1/107. stroke and seizures as the presenting signs of pediatric hiv infection.The authors report two pediatric patients with definite human immunodeficiency virus infection whose initial presentation was stroke and seizure. The first patient was a 3-year-old female who developed acute hemiparesis as the first manifestation. The other, a 2-month-old infant, had focal seizures secondary to cerebral infarction. Investigations revealed ischemic infarction of the thalamus, hypothalamus, and internal capsule in the first patient and cerebral cortex in the second. Further investigations failed to demonstrate any other causes of these cerebral infarctions. Opportunistic infection of the central nervous system was not documented. The authors emphasize that cerebrovascular accident may be the initial presentation in human immunodeficiency virus infection in children. Human immunodeficiency virus infection must be included in the differential diagnosis, and testing for the disease is mandatory in the investigation of stroke in any child who is at risk of having this infection.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
2/107. Ictal hemiparesis.Two subjects with ictal hemiparesis are described. Both children presented with evolving paresis associated with seizure activity. Structural neuroimaging remained consistently normal, although EEG demonstrated slow-wave activity, and SPECT scanning in one child showed perfusion asymmetry. Both children had resolution of the hemiparesis when seizure activity was adequately controlled. The historically proposed pathophysiology of ictal hemiparesis is that of inhibition of the somatosensory and motor areas of the cortex. The presence of an evolving hemiparesis and seizure activity associated with normal neuroimaging should prompt consideration of ictal hemiparesis. Confirmation of this rare diagnosis can only be made when seizure control leads to resolution of the paresis.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
3/107. cleidocranial dysplasia with neonatal death due to central nervous system injury in utero: case report and literature review.cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
4/107. The electroencephalogram through a software microscope: non-invasive localization and visualization of epileptic seizure activity from inside the brain.OBJECTIVE: We developed a novel non-invasive analysis to localize the source and visualize the time course of electrical activity generated inside the brain but unclear from the scalp. This analysis applies to signals with unique waveform characteristics, such as seizures. methods: The method extracts activity from an EEG data matrix as a spatiotemporal component having waveforms uncorrelated to the other concurrent activities. The method also provides the location and orientation of the dipole generating this activity. We applied this method to ten scalp seizures in three patients with temporal lobe epilepsy and single-focus seizures confirmed by intracerebral recordings. A realistic head model based on MRI was used for computation of field distributions. RESULTS: When seizure activity was still not visually identifiable on the scalp, the method demonstrated in all scalp seizures a source in the temporal neocortex corresponding clearly to the region of seizure activity in intracerebral recordings. Frequency characteristics of the estimated activities also resembled those of the intracerebral seizures. CONCLUSIONS: This method enables estimation of focal brain activity when its effect on scalp EEG is unclear to visual examination. It works in situations where currently available source analyses methods, which require noiseless definite activity, are not applicable.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
5/107. Focal polymicrogyria in mother and son.This 9-year-old boy was admitted at the age of 2 with a diagnosis of congenital hemiparesis while the rest of physical and neurological examination was normal. His score in the Wechsler intelligence scale was 80. Right fronto-parietal cortical dysplasia with hemisphere atrophy was evident by computerized tomography scanning and magnetic resonance imaging. The latter, also disclosed abnormal thick cortex which was interpreted as polymicrogyria or pachygyria. karyotype was normal. He had a hemifacial motor seizure at the age of 7. At the age of 8 frequent atonic or inhibitory seizures were presented. Asymmetric bilateral spike discharges with high voltage in the right hemisphere during the EEG recording were found. His mother, a 35-year-old woman (Full scale; adult intelligence scale: 85) also had congenital hemiparesis. She never had seizures and her EEG was normal. magnetic resonance imaging disclosed right fronto-parietal cortical dysplasia with ipsilateral hemisphere atrophy. karyotype was normal. Our cases should be interpreted as a familial presentation of the anomaly, probably with autosomal-dominant transmission.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
6/107. Surgical management of intractable epilepsy associated with cerebral neurocytoma.Neuronal neoplasms of the CNS constitute a rarely encountered group of tumors. This report concerns the surgical management of seizures encountered in four cases (ranging from 2 to 10 years-of-age at onset; consisting of two males and two females) of a recently recognized morphologically unique tumor, called 'cerebral neurocytoma'. All patients were associated solely with intractable complex partial seizures. The tumor involved the temporal lobe in two cases, and the frontal in two. magnetoencephalography (MEG) clearly demonstrated an accumulation of equivalent current dipoles originating from the interictal spikes on the cortex around the tumor. On intra-operative electrocorticography (ECoG), the epileptogenic zone was topographically distinct from the region of the tumor. No definite ECoG activities were observed at the tumor site, although this tumor did consist of small mature neuronal cells. Either a complete or a subtotal resection of the tumor and the epileptogenic cortex was performed and, post-operatively, universal freedom from seizures was demonstrated in all patients. A histological examination of the epileptogenic cortex revealed the presence of minute cortical dysplasia or tumor involvement in the hippocampus. A resection of the epileptogenic cortex along with the tumor was thus found to improve the seizure outcome in patients with neurocytoma-associated epilepsy without inducing any identifiable neurological deficits attributable to the incremental resection.- - - - - - - - - - ranking = 4keywords = cortex (Clic here for more details about this article) |
7/107. Encephalocraniocutaneous lipomatosis (Fishman syndrome): a rare neurocutaneous syndrome.Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous syndrome comprising unilateral cranial lipomas, lipodermoids of the eye and brain abnormalities. A 3-year-old boy who presented at birth with a scalp lipoma and an ipsilateral epibulbar lipodermoid is described. Infantile spasms developed at 9 months of age and cerebral imaging showed thickened and calcified cortex at the right occiput and hemiatrophy of the right hemisphere. These features were consistent with ECCL. Most children with ECCL have significant developmental delay, but we have found that control of seizures was associated with a significant improvement in developmental outcome.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
8/107. Cortical reorganization in linear nevus sebaceous syndrome: a multimodality neuroimaging study.The authors report the findings of multimodal structural, functional, and metabolic imaging in a patient with linear nevus sebaceous syndrome, intractable seizures, and right megalencephaly. Despite nearly continuous paroxysmal electrical activity from the megalencephalic region, imaging studies suggested nonfunctional tissue in this region with reorganization of cortical function to the unaffected ipsilateral hemisphere. hemispherectomy has been successfully performed in previous patients; however, it could have led to marked left hemiparesis with significant functional morbidity in this patient because of ipsilateral reorganization of the primary motor cortex.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
9/107. Intractable epilepsy after a functional hemispherectomy: important lessons from an unusual case. Case report.Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography, Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review. Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.- - - - - - - - - - ranking = 3keywords = cortex (Clic here for more details about this article) |
10/107. Chordoid meningioma.Chordoid meningioma is a relatively rare variant that is often associated with peritumoral lymphoplasmacellular infiltration causing Castleman syndrome (CS). We present a 44-year-old woman with chordoid meningioma not associated with CS. The patient presented with epilepsy and right hemiparesis (Todd's palsy) on admission. The radiological findings revealed an extraaxial mass lesion in the premotor cortex. They were compatible with a preoperative diagnosis of meningioma. No physical abnormalities related to CS were detected. A left frontal craniotomy was performed. The tumor surface was gelatinous, and it was totally resected with the attached dura mater (Simpson grade I). The patient had an uneventful recovery, and her seizures subsided. The pathological findings of the specimens revealed nests and cords of spindle and epithelioid cells with abundant myxoid matrix, mimicking the features of chordoma. On the basis of radiological, immunohistochemical, and electron microscopic findings, chordoid meningioma was verified, and a review of the literature was performed.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
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