Hypothalamic Hamartomas: Evolving Understanding and Management

Hypothalamic hamartomas (HH) are rare, basilar developmental lesions with widespread comorbidities often associated with refractory epilepsy and encephalopathy. Imaging advances allow for early, even prenatal, detection. Genetic studies suggest mutations in GLI3 and other patterning genes are involved in HH pathogenesis. About 50-80% of children with HH suffer from severe rage and aggression and a majority of cases exhibit externalizing disorders. Behavioral disruption and intellectual disability may predate epilepsy. Neuropsychological, sleep and endocrine disorders are typical. The purpose of this paper is to provide a summary of the current understanding of HH, and to highlight opportunities for future research

Epilepsia ao comer

A epilepsia reflexa ao comer é forma rara de epilepsia reflexa. Um paciente de 24 anos com crises parciais complexas reflexas ao comer foi submetido a avaliações clínica, neurológica, neurorradiológica e eletrencefalográfica. O exame, neurológico e a tomografia de crânio foram normais. Registros de EEG, incluindo monitorização vídeo-EEG durante a refeição, mostraram presença de anormalidades focais relacionadas a ambos os lobos temporais, predominando à esquerda, e sincronia bilateral secundária, predominando em regiões anteriores. Os achados ictais são semelhantes à sincronia bilateral secundária interictal exceto por sua maior duração. Monoterapias com PR, DPH e VPA não surtiram efeito. Monoterapia com altas doses de CBZ trouxe bons resultados porém com controle incompleto das crises. Como grande número de fatores precipitantes estava potencialmente envolvido, não foi possível determinar com precisão a base fisiopatológica das crises reflexas neste caso

An evoked potential mapping of transcallosal projections in the cat

In ten adult cats anesthetized with ketamine hydrochloride the neocortex was exposed and rectangular pulses (1msec, 0.5 Hz and variable intensity) were applied to discrete points of one side and transcallosal evoked potentials were recorded from the other. The stimulation and recording positions were determined on a cartesian map of most of the exposable neocortical areas and the potentials were analysed as to their components, voltage and latency. Passive spread and electrotonic potentials and the effects of increasing frequency were also analysed. The results showed large transcallosal potentials in some areas and an increase of potentials in the caudorostral direction, attaining the highest values in anteromedial areas of the suprasylvian gyrus. Confirming anatomical studies, a few silent spots were found in the motor and somesthetic cortex and in restricted posterior regions of the visual cortex, where small or zero voltages occurred. While causing weak contralateral potentials, stimulation of some posterior sites provoked high voltage potentials in anterior regions of the side being stimulated and in the corresponding area of the opposite site. These posterior sites are. poorly interconnected by the corpus callosum. The L-shaped indirect connection described in this work may be involved in some types of epilepsy and may explain the effectiveness of partial callosotomy in their treatment