RESUMO
An 18-year-old boy with refractory epilepsy and aggressiveness associated to a hypothalamic hamartoma was submitted to a stereotactically guided lesion by thermocoagulation. The target was based on magnetic resonance (MR) images merged with computed tomography scan images taken on the day of surgery while patient was on a stereotactic frame. In order to reveal structures not discernible in MR images, the Schaltenbrand digital brain atlas was merged onto the patient's images. Target and trajectory of the depth electrode were chosen based on three-dimensional imaging reconstructions. A surgical plan was devised to disconnect the hypothalamic hamartoma from the hypothalamus, medial forebrain bundle, fasciculus princeps, and dorsal longitudinal fasciculus. Our target was placed at the inferior portion of the posterolateral component of the hamartoma, bordering the normal hypothalamus. The patient evolved with marked lessening of aggressiveness. Seizure frequency was reduced from several seizures per day to less than one tonic-clonic seizure during sleep per month and only two episodes suggestive of partial complex seizures during daytime. These results have remained consistent over a 24-month postoperative follow-up. Functional neuroanatomy of hypothalamic connections involved in seizure propagation and aggressive behavior was reviewed.
Assuntos
Hamartoma/cirurgia , Doenças Hipotalâmicas/cirurgia , Transtornos Mentais/prevenção & controle , Procedimentos Neurocirúrgicos , Radiocirurgia , Convulsões/prevenção & controle , Adolescente , Anticonvulsivantes/uso terapêutico , Resistência a Medicamentos , Eletrodos Implantados , Eletroencefalografia , Hamartoma/complicações , Hamartoma/psicologia , Humanos , Doenças Hipotalâmicas/complicações , Doenças Hipotalâmicas/psicologia , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Masculino , Transtornos Mentais/etiologia , Convulsões/etiologia , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
Os autores, constatando ser o nevo comedônico dermatose rara relacionada à malformaçäo do folículo pilossebáceo, relatam um caso em que o comprometimento da regiäo mandibular direita de uma menina feodérmica de sete anos acarretou exuberantes repercussöes psicossociais. Apresentam revisäo dos aspectos mais relevantes dessa insólita entidade nosológica congênita e expöem as razöes da ulterior conduta cirúrgica adotada