Magnetoencephalographic studies of two cases of diffuse subcortical laminar heterotopia or so-called double cortex.

Two cases (a young male and a girl, suffering intractable epilepsy) of diffuse subcortical laminar heterotopia, or so-called double cortex (DC) have been investigated using magnetoencephalography (MEG). MEG confirmed involvement of both cortices (hetero- and normocortex) in the genesis of interictal spikes, and, according to the heterogeneity of DC syndrome, some differences were observed: spike initiation in the normocortex and latter involvement of the heterotopic cortex in the man, and rather a cancellation in both cortices in the girl. In addition, participation of heterotopic cortex in physiological activities could be demonstrated in the man.

Automated detection of focal cortical dysplasia lesions using computational models of their MRI characteristics and texture analysis.

Focal cortical dysplasia (FCD), a malformation of cortical development, is a frequent cause of pharmacologically intractable epilepsy. FCD is characterized on Tl-weighted MRI by cortical thickening, blurring of the gray-matter/white-matter interface, and gray-level hyperintensity. We have previously used computational models of these characteristics to enhance visual lesion detection. In the present study we seek to improve our methods by combining these models with features derived from texture analysis of MRI, which allows measurement of image properties not readily accessible by visual analysis. These computational models and texture features were used to develop a two-stage Bayesian classifier to perform automated FCD lesion detection. Eighteen patients with histologically confirmed FCD and 14 normal controls were studied. On the MRI volumes of the 18 patients, 20 FCD lesions were manually labeled by an expert observer. Three-dimensional maps of the computational models and texture features were constructed for all subjects. A Bayesian classifier was trained on the computational models to classify voxels as cerebrospinal fluid, gray-matter, white-matter, transitional, or lesional. Voxels classified as lesional were subsequently reclassified based on the texture features. This process produced a 3D lesion map, which was compared to the manual lesion labels. The automated classifier identified 17/20 manually labeled lesions. No lesions were identified in controls. Thus, combining models of the T1-weighted MRI characteristics of FCD with texture analysis enabled successful construction of a classifier. This computer-based, automated method may be useful in the presurgical evaluation of patients with severe epilepsy related to FCD.

Epileptogenicity of focal malformations due to abnormal cortical development: direct electrocorticographic-histopathologic correlations.

PURPOSE: Malformations due to abnormal cortical development (MCDs) are common pathologic substrates of medically intractable epilepsy. The in situ epileptogenicity of these lesions as well as its relation to histopathologic changes remains unknown. The purpose of this study was to correlate the cellular patterns of MCDs with the expression of focal cortical epileptogenicity as assessed by direct extraoperative electrocorticographic (ECoG) recordings by using subdural grids. METHODS: Fifteen patients with drug-resistant focal epilepsy due to pathologically confirmed MCD who underwent subdural electrode placement for extraoperative seizure localization and cortical mapping between 1997 and 2000 were included in the study. Areas of interictal spiking and ictal-onset patterns were identified and separated during surgery for further pathologic characterization (cellular and architectural). Three pathologic groups were identified: type I; architectural disorganization with/without giant neurons, type IIA; architectural disorganization with dysmorphic neurons, and type IIB; architectural disorganization, dysmorphic neurons, and balloon cells (BCs). The focal histopathologic subtypes of MCDs in cortical tissue resected were then retrospectively correlated with in situ extraoperative ECoG patterns. RESULTS: Cortical areas with histopathologic subtype IIA showed significantly higher numbers of slow repetitive spike pattern in comparison with histopathologic type I (p = 0.007) and normal pathology (p = 0.002). The ictal onset came mainly from cortical areas with histopathologic type IIA (nine of 15 patients). None of the seizures originated from neocortical areas that showed BC-containing MCD (type IIB). CONCLUSIONS: This study shows that areas containing BCs are less epileptogenic than are closely located dysplastic regions. These results suggest a possible protective effect of BCs or a severe disruption in the neuronal networks in BCs containing dysplastic lesions. Further studies are needed to elucidate the nature and the potential role(s) of balloon cells in MCD-induced epileptogenicity.

Outcome of epilepsy surgery in focal cortical dysplasia.

OBJECTIVE: To describe the outcome of surgery in patients with drug resistant epilepsy and a histopathological diagnosis of focal cortical dysplasia. METHODS AND SUBJECTS: Analysis of histories and presurgical and follow up data was carried out in 53 patients with a histological diagnosis of focal cortical dysplasia. Their mean age was 24.0 years (range 5 to 46), and they included 14 children and adolescents. Mean age at seizure onset was 12.4 years (0.4 to 36) and mean seizure duration was 11.6 years (1 to 45). RESULTS: The presurgical detection rate of focal cortical dysplasia with magnetic resonance imaging (MRI) was 96%. There were 24 temporal and 29 extratemporal resections; additional multiple subpial transections were done in 12 cases to prevent spread of seizure discharges. There was a 6% rate of complications with permanent neurological deficit, but no deaths. All resected specimens were classified by neuropathological criteria as focal cortical dysplasia. Balloon cells were seen in most cases of extratemporal focal cortical dysplasia. After a mean follow up of 50 months, 38 patients (72%) were seizure-free, two (4%) had less than two seizures a year, nine (17%) had a reduction of seizure frequency of more than 75%, and four (8%) had no improvement. Seizure outcome was similar after temporal and extratemporal surgery. The patients in need of multilobar surgery had the poorest outcome. CONCLUSIONS: Circumscribed lesionectomy of focal dysplastic lesions provides seizure relief in patients with chronic drug resistant temporal and extratemporal epilepsy. There was a trend for the best seizure outcome to be in patients with early presurgical evaluation and early surgery, and in whom lesions were identified on the preoperative MRI studies.

Hypothalamic hamartoma and epilepsy in children: illustrative cases of possible evolutions.

The progresses of neuroimaging have allowed an earlier detection of hypothalamic hamartoma in children presenting with gelastic or dacrystic seizures. Associated symptoms can include other types of seizures, precocious puberty, and behavioral or cognitive deterioration. Combination of all these features is not constant and, when present, their evolution may be variable. When epilepsy proves intractable, surgery may be a solution but is not without risks. Therefore, it can only be justified on the basis of a considerable degree of certainty on the progressive character of the disorder, both in terms of epilepsy and global development. Even though epilepsy is a major and usually the most important problem, it is not always possible to predict its course and to be able to evaluate its potential effects on development. Available data suggests that deterioration is partly related to the epileptogenic activity. We reviewed data from 16 personal cases and discussed the possible evolutions of the epilepsy syndrome on the basis of 6 illustrative cases and a review of the literature. We point out that seizures may start early in life and evolve either towards a catastrophic encephalopathy or may be transiently severe and will progressively settle down. Intermediate situations also exist as well as cases presenting with a mild epilepsy. In almost all cases cognitive difficulties are present and may be associated with behavioral disturbances. They are of variable severity, usually in relation to the severity of the epilepsy and the evolution of the EEG abnormalities. Some of our cases also illustrate that, in young children whose seizures are limited to "a sensation of a pleasant feeling", "a pressure to laugh" or "smiling", early detection of the hamartoma may still be difficult and the epilepsy pattern may be misdiagnosed as an epilepsy temporal or frontal origin. Detailed analysis of the electro-clinical evolution of representative cases highlights the variable expression of the epilepsy syndrome and renders difficult any dogmatic position on early surgery. However, recent data suggests that a surgical solution must be sought early. Prospective studies are needed to evaluate, not only outcome in terms of control the seizures without unacceptable side effects but also on the evolution of the cognitive and behavioral profile of children with HH and epilepsy are needed.

Mechanisms of epileptogenicity in focal malformations caused by abnormal cortical development.

In this article, the authors review the cellular mechanisms of epileptogenicity in malformations caused by abnormalities of cortical development as related to neuronal excitation, reduced inhibition, disorganized synaptic connections, and glial function (or dysfunction).

Cortical dysplasia and epilepsy: functional imaging using single photon emission computed tomography and positron emission tomography.

Functional imaging using single photon emission CT and positron emission tomography have made important contributions to the evaluation of patients with medically intractable epilepsy and cortical dysplasia by identifying patients who previously were not considered surgical candidates. This article reviews the role of functional imaging in the presurgical evaluation of this patient population.

The use of subdural grids in the management of focal malformations due to abnormal cortical development.

Because of the spatial limitations of scalp electroencephalographic recordings and the lack of reproducible results using functional MR imaging techniques to map various eloquent areas of the cortex, the accurate mapping of the extent of epileptogenicity and function currently are best accomplished through invasive electrocorticographic recordings and direct cortical stimulation, respectively. The purpose of this article is to give an overview of the presurgical diagnostic tools used in patients with suspected malformations caused by abnormalities of cortical development with special emphasis on subdural electrodes. The indications and the role of invasive subdural electrode evaluation are discussed.

Surgery for focal cortical dysplasia.

Interest in surgery for focal cortical dysplasia has grown with the enhanced ability to detect these lesions preoperatively with modern imaging techniques. This article focuses on the surgical management of epilepsy associated with focal cortical dysplasia. The authors highlight the approaches practiced at Yale University, review their recent series of operative cases, and discuss a representative case example to illustrate important aspects of surgical strategy.

Surgery for hemispheric malformations of cortical development.

The treatment of patients with hemispheric malformations of cortical development presents a challenging clinical problem that often requires surgical intervention. The development of a safe and effective treatment strategy for these patients requires an extensive work-up with a great deal of attention to a variety of multidisciplinary testing methods. In this article, the authors describe the presurgical evaluation of patients with hemispheric malformations of cortical development and epilepsy and review the surgical techniques currently available. They also detail some of the controversial issues regarding surgical treatment of these patients.

Hemispherectomy techniques.

Hemispherectomy techniques have undergone multiple changes. Because of these changes, several current alternatives are described. The need for an extensive procedure in young children with special pediatric requirements is the background for the development of newer and more microsurgically oriented techniques aimed at reducing the intraoperative problems and late postoperative complications. This article reviews the strengths and the disadvantages of the currently used procedures in light of special requirements for hemispheric dysplasias.

Outcome after surgical treatment.

Approximately 50% of patients will become seizure free following surgery for intractable epilepsy caused by malformations of cortical development. Another 20% have only rare seizures. The outcome is dependent on the completeness of the resection as determined by MR imaging and electroencephalography. The surgical procedures performed for malformations of cortical development in infants, children, and adults include lobar and multilobar resections or hemispherectomy.

Unusual vascular dysplasia presenting as an intra-abdominal catastrophe.

A case of severe, generalised vascular dysplasia presenting as an intra-abdominal catastrophe is reported. Associated clinical features included cutis marmorata telangiectatica congenita, congenital glaucoma, hypertension, and focal fits. The case has been reported in view of its rarity and nature of presentation.

Positive epileptiform discharges in children with neuronal migration disorders.

Most epileptiform abnormalities show a negative polarity on EEG. Focal positive spike waves have rarely been identified in seizure disorders and are generally associated with physiological and neurological impairment. Results of EEG, computed tomography, MRI, and pathologic studies of 15 children with focal neuronal migration disorders who underwent surgery for refractory localization-related epilepsy were compared to examine the association between positive discharges and other findings. Subjects were studied both ictally and interictally by scalp EEG with the International 10-20 system and zygomatic or sphenoidal electrodes, and video EEG telemetry. The 5 children with positive discharges were significantly more likely to develop hemiparesis during the preoperative period (P < or = .025). Correlations were observed between positive discharges and lesions apparent on MRI situated around the rolandic fissure (P < or = .025). Children with positive discharges had a significantly less favorable outcome after surgical treatment (P < or = .025). Positive epilepti-form discharges in children with neuronal migration disorders may signal a more dysfunctional cortex leading to a focal neurological deficit or a more extended lesion than is detected on MRI. This would explain the less favorable outcome of seizures after surgery, since the epileptogenic areas and neuronal migration lesions cannot be completely resected.

Epilepsias benignas familiares de la infancia

La epilepsia rolándica pertenece al grupo de síndromes epilépticos primarios, parciales benignos de la infancia, se caracteriza por tener un inicio entre los dos y los 13 años de edad; se presemnta en niños normales, cuya manifestación usual es una crisis parcial durante el sueño que puede llegar a generalizar. El hallazgo característico a nivel del elctroencefalograma está dado por descargas paroxísticas de punta-onda en la región centrotemporal. Este tipo de epilepsia remite espontáneamente antes de la edad adulta (1,2). Se ha visto que este desorden se presenta en un 15 por ciento a un 20 por ciento de los pacientes con epilepsia. Su presentación es más común en el sexo masculino con una relación 2:1, aunque hay autores que hablan de una relación 3:1. La edad de inicio está entre los dos y los 13 años, siendo su máxima aparición entre los 9 y los 10 años de edad. Las convulsiones casi siempre desaparecen espontáneamente después de los 16 años de edad (3,4). El propósito de esta revisión fue evaluar las diferentes manifestaciones clínicas, evolución y manejo que se realiza en este tipo de crisis, practicando un estudio descriptivo entre 1987 y 1994 de pacientes con diagnóstico de epilepsia rolándica en el servicio de neuropediatría del Hospital Militar Central

An unusual presentation of focal cortical dysplasia.

In a case of histologically proved focal cortical dysplasia, there was an absence of cortex-white matter delineation in the right parietooccipital area only on the T2-weighted images. This pattern correlated with the gross and histologic findings obtained on the resected cerebral tissue.

Operative strategies for patients with cortical dysplastic lesions and intractable epilepsy.

Cortical dysplastic lesions (CDLs) are usually identified by magnetic resonance imaging (MRI). Clinical, electrographic and histologic findings suggest that focal CDLs (FCDLs) are highly epileptogenic, often involve the rolandic cortex, and can present variable degrees of histopathologic abnormalities. An ictal or "ictal-like" bursting pattern of electrographic activity was recorded over dysplastic cortex in 65% of our patients. Resective surgery can eliminate or significantly reduce seizure frequency in many medically intractable patients, depending on lesion location, degree, and extent of histopathologic abnormalities. Best results are achieved when complete or major excision of both the MRI-visible lesion and the cortical areas displaying ictal electrographic activity can be performed. This is more likely when the degree of histopathologic abnormality is mild to moderate or when the lesion is in a temporal lobe. More severe histopathologic abnormalities and central insular or multilobar lesions usually lead to less favorable results: either major excision of the visualized lesion is impractical or the lesion is microscopically more extensive than shown by MRI. Multilobar resection or hemispherectomy for patients with infantile spasms associated with CDLs and for patients with hemimegalencephaly are often associated with dramatic improvement in seizure control. Callosotomy can be performed in selected patients with diffuse CDLs who have intractable drop attacks.

Migrational disorders of the brain.

Migrational disorders are the result of a wide range of fetal diseases that become manifest before midgestation. Recognition may not tell us everything about the etiology of these disorders, but it certainly tells us their time of origin. More diagnostic clues may be added to the case of an individual patient if the type of migrational disorder is taken into consideration.