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

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 AND PURPOSE: Cavernous angiomas are frequently encountered in patients with intractable partial epilepsies. Cavernous angioma can make highly epileptogenic foci and dual pathology. Although it is generally thought that the epileptogenic activity originated in neuronal populations adjacent to the lesion, little is known as to the exact location of the epilepsies on electophysiologic, clinical and pathologic view. We investigated nine intractable epilepsy patients with cavernous angioma regarding relation of EEG, semiology and pathology to verify where are the epileptogenic foci in cavernous angioma. METHODS: We included 9 intractable epilepsy patients with cavernous angioma who had been were undergone video-EEG monitoring. They were aged from 15 to 49 years(average:36.7+15.7)and had cavernous angioma in temporal, frontal lobe, or multiple areas(temporal:7, frontal:1, multiple:1 patients). Four patients had invasive EEG study including subdural and/or depth electrodes. Six patients had undergone epilepsy surgery. We analyzed seizure history, semiology of their seizures, interictal and ictal EEG. To know dual pathology, MRI including hippocampal volumetry, invasive EEG, and pathology were studied. RESULTS: Four patients had multiple auras. Eight patients had complex partial seizures and one had right foot clonic seizure, which were related with the location of cavernous angioma. In scalp EEG, ictal recording showed definite EEG changes, but 3 patients had no definite EEG change in some seizures. In invasive EEG with subdural and/or depth electrodes , interictal spikes were more frequently detected than scalp EEG and ictal EEG revealed not only 3 different ictal onset zones in 3 patients but also EEG seizures without clinical events in 3 patients. Regarding dual pathology, mesial temporal involvement was detected in 2 patients in MRI. Among 6 surgery patients 4 patients including 3 patients with normal hippocampus in MRI had hippocampal or dentate gyral change in pathology. Among 4 patients with invasive ictal EEG, 3 patients including 1 patient with normal hippocampus in MRI and pathology had mesial temporal involvement in ictal onset zones. CONCLUSION: Cavernous angiomas can make multiple epileptogenic foci around themselves and often dual pathology of hippocampus, which can be easily detected by invasive ictal EEG but not by imaging and even by pathology. And the foci can have frequent EEG seizures, which do not make clinical events. Precise localization of epileptogenic foci in cavernous angioma were needed to have good medical and surgical treatments.
Electrodes ; Electroencephalography ; Epilepsies, Partial ; Epilepsy ; Foot ; Frontal Lobe ; Hemangioma, Cavernous* ; Hippocampus ; Humans ; Magnetic Resonance Imaging ; Neurons ; Pathology ; Scalp ; Seizures

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

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

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

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

BACKGROUND: With the aid of high-resolution MRI, the identification of neuronal migration disorder(NMDs) is increasing and NMDs are considered as one of the major causes of extrahippocampal epilepsy. However, MRI has some limitatons in detecting small cortical lesion of NMDs. We have studied the diagnostic value and findings of brain SPECT and PET in the patients with NMDs. METHODS: Nineteen NMD patient with intrac table and partial epilepsy were studied. Diagnosis of NMDs was based on neuroimaging and pathology. Proton, Tl and T2-weighted axial, saggital and coronal MR image were obtained by 1.5 Tesla unit. Interictal and ictal SPFCT and PET imagings were performed with 99mTc-HMPAO and 18F-fluorodeoxyglucose. RESULT: Focal cortical dysplasia (FCD) and schizencephaly were detected in 4 patients, heterotopias in 3(one with 3 isolated lesions and one with bilateral temporal lobe lesions), polymicrogyria in 3, hemi-megalencephaly in 2, pachygyria in 2, forme fruste of tuberculous sclerosis(FFTS) in 1. Heterotopia was also combined with other lesions as schizencephaly, FFS and pachygyria. The MRI detected the lesions in 14 patients(73.7%). Of the 5 patients without definite abnormalities on MRI, 3 had focal polymicrogyda and 2 had FCD on pathologic examination. The interictal SPECT revealed abnormalities in 9 of 12 patients(75.0%), but could not detect 2 FCDs and one heterotopia. The ictal SPECT detected the lesions in all 11 patients. PET showed the compatible abnormalities in 17 patients(89.5%), but there was no abnormal finding in 2(1 with FCD and 1 with heterotopia). The abnormal lesions in PET were more extensive than those in MRI in the 8 patients with focal NMDs. Heterotopia showed cortical gray matter activity on PET in 6 out of 11 lesions. All other NMDs showed hypometabolism or metabolic detect in the interictal SPECT and PET. CONCLUSION: Functional imaging as SPECT & PET may be more selective than MRI to detect focal cortical lesions in NMD. NMDs show, variable metabolic pattern on functional imagings and in general the derangement in the functional imaging is more widespread than the lesions detected by MRI. We recommend the functional neuroimaging in the patients who are suspected to have partial seizure of neocortical origin and have no abnormal findings on brain MRI.
Brain ; Diagnosis ; Epilepsies, Partial ; Epilepsy ; Functional Neuroimaging ; Humans ; Lissencephaly ; Magnetic Resonance Imaging* ; Malformations of Cortical Development ; Neuroimaging ; Neuronal Migration Disorders* ; Neurons* ; Pathology ; Protons ; Seizures ; Technetium Tc 99m Exametazime ; Temporal Lobe ; Tomography, Emission-Computed, Single-Photon*