Henrique Carrete Junior

Pre-surgical evaluation and surgical treatment in children with extratemporal epilepsy.

IntroductionThis review summarizes some patterns of pre-surgical evaluation and surgical treatment of extratemporal epilepsy in pediatric patients with medically refractory seizures, whose ictal behavior is variable. The most effective treatment for intractable partial epilepsy is a focal cortical resection with excision of the epileptogenic zone (the area of ictal onset and initial seizure propagation). This might be risky, though, in the case of a widespread lesion, sometimes encroaching one or more lobes, given the risk to the functional cerebral cortex. An anterior temporal lobectomy might prove more effective then in preventing seizures with fewer potential complications. If partial extratemporal epilepsy is associated with pharmaco-resistant seizures, the preoperative evaluation and operative strategy are determined according to the epileptogenic zone and to the relationship between a substrate-directed disorder and eloquent areas. The pediatric treatment of extratemporal epilepsy is aimed at controlling the seizures, avoiding morbidity, and improving the patient’s quality of life through psychosocial integration. Since the immature brain is more plastic than when mature, the recovery of functions after surgery is greater in children than in adults.RecommendationEarly surgery is recommended for children with intractable epilepsy, and is now accepted as an important therapeutic modality also for children with chronic epilepsy.ConclusionTechnological advances in the last two decades, mainly in neuroimaging, have led many medical centers to consider surgical treatment of epilepsy, accuracy being granted by MRI-based neuronavigation systems—an interface between the lesion seen in the preoperative magnetic resonance imaging (MRI) and the operative field, often invisible to the surgeon.

Personality traits in juvenile myoclonic epilepsy: Evidence of cortical abnormalities from a surface morphometry study

Cluster B personality disorders (PD), characterized as emotional instability, immaturity, lack of discipline, and rapid mood changes, have been observed among patients with juvenile myoclonic epilepsy (JME) and have been associated with a worse seizure outcome. Proper understanding of the neurobiology of PD associated with JME could contribute to understanding the basis for earlier and more effective interventions. In the present study, volumetric and geometric features of cortical structure were assessed through an automated cortical surface reconstruction method aiming to verify possible structural cortical alterations among patients with JME. Twenty-two patients with JME with cluster B PD, 44 patients with JME without psychiatric disorders, and 23 healthy controls were submitted to a psychiatric evaluation through SCID I and SCID II and to an MRI scan. Significant cortical alterations in mesiofrontal and frontobasal regions, as well as in other limbic and paralimbic regions, were observed mainly in patients with JME with PD. The present study adds evidence to the hypothesis of frontal and limbic involvement in the pathophysiology of cluster B PD in JME, regions linked to mood and affective regulation, as well as to impulsivity and social behavior. Moreover, a multidimensional pattern of frontal, limbic, and paralimbic changes was observed through a method of structural analysis which offers different and simultaneous geometric features, allowing the elaboration of important pathophysiologic insights about cluster B PD in JME.

Blinking and eyelid myoclonia: Characteristics and correlations of eyelid movements

PURPOSE Eyelid myoclonia (EM) is considered a seizure type and has been described in several epileptic conditions. Previous studies reported that EM are precipitated only by slow eye closure, but little is known about the characteristics of blinking in patients with EM seizures and differences in precipitation of EM by different kinds of eye closure. We analyzed by video-EEG the characteristics of blinking and eye closure in these patients. METHOD Twenty patients with EM had a video-EEG protocol with eyelid sensors. Semiology and rate of blinking and EM were analyzed. Statistical analysis was performed and p-values <0.05 were considered significant. RESULTS Seventeen cases (mean age 20.7, range 3-35) were women, 10 had EM as the main seizure type and between the others, all, but one, had criteria to Juvenile Myoclonic Epilepsy. All patients presented EM, 18 spontaneously during awake, 10 on eye closure and one only during intermittent photic stimulation. EM assumed the form of flicker, flutter or jerk, accompanied by generalized discharges, spiky posterior alpha, theta rhythm or absence of any EEG abnormality. Analysis of the characteristics of blinking had no statistics differences between patients and healthy subjects. The rate of blinks and EM increased during speech and decreased during reading. EM never occurred during blinking or in the dark. CONCLUSIONS Despite normal physiology of blinking, EM can manifest as jerk, flicker or flutter, with or without EEG abnormalities and independently of IPS, suggesting that eye closure sensitivity seems to include both, a motor and a visual component.

Temporal pole signal abnormality on MR imaging in temporal lobe epilepsy with hippocampal sclerosis: a fluid-attenuated inversion-recovery study

OBJECTIVE To determine the frequency and regional involvement of temporal pole signal abnormality (TPA) in patients with hippocampal sclerosis (HS) using fluid-attenuated inversion-recovery (FLAIR) MR imaging, and to correlate this feature with history. METHOD Coronal FLAIR images of the temporal pole were assessed in 120 patients with HS and in 30 normal subjects, to evaluate gray-white matter demarcation. RESULTS Ninety (75%) of 120 patients had associated TPA. The HS side made difference regarding the presence of TPA, with a left side prevalence (p=0.04, chi2 test). The anteromedial zone of temporal pole was affected in 27 (30%) out of 90 patients. In 63 (70%) patients the lateral zone were also affected. Patients with TPA were younger at seizure onset (p=0.018), but without association with duration of epilepsy. CONCLUSION Our FLAIR study show temporal pole signal abnormality in 3/4 of patients with HS, mainly seen on the anteromedial region, with a larger prevalence when the left hippocampus was involved.

Auras in temporal lobe epilepsy with hippocampal sclerosis: Relation to seizure focus laterality and post surgical outcome

We examined the relationship between presence and frequency of different types of auras and side of lesion and post surgical outcomes in 205 patients with medically intractable mesial temporal lobe epilepsy (MTLE) with unilateral hippocampal sclerosis (HS). With respect to the number of auras, multiple auras were not associated with side of lesion (p=0.551). The side of HS was not associated with the type of auras reported. One hundred fifty-seven patients were operated. The occurrence of multiple auras was not associated with post-surgical outcome (p=0.740). The presence of extratemporal auras was significantly higher in patients with poor outcome. In conclusion, this study suggests that the presence of extratemporal auras in patients with MTLE-HS possibly reflects extratemporal epileptogenicity in these patients, who otherwise showed features suggestive of TLE. Therefore, TLE-HS patients undergoing pre-surgical evaluation and presenting clinical symptoms suggestive of extratemporal involvement should be more extensively evaluated to avoid incomplete resection of the epileptogenic zone.

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms

A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She under went a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes.

The integrity of corpus callosum and cluster B personality disorders: a quantitative MRI study in juvenile myoclonic epilepsy.

Evidence suggests increased prevalence of cluster B personality disorders (PD) among patients with juvenile myoclonic epilepsy (JME), which has been associated with worse seizure control and more psychosocial dysfunctions. A preliminary voxel-based morphometry study demonstrated corpus callosum (CC) volume reduction in patients with JME and cluster B PD, particularly in the posterior midbody and isthmus. In this study we aimed to follow up these results with region of interest analysis. Sixteen JME patients with cluster B PD, 38 JME patients without any psychiatric disorder, and 30 demographically matched healthy controls submitted to a psychiatric evaluation and a magnetic resonance imaging scan. The total and regional callosal areas were obtained from the midsagittal slice using a semi-automated program. Psychiatric evaluation was performed through SCID-I and -II. Significant reductions in the posterior region of the CC were observed in the JME with PD group relative to the other groups. These data support previous findings of callosal reductions in cluster B PD, as well as a possible involvement of CC in patients with JME and such personality characteristics.

Primary diffuse leptomeningeal gliomatosis

Dr. Andre Leite Goncalves – Division of Child Neurology / Departamento de Neurologia e Neurocirurgia Rua Botucatu 720 – 04023-900 Sao Paulo SP Brasil. E-mail: goncalvesnp@yahoo.com.br Glioma arising primarily from the leptomeninges is extremely rare and often diagnosed only in postmortem examination. It is thought to derive from heterotopic nests of neuroglial tissue within the leptomeninges, which are present in 1% of necropsies. Primary leptomeningeal glioma can be in the form of a solitary tumour or a diffuse tumour involving intracranial or spinal cord leptomeninges. We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) established by meningeal biopsy.

Fatores determinantes da viabilidade neuronal em fatias hipocampais de pacientes com epilepsia do lobo temporal mesial

Determining factors of the neuronal viability in hippocampal slices from patients with mesial temporal lobe epilepsy The aim of the present research was to investigate the determining factors of neuronal viability in hippocampal slices from TLE patients submitted to hippocampectomy in the Hospital São Paulo between 2002 and 2005. The following variables were investigated: age, epilepsy duration, seizure frequency, hippocampal atrophy, resection time, history of initial precipitant injury (IPI) and surgical outcome according Engel’s classification. We compared patients who hippocampal slices showed a preserved neuronal viability during in vitro electrophysiological recordings (“ALIVE” group) and those who hipocampal slices did not produce electrical recordings (“DEAD” group). There was no statistical difference between the two groups regarding patient’s age (p = 0,84), epilepsy duration (p = 0,71), resection time (p = 0,66) and history of IPI (p = 0,42). On the other hand, there were statistically significant differences regarding hippocampal volume (p = 0,0005), asymmetry index (p = 0,01) and seizure frequency (p = 0,008). These data suggest that very atrophic hippocampi, from patients with low seizure frequency, are likely to present a disruption of neuronal viability.

Morphometric MRI features and surgical outcome in patients with epilepsy related to hippocampal sclerosis and low intellectual quotient

OBJECTIVE The objectives of this study were to verify in a series of patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) if those with low intellectual quotient (IQ) levels have more extended areas of atrophy compared with those with higher IQ levels and to analyze whether IQ could be a variable implicated on a surgical outcome. MATERIAL AND METHODS Patients (n=106) with refractory MTLE-HS submitted to corticoamygdalohippocampectomy (CAH) (57 left mesial temporal lobe epilepsy (MTLE); 45 males) were enrolled. To determine if the IQ was a predictor of seizure outcome, totally seizure-free (SF) versus nonseizure-free (NSF) patients were evaluated. FreeSurfer was used for cortical thickness and volume estimation, comparing groups with lower (<80) and higher IQ (90-109) levels. RESULTS In the whole series, 42.45% of patients were SF (Engel Class 1a; n=45), and 57.54% were NSF (n=61). Total cortical volume was significantly reduced in the group with lower IQ (p=0.01). Significant reductions in the left hemisphere included the following: rostral middle frontal (p=0.001), insula (p=0.002), superior temporal gyrus (p=0.003), thalamus (p=0.004), and precentral gyrus (p=0.02); and those in the right hemisphere included the following: rostral middle frontal (p=0.003), pars orbitalis (p=0.01), and insula (p=0.02). Cortical thickness analysis also showed reductions in the right superior parietal gyrus in patients with lower IQ. No significant relationship between IQ and seizure outcome was found. CONCLUSIONS This is the first study of a series of patients with pure MTLE-HS, including those with low IQ and their morphometric magnetic resonance imaging (MRI) features using FreeSurfer. Although patients with lower intellectual scores presented more areas of brain atrophy, IQ was not a predictor of surgical outcome. Therefore, when evaluating seizure follow-up, low IQ in patients with MTLE-HS might not contraindicate resective surgery.