1/151. Surgical management of pediatric tumor-associated epilepsy.brain tumors are a common cause of seizures in children. Early surgical treatment can improve seizure outcome, but controversy exists regarding the most appropriate type of surgical intervention. Some studies suggest tumor resection alone is sufficient, while others recommend mapping and resection of the surrounding epileptogenic foci to optimize seizure outcome. To address this issue, we reviewed the charts of 34 pediatric patients aged 18 months to 20 years with medically intractable epilepsy and primary brain tumors. The average age at operation was 12.6 years, and patients had seizures for an average of 6.4 years. The majority of tumors were located in the temporal lobe. Seventeen patients, because of tumor location near an eloquent area, underwent extraoperative mapping using subdural electrode grids prior to definitive tumor resection. Fourteen of these patients had a gross total tumor resection, yet only two had a distinct zone of ictal onset identified and resected. The remaining 17 patients had tumors either in the nondominant hemisphere or far removed from speech-sensitive areas, and therefore did not undergo extraoperative subdural electroencephalograph mapping. Fourteen of these patients also had a gross total tumor resection, while none had intraoperative electrocorticography to guide the resection of additional nontumoral tissue. overall, of the 28 patients treated with a gross total tumor resection, 24 (86%) are seizure free, while the other four are significantly improved. Of the six patients who had a subtotal tumor removal, five have persistent seizures. The mean follow-up was 3.6 years. We conclude that in children and adolescents, completeness of tumor resection is the most important factor in determining seizure outcome. The routine mapping and resection of epileptogenic foci might not be necessary in the majority of patients. As a corollary, the use of subdural electrode grids in pediatric patients with tumor-associated epilepsy should be limited to cases requiring extraoperative cortical stimulation for localization of nearby eloquent cortex.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
2/151. Characterization of nodular neuronal heterotopia in children.Neuronal heterotopia are seen in various pathologies and are associated with intractable epilepsy. We examined brain tissue from four children with subcortical or periventricular nodular heterotopia of different aetiologies: one with severe epilepsy following focal brain trauma at 17 weeks gestation, one with hemimegalencephaly and intractable epilepsy, one with focal cortical dysplasia and intractable epilepsy, and one dysmorphic term infant with associated hydrocephalus and polymicrogyria. The connectivity of nodules was investigated using histological and carbocyanine dye (DiI) tracing techniques. DiI crystal placement adjacent to heterotopic nodules revealed numerous DiI-labelled fibres within a 2-3 mm radius of the crystals. Although we observed labelled fibres closely surrounding nodules, the majority did not penetrate them. Placement of DiI crystals within nodules also identified a limited number of projections out of the nodules and in one case there was evidence for connectivity between adjacent nodules. The cellular and neurochemical composition of nodules was also examined using immunohistochemistry for calretinin and neuropeptide y (NPY), which are normally expressed in GABAergic cortical interneurons. Within heterotopic nodules from all cases, numerous calretinin-positive neurons were identified, along with a few cell bodies and many processes positive for NPY. Calretinin-positive neurons within nodules were less morphologically complex than those in the cortex, which may reflect incomplete differentiation into an inhibitory neuronal phenotype. There were also abnormal clusters of calretinin-positive cells in the overlying cortical plate, indicating that the migratory defect which produces heterotopic nodules also affects development of the cortex itself. Thus, heterotopic nodules consisting of multiple neuronal cell types are associated with malformation in the overlying cortical plate, and have limited connectivity with other brain regions. This abnormal development of connectivity may affect neuronal maturation and consequently the balance of excitation and inhibition in neuronal circuits, leading to their epileptogenic potential.- - - - - - - - - - ranking = 2keywords = cortex (Clic here for more details about this article) |
3/151. Ictal bradycardia in a patient with a hypothalamic hamartoma: a stereo-EEG study.Little is known about bradycardia and cardiac asystole which occur during partial epileptic seizures, especially whether they relate to ictal involvement of well-defined cortical areas. Several reports based on simultaneous electrocardiographic and intracranial depth electroencephalographic monitoring have shown that either the fronto-orbital cortex or the amygdalohippocampal complex could be responsible for such cardiac variations. We performed stereo-EEG recordings in a patient with refractory localization-related epilepsy associated with a hypothalamic hamartoma. We found that other cortical areas, such as the frontocentral region and the temporal neocortex, can contribute to the genesis of ictal bradyarrhythmia. Second, the lesion per se, although located within the hypothalamus, is not involved with this phenomenon.- - - - - - - - - - ranking = 2keywords = cortex (Clic here for more details about this article) |
4/151. Treatment of seizures in subcortical laminar heterotopia with corpus callosotomy and lamotrigine.Focal and generalized cortical dysgeneses are sometimes seen on the magnetic resonance images (MRI) of patients with epilepsy. Subcortical laminar heterotopia are bilateral collections of gray matter in the centrum semiovale that resemble a band or "double cortex" on MRI. We studied one male and two female patients with subcortical laminar heterotopia who had moderate to severe developmental delay, early-onset epilepsy, and medically refractory seizures. Atonic, atypical absence, tonic, myoclonic, complex partial, and generalized tonic-clonic seizures were recorded. Interictal and ictal electroencephalographic patterns were generalized and, less commonly, multifocal. Two years after corpus callosotomy, one patient was free of generalized tonic-clonic and atonic seizures, but the other patient who had undergone callosotomy had no significant reduction in seizure frequency. With lamotrigine treatment, the patient who had not had surgery had complete cessation of monthly episodes of status epilepticus and a dramatic reduction of generalized tonic-clonic seizures, and the other patient who received lamotrigine had a 50% reduction of her atonic seizures. In patients with subcortical laminar heterotopia, atonic and generalized tonic-clonic seizures can be substantially reduced or eliminated by corpus callosotomy or treatment with lamotrigine.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
5/151. Surgical treatment of intractable epilepsy originating from the primary sensory area of the hand--case report.A 14-year-old right-handed girl presented with intractable epilepsy originating from the primary sensory area of the hand, manifesting as sensory partial seizures in the left hand with secondary generalization. Neurological examination showed no abnormal findings. magnetic resonance imaging, magnetoencephalography, and cortical stimulation using chronic subdural electrodes demonstrated a lesion located in the primary sensory cortex of the hand, in which the ictal onset zone was identified by 24-hour intracranial electroencephalographic recording. Surgical resection of the lesion and multiple subpial transections of the adjacent cortices were performed. The histological diagnosis was dysembryoplastic neuroepithelial tumor (DNT). She was completely free of seizures without permanent sensory deficits postoperatively. DNT located in the primary sensory hand area may be resectable without causing postoperative sensory deficits, if accurate functional mapping and surgical resection are performed.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
6/151. A novel mutation of the doublecortin gene in Japanese patients with X-linked lissencephaly and subcortical band heterotopia.The doublecortin (DCX) gene was recently found to be involved in patients with X-linked lissencephaly and subcortical band heterotopia or double cortex syndrome. We have studied the coding regions of the DCX gene in 11 Japanese patients with cortical dysplasia and have identified three different mutations (R186C in exon 3, R272X and R303X in exon 5) in four sporadic female cases. R272X, which has been detected in two unrelated cases, is a novel mutation. Although the number of cases studied remains limited, exon 5 may be a common mutational site in Japanese patients in contrast to many previous reports concerning exons 2 and 3.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
7/151. Outcome of multiple subpial transections for autistic epileptiform regression.Treatment options for atypical forms of landau-kleffner syndrome (LKS) are not well delineated. Many patients with typical LKS fail to respond to antiepileptic drug treatment, but some benefit from multiple subpial transections (MSTs). The authors report seven patients with autism or autistic epileptiform regression who responded in varying degrees to MSTs after failed medical management. These patients derived from an original cohort of 36 children (29 males, seven females, ranging from 2 years, 3 months to 11 years, 3 months, mean age = 5 years, 8 months) with a history of language delay or regression, as well as varying degrees of social and behavioral abnormalities, who were evaluated with video-electroencephalogram (EEG) monitoring over a 2-year period. Fifteen patients had clinical seizures (11 of the 19 children with autistic epileptiform regression and four of 12 autistic children). epilepsy was refractory to medication in seven. Surgical treatment variously involved MSTs of the left neocortex in temporal, parietal, and frontal regions, often including regions within the classic perisylvian language areas. One patient also had a left temporal lobectomy. In all seven patients, seizure control or EEG improved after MSTs. language, social, and overall behavior improved to a moderate degree, although improvements were temporary in most. Autistic epileptiform regression resembles LKS in that both may respond to MST. MST is used to treat epilepsy in eloquent regions. The responsiveness of autistic epileptiform regression to MST buttresses the argument that autistic epileptiform regression is a form of focal epilepsy.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
8/151. motor cortex localization using functional MRI and transcranial magnetic stimulation.OBJECTIVE: Congenital brain lesions producing focal seizures may be accompanied by reorganization of the areas responsible for motor and sensory functions within the brain due to a phenomenon that has been termed "neuronal plasticity." This can be studied using functional MRI (fMRI) and transcranial magnetic stimulation (TMS). Using either method, the motor cortex can be localized noninvasively, but to date there have been few studies correlating the level of agreement between the two techniques. methods: We used fMRI and TMS to localize the motor cortex in a young woman with intractable focal seizures, congenital left arm weakness, and a dysplastic right hemisphere on MRI. RESULTS: There was excellent agreement in the localization of motor representation for each hand. Both were predominantly located in the left hemisphere. fMRI also showed an area of posterior activation in the right hemisphere, but there was no evidence of descending corticospinal projections from this site using TMS, direct cortical stimulation, and Wada testing. CONCLUSIONS: Functional MRI (fMRI) and transcranial magnetic stimulation (TMS) were successfully used to localize cortical motor function before epilepsy surgery. Each technique demonstrated migration of motor function for the left hand to the left motor cortex. After resection of the dysplastic right precentral gyrus there was no permanent increase in weakness or disability. The two techniques are complementary; fMRI indicates all cortical areas activated by the motor task, whereas TMS identifies only those areas giving rise to corticospinal projections.- - - - - - - - - - ranking = 7keywords = cortex (Clic here for more details about this article) |
9/151. Somatosensory representation in patients who have undergone hemispherectomy: a functional magnetic resonance imaging study.OBJECT: Removal or disconnection of an entire cerebral hemisphere is occasionally used to treat refractory seizures. patients who have undergone a hemispherectomy provide useful models to study the reorganization of cortical somatosensory representation. This plasticity may be a consequence of the pathological lesion, the hemispherectomy itself, or both. methods: Three patients who had undergone hemispherectomy were studied with functional magnetic resonance (fMR) imaging. Responses to sensory stimulation in normal hands and hands opposite the lesioned hemisphere were studied. Multislice T2*-weighted gradient-echo echoplanar images were obtained using a 1.5-tesla MR imager. The activation condition consisted of somatosensory stimulation of the index finger. A T1-weighted anatomical MR image was acquired. The fMR and anatomical MR images were coregistered, and statistically significant activation foci (p < 0.01) were identified. Stimulation of the normal hand produced activation in the primary somatosensory cortex (SI) in all patients. Stimulation of the impaired hand resulted in activation of the ipsilateral parietal operculum (second somatosensory area [SII]) and posterior parietal lobe (Brodmann's Area 7) in all cases, but no activation was elicited in the SI in any patient. In addition, other areas within the ipsilateral frontal and parietal lobes were activated in some individuals. CONCLUSIONS: Residual somatosensory function in the hand opposite the lesioned hemisphere is mediated by the SII and other cortical regions in the intact hemisphere, without involvement of the SI.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
10/151. Magnetoencephalographic analysis of periodic lateralized epileptiform discharges (PLEDs).OBJECTIVE: To clarify the spatial and temporal distribution of the electroencephalographic (EEG) pattern termed 'periodic lateralized epileptiform discharges' (PLEDs), we performed magnetoencephalography (MEG) to analyze PLEDs in a patient with a right parietal metastasis associated with meningeal carcinomatosis. methods: A 37-channel biomagnetometer was used to simultaneously record the EEG and MEG. Equivalent current dipole (ECD) source localization was calculated based on a single-dipole model and mapped onto a magnetic resonance image. Single-photon emission computed tomography with technetium-99-hexamethyl-propyleneamine oxime (HMPAO-SPECT study) was also performed during both presence and absence of PLEDs according to an EEG monitor. RESULTS: By EEG the PLEDs, predominantly right-sided, consisted of a typical negative triphasic spike followed by a slow negative wave. By MEG the PLEDs had a sequence with 3 distinct components. ECDs in the 3 components were localized to the cortex around the lesion, although exact localization and dipole direction varied between components. HMPAO-SPECT demonstrated hypoperfusion of the lesion and adjacent cortex during both quiescence and appearance of PLEDs. CONCLUSION: Our results indicate that PLEDs originated from the hypoperfused cortex surrounding the lesion.- - - - - - - - - - ranking = 3keywords = cortex (Clic here for more details about this article) |
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