A clinical and electroencephalographic study of 107 neurologically normal children with partial seizures was undertaken to verify the existence and determine the frequency of epileptic syndromes reported in selected populations. Sixty-three children had simple partial seizures, 39 had complex partial seizures, and 5 children were unclassifiable. The syndrome of benign partial epilepsy of children with rolandic spikes (BPEC, 38 cases) was clearly identified and its uniformly benign final prognosis was confirmed even if some of these children had at times severe or poorly controlled seizures. Among the children with simple partial seizures outside the BPEC (25 cases) and complex partial seizures (39 cases), no homogeneous clinical or electroclinical subgroup could be found. Two children with benign partial epilepsy and myoclonic-astatic seizures ("atypical benign partial epilepsy of childhood") and one child with "benign epilepsy with occipital spike-waves" were identified. 74% of children with epilepsy with complex partial seizures (ECP) had a 1-year seizure-free interval, and many children with epilepsy with simple partial seizures outside the BPEC group (ESP) had no more than two seizures. A benign course is thus not limited to the BPEC but is difficult to predict. Prospective studies are necessary to confirm the existence of well-defined benign syndromes among the idiopathic partial epilepsies of childhood, which appear quite rare outside the BPEC.

The authors report at adult age 7 patients (6 men, one woman) with the syndrome of "acquired aphasia-epilepsy", 6 of which had been previously studied as children. The results of the language, neuropsychological and socio-educational evaluation detailed many years after the onset of the aphasia are the subject of this report. One man has recovered completely, one has a normal oral language but is severely dyslexic, one has recovered normal comprehension but has severe expressive language problems. Four have absent language comprehension and lack of expressive speech, and only one of them has learned and is using sign language with some efficiency. None has developed functional written language. Attempts to offer a substitutive language to children with prolonged inability to understand and use oral language appears important but is fraught with problems. Although there are no conclusive data about the role of the continuous paroxysmal EEG discharges and the effect of their suppression with drug treatment on the prognosis of the aphasia, the definite fluctuations of the aphasia in some cases, the isolated recent case reports of definitive improvement with drug treatment justify further trials in this potentially severe and chronic condition.

The authors report eight otherwise normal children who presented with transient dystonic postures and/or movements in infancy. The anomalies appeared during the first months of life, progressed during a period then gradually disappeared at follow-up (from 3 months to 5 years). Differential diagnosis with primary orthopedic problems, cerebral palsy and early progressive CNS disease may be difficult at onset of the symptoms. Transient dystonia is probably one of the numerous mechanisms responsible for some abnormalities of tone, posture and movement in infancy and may account for some of the cases labeled as "transient cerebral palsy". Our cases resemble those described by Willemse (19) as "benign idiopathic dystonia in the first year of life".

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SUMMARY The authors repbrt a follow‐up study of two boys who presented with autistic regression (after normal early development) at 13 and 22 months. Both were found on cerebral imaging to have tuberous sclerosis, with lesions involving the limbic system, bilaterally in the second child. The first child's regression coincided with the onset of partial complex seizures; disappearance of the autistic behaviour and marked improvement in cognitive development occurred with remission of the epilepsy. The second child, who had probable seizures and a late‐appearing epileptic focus on EEG, remained severely disabled. The autistic behaviour appears to be linked to pathology in the limbic system and a direct role of epilepsy in the regression is proposed. RÉSUMÉ Régression autistique de I'enfance: relation avec la pathologie limbique el I‘épilepsie Les auteurs rapportent une étude longitudinale de deux garçons ayant présenté une régression autistique (après un développement précoce normal) respectivement à 13 et 22 mois. Dans les deuxcas, furent trouvée a I'exploration imagière du cerveau, une sclérose tubéreuse avec des lésions atteignant le système limbique, bilatéralement dans le second cas. Chez le premier enfant, la régression coïncida avec le début de crises partielles complexes; une disparition du comportement autistique et une amelioration marquée du développement cognitif furent observées avec la rémission de I‘épilepsie. Le second enfant, qui faisait probablement des crises et avait un foyer épileptique d'apparition tardive á I'EEG, demeura gravemect atteint. Le comportement autistique apparait liéá une pathologie du systéme limbique et un rǒle direct de I‘épilepsie pour expliquer la régression est proposé. ZUSAMMENFASSUNG Autislische Regression bei Kindern: Relation zu pathologischen Veränderungen im limbischen System und zu Epilepsie Die Autoren berichten über die Verlaufsstudie an zwei Jungen, die eine autistische Regression (nach einer anfänglich normalen Entwicklung) mit 13 bzw 22 Monaten bekamen. Mit cerebralen bildgebenden Verfahren wurde bei beiden Kindern eine tuberóse Hirnsklerose mit Lásionen im limbischen System, bei dem zweiten Kind beidseitig, festgestellt. Bei dem ersten Kind trat die Regression mit dem Beginn komplexer Partialanfälle auf; mit der Remission der Epilepsie verschwand das autistische Verhalten und es wurde eine deutliche Besserung der kognitiven Entwicklung beobachtet. Des zweite Kind, das vermutlich Anfälle und einen spät auftretenden Krampffokus im EEG hatte, blieb schwer behindert. Das autistische Verhalten scheint an pathologische Veränderungen im limbischen System gebunden zu sein und man vermutet einen direkten Zusammenhang zwischen Epilepsie und Regression. RESUMEN Regresión autisrica en ninCos: relación con el sislerna limbico y la epilepsia Los autores aportan el resultado del curso de un estudio de dos muchachos con regresión autistica (tras un desarrollo normal) a los 13 y 22 meses respectivamente. En ambos se hallaron imágenes de esclerosis tuberosa c...