Síndrome de Rasmussen. Seguimiento de siete años. Aspectos relacionados con plasticidad cerebral en epilepsia / Rasnmussen syndrome. 7 years' follow-up. Aspects related to cerebral plasticity in epilepsy

RESUMO

Present study represents a case follow up for seven years. A case of Epilepsy Partialis Continua (EPC) in a fifteen year old girl was studied with CT, MRI, cerebral SPECT and constantly EEG procedures The SPECT study showed in right frontal lobe and right temporal lobe hypoperfusion (reduction of regional cerebral blood flow [rCBF]) cerebral zone in the interictal period and hyperperfusion (increase rCBF) in the ictal period in the same hemisphere. The EEG showed abnormal activity consisting in sharp wave and polyphasic sharp wave in frontal and temporal regions. The medical treatment was refractory at all kind of anticonvulsant drugs. In order to improve the refractory epilepsy the team decided an epilepsy surgery with right temporal lobotomy and right frontal corticetomy. This procedure were done at the eight year old and the results were satisfactory immediately after the surgery with absolute control and normal cognitive performance two weeks later the surgery. This patient was follow up for seven years with EEG constantly with normal activity, SPECT and MRI, all studies showed functional hemispherectomy, total absence in the structure and cerebral blood flow in right hemisphere with normal perfusion and structure in left hemisphere. The late control of neuropsychology assessment showed some deficits in the I.Q. cognitive performance, evaluated by the Weschler scale and Machover tests. These findings would be able to demonstrate a phenomenon of brain plasticity and neuronal restoration in the left cerebral hemisphere after to remove the abnormal cerebral epilepsy focus. This procedure might permits the restoration of inadequate neuronal environment and normalization of neural physiological stability. The postulated mechanisms of reorganization of function are unmasking, nonsynaptic diffusion neurotransmission and receptor plasticity, trophic factors, synapsins and neurotransmitters. The neuropsychological mechanism to preserve the functions would be involving in a cortical reorganization with axonal and dendritic development beside sprouting and synaptogenesis. This patient is a good example of neuronal plasticity in epilepsy surgery.