SUMMARYIn the last 10-15 years the ILAE Commission on Classification and Terminology has been presenting proposals to modernize the current ILAE Classification of Epileptic Seizures and Epilepsies. These proposals were discussed extensively in a series of articles published recently in Epilepsia and Epilepsy Currents. There is almost universal consensus that the availability of new diagnostic techniques as also of a modern understanding of epilepsy calls for a complete revision of the Classification of Epileptic Seizures and Epilepsies. Unfortunately, however, the Commission is still not prepared to take a bold step ahead and completely revisit our approach to classification of epileptic seizures and epilepsies. In this manuscript we critically analyze the current proposals of the Commission and make suggestions for a classification system that reflects modern diagnostic techniques and our current understanding of epilepsy.

There are at least five types of alterations of consciousness that occur during epileptic seizures: auras with illusions or hallucinations, dyscognitive seizures, epileptic delirium, dialeptic seizures, and epileptic coma. Each of these types of alterations of consciousness has a specific semiology and a distinct pathophysiologic mechanism. In this proposal we emphasize the need to clearly define each of these alterations/loss of consciousness and to apply this terminology in semiologic descriptions and classifications of epileptic seizures. The proposal is a consensus opinion of experienced epileptologists, and it is hoped that it will lead to systematic studies that will allow a scientific characterization of the different types of alterations/loss of consciousness described in this article.

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Subcortical band heterotopia (SBH) is seen predominantly in females, resulting from mutations in the X-linked doublecortin (DCX) gene, and can present with mild mental retardation and epilepsy. Males carrying DCX mutations usually demonstrate lissencephaly and are clinically much more severely affected. This article reports two cases of males with SBH indistinguishable from the female phenotype, both resulting from somatic mosaicism for DCX mutation.

Language can transfer to the right hemisphere in patients with early left-sided brain injury (1,2). Rare cases with acquired left hemisphere lesions and language transfer after age 9 years have been reported (1,3,4). The possibility of language transfer in adolescent patients may influence decision making in epilepsy surgery and may alter concepts of language development and plasticity.We report two adolescents with initially lefthemispheric language dominance proven by intracarotid amobarbital testing (IAT) who developed right-sided language between 9 and 15 years and between 12 and 14 years, respectively. Language was impaired in both patients because of Rasmussen chronic encephalitis beginning at 8 and 11 years, with improvement after left hemispherectomy at ages 14 and 15 years.Case one, a right-handed 8-year-old boy, had pharmacologically intractable right clonic seizures associated with left frontocentral EEG seizures. Bilateral IAT at age 9 revealed left hemisphere language dominance. Repeated magnetic resonance imaging (MRI) showed progressive left hemispheric atrophy and increased left temporooccipital signal on T 2 sequences. At age 15 years, right-sided epilepsia partialis continua and hemiparesis developed. Handedness shifted from right to left. A repeated IAT at that time showed right-sided language representation. Left functional hemispherectomy was performed. Histopathology was consistent with Rasmussen encephalitis. Postoperatively, no impaired language function was noted. Rare right facial clonic seizures were well controlled. Neuropsychological testing showed language improvement as compared with presurgical assessment (Table 1).Case two, a right-handed 11-year-old girl, had right clonic seizures and left frontocentral EEG seizures. Re- IAT at 12 years and 8 months showed left hemisphere language. Handedness shifted from right to left. The family initially declined left hemispherectomy because of concerns regarding language function. Steroids and plasmapheresis were tried instead, without effect. She was readmitted at age 13 years and 10 months with right-sided epilepsia partialis continua, dysphasia, right hemiparesis, dehydration, and inability to swallow. Immediate left functional hemispherectomy led to seizure freedom. Histopathology was typical of Rasmussen encephalitis. Postoperatively, language was slow, but she could communicate. Neuropsychological assessment demonstrated improved verbal performance as compared with preoperative assessment (Table 1).Only seven patients with Rasmussen encephalitis have been reported with apparent interhemispheric transfer of language after age 9 years. Transfer was assessed by neuropsychological means, showing initial language deterioration after hemispherectomy and progressive improvement over time in five patients (1), by late IAT showing right hemisphere language dominance at the time of surgery (4), and by functional MRI (fMRI) in one patient (3).We add the first patients with late language transfer, who had initial left hemisphere language dominance...