[Video electroencephalographic diagnosis of epileptic and non-epileptic paroxysmal episodes in infants and children at the pre-school age]. / Diagnostico videoelectroencefalografico de los episodios paroxisticos epilepticos y no epilepticos en el lactante y el nino en edad preescolar.

The main usefulness of video electroencephalographic (video-EEG) monitoring lies in the fact that it allows proper classification of the type of epileptic seizure and epileptic syndrome, identification of minor seizures, location of the epileptogenic zone and differentiation between epileptic seizures and non-epileptic paroxysmal manifestations (NEPM). In infants and pre-school age children, the clinical signs with which epileptic seizures are expressed differ to those of older children, seizures with bilateral motor signs such as epileptic spasms, tonic and myoclonic seizures predominate, and seizures with interruption of activity or hypomotor seizures, and no prominent automatisms are observed. In children with focal epilepsies, focal and generalised signs are often superposed, both clinically and in the EEG. NEPM may be benign transitory disorders or they can be episodic symptoms of different neurological or psychopathological disorders. NEPM are often observed in children with mental retardation, neurological compromise or autism spectrum disorders, who present epileptic seizures and epileptiform abnormalities in the baseline EEG. It then becomes necessary to determine which episodes correspond to epileptic seizures and which do not. The NEPM that are most frequently registered in the video-EEG in infants and pre-school age children are unexpected sudden motor contractions ('spasms'), introspective tendencies, motor stereotypic movements and paroxysmal sleep disorders.

Epilepsy surgery in children with developmental tumours.

We report our experience regarding evaluation, surgical treatment and outcomes in a population of 21 children with histopathologically confirmed developmental tumours [nine dysembryoplastic neuroepithelial tumours (DNET), ten gangliogliomas (GG) and two gangliocytomas (GC)] and related epilepsy, analyzing video-EEG, MRI and neuropsychological data, before and after surgery. Most children had focal epilepsy correlating well with lesion location. One patient had epileptic spasms and generalized discharges. Tumours were located in the temporal lobe in 13 patients. Mean age at surgery was 11.16 years. Postsurgical MRI showed residual tumour growth in one DNET. One child had a recurrent ganglioglioma with anaplastic transformation. At latest follow-up (mean 4.68 years) 95.2% of patients were seizure-free and no significant neuropsychological declines were observed. Evidence from our study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.

Continuous giggling and autistic disorder associated with hypothalamic hamartoma.

We present the case of a child affected since early infancy from recurring episodes of giggling mixed with stereotypical behaviours, mingled with head drops, and eventually with falls, in the context of an autistic disorder. Scalp video-EEG recordings revealed an epileptic encephalopathy with generalized slow spike-and-wave complexes alternating with electrodecremental periods, which generally corresponded to the onset of the aforementioned clinical sequences. A resection of a hypothalamic hamartoma was achieved at the age of two. Since then, after 22 months of follow-up, the child is totally free from the giggling and the drops, and the autistic behaviour significantly improved during the second year of follow-up. This case illustrates the difficulties to recognize some subtle ictal manifestations during infancy and childhood, and encourages the early surgical treatment of hypothalamic hamartomas when associated with epileptic encephalopathy and when technically possible.