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UMMARY Fifteen cases of lissencephaly were studied and the literature reviewed. The authors concluded that the clinical findings of lissencepaly infancy are not-specific, consisting of development delay and hypotonia. While the CT scan establishes the diagnosis, it may also be strongly suggested by an EEG showing ‘major fast dysrhythmia’, characterized by abnormally rapid, very high-voltage activity, predominantly in the alpha and beta frequency bands. Some possible mechanisms for this highly suggestive EEG pattern are proposed. RESUME Lissencephalie (agyrie-pachygyrie): donnees cliniques et etudes EEG en serie Quinze cas de lissencalie ont eteetudies, avec une analyse de la litterature. Les auteurs concluent que les donnees cliniques de la lissencephalie durant l'enfance ne sont pas specifiques, consistent en un retard de developpement et une hypotonic Tandis que la tomodensitometrie fait le diagnostic, celui-ci peut etre aussi fortement suggere par un EEG montrant “une dysrhytmie majeure rapide”, caracterisee par une activite anormalement rapide, de tres haut voltage, predominant dans les bandes de frequence alpha et beta. Quelques mecanismes possibles pour ces particularites EEG hautement suggestives sont proposes. ZUSAMMENFASSUNG Lissencephalie (Agyrie-Pachygyrie): Klinische Befunde und fortlaufende EEG Untersuchungen 15 Falle mit Lissencephalie wurden untersucht und die Literatur wurde gesichtet. Die Autoren sind der Meinung, das die klinischen Befunde der Lissencephalie im Kindesalter unspezifisch sind, sie bestehen in Entwicklungsverzogerung und Hypotonie. Obwohl die Diagnose durch das Computertomogramm gesichert wird, kann sie doch mit groser Wahrscheinlichkeit durch ein EEG mit vorwiegend hochfrequenter Arrhythmie, charakterisiert durch abnorm schnelle, sehr hohe Amplituden, vorwiegend in den Alpha und Beta Frequenzen, gestellt werden. Es werden einige mogliche Vorgange fur dieses hochst verdachtige EEG Muster diskutiert. RESUMEN Lisencefaiia (agiria-paquigiria): hallazgos cli'nicos y estudios EEG seriados Se estudiaron quince casos de lisencefaiia y se reviso la literatura. Los autores concluyen que los hallazgos clinicos de la lisencefaiia en la lactancia no son especificos y consisten en retraso en el desarrollo e hipotonia. Si bien la TAC establece el diagnostico, puede ser tambien sospechado por el EEG que muestra una disritmia rapida mayor, caracterizada por una actividad anormalmente rapida con voltaje muy alto, con predominio en las bandas de frecuencia alfa y beta. Se proponen algunos posibles mecanismos para este tipo de EEG tan sugestivo.

SUMMARY 37 children presenting with West's syndrome were studied by computerized tomography scanning. An abnormality consisting of diffuse cerebral atrophy, predominating in the fronto-temporal region, was demonstrated in 30 cases. The abnormality was sometimes associated with calcification (tuberous sclerosis) or malformation (agenesis of the corpus callosum). The aetiology of this atrophy and its relationship to clinical and electro-encephalographic signs is discussed. Computerized tomography scanning is recommended for children with West's syndrome in order to distinguish between primary and secondary cases, and for greater prognostic accuracy. RESUME Etude tomodensitometrique du syndrome de West 37 enfants avec syndrome de West ont ete etudies par la tomodensitometrie; celle-ci a revele dans 81 pour cent des cas une anomalie, representee le plus souvent par une atrophie cerebrale diffuse a predominance fronto-temporale, quelquefois associee a des calcifications (sclerose tubereuse) ou malformations (agenesie du corps calleux). L'eetiologie de cette atrophie tres particuliere et ses rapports avec l'importance des signes cliniques et eeg sont discutes. Un examen tomodensitometrique des enfants presentant un syndrome de West est recommande pour distinguer les cas primitifs des cas secondaires et donc pour mieux apprecier le pronostic de cette encephalopathie epileptogene. ZUSAMMENFUNG Komputertomographie bei der Untersuchung des West Syndrom Bei 37 Kindern mit einem West Syndrom wurde eine Komputertomographie durchge-fuhrt. In 30 Fallen stellte sich eine Fehlbildung in Form einer diffusen Hirnatrophie dar, die im Bereich der Frontalregion am ausgepragtesten war. Zusatzlich zu dieser Fehlbildung fand man in einigen Fallen Verkalkungen (tuberose Hirnsklerose) oder andere Missbildungen (Agenesie des Corpus callosum). Eine Diskussion uber die Aetiologie dieser Atrophie und ihre klinischen und elektro-encephalographischen Merkmale schliesst sich an. Die Komputertomographie wird als Untersuchung bei Kindern mit einem West Syndrom empfohlen, um zwischen sekundaren und primaren Fallen zu unterscheiden und um eien genauere Prognose stellen zu konnen.

A 10-year review of a neuropediatric department experience with childhood ischemic cerebrovascular disease identified 35 patients with arterial ischemic stroke. The ability to diagnose stroke in children has improved with the development of imaging techniques in the past few years. Children have a wide array of risk factors for ischemic strokes, since some are acquired and others are congenital. Twenty-eight associated conditions (80%) were found in our patients and we identified 17 specific causes (48.5%) among them. The cause of stroke in children is important to recognize because stroke is likely to recur depending on the etiology. (J Child Neurol 1997;12:193-199).

Summary The authors have reported a case of cerebral lipidosis (type Jansky-Bielschowsky) in which the presenting features appeared at the age of 2 years 10 months, and consisted of clonic seizures followed by atonic attacks and atypical absences. The association of seizures, severe mental disturbance and EEG abnormalities with an interseizure abnormality (diffuse slow waves) led to an initial misdiagnosis of the Lennox-Gastaut syndrome. The diagnosis which is suspected on the basis of clinical features (epilepsy, mental deterioration, pyramidal features), EEG signs (spikes on photic stimulation at low frequency or isolated) and ophthalmological investigations (ERG reduced response, abnormal VERs) was eventually made by rectal biopsy.

Arterial ischemic strokes are a relatively frequent diagnostic occurrence in pediatric neuroradiology. They occur mostly in three main etiologic contexts: 1) congenital heart disease; 2) neonatal distress ; 3) infections, focal or general inducing vasculitis, but many cases are considered idiopathic. The positive diagnosis is made by CT; in neonates, however, ultrasound appears as a promising tool. The CT features are basically similar at that age and in adults, although the site of the infarct may result from pathologies more particular to children (e.g. basal ganglia infarction due to arteritis of the carotid siphon and its branches). Infarcts may be multiple and also more frequently hemorrhagic at that age, the hemorrhagic phenomena affecting only the gray matter except in young infants in which the subcortical white matter may be affected also. Anatomical sequels include focal atrophy and asymmetry of the brain. Data regarding the etiology can be gathered from angiography which may show the degree of impairment of the arterial bed, its extent, the collateral blood supply and the morphological type of arterial lesion responsible for the cerebral damage. The most particular picture at that age is that of the often diffuse vasculitis, with its various expressions (segmentai narrowing of the lumen, dissecting aneurysm, string-of-beads appearance).