Acquired auditory agnosia in childhood and normal sleep electroencephalography subsequently diagnosed as Landau-Kleffner syndrome: report of three cases.

in Developmental Medicine & Child Neurology (in press)

Aim We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. Method ... [more ▼]

Aim We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. Method Case histories including EEG, positron emission tomography findings, and long-term outcome were reviewed. Results Auditory agnosia occurred between the age of 2 years and 3 years 6 months, after a period of normal language development. Initial awake and sleep EEG, recorded weeks to months after the onset of language regression, during a nap period in two cases and during a full night of sleep in the third case, was normal. Repeat EEG between 2 months and 2 years later showed epileptiform discharges during wakefulness and strongly activated by sleep, with a pattern of continuous spike-waves during slow-wave sleep in two patients. Patients were diagnosed with LKS and treated with various antiepileptic regimens, including corticosteroids. One patient in whom EEG became normal on hydrocortisone is making significant recovery. The other two patients did not exhibit a sustained response to treatment and remained severely impaired. Interpretation Sleep EEG may be normal in the early phase of acquired auditory agnosia. EEG should be repeated frequently in individuals in whom a firm clinical diagnosis is made to facilitate early treatment. [less ▲]

Aspects neurologiques associés au Syndrome de Down

in Tijdschrift van de Belgische Kinderarts = Journal du Pédiatre Belge (2012), 14(2), 40-43

Summary : Down syndrome is one of best caracterized genetic syndromes. Its phenotype includes specific morphological features, mental retardation and several organic impairments such as cardiac ... [more ▼]

Summary : Down syndrome is one of best caracterized genetic syndromes. Its phenotype includes specific morphological features, mental retardation and several organic impairments such as cardiac malformations, cutaneous disorders and immunologic abnormalities. Beside these disturbances neurological disorders have been less considered or thought to be the expression of the mental retardation. However it is important to pay attention to the high incidence of epilepsy such as West syndrome in infants and “Late-Onset of myoclonic epilepsy in Down Syndrome” in the adult. They might required specific treatment. ADHD as well as autistic behavior may be difficult to diagnose especially in children with severe mental retardation. They need precise attention and rigourous follow-up of their treatment. [less ▲]

La prise en charge et le suivi des nouveau-nes prematures apres leur retour a domicile.

in Revue Médicale de Liège (2008), 63(4), 199-207

Prematurity remains a public health problem with a considerable psychosocial impact. Premature infants are discharged home more fragile and more precociously than infants born at term. Post-discharge ... [more ▼]

Prematurity remains a public health problem with a considerable psychosocial impact. Premature infants are discharged home more fragile and more precociously than infants born at term. Post-discharge nutrition and growth of the preterm infants should be carefully followed because of specific needs of these infants. Infections and cardiorespiratory abnormalities are more frequent in ex-premature infants. Some cerebral lesions may be shown by brain imaging suggesting future sequelae. However, estimation of their real consequences remains imperfect and long term prognosis contains many uncertainties. Cerebral palsy seems to be less severe nowadays, but all current gravity is due to disabilities which express later: hearing disorders, visual impairments, alterations of eye-hand coordination skills, attention deficit disorders, psychological troubles and school difficulties. Multidisciplinary consultations are designed for these children because early screening and adapted care can improve long term prognosis. All this underlines the importance of prolonged follow-up program after discharge for premature infants and others who presented worse suffer from hypoxic/ischemic encephalopathy. [less ▲]

The epileptic syndromes with continuous spikes and waves during slow sleep: definition and management guidelines.

in Acta Neurologica Belgica (2006), 106(2), 52-60

The authors propose to define the epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) as a cognitive or behavioral impairment acquired during childhood, associated with a strong ... [more ▼]

The authors propose to define the epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) as a cognitive or behavioral impairment acquired during childhood, associated with a strong activation of the interictal epileptiform discharges during NREM sleep--whatever focal or generalized--and not related to another factor than the presence of CSWS. The type of syndrome will be defined according to the neurological and neuropsychological deficit. These syndromes have to be classified among the localization-related epileptic syndromes. Some cases are idiopathic and others are symptomatic. Guidelines for work-up and treatment are proposed. [less ▲]

Développement normal et anormal du cortex cérébral: un up-date

in Percentile (2004), 9(1), 3-6

Electroencéphalographie du prématuré et du nouveau-né à terme

in Percentile (1998), 3

Epilepsies rebelles de l'enfant: définition et modalités de prise en charge hospitalière

in Neurone (1997), 2(8), 285-288

Retard neuromoteur et neuropsychologique: critères de recours à une prise en charge spécialisée

in Percentile (1996), .(3), 103-107

Caractéristiques des lésions résultant d'une pathologie périnatale

in Acta Paediatrica Belgica (1996), 28(3), 203-204

La Biologie clinique en pédiatrie: hypotonie et retard moteur

in Acta Paediatrica Belgica (1996), 28(2), 95