aged between 18 months and 4 years with cerebral palsy (CP).A randomized crossover design was used to evaluate the difference between intensive NDT plus casting and a less. intensive regular occupational therapy (OT) program. Blinded assessments of hand function, quality of upper‐extremity movement, and parents' perception of hand‐function performance were carried out at baseline, 4 months (end of first intervention period), 6 months (after a 2‐month ‘washout’ period), and 10 months (end of second intervention period). Analysis of the outcomes revealed no significant differences in hand function, quality of upper‐extremity movement, or parents' perception of hand‐function performance between the two treatment groups ‐ intensive NDT plus casting or regular OT programs. There does not appear to be any beneficial effect of an increased amount of therapy for the children in this study.
Correlates of maladaptive behavior in individuals with 5p‐(cri du chat) syndrome
Authors: Clarke David J. , Dykens Elisabeth M.
Published: July 2010

This study examined the range, distinctiveness, and correlates of maladaptive behavior in 146 subjects with 5p‐ (cri du chat) syndrome using the Aberrant Behavior Checklist as a standardized measure. Hyperactivity was the most significant and frequent problem in the sample. Subjects with 5p‐syndrome also showed aggression, tantrums, self‐injurious behavior, and stereotypies; some of these problems were more pronounced in individuals with lower cognitive‐adaptive levels, as well as in those with histories of previous medication trials. Autistic‐like features and social withdrawal were more characteristic of individuals with translocations as opposed to deletions, even when controlling for the lower adaptive level of the translocation group. These findings encourage further research on the behavior of individuals with 5p‐syndrome.
Reported biomedical causes and associated medical conditions for mental retardation among 10‐year‐old children, metropolitan Atlanta, 1985 to 1987
Authors: Yeargin‐Allxopp Marshal , ‘Murphy Catherine C. , Cordrro José F. , Decoufle Pierre , Hollowell Joseph G.
Published: July 2010

This report describes biomedical causes of mental retardation(MR) among school‐age children and associated medical conditions in children for whom no cause was reported. This study involved 715,10‐year‐old children with MR (intelligence quotient [IQ] 70 or less) born between 1975 and 1977. We determined biomedical causes of MR using a hierarchical approach based on the timing of the event (i.e. prenatal, perinatal, or postneonatal). Among, children with no identified biomedical cause the occurrence of associated medical conditions was examined. No reported biomedical cause could be found in 78% of children with MR (87% mild, IQ 50 to 70; 57% severe, IQ<50). Prenatal causes were present in 12%, perinatal causes in 6%, and postneonatal causes in 4%. On the basis of these findings it was concluded that intensive use of public health prevention strategies can reduce the number of children who receive a diagnosis of MR.
A novel neurological disorder with progressive CNS calcification, deafness/ renal tubular acidosis, and microcytic anemia
Authors: Yoshimara Masaki , Hara Toshiro , Maegaki Yoshihiro , Koeda Talshyn , Okubo Kenshi , Humasaki Naotaka , Sly William S. , Takeshila Kenzo
Published: July 2010

Progressive calcification of the brain and the spinal cord at early infantile onset was observed in two siblings. They showed growth failure, psychomotor deterioration, deafness, vestibular dysfunction, microcytic hypochromic anemia, abnormal ratios of lymphocyte subpopulations, and slightly decreased bicarbonate on blood gas analysis. Distal renal tubular acidosis was demonstrated in one of them. Carbonic anhydrase II activity was normal. This new hereditary disease might have a defect in a molecule that is present in brain, spinal cord, kidney and hematocytes and is involved in H+/HCO3‐ production or transport.
Laterality of finger movements in preterm infants
Authors: Konishi Yitkuo , Takaya Rieko , Kimura Kouki , Takeuchi Keiko , Saito Masukazu , Konishi Kaoru
Published: July 2010

The laterality of head position, spontaneous finger movements, hand‐face contact, and hand‐mouth contact was studied in low‐risk preterm infants of 31 to <37 weeks postconceptional age (intrauterine + neonatal period at time of study). The head was predominantly turned towards the right side during the preterm period. Hand‐mouth contact was more frequent on the right side and coincided well with the side to which the head was turned. Other hand movements, too, were more frequent on the right side than on the left, but these were also seen in the fingers contralateral to the direction in which the head was turned. These findings suggest that even before birth a spontaneous preference for head position is present and is already associated with asymmetry in coordinated finger movements.
Prevalence and type of cerebral palsy in a British ethnic community: the role of consanguinity
Authors: Sinha Gyan , Corry Peter , Subesinghe D. , Wild J. , Levene Malcolm I.
Published: July 2010

Little is known about the prevalence of cerebral palsy (CP) within ethnic subgroups born in Britain. The Yorkshire Regional Cerebral Palsy Register has ascertained all cases of CP in children born within the Regional Health Authority boundary in 1985 to 1987 inclusive and diagnosed by 5 years of age. Birth registrations recorded by ethnic subgroups allowed us to determine the prevalence of CP within the Bradford District Health Authority (BDHA) boundaries by Asian and non‐Asian ethnic subgroups. All the children with CP in BDHA were examined by one individual and careful family pedigrees recorded. We noted that BDHA had a high prevalence of CP; 3.87 to 4.16 per 1000. The prevalences in the non‐Asian and Asian populations were 3.18, and between 5.48 and 6.42, per 1000, respectively. This difference was statistically significant (P=0.03). First cousin marriages occurred in 15 of the 39 Asian famines (51.7%) and nine of these families had another first or second degree family member with a similar type of CP to the index child. There was no consanguinity in the non‐Asian families. These data highlight the increased need for services in some ethnic populations living in Britain and the likely genetic aetiology of a significant proportion of cases of CP in Asian families.
Long‐term melatonin treatment in blind children and young adults with circadian sleep‐wake disturbances
Authors: Palm Lars , PhD. Gösta Blennow M D. , PhD Lennart Wetterberg M D.
Published: July 2010

Oral melatonin therapy was used to treat severe circadian sleep‐wake disturbances in eight children and young adults in an open study. All patients were functionally blind, six of them because of defects in the anterior visual pathway. All were mentally retarded. Baseline sleep diaries kept by the caregivers before treatment showed non‐24‐hour sleep‐wake syndrome. Diurnal variations in serum and urinary melatonin were examined. Melatonin secretion peak time was delayed in seven patients. Body temperature variation was out of phase relative to sleep and melatonin in five patients, and thus they had signs of internal desynchronisation. Melatonin given in the evening dramatically improved the sleep‐wake pattern in all patients. The effect was maintained during long‐term therapy for between 1 and 6 years in six patients. One patient fell back into the earlier sleep pattern after 6 to 8 months, and another had increasing sleep disturbance because of reflux oesophagitis, although the improvement regarding the circadian component remained. No side effects have been noted during the therapy. Oral melatonin is promising as an efficient and seemingly safe alternative for treatment of severe circadian sleep disturbances.
Early cerebral proton MRS and neurodevelopmental outcome in infants with cystic leukomalacia
Authors: Groenendaal Floris , van der Grond Jeroen , Eken Paula , van Haastert Ingrid C. , Rademaker Karin J. , Toet Mona C. , de Vries Linda S.
Published: July 2010

The present study tested the hypothesis that proton magnetic resonance spectroscopy CH‐MRS) predicted neurodevelopmental outcome in infants with cystic leukomalacia (CL). Nineteen infants with CL (grade 2, N‐7; grade 3, N‐7; grade 4, N=5), graded according to the authors' classification, were examined at corrected ages of mean 1.5±2.1 SD weeks. H‐MRS of the basal ganglia and the periventricular white matter was performed. Two infants died, 16 had an adverse neurodevelopmental outcome and one was normal at follow‐up. N‐acetylaspartate (NAA)‐.choline (Cho) ratios were mean 1.120.19 (SD) (grade 2), mean 0.9510.11 (SD) (grade 8), and mean 0.7110.13 (SD) (grade 4). These differences are significant (P<0.01, ANOVA). NAA: Cho ratios showed a positive correlation with developmental quotient (DQ) at the age of 1 year (P<0.05). In 13 infants lactate (Lac) was found. Lac: NAA ratios showed a negative correlation with NAA: Cho ratios, but not with DQ. We conclude that a low NAA. Cho ratio predicted a poor outcome, whereas some infants developed unfavourably despite a normal NAA: Cho ratio. We speculate that partial volume effects might explain this observation.
Febrile seizures in a South Indian district: incidence and associations
Authors: Hackett Richard , Psych Latha Hackett MRC , MA. Preeta Bhakta
Published: July 2010

One thousand four hundred and three children participated in a home‐based survey of psychiatric disorders in 8‐ to 12‐ year‐old children in Calicut District, Kerala, India. One thousand one hundred and ninety‐two consecutive children underwent neurological and psychometric assessments. The projected number of children with a history of febrile seizures was 120 giving a lifetime incidence of 10.1%. Recurrent febrile seizures predominated and these were strongly associated with a history of perinatal adversity. Febrile seizures were independently associated with indices of infective illness and mothers' education. Epilepsy developed in 2.7% of children with febrile seizures, but no evidence was found that febrile seizures had adverse intellectual or behavioural sequelae.
Treatment of multiple sleep problems in children with developmental disabilities: faded bedtime with response cost versus bedtime scheduling
Authors: PhD. Cathleen C Piazza , PhD. Wayne W Fisher , Sherer Michelle
Published: July 2010

Sleep problems are a common concern for persons with mental retardation and severe behavior problems, yet few empirically validated treatment options exist. In the current investigation, the efficacy of a faded bedtime with response cost treatment was compared with a bedtime scheduling procedure in treating the multiple sleep problems of two groups of children with mental retardation, sleep problems, and other severe behavior problems. Faded bedtime with response cost (FBRC) consisted of systematic delay of bedtime, removal from bed if sleep was not initiated within 15 minutes (response cost), and a fading procedure to gradually advance the bedtime. The bedtime scheduling procedure consisted of a consistent sleep and wake time and prevention of daytime sleep. The sleep of children in the FBRC group improved significantly more than the sleep of children in the bedtime scheduling group. Results are discussed in terms of behavioral and biological mechanisms which may contribute to the efficacy of FBRC.
Progressive facial hemiatrophy: MRI appearances
Authors: Taylor Helena M. , Robinson Richard , Cox Timothy
Published: July 2010

The cranial CT and MRI appearances of a 14‐year‐old girl with Parry‐Romberg syndrome and epilepsy are described. The findings are compared with the two published descriptions of MRI and CT in such patients. MRI appearances in our patient differ from those published and may be consistent with a vascular malformation. The one published report on the intracranial histopathology of a patient with Parry‐Romberg syndrome and epilepsy states that a microscopic vascular malformation was found. We discuss the relationship between radiological and pathological findings. The possible aetiologies of Parry‐Romberg syndrome (vascular malformation, immunological, trauma, sympathetic innervation, hereditary, slow virus) are discussed. We suggest that Parry‐Romberg syndrome could be regarded as a neurocutaneous syndrome, a component of which includes intracerebral vascular dysplasia. In this and the other three cases of Parry‐Romberg syndrome with epilepsy, the sensitivity of MRI in detecting intracranial lesions is demonstrated. Recommendations for imaging these patients are proposed.
Computed tomography and single‐photon emission computed tomography as diagnostic tools in acquired brain injury among children and adolescents
Authors: Emanuelson Ingrid M. , von Wendt Lennart , Bjure Jan , Wiklund Lars‐Martin , Uvebrant Paul
Published: July 2010

Twenty children with acquired brain injuries were investigated with CT and SPECT. The findings were related to the clinical outcome judged at discharge following acute care after the injury and at follow‐up 5 years later. The abnormalities that were found were classified for each lobe on a scale ranging from 0 (normal) to 5 (severe abnormality). The patients were divided into two groups showing mild and severe injury. CT and SPECT revealed similar results in the patients with severe injury, but in the group of mildly injured children the number of affected lobes and scores indicated by SPECT were significantly higher than those indicated by CT. SPECT also differentiated more effectively between the two outcome groups. This supports the hypothesis that SPECT could be used as an instrument to objectivise minor sequelae and that SPECT and CT are both useful methods for forecasting outcome.
Test of two views of impulsivity in hyperactive and conduct‐disordered children
Authors: Leung Patrick W L. , Connolly Kevin J.
Published: July 2010

The nature of impulsivity in hyperactive and conduct‐disordered children was examined in two experiments, one involving a priming task, the other a delayed reaction time task. Four groups of children, aged 7 to 8 years and with IQs in the normal range, were recruited for study: (1) a pure hyperactive group (HA), (2) a hyperactive/conduct‐disordered group (HA+CD), (3) a pure conduct‐disordered group (CD), and (4) a normal control group (N). When the stimulus configuration and presentation were simple and well organized, none of the three clinical groups displayed any sign of impulsivity at the input/perceptual stage; there was no tendency to rush responding before adequate consideration of the relevant stimuli, i.e. a trading of accuracy for speed. Instead, the HA children were found to be disinhibited at the output/motor stage, i.e. failing to temporarily withhold activated responses. This deficit was found to be specific to the HA children; it was not observed in the CD and HA+CD children.
Attention deficits and autistic spectrum problems in children exposed to alcohol during gestation: a follow‐up study
Authors: Aronson Marita , Hagberg Bibbi , Gillberg Christopher
Published: July 2010

Children born to mothers who had abused alcohol throughout pregnancy had severe behavioural and intellectual problems which remained at age 11 to 14 years. Of 24 children examined, 10 had attention deficit hyperactivity disorder (ADHD) with or without developmental coordination disorder, two had Asperger syndrome, and one had an autistic‐like condition not meeting the criteria for Asperger syndrome. Six of these 24 attended special schools for the mentally retarded and a further 11 were given special education, leaving only seven attending regular schools without any type of support. The children had difficulties in mathematics, logical conclusions, visual perception, spatial relations, short‐term memory, and attention. Sixteen children lived in foster homes. There was a clear correlation between the occurrence and severity of the neuropsychiatric disorder and the degree of alcohol exposure in utero.
Adaptive and maladaptive behaviour in children with epileptic encephalopathies: correlation with cerebral glucose metabolism
Authors: Ferrie CD , Madigan C. , Tilling K. , Maisey M.N , Marsden P K. , Robinson R O.
Published: July 2010

In the childhood epileptic encephalopathies mental impairment is common and severe. Traditional cognitive assessment is difficult because of the low level of performance, autistic features, and the unpredictable effect of seizures. An alternative is to measure adaptive and maladaptive behaviour using instruments administered to the caregivers. Adults with different types of dementia have characteristic patterns of cortical glucose hypometabolism. Thirty‐two children were studied using visual and semiquantitative analysis of 18fluorodeoxyglucose positron emission tomographic (PET) scans. The Vineland Scales and the Conners' Questionnaires were used to assess adaptive and maladaptive behaviour. The mean adaptive behaviour composite score was 37.3±15.6; all but one subject had a low adaptive level. A profile of relative strength in socialisation and weakness in daily living skills emerged. Up to two‐thirds of children had abnormal behaviour patterns, particularly attention‐deficit disorders and hyperactivity. Adaptive and maladaptive behaviour was not related to the presence or absence of focal cortical PET abnormalities. However, adaptive behaviour scores showed an inverse correlation with the degree of metabolic abnormality in the frontal lobes.
Clinical characteristics and long‐term outcome of migraine with aura in children and adolescents
Authors: Balottin U. , Borgatti R. , Zamrino C A. , Lansi G.
Published: July 2010

A population of 45 young subjects, 16 males and 29 females, affected by migraine with aura were studied prospectively. The mean follow‐up was 7 years 6 months. The clinical characteristics and modifications during migraine aura attacks were evaluated. The aura propagated slowly with a pattern corresponding to a cortical pathway in the posteroanterior direction in 71% of the series, and was visual alone in 40%. Analysis of the relation between the course, and therefore prognosis, and the aura type showed that headache has a better prognosis when symptoms continue to be only visual. The International Headache Society (IHS) classification subdivides migraine with aura into six categories based on the duration of symptoms and the clinical characteristics (hemiplegic, basilar). Our results suggest that subjects with only visual aura should be considered separately, possibly as a seventh category, because they may have a more favourable prognosis.
Do neurological disorders in childhood pose a risk for mental health in young adulthood?
Authors: MD Eeva‐Riitta Kokkonen , DrMSc Jorma Kokkonen MD , DrMSc Anna‐Liisa Saukkonen MD
Published: July 2010

To assess whether juvenile‐onset epilepsy or motor disability is complicated by an increased number of mental health disorders or experience of psychosomatic symptoms in young adulthood, we studied 81 subjects with epilepsy and 52 with motor disabilities at the age of 19 to 25 years and compared them with 211 controls. The main diagnostic tool, the Present State Examination, was administered to those attending the interviews in person who were of normal intelligence; there were 62, 38, and 123 subjects in the three categories, respectively. Compared with the controls, the subjects with epilepsy showed an equal prevalence of psychiatric disorders whereas those with motor disabilities had a significantly higher prevalence, particularly of depressive disorders. The reported prevalence of psychosomatic symptoms confirmed this main result. Psychological illness affected everyday life of two out of five subjects with motor disabilities, but only half of those in the other two groups. It is concluded that motor disability since childhood, but not epilepsy, could be a factor that increases susceptibility to psychiatric morbidity, especially depression, and causes a large number of psychosomatic symptoms. The results challenge staff of clinics working with such adolescents to find individual approaches in preventing the negative influence of psychological disorders on social life.
Bilateral oedema of the basal ganglia in an echovirus type 21 infection: complete clinical and radiological normalization
Authors: MD A Freund , MO R Zass , MD G Kurlemann , MD Q Schuierer , MD K Ullrich
Published: July 2010

A 4‐year‐old girl with bilateral striatal oedema in association with an echovirus type 21 infection is reported. In the course of a prolonged upper respiratory‐tract infection, the patient developed muscular hypotonia, resting tremor, ataxia, sleepiness, hyperesthesia, and indistinct speech. T2‐weighted cranial MRI revealed bilateral oedema of the basal ganglia and the cerebellar peduncles. At follow‐up after 3 months MRI changes and clinical symptoms had fully resolved.
Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non‐Japanese cases
Authors: Campistol J. , Gassió R. , Pineda M. , Fernandez‐Alvarez E.
Published: July 2010

This report describes two Spanish cases of acute necrotizing encephalopathy of childhood, a rare disease first described in Japan by Mizuguchi and colleagues. Similar cases have been reported from Taiwan and other countries of the Far East. Two cases have been reported from the UK and one from the USA. The disease affects young children and is characterized by acute encephalopathy after a viral illness, with seizures and decreased levels of consciousness. The hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, internal capsules, upper brainstem tegmentum, and cerebral white matter. The aetiology is unknown but an infectious or parainfectious process seems likely. Because of the predominant involvement of the thalami, we propose the term ‘infantile bilateral thalamic necrosis’, a more specific term and one which distinguishes the entity from other basal ganglia diseases in childhood.
Pons tumour behind a phenotypic Rett syndrome presentation
Authors: Vanhala R. , Gaily E. , Paetau A. , Riikonen R.
Published: July 2010

We describe a girl with a brain‐stem tumour and symptoms very similar to those of Rett syndrome (RS). Her early history was uneventful and development was normal (except for hypotonia). At the age of 6 months her development slowed. Subsequently, deterioration occurred and the features characteristic of RS were seen: loss of purposeful hand use, stereotypic hand movements, impaired social contact, decelerated head growth, and ataxia. Cerebral CT at the age of 3 years and 4 months revealed a tumour in the region of the pons and hydrocephalus. We suggest that a pons/midbrain tumour appearing at an early age may affect the developing CNS and cause symptoms similar to those of RS. A possible causal connection between midbrain pathology and RS is supported by this case.
Obstetric brachial plexus injuries: assessment protocol and functional outcome at age 5 years
Authors: Sundholm Lena Krumlinde , Eliasson Ann‐Christin , Foissbeig Hans
Published: July 2010

One hundred and five children with the whole spectrum of obstetric brachial plexus (OBP) injuries, from severe to full recovery, were examined at the age of 5 years with regard to motor and sensory functions as well as to use of the affected limb. Since root involvement level does not fully reflect the degree of disability, a classification based on range of motion and grip‐strength was formulated and found to correspond well with functional abilities. The results from this study indicate that the eventual outcome in upper‐plexus lesions is more complex than is commonly believed. Hand function is affected due to the effect of limited shoulder movements on hand positioning. Grip strength was also reduced in many of these children. All the children with total‐plexus lesions had diminished grip strength and half of them had impaired tactile sensibility. In most children with total‐plexus lesions, performance of activities in daily life was affected as were bimanual activities requiring use of the involved limb. Hand preference was affected in children with a right‐sided injury. From a clinical perspective, as well as for research, it is important to describe OBP injuries not only in terms of impairment but also of disability.
Children with Apert syndrome: behavioural problems and family stress
Authors: Sarimski Klaus
Published: July 2010

Apert syndrome is characterized by severe craniosynostosis, midface hypoplasia, and symmetric syndactyly of the hands and sometimes of the feet. In this study, postal questionnaires were completed by parents of 41 children with Apert syndrome. Data from this preliminary survey revealed that the children functioned fairly well socially despite their disabilities and potential for psychological difficulties. Parents suffered from stress caused by problems over acceptance of the child'sappearance, the child'sbehavioural problems, and parental low self‐esteem. Ten mothers and eight fathers scored in the deviant range for parental stress, suggesting a need for psychological counselling.
Lower‐Extremity strength profiles in spastic cerebral palsy
Authors: Wiley Mary Elizabeth , Damiano Diane L.
Published: July 2010

Although weakness has been identified in cerebral palsy (CP) in isolated muscle groups, the magnitude of weakness in multiple muscles and the patterns of weakness across joints have not been documented. The maximum voluntary contraction of eight muscle groups in the lower extremities of 15 children with spastic diplegia, 15 with spastic hemiplegia, and 16 age‐matched peers was determined using a hand‐held dynamometer. Children with spastic diplegia were shown to be weaker than age‐matched peers in all muscles tested, as were the children with hemiplegia on the involved side, with strength differences also noted on the uninvolved side. Weakness was more pronounced distally in the groups with CP, and the hip flexors and ankle plantarflexors in spastic CP tended to be relatively stronger than their antagonists as compared with the strength ratios of the comparison group. In conclusion, children with spastic CP demonstrate quantifiable lower‐extremity weakness and muscle imbalance across joints.
Postural adjustments during sitting at preschool age: presence of a transient toddling phase
Authors: Hadders‐Algra Mijna , Brogren Eva , Forssberg Hans
Published: July 2010

The present study investigated developmental changes in postural adjustments during preschool age. Postural responses during sitting on a moveable platform were assessed in 21 healthy children aged 1% to 4% years. Multiple surface EMGstof neck, trunk, and leg muscles were recorded during forward and backward translations. Comparable data were available for 11 infants seen three times between the ages of 5 and 10 months. The data revealed the existence of a transient period between the ages of 9 to 10 months and 21/2 to 3 years, during which perturbations in a sitting position are accompanied by high activity in the direction‐specific agonist muscles and in the antagonist muscles. After this period, agonist activity became more variable, particularly so during backward translations, and antagonist activity disappeared. These changes could be attributed to biomechanical factors and to maturation of the nervous system.
Acquired brachial‐plexus neuropathy in the neonate: a rare presentation of late‐onset group‐B streptococcal osteomyelitis
Authors: Sadleir Lynette G , Connolly Mary B
Published: July 2010

Acquired brachial‐plexus neuropathy outside the immediate neonatal period is uncommon. Pseudopalsy of a limb, associated with osteomyelitis, is well recognized. Acquired brachial‐plexus neuropathy as the initial presentation of osteomyelitis of the humerus in the neonatal period is described. Three infants presented at 3, 15, and 21 days respectively, with acute monoplegia consistent with brachial‐plexus neuropathy. The infants were afebrile and generally well. Initial radiographs of the humerus were normal and blood cultures grew group‐B streptococcus in all infants. Nerve conduction studies were consistent with brachial‐plexus neuropathy. Following intravenous antibiotics, there was complete recovery in all infants. Osteomyelitis of the humerus should be considered in infants in whom there are no overt signs of sepsis and who present with brachial‐plexus neuropathy. Early diagnosis and appropriate treatment should result in a complete neurological recovery.
Childhood headaches: discrete entities or continuum?
Authors: Viswanathan V. , Bridges Sarah J , Whitehouse W. , Newton RW
Published: July 2010

The aims of this study were to investigate the signs and symptoms of recurrent headaches in children and to identify if there are any discrete groups of children whose headaches corresponded to the World Federation of Neurology (1969) definition of migraine. One‐hundred and fifty children recruited from the neurology clinics at Royal Manchester, Booth Hall, and Birmingham Children's Hospitals were interviewed to complete a standardized questionnaire. The data were examined using cluster analysis followed by comparative analysis of the headache signs and symptoms in the different groups identified. No stable groups were identified (i.e., no group was reliably identified by different methods of cluster analysis) which corresponded to the World Federation of Neurology definition of migraine. This would suggest that this definition is not appropriate for the sample investigated. Three groups of children evolved after cluster analysis. None of these groups was in agreement with the International Headache Society classification of headaches. The groups were neither ‘stable’ clusters nor ‘useful’ in predicting prognosis. This outcome supports the continuum theory of headache syndromes.
Gangliosides in cerebrospinal fluid in children with autism spectrum disorders
Authors: Nordin Viviann , Lekman Annika , Johansson Maria , Fredman Pam , Gillberg Christopher
Published: July 2010

Gangliosides are sialic acid‐containing glycolipids found in all cells, especially abundant in nerve cells and mainly situated on outer‐membrane surfaces. The aim of this study was to provide data on the concentration of gangliosides in the CSF of children and adolescents with autism spectrum disorders (ASD) ‐ 66 with autistic disorder, and 19 with other autism spectrum disorders. The comparison group consisted of 29 children and adolescents, whose CSF had been sampled to exclude acute infectious CNS disorder. The concentrations of the gangliosides GM1, GD1a, GD1b, and GT1b were determined using a microimmunoaffinity technique. The ASD group had a significantly higher concentration of ganglioside GM1 compared with the comparison group. The GM1 increase could not be explained as secondary to other clinical factors. Mean ganglioside levels did not differentiate subgroups with autistic disorder and those with a more atypical clinical picture, nor subgroups with known medical disorders and those with idiopathic autism. Altered patterns of gangliosides in the CNS might reflect important correlates of pathogenesis in autism.
Saccadic strategies in children with hemianopia
Authors: Mezey LE , Harris C M , Shawkat FS , Timms C. , Kriss A. , West P. , Taylor D S I
Published: July 2010

Multiple hypometric (undershooting) saccades are generally reported as a compensatory strategy in adults with homonymous hemianopia. However, hypermetric (overshooting) saccades have been reported to develop spontaneously as a beneficial strategy in response to predictable targets. We examined the saccades of 10 children (aged 5 to 16 years) with homonymous hemianopia to determine the type of compensatory eye‐movement strategies employed 6 months to 16 years after hemianopia onset. Homonymous hemianopia was identified using perimetry and/or pattern visual evoked potentials and supported with results of neuroimaging. Eye movements were recorded using bitemporal electrooculography. Saccades were elicited to a red light source in a semipredictable paradigm. We found that hypermetria was not a consistent compensatory strategy in our patients. In spite of the predictability of our paradigm and the long follow‐up period, multiple hypometric saccades into the blind field appeared to be the preferred strategy.