The VNPP in GGE is a useful tool in diagnosis and characterization of reflex traits, and is a safe procedure. Its use might preclude multiple R-EEG exams.
Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as "tonic spasm seizures". Here, we present a 5-year-old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video-EEG monitoring revealed high-voltage slow waves superimposed by low-voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time-locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET-CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term "periodic spasms" and "focal spasms". In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. [Published with video sequence]A cluster of three tonic spasms, characterized by sudden flexion of the head and proximal muscles, lasting 5, 4.3 and 1 second, respectively. The spasms were accompanied simultaneously by upward deviation of the eyes and oral automatisms at the end.Key words for the video research on www.epilepticdisorders.com
Insulectomy is an established microsurgical technique for treatment of insular epilepsy refractory to clinical management. The insular origin of epilepsy is unusual. However with depth investigation through hybrid electrodes, it is becoming possible to improve its diagnosis. The authors emphasize the insular functions as well as the anatomical landmarks for surgery. The main complications and physiological basis are discussed. RESUMO Insulectomia é uma técnica cirúrgica bem estabelecida que tem sido cada vez mais usada para o tratamento de epilepsias insulares e peri-insulares refratárias ao tratamento medicamentoso. A investigação neurofisiológica por meio de eletrodios de profundidade tem demonstrado um aumento na incidência de casos com indicação cirúrgica. Os autores enfatizam as funções autonômicas insulares, bem como os limites anatômicos da ressecção. As principais complicações e bases fisiológicas para indicação serão discutidas.Palavras-chave: Ínsula; Insulectomia; Epilepsia do lobo temporal; Eletródios híbridos.
Summary:Zika virus (ZIKV) has been shown to be highly neurotropic; neurologic disorders are a common complication of this infection. Encephalitis—an inflammation of the brain parenchyma associated with neurologic dysfunction—is a rare complication of ZIKV infections. It affects patients from young to elderly ages. Clinical presentation of ZIKV encephalitis may be heterogeneous, including altered mental status (decreased or altered level of consciousness, lethargy, or personality change), seizures, and focal deficits. Complementary diagnostic investigation should include neuroimaging, lumbar puncture, and EEG. Neuroimaging findings in ZIKV encephalitis are not specific and may be diverse, including normal findings, hyperintense lesions on MRI involving cortical or subcortical structures, symmetric or asymmetric lesions involving supra or infratentorial regions, and more widespread involvement such as brain swelling. A remarkable scarcity of neurophysiological data on ZIKV encephalitis was found in the literature. In line with other diagnostic examinations, there are no neurophysiological findings suggestive or specific of the disease. EEG in ZIKV encephalitis showed different results: normal or diffuse disorganization of background activity, asymmetry with abnormal focal slow waves, focal epileptic discharges or generalized spike–wave and multispike–wave complexes, and periods of generalized voltage attenuation.
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