Clinical and electrographic presentation of a case of hemimegalencephaly

Autor: Barbara M.R. Sousa, Maria do Carmo V. Santos, Gisele N.M. Sion, Eduardo A.R. Silva, Aline Curcio de Moraes, Wesley M. Vieira, Bruno Silva Costa, Antonio P. Gomes Neto, Cesar A. Lamberti, Pedro M.G. Molina
Rok vydání: 2018
Předmět:
Zdroj: Neurophysiologie Clinique. 48:138-139
ISSN: 0987-7053
DOI: 10.1016/j.neucli.2018.05.030
Popis: Objectives Epilepsy is a common and potentially serious and disabling disease. Congenital malformations of the brain are often associated with severe presentations and difficult drug control. Our objective is to report a case of a child with hemimegalencephaly, the clinical and electroencephalographic presentation before and after neurosurgery. Methods RAR, a 2-year-old girl with difficult-to-control epilepsy using a combination of high doses of anticonvulsants, presented daily crisis from the neonatal period. She was monitored with video-EEG before and after brain surgery. Clinical presentation and electroencephalographic changes helped to indicate the procedure. Results The video-EEG showed crises characterized by behavioral arrest, eyes up and stillness. Inter-ictal EEG had continuous poly-spykes in the right hemisphere and focal EEG accentuates epileptiform activity over the right quadrant region. Magnetic resonance imaging of the requested brain with right hemimegalencephaly. The patient underwent right hemispherectomy in December 2017, provided no recurrence of crisis. Her behavior and development is also better. She will be admitted shortly for a new video-EEG monitoring. Conclusion Hemimegalencephaly is a malformation of cortical development characterized by excessive hamartomatous growth of all or part of a cerebral hemisphere and frequently associated with refractory epilepsy. Patients may present with early encephalopathy, delayed neuropsychomotor development, motor deficits and learning disabilities. Anatomic or functional hemispherectomy is indicated if epilepsy is clinically intractable.
Databáze: OpenAIRE