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Purpose: This study aimed to elucidate the clinical features of water-electrolyte disturbance (WED) as a sequela of hemispherotomy., Methods: We performed a retrospective chart review to identify the clinical features of diabetes insipidus (DI) as a complication in < 12-month-old patients who underwent hemispherectomy or hemispherotomy for severe epilepsy between 2007 and 2018. Central DI was diagnosed if a patient developed polyuria (urine output > 5 mL/kg/h), abnormally high serum osmolality (> 300 mOsm/kg), high serum sodium level (> 150 mEq/L), either abnormally low urine specific gravity (< 1.005) or low urine osmolality (< 300 mOsm/kg) or both, and effective control of polyuria with arginine vasopressin (AVP). The clinical course of post-hemispherotomy WED, complications other than WED, and seizure outcomes were analyzed., Results: The review identified that 3 of 23 infants developed WED. All patients developed polyuria within 2 days after surgery, with high serum osmolality and hypotonic urine; AVP was effective in treating these symptoms. The clinical course was compatible with central DI. Two patients subsequently developed hyponatremia in a biphasic or triphasic manner. All patients had multiple seizures that were probably related to WED. Two patients developed asymptomatic cerebral sinovenous thrombosis, possibly because of the surgical procedure and dehydration; anticoagulant treatment was provided. All patients were treated for WED for up to 2 months and had no residual pituitary dysfunction., Conclusion: Systemic complications other than intracranial ones can occur in patients who have undergone hemispherotomy. Perioperative systemic management of young infants undergoing this procedure should include careful water and electrolyte balance monitoring.

Background: Hippocampal sclerosis (HS) is one of the major causes of intractable epilepsy. Astrogliosis in epileptic brain is a peculiar condition showing epileptogenesis and is thought to be different from the other pathological conditions. The aim of this study is to investigate the altered expression of astrocytic receptors, which contribute to neurotransmission in the synapse, and channels in HS lesions., Methods: We performed immunohistochemical and immunoblotting analyses of the P2RY1, P2RY2, P2RY4, Kir4.1, Kv4.2, mGluR1, and mGluR5 receptors and channels with the brain samples of 20 HS patients and 4 controls and evaluated the ratio of immunopositive cells and those expression levels., Results: The ratio of each immunopositive cell per glial fibrillary acidic protein-positive astrocytes and the expression levels of all 7 astrocytic receptors and channels in HS lesions were significantly increased. We previously described unique astrogliosis in epileptic lesions similar to what was observed in this study., Conclusion: This phenomenon is considered to trigger activation of the related signaling pathways and then contribute to epileptogenesis. Thus, astrocytes in epileptic lesion may show self-hyperexcitability and contribute to epileptogenesis through the endogenous astrocytic receptors and channels. These findings may suggest novel astrocytic receptor-related targets for the pharmacological treatment of epilepsy.

Objective: To compare the seizure and developmental outcomes in infants and young children with epileptic encephalopathy who have undergone surgical and medical treatments., Methods: An international, multicenter, observational cohort study was undertaken. A total of 317 children aged <6 years, who had frequent disabling seizures despite intensive medical treatments, were registered. Among the enrolled children, 250 were treated medically (medical group), 31 underwent resective surgery (resective group), and 36 underwent palliative surgery [callosotomy (n=30) or vagal nerve stimulation (n=6); palliative group] on admission. Seizure and developmental outcomes were obtained for 230 children during the 3-year follow-up period. Cox proportional hazard model was used to adjust for clinical backgrounds among treatment groups when comparing the seizure-free survival rates., Results: At the 3-year follow-up, seizure-free survival was 15.7%, 32.1%, and 52.4% in the medical, palliative, and resective groups, respectively. The adjusted hazard ratios for seizure recurrence in the resective and palliative groups versus the medical group were 0.43 (95% CI, 0.21-0.87, P=0.019) and 0.82 (95% CI, 0.46-1.46, P=0.50), respectively; the former was statistically significant. Regarding the developmental outcome, the mean DQs in the resective group increased significantly compared to those in the medical group during the follow-up (P<0.01). As for subgroup analysis, better seizure and development outcomes were demonstrated in the resective group compared to the medical group in children with nonsyndromic epilepsies (those to which no known epilepsy syndromes were applicable)., Significance: These results suggest that surgical treatments, particularly resective surgeries, are associated with better seizure and developmental outcomes compared with successive medical treatment. The present observations may facilitate the identification of infants and young children with epileptic encephalopathy who could benefit from surgery., (Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Balloon cells are a pathognomonic cellular feature of various cortical malformations, including focal cortical dysplasia type IIb (FCD IIb), cortical tubers of tuberous sclerosis (TSC) and hemimegalencephaly (HME). In the present study, we investigated the immunohistochemical expression of p57/Kip2, a member of the Cip/Kip family of cyclin-dependent kinase inhibitory proteins, in balloon cells in surgical specimens taken from 26, 17 and six patients with FCD IIb, TSC and HME, respectively. Characteristic dot-like reactivity with a faint, intense, reticular and process-like pattern was confined to the proximal portion of the cytoplasmic processes of the cells. Immunoelectron microscopy revealed the p57/Kip2 reactivity on intermediate filaments in the proximal portion of the processes. The immunohistochemical profile appeared similar to that of CD34; however, a double immunofluorescence study demonstrated that no cells showed reactivity for both p57/Kip2 and CD34. The frequencies of the p57/Kip2-positive cells in FCD IIb and HME were significantly higher than those in TSC, suggesting that the balloon cells may be heterogeneous. These findings suggest some functional significance of the protein on the cytoplasmic processes of balloon cells and appear consistent with the notion that the cells are abnormally differentiated progenitor cells., (© 2015 Japanese Society of Neuropathology.)

The Asia-Oceanian region is the most populous region in the world. Although there has been substantial economic development and improvement in health services in recent years, epilepsy remains generally an underrecognized and understudied condition. To help promote research in the region, the Commission on Asian and Oceanian Affairs (CAOA) of the International League Against Epilepsy (ILAE) appointed the Research Task Force (RTF) to facilitate the development of research priorities for the region. Research that focuses on issues that are unique or of particular importance in the Asia-Oceanian region is encouraged, and that captures the impact of the dynamic socioeconomic changes taking place in the region is emphasized. Based on these considerations, we propose research "dimensions" as priorities within the Asia-Oceanian region. These are studies (1) that would lead to fuller appreciation of the health burden of epilepsy, particularly the treatment gap; (2) that would lead to better understanding of the causes of epilepsy; (3) that would alleviate the psychosocial consequences of epilepsy; (4) that would develop better therapies and improved therapeutic outcomes; and (5) that would improve the research infrastructure., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)

Epilepsy is one of the major neurologic diseases, and astrocytes play important roles in epileptogenesis. To investigate possible roles of astrocyte-related receptors in patients with intractable epilepsy associated with focal cortical dysplasia (FCD) and other conditions, we examined resected epileptic foci from 31 patients, including 23 with FCD type I, IIa, or IIb, 5 with tuberous sclerosis complex, and 3 with low-grade astrocytoma. Control samples were from 21 autopsied brains of patients without epilepsy or neurologic deficits and 5 patients with pathologic gliosis without epilepsy. Immunohistochemical and immunoblot analyses with antibodies against purinergic receptor subtypes P2RY1, P2RY2, P2RY4, potassium channels Kv4.2 and Kir4.1, and metabotropic receptor subtypes mGluR1 and mGluR5 were performed. Anti-glial fibrillary acidic protein, anti-NeuN, and anti-CD68 immunostaining was used to identify astrocytes, neurons, and microglia, respectively. Most glial fibrillary acidic protein-immunopositive astrocyte cells in the brain samples from patients with epilepsy were P2RY1-, P2RY2-, P2RY4-, Kv4.2-, Kir4.1-, mGluR1-, and mGluR5-positive, whereas samples from controls and pathologic gliosis showed lower expression levels of these astrocyte-related receptors. Our findings suggest that, although these receptors are necessary for astrocyte transmission, formation of the neuron-glia network, and other physiologic functions, overexpression in the brains of patients with intractable epilepsy may be associated with activation of intracellular and glio-neuronal signaling pathways that contribute to epileptogenesis.

Build-up of community health coalition system is now an essential part of medicine. However, little attention has been paid to epilepsy care in Japan, which resulted in a chaotic and difficult situation to find epilepsy-care physicians in the community. The reason is that responsible medical specialty in charge has been ambiguous historically in Japan and a lack of post-in-charge in the government to plan epilepsy care system is aggravating this condition. To solve this issue, epilepsy care network connecting the primary, secondary and tertiary epilepsy care physicians should be established and open to the community. In this context, our Epilepsy Care Network-Japan was started on July 2012 proposing a new epilepsy care algorithm suitable for our complex medical community.

Cerebral cavernous malformations (CCMs) are well-defined, mostly singular lesions present in 0.4-0.9% of the population. Epileptic seizures are the most frequent symptom in patients with CCMs and have a great impact on social function and quality of life. However, patients with CCM-related epilepsy (CRE) who undergo surgical resection achieve postoperative seizure freedom in only about 75% of cases. This is frequently because insufficient efforts are made to adequately define and resect the epileptogenic zone. The Surgical Task Force of the Commission on Therapeutics of the International League Against Epilepsy (ILAE) and invited experts reviewed the pertinent literature on CRE. Definitions of definitive and probable CRE are suggested, and recommendations regarding the diagnostic evaluation and etiology-specific management of patients with CRE are made. Prospective trials are needed to determine when and how surgery should be done and to define the relations of the hemosiderin rim to the epileptogenic zone., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Purpose: We studied children younger than 6years old who developed catastrophic epilepsy and were registered in the FACE study group to clarify their clinical characteristics and prevalence of seizure as well as epilepsy types., Subjects: Subjects were prospectively recruited from children with epilepsy who satisfied the following criteria and underwent intensive examination between 2009 and 2012 in 14 collaborative centers: (1) younger than 6years old and (2) more than 10 seizures/month refractory to all available medical treatments including ACTH therapy, leading to significant psychosocial morbidity., Methods: We analyzed epilepsy onset age, predominant seizure type, etiology, neuropsychological findings, and syndromic classification according to the pre-determined registration format., Results: A total of 314 children were enrolled in this study. Epilepsy onset age in 239 cases (80%) was younger than 12months. The most frequent seizure type was epileptic spasms (ES), followed by generalized tonic seizures (GTS), which accounted for 42% and 20%, respectively. West syndrome (WS) was the most frequent epileptic syndrome and accounted for 37%, followed by unclassified epilepsy at 21%, neocortical epilepsy at 19%, Lennox-Gastaut syndrome at 12%, Dravet syndrome at 4%, Rasmussen syndrome at 2%, and others. The two most frequent causes of epilepsy were cortical dysplasia and chromosomal anomalies, as shown in 16% and 6%, respectively. However, the etiology of nearly one half of all patients remained unknown. Psychomotor development was already worse than a moderate degree in 62% of subjects at the first examination., Conclusion: The highest proportion of catastrophic epilepsy was WS and its related syndromes featuring ES and GTS, followed by neocortical epilepsy, whose psychomotor development was significantly retarded at examinations., (Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Objective: Hemimegalencepahly (HME) and Hemispheric Cortical Dysplasia (HCD) are rare congenital diseases that occur with intractable epilepsy. They manifest by early epilepsy, mental retardation, hemianopsia and contralateral hemiplegia. Hemispheric disconnection (mainly anatomical hemispherectomy, peri-insular hemispherotomy, modified lateral hemispherotomy and vertical parasagittal hemispherotomy) have been reported to be efficient on seizures and also to prevent additional cognitive injury and developmental delay., Method: We reviewed literature about clinical presentation, predictors of outcome and expectation about epileptic seizures and cognitive outcome., Results: Clinical presentation and seizures outcome have been described in almost 600 children for the last thirty years. Epilepsy improved in most cases depending on the series and the follow-up duration. Percentage of seizure-free patients with HME or HCD was lower than in other groups (Rasmussen Encephalitis, Vascular Sequellae). Post-operative complications decreased with the hemispherotomy surgical procedures. EEG abnormalities on the "save" hemisphere did not negatively influence postsurgical outcome. Seizure free outcome did not seem to depend on the surgical procedure but the presence of residual insular cortex seemed to be associated with persistent postoperative seizures. Contralateral MRI abnormalities seemed to be associated with poorer prognosis for seizure free outcome and lack of cognitive improvement., Conclusion: Hemispheric disconnection remains the best treatment in order to control epileptic seizures. Hemispheric surgical procedures are safe and can be performed from the first month of life. Prospective studies of cognition are needed to emphasize benefits on long term outcome., (Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Focal cortical dysplasia (FCD) and hemimegalencephaly (HME) are major causes of intractable epilepsy in children. The probable pathogenesis of FCD and HMG is the abnormal migration and differentiation of neurons. The aim of the present study was to clarify the abnormal cytoarchitecture, based on neuronal immaturation. Tissue samples were obtained from 16 FCD and seven HME patients, aged between 2 months and 12 years, who had been diagnosed as typical FCD and HME, following surgical treatment for intractable epilepsy. Paraffin-embedded sections were stained with the antibodies of three layer-markers that are usually present only during the fetal period, namely SATB2 (expressed in the upper layer of the normal fetal neocortex), FOXP1 (expressed in the 5th layer), and TBR1 (expressed in the 6th layer). In FCD, SATB2-positive (+) cells located in the middle and deep regions of FCD Ia and Ib, but only in the superficial region of FCD IIa and IIb. FOXP1+ cells diffusely located in the neocortex, especially the upper layer of FCD IIa and IIb. TBR1+ cells mainly located in the middle and deep regions, and also white matter. In FCD IIb, TBR1+ cells were in the superficial region. In HME, SATB2+ and FOXP1+ cells were found diffusely. TBR1+ cells were in the middle and deep regions. On the basis of continued expression of fetal cortical layer-specific markers in FCD and HME brains, the abnormal neocortical formation in both is likely to be the result of disrupted neuronal migration and dysmaturation. The expression pattern is different between FCD and HME., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Purpose: To detect the epileptogenic region causing epileptic spasms in an infant with tuberous sclerosis (TS)., Methods: We applied a multiple band frequency analysis to video electroencephalographic (EEG) recordings of the infant's scalp. We also performed computed tomography (CT), magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT), and magnetoencephalography (MEG) of the brain in order to ascertain the epileptic focus., Results: During the periodic spasms, we identified fast ictal activity with frequencies of 60-70 Hz in the right centroparietal region. This region was part of the area surrounding the largest cortical tuber that was identified on CT and MRI and was located in the right frontal lobe. An area of increased blood perfusion that was observed with SPECT and dipole sources that were determined with interictal MEG were also located in this area. In addition, ictal frequency oscillations (FOs) with high gamma activity were identified over the cortex surrounding the largest tuber. After a lesionectomy of this tuber, the periodic spasms disappeared, and no FOs were detected over this area., Conclusions: Scalp EEG, which identified the ictal onset zone by detecting fast activity that was suggestive of FOs, was useful for detecting the epileptogenic region in an infant with TS.

Number of epilepsy patient in Japan officially surveyed by our government in 2008 is 219,000, which is only 0.17% of the total population and less than one third of the prevalence rate reported in Western countries. Number of epilepsy surgery per year in Japan is also low and less than half of other countries such as US, UK and Korea. These numbers may suggest that epilepsy care in Japan is not sufficient to cover all hidden medical needs of people with epilepsy at present. To solve this issue, our research group funded by the government have started to build an epilepsy care network among primary care physicians, secondary care neurology specialists and tertiary care epilepsy centers by utilizing a web site: Epilepsy Care Network-Japan (http://www.ecn-japan.com/) from July 2012. We are also proposing an epilepsy care algorithm suitable for our complex medical community consisted with various neurology specialists such as pediatric and adult neurologists, neurosurgeons and psychiatrists. Building Epilepsy Care Network in Japan may facilitate better medical and social support for people with epilepsy in Japan.

To evaluate the effect of corpus callosotomy (CC) on attention deficit and behavioral problems in pediatric patients with intractable epilepsy, we retrospectively investigated sequential patients who had undergone CC to control seizures. Between August 2005 and April 2010, a total of 15 patients aged between 3.1 and 17.9 years underwent CC at our institute. All the patients experienced either drop attacks or head nodding, which were considered to be therapeutic targets of CC. A standardized instrument, the Child Behavior Checklist (CBCL), was used to assess behavioral and emotional problems before and after surgery. On postoperative EEGs, 8 (53%) showed improvement and 7 (47%) showed no change in epileptiform discharges. The Attention Problems scale and total score on the CBCL significantly improved in patients whose postoperative EEGs showed improvement. In addition to amelioration of target seizures, CC can improve attention impairments in association with improvement in the postoperative EEG., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The epileptogenesis of the striatum is unknown. We describe the case of a 12-year-old girl with intractable epilepsy who was treated by surgical interventions. Magnetic resonance imaging (MRI) showed ambiguous corticomedullary boundary in the left frontal lobe, and magnetoencephalography (MEG) revealed spike dipoles in the vicinity of the left ventral striatum. The epileptic seizures disappeared after partial resection of the frontal lobe, but recurred within 2 months and remained intractable. Neuropathological examination confirmed the presence of focal cortical dysplasia in the resected brain tissue. Ictal single photon emission computed tomography at this period displayed hyperperfusion of the left anterior striatum. At the second surgery, intraoperative electrocorticography exhibited spike discharges from the anterior striatum. After the removal of this structure and adjacent brain tissues, the patient remains seizure-free for 33 months, without any neurological deficits. Histopathological examination of the resected tissue revealed a large number of dysmorphic neurons distributed widely in the cerebral cortex, subcortical white matter, striatum, and insular cortex. These findings suggest that microscopic dysplasia of basal ganglia can accompany certain cases of focal cortical malformations, and may play a critical role in the epileptogenesis through their interaction with cortical structures., (Copyright 2010 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

To determine a management strategy for the epilepsy in children with bilateral cortical malformations, clinical data of 23 patients (age, 3-23 years, M:F=7:16) were retrospectively reviewed. Among these patients, 15 were bedridden and 16 were profoundly retarded and could not even smile. The patients were categorized into the following five groups based on the findings of neuroimaging, seizure types, and electroencephalographic patterns. Group 1: Diffuse cortical malformation with epileptic spasms and secondarily generalized tonic seizures, group 2: diffuse cortical malformation with erratic twitches, group 3: bilaterally extended but not diffuse cortical malformations, group 4: bilateral polymicrogyria with persistent epileptic spasms (Aicardi syndrome), and group 5: bilateral cortical malformation with drop attacks (subcortical band heterotopia and congenital bilateral perisylvian syndrome). Eleven patients suffered from infantile spasms; adrenocorticotropic hormone was effective in group 1 but ineffective in group 4. Treatment of tonic seizures in groups 1-3 and erratic twitching in group 3 with phenobarbital, zonisamide and potassium bromide was beneficial. Epileptic spasms and tonic seizures were prominent in group 4 and were refractory to medical treatment, except that zonisamide, clobazam, and a ketogenic diet were partially or transiently effective. Complex partial and astatic/atonic seizures in group 5 were refractory to medications other than that carbamazepine and clobazam provided limited benefits. Total callosotomy resulted in better seizure control for three patients in group 5, and functional hemispherectomy was effective for one patient in group 4. These results provide the basis for the appropriate choice of medical and surgical treatment for managing bilateral, widespread cortical malformations.

Purpose: To examine the distribution of KCC2, a neuron-specific K(+)-Cl(-) cotransporter, in human cortical dysplasia (CD)., Methods: The immunohistochemical expression of KCC2 was investigated in 18 CD specimens obtained during epilepsy surgery. The histopathologic diagnoses were focal CD (FCD) type I (eight cases), FCD type II (six cases), and hemimegalencephaly (HME; four cases). Tissue sections were immunostained for KCC2 and compared with control sections., Results: In the mature nondysplastic cortex, all the layers showed diffuse neuropil staining for KCC2. The somata were stained much less, although subcortical ectopic neurons displayed dense staining in the cytosol (intrasomatic staining). In FCD type I, the cortex showed neuropil staining for KCC2 with less-stained somata. Aberrant giant pyramidal neurons were also less stained at the soma, whereas immature neurons showed intrasomatic staining. Increased numbers of ectopic neurons with intrasomatic staining were noted in the subcortical white matter. In FCD type II, dysmorphic neurons displayed dense intrasomatic staining with reduced staining of the neighboring neuropils. Balloon cells did not stain for KCC2. Dysmorphic neurons in HME also showed intrasomatic staining., Conclusions: Neurons in CD tissues expressed KCC2. However, the subcellular distribution of KCC2 was altered, which might have affected the ionic homeostasis of Cl(-) and K(+) involved in epileptic activity within CD tissues.

To clarify changes in glutamate metabolism in the brain with chronic epileptic activities, 13C-magnetic resonance spectroscopy observation of glutamate and glutamine synthesis after oral administration of [1-13C] glucose (Glc C1) (0.75 g/kg) was performed in intractable occipital lobe epilepsy patients (n=5) and controls (n=10). 1H[13C]-spectra were obtained from two voxels of 64 ml placed on the bilateral parieto-occipital lobes of the study participants. Time courses for 13C-incorporation into 4-glutamate and 3-glutamate (Glu C4, C3) and 4-glutamine (Gln C4) were obtained and the concentrations of Glu C4, C3 and Gln C4 at the time between 120 and 150 min after Glc C1 administration was calculated. Concentration of Gln C4 was increased in the epilepsy patients [control: 0.39 mM (SD 0.14), epilepsy: 0.60 mM (SD 0.15), P<0.05], whereas those of Glu C4 and Glu C3 were not. The present study revealed increased glutamine synthesis compared with glutamate formation in a widespread cortical area with sustained epileptiform activities, possibly a result of chronic excessive glutamate release from neurons and subsequent uptake into astrocytes.

The goal of presurgical evaluation of intractable epilepsy is to identify epileptogenic regions in the brain. From our experience of 38 cases of resective epilepsy surgery from the last 3 years, ictal SPECT was considered the most sensitive at detecting focal changes relating to seizures compared to other neuroimaging modalities, such as MRI, FDG-PET, SPECT and MEG. At interictal state, on the other hand, FDG-PET was most sensitive, especially in cases with focal cortical dysplasia, which is often MRI-invisible. In dysplastic tumors, MRI showed the highest concordance rate to clinically verified epileptogenic regions. Activation studies using functional neuroimaging such as PET and fMRI is useful to evaluate brain functions at epileptogenic regions presurgically. The role of functional brain imaging in epilepsy surgery is considered to be: (i). case selection for resective surgery, (ii). case selection for invasive EEG monitoring, and (iii). navigation of electrode placement and cortical resection.

Good surgical outcome can be expected for intractable epilepsy in children by resective surgery when a focal cortical abnormality is demonstrated by neuroimaging studies such as MRI, SPECT, and PET. Improvement in behavioral and cognitive development can be expected with seizure cessation after surgery. Early surgical intervention for intractable epilepsy in children is considered a safe and effective choice of treatment to avoid cognitive delay and learning disability.

The dysembryoplastic neuroepithelial tumor (DNT), characterized by its favorable prognosis, must be precisely differentiated from other gliomas to prevent overtreatment by radio/chemotherapy. We studied, here, pathological features of five surgical cases of DNT with intractable epilepsies. There were two males and three females with a median age of 20.4 years (range 12-41). We evaluated lesional topography, gross appearances and microscopic findings including immunohistochemical data. One tumor was located in the temporal lobe, another two in the parietal lobe, another in the occipital lobe, and the other one in the occipito-parietal junction. The lesions were predominantly intracortical in all cases. Macroscopically, two tumors were grayish-soft, another two were yellowish-elastic, and the other one was resected as soft-mucinous fragments. Microscopically, the main glial components were oligodendrocyte-like cells (OLCs) in all cases, except for one case in which atypical glial cells were also prominent. Histopathological characteristics of the DNTs included the specific glioneuronal elements (the mixture of OLCs and neurons) in four cases, alveolar pattern (all cases), microcystic degeneration (all cases), multinodular architecture (four cases), and adjacent cortical dysplasia (all cases). Immunohistochemically, Ki-67 labelling indices ranged from 0 to 0.8%. OLCs were positive for glial fibrillary acidic protein in two cases, for S-100 protein in all cases, for synaptophysin in two cases, and for class III beta tubulin in all cases. Pathological features of our cases were characterized by its heterogeneous histological appearances and divergent cellular differentiation.

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.

Abstract: Aim: The balance of excitation and inhibition of neurons and neuronal network is very important to perform complete neuronal function. Damage or loss of inhibitory γ-aminobutyric acid (GABA)-ergic interneuron is associated with impaired inhibitory control of cortical pyramidal neurons, leading to hyperexcitability and epileptogenesis. Ectopic neurons in the basal ganglia are to be one of the pathological features of epileptogenesis. In the present study, we investigated distribution of interneuron subtypes between neocortex and caudate nucleus. Methods: We performed immunohistochemistry of GABA, glutamic acid decarboxylase (GAD), calretinin (CR), calbindin (CB), parvalbumin (PV) and neuropeptide. We used surgical materials of four focal cortical dysplasia (FCD) cases, having lesions of neocortex and caudate nucleus, and eight age-matched autopsy controls. Results: The pathology showed three FCD IIa, containing dysmorphic neurons, and one FCD IIb, balloon cells. In the neocortex, the concentrations (each positive cell number/all cell numbers in the evaluated field) of GAD+, CR+ and CB+ cells were significantly lower in FCD than in controls. On the contrary, in the caudate nucleus those of CR+ and CB+ cells were significantly more in FCD than in controls. Conclusion: The interneuron imbalance between the neocortex and basal ganglia may affect the epileptogenesis of FCD. [Copyright &y& Elsevier]