BACKGROUND: The Wada test is an essential part of the preoperative evaluations in candidates for epilepsy surgery. Impaired reactions (IR) to intracarotid sodium amytal infusions include: confusion, disorientation, and decreased respon-siveness, thereby confounding test results. We retrospectively analyzed the factors affecting the impaired reactions (IR) regarding both individual and methodologic factors. METHODS: Subjects included 202 patients (age;28.5+/-9.48, range:8 to 64 years, M:F=113:89) with intractable epilepsy. Patients with IR (IR group) and those without IR (Non-IR group) were compared with respect to individual factors such as: age, sex, epilepsy syndromes, injected hemispheres, dominan-cy as well as methodologic factors including amobarbital doses. With regards to the dose of amobarbital, the total accu-mulated dose as well as the initial injected dose, were used. RESULTS: IR was observed in 50 patients (24.7%). Compared to the non-IR group, the IR group had more prolonged, contralateral weakness and total test times, but the same duration of EEG changes. IR occurred more frequently with injections into the left hemisphere (p<0.001), the dominant hemi-sphere (p<0.0001), and contralateral hemisphere of epileptic focus (p<0.07). The IR group received significantly higher amounts of amobarbital than the non-IR group (p<0.05). Considering the oversedation as well as the undersedation of amobarbital together, 100mg of amobarbital was the most appropriate dose in the Wada test. CONCLUSIONS: These results suggest that dominant hemisphere injections and usage of higher doses of amobarbital can frequently produce IR in the Wada test. The ideal dose of amobarbital in the Wada test for Korean patients should be 100mg.
Amobarbital ; Electroencephalography ; Epilepsy ; Humans ; Retrospective Studies

Epilepsy is one of the most common episodic neurological diseases characterized by recurrent epileptic seizures. The seizures occur by synchronization of a neuronal network, which may cause disturbances in intracellular ion homeostasis, neuronal excitability, network remodeling, and neuronal death. The neuronal death following epileptic seizures results from the execution of cellular programs that are similar to those in developmentally programmed cell death. Research into cell death after seizures has identified the molecular machinery of apoptosis including the caspases and bcl-2 family proteins. The author reviews the clinical experimental evidences of programmed death pathway function in epileptic seizures. Four neuronal death pathways after epileptic seizures are proposed; non-programmed necrotic, programmed necrotic, programmed apoptotic extrinsic, and programmed apoptotic intrinsic pathways. Epileptogenesis is speculated based on the programmed pathways. Research on seizure-induced neuronal damage has developed considerably in recent years and that may open new ways to improve neuroprotective and antiepileptic treatments for patients with epilepsy.
Apoptosis ; Caspases ; Cell Death* ; Epilepsy* ; Homeostasis ; Humans ; Neurons ; Seizures

Epilepsy surgery can be a safe, effective treatment for individuals with intractable partial epilepsy. There is increasing evidence that brain abnormalities in focal epilepsy are not restricted to a single area. The longstanding debate around the relationship between structural lesions and the epileptic zone remains unresolved. Patients with DNT (dysembryoplastic neuroepithelial tumor), which is an essentially benign tumor, can be cured by epilepsy surgery-oriented approach. Cortical dysplasia is frequently associated with DNT and seems to contribute to epileptogenic activity of DNT. Surgical treatment should be aimed at removal of the associated cortical dysplasia as well as DNT itself for ideal treatment of the disease. Simple lesionectomy of cavernous angioma would relieve seizures significantly, but not always. The concept of epilepsy surgery needs to be recruited in the treatment of cavernous angioma with seizures because diffusion of hemosiderin into the surrounding brain tissue and formation of cortical scars can make epileptogenic areas. Cortical dysplasia is a highly epileptogenic lesion constituting an important cause of medically intractable epilepsy and surgery is a treatment of choice in a selected group of patients. Identification and complete resection of the lesion and ictal onset zone are necessary to achieve a good surgical results. Intractable epilepsy accompanied by benign brain lesions can be treated surgically using the entire armamentarium of presurgical investigations. Deliberate resective procedures aimed at complete removal of dysplastic tissue and epiletogenically active areas on and around the lesion ensure excellent seizure control without permanent neurologic deficit.
Brain* ; Cicatrix ; Diffusion ; Epilepsies, Partial ; Epilepsy* ; Hemangioma, Cavernous ; Hemosiderin ; Humans ; Malformations of Cortical Development ; Neurologic Manifestations ; Seizures

PURPOSE: The ictal perfusion patterns of cerebellum and basal ganglia have not been systematically investigated in patients with temporal lobe epilepsy (TLE). Their ictal perfusion patterns were analyzed in relation with temporal lobe and frontal lobe hyperperfusion during TLE seizures using SPECT subtraction. MATERIALS AND METHODS: Thirty-three TLE patients had interictal and ictal SPECT, video-EEG monitoring, SPGR MRI, and SPECT subtraction with MRI co-registration. RESULTS: The vermian cerebellar hyperperfusion (CH) was observed in 26 patients (78.8%) and hemispheric CH in 25 (75.8%). Compared to the side of epileptogenic temporal lobe, there were seven ipsilateral hemispheric CH (28.0%), fifteen contralateral hemispheric CH (60.0%) and three bilateral hemispheric CH (12.0%). CH was more frequently observed in patients with additional frontal hyperperfusion (14/15, 93.3%) than in patients without frontal hyperperfusion (11/18, 61.1%). The basal ganglia hyperperfusion (BGH) was seen in 11 of the 15 patients with frontotemporal hyperperfusion (73.3%) and 11 of the 18 with temporal hyperperfusion only (61.1%). In 17 patients with unilateral BGH, contralateral CH to the BGH was observed in 14 (82.5%) and ipsilateral CH to BGH in 2 (11.8%) and bilateral CH in 1 (5.9%). CONCLUSION: The cerebellar hyperperfusion and basal ganglia hyperperfusion during seizures of TLE can be contralateral, ipsilateral or bilateral to the seizure focus. The presence of additional frontal or basal ganglia hyperperfusion was more frequently associated with contralateral hemispheric CH to their sides. However, temporal lobe hyperperfusion appears to be related with both ipsilateral and contralateral hemispheric CH.
Basal Ganglia* ; Cerebellum* ; Epilepsy, Temporal Lobe* ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Perfusion ; Seizures ; Temporal Lobe* ; Tomography, Emission-Computed, Single-Photon

Epilepsy is one of the most common episodic neurological diseases, and patients with epilepsy may experience a range of neurological, psychological, and behavioral problems. Recurring seizures potentially contribute to the progressive severity of epilepsy, cognitive and behavioral consequences. The clinical and experimental evidences involving radiological, pathological, and biochemical studies suggest that seizures can potentially injure the brain via a number of diverse molecular, cellular, and network mechanisms. The damage includes neuronal death, axodendritic changes, molecular changes of synaptic membrane, and gliosis and increased neurogenesis. Those changes induce rewiring of the network and reorganization of synapses, causing alteration of the functional and morphological properties as the mechanism of epilepsy. As the most overt form of alterations, the neuronal death may result from the execution of cellular programs that are similar to the molecular machinery of programmed cell death including the caspases and bcl-2 family proteins. In epileptic seizure, the neurons are overexcited and run out of energy. The low energy state is closely related with the necrotic pathway. The features suggest that the neuronal death in epilepsy may follow characteristic mechanism, suggesting necrotic programmed cell death pathway. Therapeutic modification of seizure-induced death could open new strategy in epilepsy treatment.
Brain ; Caspases ; Cell Death ; Epilepsy* ; Gliosis ; Humans ; Neurogenesis ; Neurons* ; Seizures ; Synapses ; Synaptic Membranes

Epidermolytic keratosis palmaris is a rare disease which shows clinical findings of Unna Thost keratoderma and histopathologic of epidermolytic hyperkeratosis. We report herein a case of epidermolytic keratosis palmaris et plantaris in a 16-month-old female baby. Light microscopy shows marked hyperkeratosis, large irregular keratohyalin granules, and large clear spaces in the granular and upper spinous layers. Eletron microscopic findings shows that the clear spaces are areas of cytoplasm filled with a fibrillar material and cellular organelles. Abnormal clumping of tonofilament and keratohyalin is also present.
Cytoplasm ; Female ; Humans ; Hyperkeratosis, Epidermolytic ; Infant ; Intermediate Filaments ; Keratoderma, Palmoplantar* ; Keratosis* ; Microscopy ; Organelles ; Rare Diseases

Sometimes it is not easy to make the diagnosis of myasthenia gravis (MG). The aim of our study was to understand the diagnostic sensitivities of tensilon, repetitive nerve stimulation (RNS), single fiber EMG (SFEMG) tests, acetylcholine receptor (AChR) antibodies (Ab), and to know their comparative significances for making diagnosis of MG. Those tests were safely completed in 40 myasthenia gravis, which consisted of 17 ocular, 21 generalized, 1 acute severe, 1 late severe MG. In all 40 subjects at least one of the tests was abnormal. The positive rates of tensilon, RNS tests, SFEMG, AChR Ab were 92.5%, 65.0%, 90.0%, and 82.5% respectively. The sensitivities of each tests were high in severe MG group, compared with those in mild MG group, because the positive rates were decreased from 100.0% to 89.2% in tensilon tests, from 83.3% to 57.1% in RNS test, from 100.0% to 89.2% in SFEMG tests and 91.7% to 78.6% in AChR Ab test. The positive results of RNS test was increased from 32.5% on abductor digiti quinti (ADQ) to 65.0% on orbicularis oculi (OOC). Among 3 cases with negative tensilon test, the RNS test in 1 case, the SFEMG tests were positive in all 3 cases, and the AChR Ab assay 1case. Among 3 cases with negative SFEMG, the tensilon tests were positive in 3 all cases, the RNS test in 1case and the AChR Ab assay in 1 cases. Among 5 cases, with negative RNS teses and negative AChR Ab assay, the tensilon tesrs wre positive in 3 cases and the SFEMG tests in 3 cases. Therefore we conclude that tensilon and SFEMG tests were more sensitive than RNS tests and AChR Ab assay. SFEMG test would be indicated to diagnose MG, especially in those mild MG cases with negative stensilon test. Also AChR Ab assay could improve diagnostic yields.
Acetylcholine ; Antibodies ; Diagnosis ; Edrophonium ; Myasthenia Gravis*

BACKGROUND: Anteromesial temporal resection (AMTR) is well established as effective in patients with intractable mesial temporal epilepsy. However, little electroclinical information is available relevant to poor surgical outcome after AMTR. We examined the postoperative electroclinical features based on postoperative MRI and video-EEG monitoring (VEM) in patients with poor surgical outcome. METHODS: We reviewed clinical features and postoperative VEM results in 20 patients with failure in AMTR. According to the postoperative electroclinical features, we classified them into mesial temporal (MT), bitemporal (BT), extramesial temporal (XMT), combined (C), and unclassified groups. The postoperative VEM results were compared among the groups. Surgical outcome was assessed in five patients who underwent reoperation. RESULTS: Patients comprised 6 MT, 2 BT, 6 XMT, 1 C, and 6 unclassified. Aura and automatism were more frequent in MT (50.0%, 83.3%) than in XMT (16.7%, 33.3%). Theta to delta rhythm, during the ictal onset and build-up period, was more frequent in MT (83.3%, 66.7%) than in XMT (33.3%, 33.3%). The ictal onset and build-up pattern of ictal EEG were most frequently localized to the frontotemporal region in MT (66.7%, 100.0%), while there was no predominantly localized region in XMT. The surgical outcome after reoperation was better in MT group than in XMT and C groups. CONCLUSIONS: Postoperative MRI and VEM are useful to assess the postoperative electroclinical features in failed AMTR. Reoperation of the residual mesiotemporal structures after confirming epileptogenic foci may have good surgical outcome.
Automatism ; Delta Rhythm ; Electroencephalography ; Epilepsy ; Humans ; Reoperation ; Seizures

BACKGROUND: The detection of the phase reversal of somatosensory evoked potentials (SEP) has been well known to be a safe and reproducible method for defining the sensorimotor cortex. Brain stimulation (BS) has also been used to identify functional loci. The two tests, however have not to be compared in the cortical localization of the sensorimotor cortex yet. METHODS: We recorded the cortical potentials to median nerve stimulation from subdural grids which were in the frontoparietal areas of 12 epilepsy patients. After looking for phase reversals, we compared positive motor and sensory responses by brain stimulation from each electrode. In addition, the maximal amplitude areas of precentral positivity (max-P20) and postcentral negativity (max-N20) were analyzed, which were then compared with BS results. RESULTS: All patients showed phase reversals (average:4.7+1.61) on cortical SEP to median nerve stimulation. The electrodes before the phase reversal line had positive motor responses in 75% among electrodes with positive motor responses. The electrodes after the phase reversal line had positive sensory responses in 88.9% among electrodes with positive sensory responses. The maximal amplitude areas were recorded within a 2cm distance to the central sulcus. Max-P20's were located on the areas where BS showed a finger in 5 patients, hand in 3, forearm in 2, and tongue motor response in 2. Max-N20's were recorded on the areas where BS revealed a hand in 4 patients, forearm in 1, and undetected sensory responses in 7. CONCLUSIONS: These results suggest that SEP is different from BS in the functional localization of the perirolandic area. Thus, combined functional evaluation of the periolandic area by SEP as well as by BS is helpful in assessing sensorimotor functions.
Brain* ; Electrodes ; Epilepsy* ; Evoked Potentials, Somatosensory* ; Fingers ; Forearm ; Hand ; Humans ; Median Nerve ; Tongue

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