Transient magnetic resonance diffusion abnormalities in West syndrome: the radiological expression of non-convulsive status epilepticus?


:The purpose of this study was to report patients with pharmacoresistant West syndrome of unknown cause whose magnetic resonance imaging (MRI) with diffusion weighted imaging (DWI) showed a transient decrease of diffusion in subcortical structures. Of 20 patients investigated over a 2-year period, three males and three females constitute the present series. They had daily clusters of infantile spasms with hypsarrhythmia for 4 to 24 months before the first investigation. Four were severely hypotonic. All aetiological studies involving intermediary metabolism, peroxysomes, mitochondria, and neurotransmitters in cerebrospinal fluid were negative. Patients underwent DWI when first examined at the mean age of 13 months, and on follow-up examination 6 to 18 months later. Diffusion was decreased in the pallidi and posterior brainstem. It was also decreased for five patients in thalami and for three in dentate nuclei. Repeat MRI, performed when spasms were still present but hypsarrhythmia had ceased, did not show the same abnormalities. Because of recruitment bias, this series probably overestimates the true incidence of such DWI abnormalities. The eventuality of toxic lesions, including some inborn error of metabolism or drug toxicity, is considered unlikely although it could not be excluded. The contribution of the epileptic encephalopathy itself appears the most likely course.


Dev Med Child Neurol


Desguerre I,Marti I,Valayannopoulos V,Bahi-Buisson N,Dulac O,Plouin P,Delonlay P,Hertz-Pannier L,Boddaert N




Has Abstract


2008-02-01 00:00:00














  • Folate-induced reversal of leukoencephalopathy and intellectual decline in methylene-tetrahydrofolate reductase deficiency: variable response in siblings.

    abstract::Homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency may present with variable neurological manifestations. Radiological features include white matter changes (leukoencephalopathy). Clinical, biochemical, and radiological response to treatment may again be variable. Here we present a 12-year follo...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Tallur KK,Johnson DA,Kirk JM,Sandercock PA,Minns RA

    更新日期:2005-01-01 00:00:00

  • Explicit memory in low-risk infants aged 19 months born between 27 and 42 weeks of gestation.

    abstract::The aim of this study was to determine whether there are primary effects of prematurity on the development of explicit memory. Elicited imitation of action sequences was used to compare immediate and 15-minute delayed memory in term and preterm infants (19 months corrected age; n=48) who were at low risk: none had exp...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: de Haan M,Bauer PJ,Georgieff MK,Nelson CA

    更新日期:2000-05-01 00:00:00

  • Dynamic motor control is associated with treatment outcomes for children with cerebral palsy.

    abstract:AIM:To estimate the impact of dynamic motor control on treatment outcomes in children with cerebral palsy. METHOD:We used multiple regression on a retrospective cohort of 473 ambulatory children with cerebral palsy who underwent conservative treatment, single-level orthopaedic surgery, single-event multi-level orthopa...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Schwartz MH,Rozumalski A,Steele KM

    更新日期:2016-11-01 00:00:00

  • Speech and language interventions for infants aged 0 to 2 years at high risk for cerebral palsy: a systematic review.

    abstract:AIM:We evaluated the level of evidence of speech, language, and communication interventions for infants at high-risk for, or with a diagnosis of, cerebral palsy (CP) from 0 to 2 years old. METHOD:We performed a systematic review of relevant terms. Articles were evaluated based on the level of methodological quality an...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章,评审


    authors: Chorna O,Hamm E,Cummings C,Fetters A,Maitre NL

    更新日期:2017-04-01 00:00:00

  • Auditory brainstem responses of children with developmental language disorders.

    abstract::Brainstem auditory evoked potentials (BAEPs) were recorded from 48 children with language disorders (aged four to nine years old, IQs normal to borderline, normal audiometric thresholds and attending special school) and 20 healthy children (four to eight years old, with normal IQs, audiometry and school-level). The la...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Roncagliolo M,Benítez J,Pérez M

    更新日期:1994-01-01 00:00:00

  • GLUT1 deficiency syndrome--2007 update.

    abstract::GLUT1 deficiency syndrome (GLUT1DS, OMIM 606777) is a treatable epileptic encephalopathy resulting from impaired glucose transport into the brain. The essential biochemical finding is a low glucose concentration in the cerebrospinal fluid (CSF; hypoglycorrhachia; mean 1.7 [SD 0.3mmol/L]) in the setting of normoglycaem...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章,评审


    authors: Klepper J,Leiendecker B

    更新日期:2007-09-01 00:00:00

  • Longitudinal study of growth and development of young Jamaican children recovering from severe protein-energy malnutrition.

    abstract::The developmental level and nutritional status of a group of 17 children aged between six and 24 months who were admitted to hospital with severe protein-energy malnutrition were studied from admission to hospital until 36 months after returning home. They were compared with a group of 14 adequately nourished children...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Grantham-McGregor SM,Powell C,Stewart M,Schofield WN

    更新日期:1982-06-01 00:00:00

  • Psychosocial and cultural factors associated with the management of spina bifida cystica in Nigeria.

    abstract::A prospective study of the psychosocial and cultural factors influencing the management of spina bifida cystica was carried out on Nigerian children (48 males, 28 females) seen at the University College Hospital, Ibadan, between January 1982 and December 1983. The parents of 74 of the 76 children had never heard of th...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Oyewole A,Adeloye A,Adeyokunnu AA

    更新日期:1985-08-01 00:00:00

  • Tuberous sclerosis and infantile spasms.

    abstract::The short- and long-term outcome and special problems of 24 children with infantile spasms and tuberous sclerosis (TS) was studied. The diagnosis of TS is frequently missed: white spots on the skin have to be carefully looked for. In the present study, these spots were always found, and calcifications or hypodense are...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Riikonen R,Simell O

    更新日期:1990-03-01 00:00:00

  • CNS effects of mechanically produced spina bifida.

    abstract::Neural-tube defects were produced by one of two mechanical perturbations in an amphibian model system: newly-formed neural tubes were slit dorsally, or an intrinsic mass (an eye primordium transplanted to the neural plate) was introduced to prevent dorsal closure. Electron microscopic analyses showed that the resultin...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Katz MJ

    更新日期:1984-10-01 00:00:00

  • Atelencephalic microcephaly.

    abstract::A two-month-old child born with marked microcephaly (head circumference 17cm) was found at autopsy to have gliomesodermal tissue replacing the telencephalon (atelencephaly). This finding, together with genital anomalies and hypoplasia of the extremities, suggests arrest at the fifth week of fetal life. The clinical an...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Garcia CA,Duncan C

    更新日期:1977-04-01 00:00:00

  • Gabapentin for pain, movement disorders, and irritability in neonates and infants.

    abstract::We aimed to report our institution's experience with gabapentin therapy to manage agitation and pain in the neonatal intensive care unit (NICU) setting. This was a retrospective, single-center study of NICU patients admitted between January 2015 and December 2017, who received gabapentin. Data on neonatal agitation, p...

    journal_title:Developmental medicine and child neurology



    authors: Burnsed JC,Heinan K,Letzkus L,Zanelli S

    更新日期:2020-03-01 00:00:00

  • Reduced accommodative function in dyskinetic cerebral palsy: a novel management strategy.

    abstract::A 9-year-old boy with dyskinetic cerebral palsy secondary to neonatal encephalopathy is described. He presented with blurring of near vision which had begun to impact on his school work. Objective assessment of accommodation showed that very little was present, although convergence was almost normal. The near-vision s...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Ross LM,Heron G,Mackie R,McWilliam R,Dutton GN

    更新日期:2000-10-01 00:00:00

  • Influence of equinus treatments on the vertical displacement of the body's centre of mass in children with cerebral palsy.

    abstract::We assessed the influence of equinus gait treatments on the vertical displacement of the body's centre of mass (COM) in 21 patients with cerebral palsy (14 males, 7 females; mean age 8 y 9 mo [SD 2 y]; range 3 y 7 mo-17 y) presenting different topographical types (quadriplegia, n = 1; diplegia, n = 6; right hemiplegia...

    journal_title:Developmental medicine and child neurology

    pub_type: 临床试验,杂志文章


    authors: Massaad F,van den Hecke A,Renders A,Detrembleur C

    更新日期:2006-10-01 00:00:00

  • Conservative interventions for positional plagiocephaly: a systematic review.

    abstract::This review aimed to synthesize current research evidence to determine the effectiveness of conservative interventions for infants with positional plagiocephaly. A systematic review was conducted, where papers were sourced from 13 library and internet databases. Research was included if published in English between 19...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章,评审


    authors: Bialocerkowski AE,Vladusic SL,Howell SM

    更新日期:2005-08-01 00:00:00

  • Electrophysiological approach to the study of essential tremor in children and adolescents.

    abstract::Surface electromyography and accelerometry provide essential information on the neurophysiological characteristics of essential tremor. There are many reports on neurophysiological features in adult-onset essential tremor, but to our knowledge there have been no similar investigations of essential tremor in children. ...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Fusco C,Valls-Solé J,Iturriaga C,Colomer J,Fernández-Alvarez E

    更新日期:2003-09-01 00:00:00

  • MRI findings in patients with spastic cerebral palsy. II: Correlation with type of cerebral palsy.

    abstract::The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. Eighty-one patients had diplegia, 45 had quadriplegia, and 26 had hemiplegia. Of patients with diplegia, 72 had periventricular leuk...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Okumura A,Kato T,Kuno K,Hayakawa F,Watanabe K

    更新日期:1997-06-01 00:00:00

  • The development of emotion and empathy skills after childhood brain injury.

    abstract::Lasting socio-emotional behaviour difficulties are common among children who have suffered brain injuries. A proportion of difficulties may be attributed to impaired cognitive and/or executive skills after injury. A recent and rapidly accruing body of literature indicates that deficits in recognizing and responding to...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章,评审


    authors: Tonks J,Slater A,Frampton I,Wall SE,Yates P,Williams WH

    更新日期:2009-01-01 00:00:00

  • Intravenous diazepam infusion in the management of planned intrathecal baclofen withdrawal.

    abstract::Intrathecal baclofen is used in the management of spasticity associated with cerebral palsy (CP). Sudden cessation of intrathecal baclofen may give rise to the potentially life-threatening baclofen withdrawal syndrome. We report three cases in which intravenous diazepam is used effectively in the management of planned...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Cruikshank M,Eunson P

    更新日期:2007-08-01 00:00:00

  • Environmental effects on motor development: the case of "African infant precocity".

    abstract::In order to clarify the extent and cause of African infants' precocity in motor development, as reported by Geber and others, 64 babies and their families were intensively studied in a rural Kenyan community. It was found that the motor skills of sitting and walking, which the Kenyan babies acquired early (by American...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Super CM

    更新日期:1976-10-01 00:00:00

  • Relationship of finger function to beginning writing: application to diagnosis of writing disabilities.

    abstract::Finger function tasks designed to elicit soft neurological signs or to measure sensory-motor integration were administered to 100 first-, 100 second- and 100 third-grade children as part of a multivariate study of normal writing acquisition in an unreferred sample. The finger-succession task--a measure of motor planni...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Berninger VW,Rutberg J

    更新日期:1992-03-01 00:00:00

  • Assessing the impact of a child with spina bifida on the family.

    abstract::This study examined the characteristics of families which may be more vulnerable than others to the impact of caring for a child with spina bifida. Morbidity and the use of health services were unexpectedly high. The impact on the family was related less to clinical diagnoses than to the characteristics of the child's...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: McCormick MC,Charney EB,Stemmler MM

    更新日期:1986-02-01 00:00:00

  • Neurotrichosis: hair-shaft abnormalities associated with neurological diseases.

    abstract::Six children with morphological hair-shaft abnormalities and neurological disease are presented, including two with Pollitt syndrome, one with biotin deficiency, two with Menkes diseases and one with argininosuccinic aciduria. The child with biotin deficiency grew normal hair following oral biotin therapy. Although th...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Coulter DL,Beals TF,Allen RJ

    更新日期:1982-10-01 00:00:00

  • Relationship between lead exposure indicators and neuropsychological performance in children.

    abstract::This study surveyed 237 schoolchildren in a lead-polluted industrial area in northern Italy to assess the relationship between various biological indicators (lead in blood, hair and teeth, and delta-aminolevulinic dehydratase [ALA-D] activity) and some neuropsychological functions, assessed by a battery of five psycho...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Bergomi M,Borella P,Fantuzzi G,Vivoli G,Sturloni N,Cavazzuti G,Tampieri A,Tartoni PL

    更新日期:1989-04-01 00:00:00

  • Botulinum toxin type A injections can be an effective treatment for pain in children with hip spasms and cerebral palsy.

    abstract:AIM:Botulinum toxin type A (BoNT-A) injections were used in the treatment of lower-limb spasticity in children with cerebral palsy (CP). Anecdotal evidence suggests a reduction in pain after this treatment in children who had pain localized to a displaced hip joint. We report on our current clinical practice. METHOD:T...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Lundy CT,Doherty GM,Fairhurst CB

    更新日期:2009-09-01 00:00:00

  • The epidemiology of spina bifida in south-western Ohio--1970-1979.

    abstract::Multiple epidemiological variables of 131 children with spina bifida born during 1970 and 1979 in a seven-county urban/rural region of south-western Ohio were analyzed retrospectively via personal interviews, hospital and clinic records, and birth and stillbirth certificates. The estimated incidence of spina bifida wa...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Crowe CA,Heuther CA,Oppenheimer SG,Barth LD,Jeffrey E,Reinhart S

    更新日期:1985-04-01 00:00:00

  • Long-term outcome of vein of Galen malformation.

    abstract:AIM:To describe the long-term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM). METHOD:This was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 y...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Taffin H,Maurey H,Ozanne A,Durand P,Husson B,Knebel JF,Adamsbaum C,Deiva K,Saliou G

    更新日期:2020-06-01 00:00:00

  • Herpes simplex virus-induced anti-N-methyl-d-aspartate receptor encephalitis: a systematic literature review with analysis of 43 cases.

    abstract:AIM:To conduct a systematic literature review on patients with biphasic disease with herpes simplex virus (HSV) encephalitis followed by anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHOD:We conducted a case report and systematic literature review (up to 10 December 2016), focused on differences between h...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章,评审


    authors: Nosadini M,Mohammad SS,Corazza F,Ruga EM,Kothur K,Perilongo G,Frigo AC,Toldo I,Dale RC,Sartori S

    更新日期:2017-08-01 00:00:00

  • Isosorbide in the medical management of hydrocephalus in children with myelodysplasia.

    abstract::Fourteen children with hydrocephalus associated with myelodysplasia were given isosorbide (8 to 12 g/kg/day) and compared with 17 children managed without it. Three of the treated and three of the untreated children did not require shunting. The mean interval between back closure and shunt placement was 33 days for th...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Liptak GS,Gellerstedt ME,Klionsky N

    更新日期:1992-02-01 00:00:00

  • Visuoperceptual profiles of children using the Flemish cerebral visual impairment questionnaire.

    abstract:AIM:To investigate the underlying factor structure of the 46-item Flemish cerebral visual impairment (CVI) questionnaire, differentiate the factor scores of children with and without CVI, and examine the impact of comorbidities on factor scores. METHOD:The records of 630 children (386 males, 244 females; median age 77...

    journal_title:Developmental medicine and child neurology

    pub_type: 杂志文章


    authors: Ben Itzhak N,Vancleef K,Franki I,Laenen A,Wagemans J,Ortibus E

    更新日期:2020-08-01 00:00:00