B Hagberg

Uppsala University Hospital, Uppsala, Uppsala, Sweden

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Publications (159)400.42 Total impact

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    ABSTRACT: This population-based study refers to 78 Swedish children with non-progressive ataxia from a total population of 3.1 million inhabitants. Inclusion criteria were ataxic gait without any signs of spasticity, dyssynergia, dysmetria and intention tremor. CT and/or MRI studies were available from 70 patients (90%). Infratentorial pathology was revealed in 27%. and findings were considered normal in 61%. If CT was normal, of recent date and of good quality, MRI did not add any new information. In half of the cases with pathological CT, however, MRI provided new information. The origin was considered prenatal in 45% (familial in 17%), perinatal in 4% and unclassifiable in 51%. 60% were mentally retarded; in the rest, cognitive development was near normal (18%) or normal (22%). Speech development was delayed in 88%, and 58% had visual dysfunction.RÉSUMÉAtaxie non évolutive. Olivine, pathologie cérébrate et handicaps chez 78 enfants suédoisCette etude de population porte sur 78 enfants suédois avec ataxie non évolutive, provenant ď unc population de 3.1 millions de habitants. Lcs critères ď appartenance comprcnaient une démarche ataxiquc avec ou sans signes de spasticité, une dyssynergie, une dysmétrie et un tremblement intcntionnel. Des scanners et/ou une imagerie IRM étaient présents pour 70 enfants (90%). Une pathologie sous-tentorielle fut trouvée dans 27% des cas, et les données étaient considérées comme normales dans 61% des cas. Si le scanner était normal, recent et de bonne qualityé, ITRM ne fournissait pas ď informations supplémentaires. Cependant dans la moitié des cas de scanners anormaux, ľ IRM apportait de nouvclles données. ľ origine a été considérée comme pré-natalc dans 45% des cas (17% des cas familiaux), 4% péri-natale et non classablc dans 51%. 60%étaient retardés mentaux; pour les autres, le développement cognitif était proche de la normale (18%) ou normal (22%). Le développement du langage était retardé dans 88% des cas. et il y avail une dysfonction visuelle dans 58% des cas.ZUSAMMENFASSUNGNicht progressive Ataxie. Ursacheit. Hirnpathologie mul Störungen bei 78 schwedischen KindernIm Rahmen dieser Populationsstudie wurden 78 schwedische Kinder mit einer nicht progressiven Ataxie aus einer Gesamtbevölkerung von 3.1 Millioncn untcrsucht. Die Kritericn für die Aufnahme in die Studie waren ataktischer Gang ohne Zeichen einer Spastik, Dyssynergie, Dysmetrie und Intcnsionstremor. CT - und/oder MRT Untcrsuchungen lagen bei 70 Patienten vor (90%). Bei 27% fand sich eine infratentorielle Patholgie und bei 61% waren die Befunde normal. War das CT normal, vor kurzem durchgefiihrt und qualitativ in Ordnung, ergaben sich aus dem MRT keine neuen Informationen. Bei der Hlfte der Flle mit pathologischem CT jedoch crgab das MRT neue Informationen. Die Ursache wurde in 45% (17% familir) als prnatal und in 4% als perinatal angesehen und war in 5% nicht einzuordncn. 60% waren gcistig retardicrt. die restlichen hatten einc nahezu normale (18%) bzw. normale (22%) kognitive Entwicklung. Die Sprachentwicklung war bei 80% vcrzögert und 58% hatten visuelle Störungen.RÉSUMÉAtaxia no progresiva. Origen, patología cerebral yalteraciones en 78 niños suecosEl estudio de población base se refiere a 78 niños suecos con ataxia no progresiva en una población total de 3.1 millones de habitantcs. Los criterios de inclusion fueron una marcha atáxica sin signos de espasticidad. dis-sinergia, dismctria y temblor intencional. En 70 pacientes (90%) se practiceó TAC o IRM. Se halló una patología infratentorial en el 27% y los hallazgosfueron eonsiderados normales en el 61%. Si la TAC era normal, realizada recientemente y de buena calidad. la IRM no proporcionó ninguna nueva información. Sin embargo, en la mitad de los casos con TAC patológica, la IRM proporcionó nueva información. Se consideró que el origen era prenatal en el 45% (familiar en el 17%). perinatal en el 4% y sin clasificaren el 51%. El 60% eran retrasados mentales; en el resto el desarrollo cognitivo era casi normal (18%) o normal (22%). El desarrollo del lenguaje estaba retrasado en el 88% y el 58% tenía una disfunción visual.
    Developmental Medicine & Child Neurology 11/2008; 38(4):285 - 296. · 2.68 Impact Factor
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    ABSTRACT: The results of a collaborative study of bilateral spastic cerebral palsy (BSCP) between south-west Germany and western Sweden are reported, comprising 249 children in south-west Germany and 264 children in western Sweden. A severe gross motor disability was present in 65 per cent of the German and 62 per cent of the Swedish children; learning difficulties or mental retardation in 73 and 76 per cent; active epilepsy in 28 and 26 per cent; and severe visual disability in 20 and 19 per cent, respectively. Severe disabilities were especially pronounced in children with normal birthweights, in whom the most severe subtypes of BSCP were also found. Leg-dominated BSCP was the predominant subtype among low-birthweight children, but also occurred in more than half of the normal-birthweight children. The authors conclude that the two series were comparable, and that reliable results between countries can be obtained if clear-cut classifications and definitions are used.RÉSUMÉIMC spastique bilatérale: étude de population comparant l'Allemagne du Sud-Ouest et la Suède occidentale. I: Aspects cliniques et incapacitésLes résultats d'une étude en collaboration portant sur l'IMC, spastique bilatérale (BSCP) en Allemagne du Sud-Ouest et Suède occidentale sont rapportés; l'étude portait sur 249 enfants pour l'Allemagne du Sud-Ouest et 264 enfants pur la Suède occidentale. Une incapacité motrice sévère était présente chez 65 pour cent des enfants allemands et 62 pour cent des enfants suédois; les difficultés d'apprentissage ou le retard mental dans respectivement 73 et 76 pour cent des cas; l'épilepsie active dans 28 el 26 pour cent; et l'incapacité visuelle sévère dans respectivement 20 et 19 pour cent des cas. Les déficits sévères étaient spécialement prononcés chez les enfants à poids de naissance normal, chez qui les soustypes de BSCP les plus graves étaient également rencontrés. Une BSCP prédominant aux members inférieurs était le soustype prédominant chez les enfants de faible poids de naissance, mais apparaissait également dans plus de la moitié des cas d'enfants à poids de naissance normal. Les auteurs concluent que les deux séries étaient comparables et que des comparaisons fidèles entre pays peuvant ětre obtenues si des classifications et des définitions précises sont utilisées.ZUSAMMENFASSUNGBilaterale spastische Cerebralparese: eine Vergleiclisstudie zwischen Sudwestdeutschland und Westschweden. I: Klinische Muster und BehinderungenEs werden die Ergebnisse einer kollaborativen Studie über bilaterale spastische Cerebralparese (BSCP) zwischen Sudwestdeutschland und Westschweden dargestellt; 249 deutsche und 264 schwedische Kinder wurden untersucht. Schwere grobmotorische Behinderungen fand man bei 65 Prozent der deutschen und 62 Prozent der schwedischen Kinder; Lernschwierigkeiten oder geistige Retardierung bei 73 bzw. 76 Prozent; aktive Epilepsie bei 28 bzw. 26 Prozent; und schwere Sehstörungen bei 20 bzw 19 Prozent. Schwere Behinderungen waren besonders ausgeprägt bei Kindern mit normalem Geburtsgewicht, bei denen auch die schwersten Formen der BSCP gefunden wurden. Die Beinbetonte BSCP war die vorherrschende Form bei Kindern mit niedrigem Geburtsgewicht, sie trat aber auch bei mehr als der Hälfte der normalgewichtigcn Kinder auf. Die Autoren kommen zu dem Schlu, da die zwei Studien vergleichbar waren, und da zuverlässige Ergebnisse erzielt werden kónnen, wenn man klare Einteilungen und Definitionen verwendet.RESUMENParálisis cerebral espáslica bilateral: un estudio basado en la poblacién comparando el Sudoeste de Alemanis y el Oeste de Suecia. I: Patrones clícos y discupacidadesSe reficren los resultados de Un estudio en colaboración sobre la parǎlisis cerebral espastica bilateral (PCEB) entre el Sudoeste de Alemania y el Oeste de Suecia. Comprende 249 niǹos en el Sudoeste de Alemania y 264 en el Oeste de Suecia. Una discapacidad motora grosera grave estaba presente en el 65 por ciénto de los niños alemanes y el 62 por ciento de los suecos; dificultad para aprender o retraso mental en el 73 y 76; epilepsia activa en el 28 y 26; e incapacidad visual grave en el 20 y 19 por ciento respectiveḿente. Las discapacidades graves eran especialmente pronunciadas en niños con pesos normales, en los que también se hallaron los más graves subtipos de PCEB. El subtipo prédominante entre los niños de bajo peso era el de predominio podálico, pero también se daba en más de la mitad de los niños con peso de nacimiento normal. Los autores coricluyen que las dos séries eran comparables y pueden obtenerse resultados fiables entre diferentes paises si se usan clasificaciones y definiciones claras.
    Developmental Medicine & Child Neurology 11/2008; 35(12):1037 - 1047. · 2.68 Impact Factor
  • Bengt Hagberg
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    ABSTRACT: The clinical effect of pRÉSUMÉd minimal brain damage was studied in 26 apparently normal children and young adults who had suffered from a transient expansive hydrocephalus in infancy. Many of them had been late in motor and mental development, especially in their early years, and 5 had had convulsions. A dysplastic body build was present in 3 cases and 2 children had shown precocious püberty. Signs of ataxia were common (12 cases), but spastic pareses were only occasionally met with. Squint was found in 11 cases, and there were single cases of optic atrophy and nystagmus. Terman-Merrill tests showed variations in intelleétual capacity wider than would be expected in a healthy population. Behaviour déviations were common especially in the lower IQ groups and in children with neurological abnormalities. The minor neurological and mental déviations associated with slight early brain damage are often misunderstood.RÉSUMÉDommage minimum du cerveau dans l'hydrocéphalie enfantile arrêtée spontandmentL'effet clinique de l'atteinte présumée minime du cerveau a étéétudiié chez 26 enfants apparemment normaux et jeunes adultes connus pour avoir souffert d'une hydrocéphalie expansive transitoire pendant leur enfance. Beaucoup des cas étudié ont été retardés dans leur développement moteur et mental, surtout au cours de leurs premiéres années. On a recontré 3 cas de construction dysplastique du corps et 2 cas de püberté précoce. Les signes atactiques étaient communs (12 cas), tandis que les parésies spastiques n'étaient rencontrées qu'occasionnellement. On a compté 11 cas de strabisme. Atrophie optique et nystagmus ont été découverés, et 5 patients ont présenté des convulsions. Des tests de Terman-Merrill appliqués aux patients ont montré que les variations de leurs capacités intelleetuelles étaient plus grandes que chez une population en bonne santé. Les déviations de comportement étaient communes, rencontrées plus souvent dans les groupes à plus faible quotient d'intelligence et parmi les cas présentant des anomalies neurologiques et mentales.
    Developmental Medicine & Child Neurology 11/2008; 4(6):583 - 587. · 2.68 Impact Factor
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    ABSTRACT: Epidemiological data of a collaborative study on children with bilateral spastic cerebral palsy (BSCP) 3 between south-west Germany and western Sweden are reported. The study period covered the birth 5 years 1975 to 1986. Overall, the rate of BSCP increased during the birth year periods 1975-77 and 1978-80, but decreased thereafter. The rise was due to an increase of BSCP in low-birthweight (LBW) children, especially very LBW (VLBW) children. Mortality rates in LBW, and particularly VLBW, children decreased significantly during the whole study period in both countries. The BSCP rate, after the initial increase, showed a decrease during the second half of the study period in LBW children. Results are interpreted in favour of a predominantly prenatal aetiology in normal birthweight and of a predominantly peri- and neonatal aetiology in LBW children.RÉSUMÉIMC Spastique bilatérale: étude comparative de population entre l'Allemagne du sud-ouest et la Suède Occident ale. II: EpidémiologieLes donnèes epidemiologiques d'une étude en collaboration, des enfants présentant une forme spastique bilatérale d'I.M.C. (BSCP) entre l'Allemagne du Sud-ouest et la Suède occidentale sont rapportées. La période d'étude couvrait une naissance entre 1975 et 1986. Globalement, le taux de BSCP augmentait durant les périodes de naissance 1975-77 et 1978-80 puis diminuait ensuite. L'augmentation était liée à un accroissement de BSCP chez les enfants de faible poids de naissance (LBW) et tout spécialement de très faible poids de naissance (VLBW). Les taux de mortalité pour les enfants LBW, et particulierèment VLBW, diminuaient significativement durant la période totale d'observation dans les deux études. Le taux de BSCP chez les enfants LBW, après l'augmentation initiale, présentait une diminution durant la seconde moitié de la période d'etude. La répartition entre soustypes de BSCP pour les enfants LBW indiquait une dimunition plus précoce pour les formes prédominant aux mcmbres inférieurs que pour les autres formes; pour les enfants avec unZUSAMMENFASSUNGBilaterale spastische Cerebralparese: eine vergleichende Populationsstudie zwischen Südwestdeutschtand und West-sehweden. II: EpidemiologieEs werden epidemiologische Daten einer kollaborativen Studie an Kindern mit bilateraler spastischer Cerebralparese (BSCP) zwischen Siidwestdeutschland und Westschweden vorgestellt. Die Studiendauer erstreckte sich über die Geburtsjahre 1975 bis 1986. Allgemein nahm die BSCP in den Zeiten von 1975-77 und 1978-80 zu, danach jedoch ab. Der Anstieg war durch eine Zunahme der BSCP bei Kindern mit niedrigem Geburtsgewicht (LBW) und besonders bei Kindern mit sehr niedrigem Geburtsgewicht (VLBW) bedingt. Die Mortalittsrate bei LBW-und besonders bei VLBW-Kindern ist im Verlauf der Studie in beiden Lndern signifikant gesunken. Die BSCP-Rate zeigte nach dem anfnglichen Anstieg bei den LBW-Kindern eine Abnahme in der zweiten Hlfte der Studie. Bei den BSCP Untergruppen für LBW-Kinder zeigte sich eine Verminderung der Bein-betonten Formen eher als in den anderen Untergruppen; Für Kinder mit normalem Geburtsgewicht gab es keinen eindeutigen Trend.RESUMENParálisis cerebral espástica bilateral: estudio comparativo de población entre el sud-oeste de Alemania y la Suecia occidental. II: EpidemiologíaSe aportan los datos epidemiológicos recogidos en un estudio en colaboración entre ninos con parálisis cerebral espástica (PCEB) del sud-este de Alemania y del oeste de Suecia. El periodo estudiado cubria los nacimientos de los años 1975 a 1986. En conjunto, el porcentaje de PCEB aumentaba en los periodos 1975-77 y 1978-80 pero disminuia después. EI aumento se debió a un aumento de las PCEB en los niños con bajo peso al nacer (BPN) especialmente en los de muy bajo peso (MBPN). La mortalidad en los BPN y en particular en los MBPN disminuyó significativamente a lo largo de todo el periodo de estudio en ambos paises. El porcentaje de PCEB, después delaumento inicial, mostró una disminución en la segunda mitad del periodo en estudio, sobre todo en niños con BPN. Las tendencias de los diversos tipos de PCE en los BPN mostraron una disminución en las formas en que dominaba la efectación de las piernas, más que en las otras formas. En los niños con peso normal al nacer, las tendencias eran inconsistentes.
    Developmental Medicine & Child Neurology 11/2008; 36(6):473 - 483. · 2.68 Impact Factor
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    ABSTRACT: In this third report from the collaborative study of children with bilateral spastic cerebral palsy born between 1975 and 1986, aetiology was analysed. Evidence for a prenatal aetiology increased with gestational age, whereas evidence for a peri-/neonatal aetiology decreased. The largest subgroup, the leg-dominated subtype, showed the same distribution of aetiology as the total group. A prenatal aetiology was found mainly among term and moderately preterm children with a four-limb-dominated subtype; a peri-/neonatal aetiology among very preterm children with a three- or four-Iimb-dominated subtype or among term children with a dyskinetic-spastic subtype. The findings support the hypothesis generated from the authors' epidemiological results of a peri-/neonatal aetiology being predominant among preterm, and a prenatal aetiology among term, children with bilateral spastic cerebral palsy.RÉSUMÉIMC spastique bilatérale: étude comparative entre Allemagne du sud-ouest el Suede occidentale. Ill: EtiologieDans ce troisième article d s'étude en collaboration des enfants avec IMC spastique bilatérale nés entre 1975 et 1986 létiologie a été analysée. l'évidence d'une étiologie prénatale augmentait avec l'âge de gestation tandis que l'évidence d'une étiologie péri- ou néonataie diminuait. Le sous groupe le plus important, sous type de prédominance aux MI, présentait la même distibution d'étiologie que le groupe total. Une étiologie prénatale était retrouvée chez les enfants nés à terme ou modérément prématurés dans le soustype d'atteinte dominante des quatre membres; une étiologie péri-, néonatale parmi les grands prématurés avec un sous type d'atteinte de trois ou quatre membres ou parmi les enfants de soustype dyskinétique-spastique. Ces données favorisent l'hypothèse émise par les auteurs à partir des résultats épidémiologiques d'une étiologie péri-, néonataie prédominant chez les prématurés, et d'une étiologie prénatale chez les enfants nés a terme, pour l'IMC spastique bilatérale.ZUSAMMENFASSUNGBilaterale spastische Cerebralparese: eine Vergleichsstudie zwischen südwest Deutschland und West Schweden. III: ÄtiologieIn diesem dritten Bericht über die (collaborative Studie an Kindern mit bilateraler spastischer Cerebralparese, die zwischen 1975 und 1986 geboren wurden, wurde die Ätiologie analysiert. Die Wahrscheinlichkeit einer prnatalen Ätiologie wurde mit zunehmendem Gestationsalter größer, whrend sich die einer periVneonatalen Ätiologie verringcrte. Bei der größten Untergruppe, dem beinbetonten Typ, fand sich dasselbe Ätiologieschema wie bei der Gesamtgruppe. Bei reif- oder etwas zu früh geborenen Kindern vom tetraplegischen Typ fand sich eine prnatale Ätiologie; bei sehr viel zu früh geborenen Kindern vom tri- oder tetraplegischen Typ oder bei reifgeborenen Kindern vom dyskinetisch-spastischen Typ fand sich eine peri-/neonatale Ätiologie. Die epidemiologischen Untersuchungen der Autoren stützen die Hypothese, daß bei Kindern mit bilateraler spastischer Cerebralparese eine peri-/neonatale Ätiologie bei frühgeborenen und eine prnatale bei reif geborenen vorherrscht.RESUMENParálisis cerebral espástica bilateral: estudio comparalivo entre el suroeste de Alemania y el oeste de Suecia. III: EtiologiaEn este tercer report sobre el estudio en colaboración de niños con parálisis espástica bilateral, nacidos entre 1975 y 1986, se analizó la etiologia. Le evidencia de una etiología prenatal aumentó con la edad gestacional, al mismo tiempo que disminuia la etiologáia periVneonatal. El subgrupo más grande, en el que predominaba la afectación de las extremidades inferiores, mostró la misma distribución etiológica que el grupo total. Una etiologia prenatal se halló en los niños a término y modcradamente pre-término en el grupo en que estaban afectados las cuatro extermidades; una etiología peri-/neonatal en los niños muy a pretermino con tres o cuatro miembros afectados o entre los niños con subtipo discinetico-espastico. Los hallazgos apoyan la hipotesis, originada en los hallazgos epidemiologicos de los autores, de que la etiología peri-/neonataI predomina en los a pretermino y una etiología prenatal en los a término, en los casos de parálisis espástica bilateral.
    Developmental Medicine & Child Neurology 11/2008; 37(3):191 - 203. · 2.68 Impact Factor
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    ABSTRACT: SummaryA case report is presented of a 6-year-old girl with a severe progressive encephalopathy of late infantile onset and with changes indicating marked disturbances in the metabolism of linolenic acid.
    Acta Paediatrica 01/2008; 57(6):495 - 499. · 1.97 Impact Factor
  • BENGT HAGBERG, ARNE HAMFELT, OLLE HANSSON
    Acta Paediatrica 01/2008; 55(4):371 - 384. · 1.97 Impact Factor
  • Acta Paediatrica 01/2008; 54(2):116 - 130. · 1.97 Impact Factor
  • B. HAGBERG, G. HAGBERG, I. OLOW
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    ABSTRACT: Hagberg, B., Hagberg, G. and Olow, I. (Department of Paediatrics II, Children's Hospital and the Habilitation Unit of Bräcke-Östergård, Gothenburg, Sweden). The changing panorama of cerebral palsy in Sweden 1954–1970. II. Analysis of the various syndromes. Acta Paediatr Scand 64:193, 1975 From an unselected series of 560 Swedish cases of cerebral palsy, born 1954-70, various data of etiologic and pathogenetic interest were analysed in detail. Untraceable and prenatal factors were found to dominate within the group of spastic hemiplegia. Placental dysfunction in small-for-date babies and severe asphyxia were thought to be the two main pathogenetic factors among the patients with spastic tetraplegia. In spite of a significant decrease in the number of low birth weight children within the group of spastic diplegia, this syndrome was still very characteristic for the child born immature. Ataxic diplegic forms were found to have greater pathogenetic similarities to spastic diplegia than to simple ataxia. In two-thirds of the children the latter syndrome was characterized by normal pregnancy, delivery and birth weight and an untraceable (genetic?) factor. Oyskinetic syndromes were mostly encountered after perinatal asphyxia.
    Acta Paediatrica 01/2008; 64(2):193 - 200. · 1.97 Impact Factor
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    ABSTRACT: Lbid berg, U., Hagberg, B., Olsson, Y. and Sourander, P. (Department of Paediatrics II and Institute of Pathology, University of Göteborg, and Institute of Pathology, University of Uppsala, Sweden), Injury of the spinal cord at birth. A report of two cases. Acta Paediatr Scand, 64:546, 1975.–Spinal cord injury may occur as a severe complication to delivery. In the vast majority of such cases the injury results from a traumatic breech delivery, but cases of injuries after cephalic presentation and fetal malposition have also been described. Two cases are reported. One of the infants died at the age of 8 months and neuropathologies! examination of the brain and spinal cord was performed. The other child, now 6 years old, is still alive. Incidence, mechanism of injury, clinical and morphological features, and treatment are briefly discussed.
    Acta Paediatrica 01/2008; 64(3):546 - 550. · 1.97 Impact Factor
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    ABSTRACT: Hagberg, B., Haltia, M., Sourander, P., Svennerholm, L. and Eeg-Olofsson, O. (Departments of Paediatrics II, Pathology I and Neurochemistry, University of Gothenburg, Sweden, and Pathology II, University of Helsinki, Finland). Poly-unsaturated fatty acid lipidosb—an infantile form of so-called neuronal ceroidlipo-fuscinosis. Acta Paediatr Scand, 63:753, 1974.—Clinical, histological and ultrastructural findings in three children of Finnish origin and with a severe progressive encephalopathy are reported. The main symptoms were rapid developmental regression from about one year of age, loss of speech, severe visual failure and pronounced secondary microcephaly. A decerebrated state was reached within 1–3 years. Laboratory tests revealed a successively decreasing CSF τ-fraction. The fatty acid composition of Serum lecithin showed an increased amount of arachidonic acid in the early stage of the disease, a pattern consistent with the biochemical changes in the brain.The morphological characteristics consisted of extreme cerebral and cerebellar atrophy, massive neuronal destruction associated with a pronounced macrophage and astrocytic reaction, and strongly PAS-reacting and autofluorescent granular deposits in the cytoplasm of the remaining cells. Electron microscopy revealed cytoplasmic osmiophilic deposits in the form of aggregates of globules with a uniform and finely granular ultra-structure.The condition was considered to be a nosological entity with a uniform clinical picture and characteristic ultrastructural changes in the brain. The evidence produced suggests that the disease described is identical with a progressive heredodegenerative disorder, known to have been diagnosed during recent years in more than 50 Finnish infants and small children and earlier described under the name of infantile type of so-called neuronal ceroidlipifuscinosis.
    Acta Paediatrica 01/2008; 63(5):753 - 763. · 1.97 Impact Factor
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    ABSTRACT: Lipid composition was studied on cerebral tissue from nine children who had died of a progressive encepha-lopathy called the infantile form of neuronal ceroid lipofus-cinosis (INCL) or polyunsaturated fatty acid lipidosis (PFAL). In the terminal stage of the disease, the concentrations of all lipid classes were found to be significantly reduced in the cerebral and cerebellar cortex and white matter. The concentration of gangliosides of the cerebral cortex was 15% and that of cerebrosides (galactosylceramide) in white matter 0.2–5% of the normal values for the children's ages. The reduction of gangliosides mainly affected those of the gangliotetraose series, particularly GDI a. The fatty acids of the linolenic acid series were strongly reduced in ethanolamine and serine phosphoglycerides. A very large increase up to 100-fold of oligoglycosphingolipids of the globo series and two fucose-containing lipids of the neo-lacto series was found in the forebrain of the three advanced cases examined. The brain tissue also contained very high concentrations of mono-, d -, and trisialogangliosides of the lacto and neolacto series, gangliosides with type 1 chain, dominating. The structures of the gangliosides were tentatively identified by gas chromatography-mass spectrometry and monoclonal antibodies with carefully determined epitope specificity. The gangliosides and neutral glycosphingolipids had very similar fatty acid composition, consisting of about 40% stearic acid and 40% C24-acids.
    Journal of Neurochemistry 10/2006; 49(6):1772 - 1783. · 3.97 Impact Factor
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    ABSTRACT: This is the ninth report from the western-Swedish study of the prevalence and origin of cerebral palsy. A population-based study covering the 88 371 live births in the area in 1995-1998. Birth characteristics, neuroimaging findings and risk factors in children with cerebral palsy were recorded, prevalence was calculated, and aetiology was analysed. The study comprised 170 children with cerebral palsy, i.e. a prevalence of 1.92 per 1000 live births. Excluding eight post-neonatally derived cases, the gestational age-specific prevalences were 77 per 1000 for children born before 28 wk of gestation, 40 for children born at 28-31 wk, 7 for children born at 32-36 wk and 1.1 for children born after 36 wk of gestation. Spastic hemiplegia, diplegia and tetraplegia accounted for 38%, 35% and 6%, respectively, dyskinetic cerebral palsy for 15%, and ataxia for 6%. For the first time, hemiplegia was now most common, due to the decline in preterm diplegia. There was a further increase in full-term dyskinetic cerebral palsy. The origin of cerebral palsy in children born at term was considered to be prenatal in 38%, peri/neonatal in 35% and unclassifiable in 27%, while in children born preterm it was 17%, 49% and 33%, respectively. The decreasing trend from the period 1991-1994 continued, both in children born at term and especially in those born preterm. However, the increase in dyskinetic cerebral palsy in children born at term was a matter of concern. In this group, a perinatal hypoxic ischaemic encephalopathy had been present in 71%.
    Acta Paediatrica 04/2005; 94(3):287-94. · 1.97 Impact Factor
  • American Journal of Obstetrics and Gynecology - AMER J OBSTET GYNECOL. 01/2003; 189(6).
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    ABSTRACT: Previous studies have indicated that foetomaternal infection increases the risk of spastic cerebral palsy (CP) in term infants, whereas this association appears to be less evident in preterm infants. The aim of this study was to analyse infection-related risk factors for spastic CP in preterm infants. A population-based series of preterm infants with spastic CP, 91 very preterm (<32 wk) and 57 moderately preterm (32-36 wk), born in 1983-90, were included and matched with a control group (n = 296). In total, 154 maternal, antenatal and intrapartal variables were retrieved from obstetric records. In the entire group, histological chorioamnionitis/pyelonephritis, long interval between rupture of membranes and birth, admission-delivery interval <4 h and Apgar scores of <7 at 1 min just significantly increased the risk of CP, and Apgar scores of <7 at 5 and 10 min were strongly associated with an increased risk. Abruptio placentae, Apgar scores <7 at 1 min and pathological non-stress test (reason for delivery) were significant risk factors of CP only in the moderately preterm and hemiplegic groups, whereas fever before delivery was a significant risk factor in the very preterm and spastic diplegic groups. Antibiotics during pregnancy was associated with CP only in the spastic diplegic CP group. Conclusion: Antenatal infections marginally increased the risk of CP. Low Apgar score and abruptio placentae were associated with CP, especially in moderately preterm infants with hemiplegic CP.
    Acta Paediatrica 01/2002; 91(8):946-51. · 1.97 Impact Factor
  • M Berg, B Hagberg
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    ABSTRACT: Through 1998-1999 the Swedish research team have systematically mapped 45 of 54 females with Rett syndrome (RS) aged 5-57 years (Berg M. Uppföljning av flickor med Rett syndrom i Västsverige. Socialmedicinsk kartläggning, Stencilerad rapport, Sahlgrenska universitetssjukhuset, Göteborg, 1999; Läkartidningen (Swedish Medical Journal) 96 (1999) 5488). The emphasis is on the RS females' adjustment to grown-up living. At an adult age the large majority moved over to a Swedish type of small group home. We visited these girls in their group homes and got information about their adaptation to this special home situation. We noted their behaviour and contact with the staff and interviewed their parents. The results showed that the young women usually had adjusted well. The adjustment seemed to depend on the degree of planning before the girls moved from their parents to special group homes. These seem to provide a good environment for the RS females, in respect of both physical and social aspects. Although neuropaediatrics in Sweden today has advanced in RS knowledge, the result from this research shows that in Swedish society there is still a great ignorance of RS, even among medical professionals. In particular there is a gap of knowledge when the medical responsibility had to be transferred over to adult medicine. Many of the parents emphasized the great importance of how they were listened to by professionals in public medical and social services. It is essential that professionals always listen to information from parents and personal assistants.
    Brain and Development 01/2002; 23 Suppl 1:S224-6. · 1.67 Impact Factor
  • B Hagberg, M Berg, U Steffenburg
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    ABSTRACT: Rett syndrome, today known as a worldwide important cause behind severe mental retardation in females, has been seen in Sweden since the 1960s (the senior author). This study gives population representative clinical and sociomedical follow-up data from West Sweden (1/4 of the population in Sweden). The series comprises 54 females diagnosed in 1971-1998, 5-60 years old, median age 20. Mortality in 1971-2000 was 18% (10/54), median age at death 20. Death usually had been sudden and unexpected. The sociomedical follow-up emphasized the adult group. The large majority had usually moved to small group homes, a good and well-accepted environment once the staff had learned the particularities of these so uniquely neurodevelopmentally disabled women.
    Brain and Development 01/2002; 23 Suppl 1:S28-31. · 1.67 Impact Factor
  • F Xiang, Y Stenbom, M Anvret, B Hagberg
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    ABSTRACT: Mutations in the MECP2 gene are known to be associated with Rett Syndrome (RTT) in the large majority of sporadic cases. Four Swedish families with a total of eight RTT patients (two in each family), were screened and found negative for MECP2 mutations. The series included females with both classical and forme fruste phenotypes. Rett syndrome thus might still be complex and genetically multifactorial.
    Neuropediatrics 09/2001; 32(4):217-8. · 1.19 Impact Factor
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    ABSTRACT: Rett syndrome (RS) is a neurodevelopmental disorder almost exclusively affecting females. We have studied the mutation spectrum of the responsible gene MECP2, encoding methyl-CpG-binding protein 2 (MeCP2), in 16 sporadic classical RS females from Sweden. In 13 of 16 patients (81%) we detected nonsense or missense mutations in the coding parts of MECP2. This mutation rate is in agreement with other reports (65-80%). In all, 12 different mutations and one polymorphism were found; three of the mutations have not been reported previously. The missense mutations were restricted to highly conserved regions of the gene. None of the mutations was detected in parents; thus, they had probably arisen de novo. In contrast, two normal variants, one intron deletion and one silent mutation, were seen singly only in two patients' mothers; neither has been reported previously. One patient showed two different mutations closely located, i.e. 802C > T (R268W) together with 808C > T (R270X). Another patient had a mutation in the stop codon 1459T > C (X487R), leading to a gene product prolonged with 27 amino acids. In conclusion, our results indicate that the majority of Swedish RS patients (81%) have mutations in MECP2 that are sporadic cases with de novo mutations. Moreover, both missense and nonsense mutations occur, but in different parts of the gene, probably reflecting the function of the domains in MeCP2. This study has improved our ability to offer these families an early confirmation of Rett diagnoses.
    European Child & Adolescent Psychiatry 07/2001; 10(2):117-21. · 3.70 Impact Factor
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    ABSTRACT: This 8th Swedish population-based cerebral palsy (CP) report comprises 241 children born 1991-94. The live birth prevalence was 2.12 per 1000. Excluding 7 postnatally-derived cases, the gestational age-specific prevalences were 86 for extremely preterm children, 60 for very preterm and 6 for moderately preterm, and 1.3 for term children per 1000. Spastic hemiplegic, diplegic and tetraplegic subtypes accounted for 33%, 44% and 6%, dyskinetic CP for 12% and simple ataxia for 4%. Neuroimaging had been performed in 90%. Probable aetiology was identified in 73% of preterm and 86% of term children. Among preterm children it was considered prenatal in 12%, peri/neonatal in 61% and unclassifiable in 27%, while it was 51%, 36% and 14% among term children. CONCLUSION: The live birth prevalence for CP in the birth year period 1991-94 continued to decrease slightly. Gestational age-specific prevalences increased marginally in extremely and very preterm births, continued to decrease in moderately preterm births and decreased slightly in term births. Probable aetiology and timing of the brain insult could be revealed in 81%, birth asphyxia being the likely cause in 28% of term children.
    Acta Paediatrica 04/2001; 90(3):271-7. · 1.97 Impact Factor

Publication Stats

4k Citations
400.42 Total Impact Points

Institutions

  • 2008
    • Uppsala University Hospital
      Uppsala, Uppsala, Sweden
  • 1992–2008
    • University of Tuebingen
      • Department of Neurology
      Tübingen, Baden-Württemberg, Germany
  • 1961–2008
    • Uppsala University
      Uppsala, Uppsala, Sweden
  • 1960–2008
    • University of Gothenburg
      • Division of Paediatrics
      Goeteborg, Västra Götaland, Sweden
    • Länssjukhuset Ryhov
      Jönköping, Jönköping, Sweden
  • 2001
    • Karolinska Institutet
      • Institutionen för klinisk neurovetenskap
      Solna, Stockholm, Sweden
  • 1995–1996
    • Sahlgrenska University Hospital
      Goeteborg, Västra Götaland, Sweden
  • 1990–1995
    • Karolinska University Hospital
      • • Department of Clinical Genetics
      • • Department of Neurology
      Stockholm, Stockholm, Sweden
    • Baylor College of Medicine
      Houston, Texas, United States
    • Jönköping University
      Jönköping, Jönköping, Sweden
  • 1991
    • Universitair Ziekenhuis Leuven
      • Department of Pedriatrics
      Leuven, VLG, Belgium
  • 1988
    • University of Turku
      • Department of Paediatrics
      Turku, Western Finland, Finland
  • 1962
    • Akademiska Sjukhuset
      Uppsala, Uppsala, Sweden