OBJECTIVETo analyze the relationship between the ictal onset zone and dominant interictal epileptiform foci in tuberous sclerosis complex (TSC) patients.

METHODClinical data of 20 patients with TSC which had epileptic seizures during Video-EEG monitoring was assessed. Consistency and dominance of focal interictal epileptiform activity and the ictal onset zone were identified. Concordance between interictal and ictal findings was analyzed.

RESULTOf the 20 patients, 7 were female, and 13 were male. The age of epilepsy onset was from 15 d to 6 years. The Video-EEG monitoring age was from 6 months to 11 years. Family history was found in three cases. Abnormality in neuroimaging existed in 17 of 18 patients who were examined. Interictal EEG showed hypsarrhythmia in 3 patients, multifocal epileptiform activity with a dominant focus in 12 patients, both focal and generalized discharges in 2 patients, and only focal discharges in 3 patients. The seizures types during EEG monitoring included epileptic spasms, partial seizure, atypical absence, and generalized or focal myoclonic seizure. The most common seizure type was partial seizure and then epileptic spasms. EEG in 4 patients with epileptic spasms showed ictal generalized discharges and interictal hypsarrhythmia or generalized discharges. Clinical manifestation of epileptic spasms was asymmetric in 3 patients. Lateralization and location of interictal and ictal discharges were consistent in 2 of the 3 patients, while only lateralization consistency in 1 of the 3 patients. Partial seizures as the only seizure type were monitored in 13 patients. Of the 13 patients, lateralization and location of interictal and ictal discharges were inconsistent in 2 patients (15%), consistent in 8 patients (62%), lateralization or location consistent in 2 patients (15%). One case could not be analyzed because of uncertainty of lateralization and location of seizure onset.

CONCLUSIONIn the majority of patients with TSC, multifocal interictal epileptiform activity is present, in which a most dominance of focal epileptiform activity could be found. For some epileptic seizures or the majority of partial seizures, the ictal onset zone is concordant with the dominance of focal interictal epileptiform foci. The concordance might have positioning reference significance for preoperative evaluation of epilepsy surgery.

Brain ; pathology ; physiopathology ; Brain Mapping ; methods ; Cerebral Cortex ; pathology ; physiopathology ; Child ; Child, Preschool ; Electroencephalography ; statistics & numerical data ; Epilepsies, Partial ; diagnosis ; etiology ; physiopathology ; Female ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Predictive Value of Tests ; Retrospective Studies ; Seizures ; diagnosis ; etiology ; physiopathology ; Tuberous Sclerosis ; complications ; diagnosis ; physiopathology

PURPOSE: We investigated the localizing and lateralizing values of auras in patients with lesional partial epilepsy on an outpatient basis. MATERIALS AND METHODS: A total of 276 subjects were retrospectively selected for this study if they had a unilateral single lobar lesion based on magnetic resonance image (MRI) results, and their scalp electroencephalography (EEG) findings were not discordant with the MRI-defined lobar localization and lateralization. According to the lesion locations, subjects were considered as having mesial temporal (MTLE), lateral temporal (LTLE), frontal (FLE), parietal (PLE), or occipital (OLE) lobe epilepsies. Auras were classified into 13 categories. RESULTS: A hundred and seventy-six subjects (63.8%) had experienced at least one aura. FLE subjects had the fewest number of auras. Epigastric and psychic auras were frequent among MTLE subjects, while visual auras were common in those with PLE and OLE. Somatosensory auras and whole body sensations were more frequent in the subjects with PLE than those without. Autonomic auras were more common in MTLE subjects than in LTLE subjects. Dysphasic auras were more frequently found in left-sided epilepsies. Five pairs of aura categories showed concurrent tendencies, which were the epigastric and autonomic auras, autonomic and emotional auras, visual and vestibular auras, auditory and vestibular auras, and whole-body sensation and auditory auras. Autonomic and emotional auras had a concurrent tendency in left-sided epilepsies, but not in right-sided epilepsies. CONCLUSION: Our results support the previously known localizing value of auras, and suggest that dysphasic auras and the association of emotional and autonomic auras may have a lateralizing value.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Epilepsies, Partial/*pathology ; Epilepsy/*pathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

This study was performed to assess the usefulness of magnetoencephalography (MEG) as a presurgical evaluation modality in Korean pediatric patients with lesional localization-related epilepsy. The medical records and MEG findings of 13 pediatric patients (6 boys and 7 girls) with localization-related epilepsy, who underwent epilepsy surgery at Seoul National University Children's Hospital, were retrospectively reviewed. The hemispheric concordance rate was 100% (13/13 patients). The lobar or regional concordance rate was 77% (10/13 patients). In most cases, the MEG spike sources were clustered in the proximity of the lesion, either at one side of the margin (nine patients) or around the lesion (one patient); clustered spike sources were distant from the lesion in one patient. Among the patients with clustered spike sources near the lesion, further extensions (three patients) and distal scatters (three patients) were also observed. MEG spike sources were well lateralized and localized even in two patients without focal epileptiform discharges in the interictal scalp electroencephalography. Ten patients (77%) achieved Engel class I postsurgical seizure outcome. It is suggested that MEG is a safe and useful presurgical evaluation modality in pediatric patients with lesion localization-related epilepsy.
Adolescent ; Brain/radionuclide imaging ; Brain Diseases/pathology ; Child ; Child, Preschool ; Epilepsies, Partial/pathology/*surgery ; Female ; Ganglioglioma/pathology ; Humans ; Infant ; Magnetic Resonance Imaging ; *Magnetoencephalography ; Male ; Malformations of Cortical Development/pathology ; Neoplasms, Neuroepithelial/pathology ; Positron-Emission Tomography ; Retrospective Studies ; Seizures/diagnosis

OBJECTIVETo evaluate the alteration of subunits composition in NMDA receptor and the alterations of the expression and distribution of NMDA receptors and parvalbumin (PV)-positive neurons in focal cortical dysplasia (FCD) cortices.

METHODSTwenty cases of FCD samples (including all four subtypes of FCD) obtained during epilepsy surgery and 4 controls were analysed by immunohistochemical staining for NR1, NR2A/B and PV.

RESULTSIncreased expression of NR1 was detected in the giant neurons and dysmorphic neurons in FCD; while pronounced expression of NR2A/B was detected in immature neurons, giant neurons and dysmorphic neurons of FCD, especially in somata and processes of the immature neurons. Compared with the controls, FCD cortices showed prominent scattered arrangement of PV positive neurons and fibers, dramatically decreased number of PV positive interneurons and PV background staining, especially in foci of FCD II subtype.

CONCLUSIONThere are increased expressions of NR1 and NR2A/B subunits in FCD abnormal neurons, as well as scattered and reduced expressions of PV positive neurons and fibers in FCD cortices.

Adolescent ; Biomarkers, Tumor ; metabolism ; Cerebral Cortex ; metabolism ; pathology ; Child ; Child, Preschool ; Epilepsies, Partial ; pathology ; Female ; Gene Expression Regulation ; Humans ; Infant ; Interneurons ; metabolism ; Male ; Malformations of Cortical Development ; pathology ; Neurons ; metabolism ; Parvalbumins ; analysis ; isolation & purification ; Receptors, N-Methyl-D-Aspartate ; genetics ; metabolism

Child ; Epilepsies, Partial ; etiology ; Hamartoma ; complications ; diagnosis ; pathology ; Humans ; Hypothalamic Diseases ; complications ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Male ; Puberty, Precocious ; etiology

OBJECTIVETo study the clinicopathologic features and radiologic findings of dysembryoplastic neuroepithelial tumor (DNT).

METHODSThe clinical presentations, radiologic findings, histologic features and immunophenotype of 9 cases of DNT were analyzed.

RESULTSThe age of patients ranged from 12 to 51 years (mean age = 32 years). Most presented with partial seizures, sometimes accompanied by transient aphasia, agraphia and decreased visual acuity. One case was asymptomatic and discovered incidentally during a routine check-up. All patients had no neurological deficit found on physical examination. All tumors were located in the supratentorial cerebral cortex. There was no peritumoral edema or space-occupying effect on radiologic examination. The tumors involved either frontal lobe (number = 4), temporal lobe (number = 4), frontoparietal lobe (number = 1) . Two cases showed cystic changes. Two histologic variants of DNT were recognized: simple (number = 3) and complex (number = 6). Simple variant was composed mainly of the glioneuronal element, accompanied by surrounding oligodendrocyte-like cells, and the complex variant contained a low-grade glioma component, in addition to the glioneuronal element and sometimes foci of cortical dysplasia.

CONCLUSIONSDNT is a benign tumor with excellent prognosis after surgical excision. Local recurrence is rare. Complex variant of DNT needs to be distinguished from other types of low-grade glioma.

Adolescent ; Adult ; Brain Neoplasms ; complications ; metabolism ; pathology ; surgery ; Cerebral Cortex ; metabolism ; pathology ; Child ; Child, Preschool ; Epilepsies, Partial ; etiology ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive ; complications ; metabolism ; pathology ; surgery ; Neurofilament Proteins ; metabolism ; Oligodendroglia ; pathology ; ultrastructure ; S100 Proteins ; metabolism ; Survival Rate ; Synaptophysin ; metabolism

Aicardi syndrome is a severe congenital disorder characterized by infantile spasms, chorioretinal lacunae, and agenesis or hypogenesis of the corpus callosum. A 6 month old female had developed abnormal eye movement and seizures of the complex partial type and myoclonic type. MRI pictures of the patient revealed the presence of genu associated with agenesis of the rest of corpus callosum. A funduscopic examination revealed bilateral small, solitary, pale areas with sharp borders, some of which had minimal surrounding pigmentation (chorioretinal lacunae), especially clustered around the disc, and they were more prominent on the left side. We report here on the unusual findings of a complex partial seizure, myoclonic seizure and the atypical EEG finding in addition to the well-known characteristic clinical and imaging findings of a patient with Aicardi syndrome.
Anticonvulsants/therapeutic use ; Arachnoid Cysts/*pathology ; Corpus Callosum/*abnormalities ; Electroencephalography ; Epilepsies, Myoclonic/drug therapy/*pathology ; Epilepsies, Partial/drug therapy/*pathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Retinal Diseases/*pathology ; Spasms, Infantile/drug therapy/*pathology ; Syndrome

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Electroencephalography ; Epilepsies, Partial ; diagnosis ; drug therapy ; Humans ; Infant ; Occipital Lobe ; pathology ; Prognosis

PURPOSE: To investigate the various ictal perfusion patterns and find the relationships between clinical factors and different perfusion patterns. MATERIALS AND METHODS: lnterictal and ictal SPECT and SPECT subtraction were performed in 61 patients with partial epilepsy. Bath positive images showing ictal hypoperfusion and negative images revealing ictal hypoperfusion were obtained by SPECT subtraction. The ictal perfusion patterns of subtracted SPECT were classified into focal hypoperfusion, hypoperfusion-plus, combined hypoperfusion-hypoperfusion, and focal hypoperfusion only. RESULTS: The concordance rates with epileptic focus were 91.8% in combined analysis of ictal hypoperfusion and hypoperfusion images of subtracted SPECT, 85.2% in hypoperfusion images only of subtracted SPECT, and 68.9% in conventional ictal SPECT analysis. Ictal hypoperfusion occurred less frequently in temporal lobe epilepsy (TLE) than extratemporal lobe epilepsy. Mesial temporal hypoperfusion alone was seen only in mesial TLE while lateral temporal hypoperfusion alone was observed only in neocortical TLE. Hippocampal sclerosis had much lower incidence of ictal hypoperfusion than any other pathology. Some patients showed ictal hypoperfusion at epileptic focus with ictal hypoperfusion in the neighboring brain regions where ictal discharges propagated. CONCLUSION: Hypoperfusion as well as hypoperfusion in ictal SPECT should be considered for localizing epileptic focus. Although the mechanisrn of ictal hypopertusion could be an intra-ictal early exhaustion of seizure focus or a steal phenomenon by the propagation of ictal discharges to adjacent brain areas, further study is needed to elucidate it.
Baths ; Brain ; Epilepsies, Partial* ; Epilepsy ; Epilepsy, Temporal Lobe ; Humans ; Incidence ; Pathology ; Perfusion* ; Sclerosis ; Seizures ; Tomography, Emission-Computed, Single-Photon*

BACKGROUND: Intractable partial epilepsy is the most frequent manifestation in cortical dysplasia (CD). The surgical removal of the epileptogenic focus in CD is the main therapeutic option for achieving seizure control. However, it is dif-ficult to predict surgical outcome by current diagnostic methods. We investigated 35 subjects with intractable epilepsy due to CD, in order to know the relationship the extent of lesion in a magnetic resonance imaging (MRI), the epilepto-genic foci in invasive electroencephalography (IEEG), pathologic grading, and surgical outcome. METHODS: We reviewed clinical findings (the seizure history, MRI), IEEG findings (interictal and ictal EEG), pathologic findings and surgical outcomes retrospectively and compared them. All the patients ranged in age from 2 to 45 years (mean: 21.8+10.8years). The time of their seizure onset ranged from 6 months to 31 years (mean:9.4+6.6years). The postopera-tive follow-up period ranged from 7 to 45months (mean:26+14months). RESULTS: Regarding MRI findings, the lesions were focal (within one lobe) in 10, diffuse (more than one lobe) in 7, and no abnormalities in 18 subjects. The ictal onset zones of ictal IEEG had one focus in 18, two in 7, three in 7, and four in 3 subjects. The area of focus was focal in 15, regional in 14, lobar in 4, and in diffuse areas in 2 subjects. Pathologic grading revealed mild in 18, moderate in 5, and severe changes in 13 subjects. Their surgical outcomes were: Engel's class I in 20, class II in 7, class III in 6, and class IV in 2 subjects. The relationships among clinical features, IEEG, regarding pathology and surgical outcomes, revealed that severe pathologic grading had a significant correlation with earlier ictal onset zones (p=0.003), and diffuse MRI lesions. Diffuse ictal onset zones had poor surgical outcomes (p<0.010). CONCLUSIONS: Cortical dysplasia may have a diverse pattern of epileptogenic foci in MRI and IEEG. The earlier the seizure onset is, the severe pathologic grading becomes. When the lesions in MRI as well as the ictal onet zones in IEEG were diffuse, the surgical outcome was poor.
Electroencephalography* ; Epilepsies, Partial ; Epilepsy* ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging* ; Malformations of Cortical Development* ; Pathology ; Retrospective Studies ; Seizures