Association of the HLA-DRB1 alleles with characteristic MRI features of Asian multiple sclerosis.

BACKGROUND: In Asian patients with multiple sclerosis (MS), a paucity of brain lesions and longitudinally extensive spinal cord lesions (LESCLs) extending three or more vertebral segments are characteristic findings on magnetic resonance imaging (MRI). We aimed to disclose possible factors contributing to the development of such MRI features. METHOD: Genotyping of HLA-DRB1 and -DPB1 alleles was performed in 121 consecutive Japanese patients with clinically definite MS based on the Poser criteria and 125 healthy controls. Possible factors associated with MRI features were determined by multiple logistic analysis. Patients with MS were classified based on the presence or absence of brain lesions fulfilling the Barkhof criteria (Barkhof brain lesions) and LESCLs. Barkhof brain lesion-negative (-) patients had a markedly lower frequency of HLA-DRB1*0901 than controls (P(corr) < 0.05), whereas the frequency of DRB1*1501 was increased in the Barkhof brain lesion-positive (+) group, although this increase was not significant after correction. No Barkhof(-)LESCL(+) patients carried DRB1*0901 (P(corr) < 0.05), despite this being the most common allele in Japanese. The Barkhof(-)LESCL(-) group showed a significant increase in the frequency of DRB1*0405 compared with controls (P(corr) < 0.05). None of the DPB1 alleles were significantly different among the groups. Using multiple logistic analysis, the absence of oligoclonal bands was positively associated with an absence of Barkhof brain lesions, whereas a higher EDSS score was positively associated with the presence of LESCLs; however, the presence of anti-aquaporin-4 antibodies was not associated with either feature. CONCLUSION: The characteristic MRI features in Asians are partly related to distinct HLA-DRB1 gene alleles and an absence of oligoclonal bands.

Performance evaluation of radiologists with artificial neural network for differential diagnosis of intra-axial cerebral tumors on MR images.

BACKGROUND AND PURPOSE: Previous studies have suggested that use of an artificial neural network (ANN) system is beneficial for radiological diagnosis. Our purposes in this study were to construct an ANN for the differential diagnosis of intra-axial cerebral tumors on MR images and to evaluate the effect of ANN outputs on radiologists' diagnostic performance. MATERIALS AND METHODS: We collected MR images of 126 patients with intra-axial cerebral tumors (58 high-grade gliomas, 37 low-grade gliomas, 19 metastatic tumors, and 12 malignant lymphomas). We constructed a single 3-layer feed-forward ANN with a Levenberg-Marquardt algorithm. The ANN was designed to differentiate among 4 categories of tumors (high-grade gliomas, low-grade gliomas, metastases, and malignant lymphomas) with use of 2 clinical parameters and 13 radiologic findings in MR images. Subjective ratings for the 13 radiologic findings were provided independently by 2 attending radiologists. All 126 cases were used for training and testing of the ANN based on a leave-one-out-by-case method. In the observer test, MR images were viewed by 9 radiologists, first without and then with ANN outputs. Each radiologist's performance was evaluated through a receiver operating characteristic (ROC) analysis on a continuous rating scale. RESULTS: The averaged area under the ROC curve for ANN alone was 0.949. The diagnostic performance of the 9 radiologists increased from 0.899 to 0.946 (P < .001) when they used ANN outputs. CONCLUSIONS: The ANN can provide useful output as a second opinion to improve radiologists' diagnostic performance in the differential diagnosis of intra-axial cerebral tumors seen on MR imaging.

Perfusion imaging of brain tumors using arterial spin-labeling: correlation with histopathologic vascular density.

BACKGROUND AND PURPOSE: We investigated the relationship between tumor blood-flow measurement based on perfusion imaging by arterial spin-labeling (ASL-PI) and histopathologic findings in brain tumors. MATERIALS AND METHODS: We used ASL-PI to examine 35 patients with brain tumors, including 11 gliomas, 9 meningiomas, 9 schwannomas, 1 diffuse large B-cell lymphoma, 4 hemangioblastomas, and 1 metastatic brain tumor. As an index of tumor perfusion, the relative signal intensity (SI) of each tumor (%Signal intensity) was determined as a percentage of the maximal SI within the tumor per averaged SI within normal cerebral gray matter on ASL-PI. Relative vascular attenuation (%Vessel) was determined as the total microvessel area per the entire tissue area on CD-34-immunostained histopathologic specimens. MIB1 indices of gliomas were also calculated. The differences in %Signal intensity among different histopathologic types and between high- and low-grade gliomas were compared. In addition, the correlations between %Signal intensity and %Vessel or MIB1 index were evaluated in gliomas. RESULTS: Statistically significant differences in %Signal intensity were observed between hemangioblastomas versus gliomas (P < .005), meningiomas (P < .05), and schwannomas (P < .005). Among gliomas, %Signal intensity was significantly higher for high-grade than for low-grade tumors (P < .05). Correlation analyses revealed significant positive correlations between %Signal intensity and %Vessel in 35 patients, including all 6 histopathologic types (rs = 0.782, P < .00005) and in gliomas (rs = 0.773, P < .05). In addition, in gliomas, %Signal intensity and MIB1 index were significantly positively correlated (rs = 0.700, P < .05). CONCLUSION: ASL-PI may predict histopathologic vascular densities of brain tumors and may be useful in distinguishing between high- and low-grade gliomas and in differentiating hemangioblastomas from other brain tumors.

Phosphoprotein enriched in astrocytes-15 is expressed in mouse testis and protects spermatocytes from apoptosis.

Phosphoprotein enriched in astrocytes (PEA-15) is a 15 kDa acidic serine-phosphorylated protein expressed in different cell types, especially in the CN. We initially detected the expression of PEA-15 in primary cultures of Sertoli cells. To assess the presence and localization of PEA-15 in the mouse testis, we studied the expression pattern of the PEA-15 protein by immunohistochemistry and mRNA by in situ hybridization. Both the protein and the mRNA of PEA-15 were localized in the cytoplasm of Sertoli cells, all types of spermatogonia, and spermatocytes up till zygotene phase of the meiotic prophase. Subsequently, with ongoing development of the spermatocytes, the expression decreased and was very low in the cytoplasm of diplotene spermatocytes. To analyze the possible role of PEA-15 in the developing testis, null mutants for PEA-15 were examined. As the PEA-15 C terminus contains residues for ERK binding, we studied possible differences between the localization of the ERK2 protein in wild type (WT) and PEA-15(-/-)mice. In the WT testis, ERK2 was localized in the cytoplasm of Sertoli cells, B spermatogonia, preleptotene, leptotene, and zygotene spermatocytes, whereas in the KO testis, ERK2 was primarily localized in the nuclei of these cells and only little staining remained in the cytoplasm. Moreover, in PEA-15-deficient mice, significantly increased numbers of apoptotic spermatocytes were found, indicating an anti-apoptotic role of PEA-15 during the meiotic prophase. The increased numbers of apoptotic spermatocytes were not found at a specific step in the meiotic prophase.

MR imaging of human herpesvirus-6 encephalopathy after hematopoietic stem cell transplantation in adults.

BACKGROUND AND PURPOSE: Human herpesvirus-6 (HHV-6)-associated encephalopathy tends to develop in immunocompromised patients. Neurologic symptoms, such as disorientation, short-term memory loss, convulsion, coma, and hypopnea could occur, but they may be nonspecific. We retrospectively reviewed MR images of 6 adults with HHV-6-associated encephalopathy to study characteristic imaging findings that could be useful in making the diagnosis. MATERIALS AND METHODS: Between 2003 and 2005, we encountered 6 cases of HHV-6-associated encephalopathy (3 men and 3 women; age range, 36-55 years) in 3 hospitals. The diagnosis was made clinically according to the neurologic symptoms accompanied by high-level copies of HHV-6 DNA in CSF or peripheral blood by quantitative polymerase chain reaction without the detection of any other infectious pathogen. RESULTS: All 6 patients had abnormal hippocampus/amygdala findings on presentation, and no other regions were involved. In the early period (0-2 days from onset), abnormal high signal intensity on fluid-attenuated inversion recovery (FLAIR) imaging (2 of 3, 67%) and on diffusion-weighted images accompanied by apparent diffusion coefficient (ADC) reduction (2 of 2, 100%) were observed. In the middle period (3-30 days), abnormal low signal intensity on T1-weighted images (5 of 6, 83%) and abnormal high signal intensity on T2-weighted images (4 of 6, 67%) and FLAIR (5 of 6, 83%) were confirmed. In the late period (> 30 days), we saw the resolution of signal intensity abnormalities and the appearance of atrophic change (4 of 4, 100%) of the affected regions. CONCLUSION: HHV-6-associated encephalopathy in adults tends to affect the mesial temporal lobe. MR imaging is useful for detecting HHV-6 encephalopathy and distinguishing it from the other diseases of the central nervous system in immunocompromised patients.

Transforming growth factor alpha acts as a gliatrophin for mouse and human astrocytes.

Astrocyte death has been implicated in several neuropathological diseases, but the identification of molecules susceptible of promoting astrocyte survival has been elusive. We investigated whether transforming growth factor alpha (TGFalpha), an erbB1/EGFR ligand, which promotes glioma progression and affects astrocyte metabolism at embryonic and adult stages, regulates astrocyte survival. Primary serum-free astrocyte cultures from post-natal mouse and fetal human cortices were used. Transforming growth factor alpha protected both species of astrocytes from staurosporine-induced apoptosis. In serum-free medium, mouse astrocytes did not survive beyond 2 months while TGFalpha-treated astrocytes survived up to 12 months. Transforming growth factor alpha also promoted long-term survival of human astrocytes. We additionally extended TGFalpha proliferative effects to human astrocytes. After 3 days of permanent application, TGFalpha induced a major downregulation of both erbB1 and erbB2. This downregulation did not impair the functional activation of the receptors, as ascertained by their tyrosine phosphorylation and the continuous stimulation of both ERK/MAPK and PI3K/Akt pathways up to 7 days, the longest time examined. The full cellular effects of TGFalpha required activation of both transduction pathways. Enhanced proliferation and survival thus define TGFalpha as a gliatrophin for mammalian astrocytes. These results demonstrate that in normal, non-transformed astrocytes, sustained and functional erbBs activation is achieved without bypassing ligand-induced receptors downregulation.

Cerebral hemodynamics in Moyamoya disease: correlation between perfusion-weighted MR imaging and cerebral angiography.

BACKGROUND AND PURPOSE: In Moyamoya disease, the relationship between cerebral hemodynamics and angiographic findings has not been fully evaluated. The purpose of this study is to evaluate hemodynamics in Moyamoya disease with perfusion-weighted MR imaging (PWI) and cerebral angiography. METHODS: Twenty patients with Moyamoya disease were the subjects. Mean transit time (MTT) derived from PWI was calculated in the medial frontal lobes, the posterior frontal lobes, the occipital lobes, and the basal ganglia. From the angiographies, we classified the degrees of internal carotid artery (ICA) and posterior cerebral artery (PCA) stenoses as well as the degrees of Moyamoya vessels and leptomeningeal anastomosis (LMA). MTT in each region was compared with the angiographic findings. RESULTS: MTT positively correlated with the degree of ICA stenosis in the medial frontal (P < .01), posterior frontal (P < .001), and occipital (P < .001) lobes, as well as in the basal ganglia (P < .001). MTT correlated with the degree of PCA stenosis in the medial frontal (P < .001), posterior frontal (P < .001), and occipital (P < .001) lobes, as well as in the basal ganglia (P < .001). MTT correlated with the degree of Moyamoya vessels in the medial frontal (P < .05) and posterior frontal (P < .01) lobes. A multivariate analysis revealed that ICA and PCA stenoses and Moyamoya vessels were independent factors that prolonged MTT. CONCLUSION: Both ICA and PCA stenoses may influence overall cerebral perfusion in Moyamoya disease. The development of Moyamoya vessels may indicate hemodynamic impairment.

Deterioration of pre-existing hemiparesis brought about by subsequent ipsilateral lacunar infarction.

Mechanisms of post-stroke recovery are still poorly understood. Recent evidence suggests that cortical reorganisation in the unaffected hemisphere plays an important role. A 59 year old man developed a small lacunar infarct in the left corona radiata, which then caused marked deterioration in a pre-existing left hemiparesis that had resulted from an earlier right putaminal haemorrhage. Functional magnetic resonance imaging showed that the paretic left hand grip activated the ipsilateral left motor areas, but not the right hemispheric motor areas. This suggests that partial recovery of the left hemiparesis had been brought about by cortical reorganisation of the left hemisphere and intensification of the uncrossed corticospinal tract. The subsequent small infarct may have damaged the uncrossed tract, thereby causing the pre-existing hemiparesis to deteriorate even further.

Cerebral abnormalities in lumbosacral neural tube closure defect: MR imaging evaluation.

OBJECTS: It was decided that patients with caudal neural tube closure defect should be evaluated by means of cranial MR scans, with special attention devoted to cerebral malformations other than craniospinal abnormalities. METHODS: Twenty-four patients with lumbosacral neural tube closure defects, classified into myeloschisis (MS, 9 cases), meningo(myelo)cele (MMC, 10 cases) and spina bifida occulta with lumbosacral lipoma (LL, 5 cases), were evaluated in this way, and cerebral anomalies were found to be present in all MS patients, 8 with MMC and 2 with LL. The cerebral hemispheres were mildly to moderately hypoplastic in 6 patients, and hydrocephalus was present in 6 patients with MS and in 5 with MMC; LL was not associated with ventriculomegaly in any patient. Polymicrogyria was present in 6 patients with MS, in 3 with MMC, and in none of those with LL. Heterotopic gray matter was observed in only 2 patients with MS. Partial agenesis of the corpus callosum was found in 6 MS patients and in 6 patients with MMC, but was not seen in patients with LL. Cervicomedullary anomalies, such as herniation of the cerebellar tonsils through the foramen magnum, were found in 7 patients with MS, in 4 with MMC and in 2 with LL. While patients with LL were accompanied by only mild forebrain anomalies, cerebral abnormalities in MS and MMC were generally more severe in degree than those in LL. Among 21 patients in whom intellectual and performance status was evaluated, 9 of the 17 patients with cerebral anomalies had some degree of retardation, and the 4 without cerebral anomalies were normal. CONCLUSIONS: Caudal neural tube closure defect is not a single developmental abnormality, and can be regarded as one part of the spectrum of malformations causing primary insults that are as yet unidentified to the developing central nervous system.

Cerebral ganglioglioma with epilepsy: neuroimaging features and treatment.

Gangliogliomas are an increasingly recognized cause of epilepsy in children. In this study the clinical, neuroimaging, and neurophysiological data of five patients with cerebral ganglioglioma and epilepsy are reviewed retrospectively. The average age of these patients was 4.4 years at onset and the average duration of seizures before diagnosis was 11 months. Tumors were located in the frontal (3), parietal (1), and occipital (1) lobes. While one cystic and four solid tumors showed various densities on CT and MRI, one frontal lesion was not demonstrated by CT scan but clearly shown by MRI. Scalp electroencephalography (EEG) showed neither localized nor epileptiform abnormalities in three patients, while the remaining two had these abnormalities. In one patient, invasive chronic electrocorticography (ECoG) recordings with subdural electrodes revealed an ictal onset zone located in the hand motor area. In all patients, intraoperative ECoG failed to reveal any epileptiform activities, and tumor removal alone was performed. For a mean of 3.4 years after surgery, all patients are alive and seizure-free, with stable imaging findings. Tumor resection may be the most important factor for optimal seizure control and prevention of tumor recurrence despite the fact that EEG and ECoG findings may conflict on tumor location.

Manganese intoxication during intermittent parenteral nutrition: report of two cases.

BACKGROUND AND METHODS: The administration of trace elements is thought to be needed in patients receiving long-term parenteral nutrition. Recently, manganese intoxication or deposition was documented in such patients. We report two cases of manganese intoxication during intermittent parenteral nutrition including manganese. Manganese had been administered for 4 years at a frequency of one or two times per week in one case and for 5 years at a frequency of one or two times per month in the other case. Both cases showed mild symptoms with headache and dizziness. One case had mild hepatic dysfunction and the other did not. The whole-blood manganese level increased in one case, but not in the other case. T1-weighted magnetic resonance images revealed symmetrical high-intensity areas in basal ganglia and thalamus in both cases. After the administration of manganese was stopped, these symptoms all disappeared and the magnetic resonance images abnormalities gradually improved in both patients. Mild long-term manganese intoxication is thus considered to occur regardless of the frequency of using a manganese supplement. CONCLUSIONS: Patients should be carefully monitored when receiving long-term parenteral nutrition including manganese, even when the manganese dose is small and the frequency of receiving a manganese supplement is low.

Subependymal giant cell astrocytoma: clinical and neuroimaging features of four cases.

The clinical history, neuroimaging features, treatments, and outcome of 4 patients with histologically verified subependymal giant cell astrocytomas (SEGA) were retrospectively reviewed. The average age at the time of surgery was 13.3 years. Headache related to raised intracranial pressure was the first and only sign in 2 patients, with the remaining 2 being admitted because of sequential neuroimaging studies over several years revealing the growth of 'subependymal nodules' into intraventricular tumours. In each case the tumour was in the region of Monro's foramen and was associated with ventricular dilatation. On computed tomography (CT), multiple subependymal nodules were found in 3 patients, and these well circumscribed isodense SEGAs were markedly enhanced by contrast medium. On magnetic resonance imaging (MRI), which was obtained in 3 patients, 2 SEGAs were isointense with the cerebral cortex and one with the white matter on T1-weighted images, and on T2-weighted images, 2 were isointense with the cortex and one with the white matter. At surgery the tumours appeared to originate from the inferolateral wall of the lateral ventricle in the region of the head of the caudate nuclei. Total macroscopic removal was achieved in 3 patients, and subtotal removal in one patient. Follow up ranged from 4.6 to 13.2 years, and all patients have exhibited similar physical and mental conditions to preoperative. So far there has been no evidence of any recurrences. The diagnosis and the surgical indications for SEGA are discussed, with periodic monitoring with neuroimaging studies being recommended even for asymptomatic patients with 'subependymal nodules'.

Fetal germinal matrix and intraventricular haemorrhage diagnosed by MRI.

Subependymal germinal matrix haemorrhage and intraventricular haemorrhage (GMIVH) is a common complication of delivery in preterm neonates but has rarely been observed in the fetus. We report two cases of GMIVH in fetuses of 36 weeks gestation, clearly demonstrated by antenatal MRI. In both cases progressive ventriculomegaly was observed, and ventriculoperitoneal shunts were placed at 19 and 6 months of age, respectively. Prenatal GMIVH may be a cause of congenital hydrocephalus, for which no aetiology has yet been found.

[Clinical trial of bradykinin-enhancing chemotherapy for a recurrent malignant glioma: a case report].

Patients with malignant glioma undergo a combined treatment with surgical resection, radiotherapy, and chemotherapy. Although those treatments usually show some restraining effects on the tumor, a relapse occurs in most of the patients within a few years. We have investigated the feasibility and safety of intra-arterial chemotherapy for malignant brain tumors by enhancing vascular permeability using intra-arterial bradykinin infusion. In 2001, The Committee of Ethics in Kyushu University approved our clinical trial of the bradykinin-enhancing chemotherapy for recurrent malignant gliomas. We here report the first case of our clinical trial. A 31-year-old man, who had undergone surgical resection followed by chemotherapy and irradiation for malignant progression of the left frontal astrocytoma over a period of 2 years, had a relapse of the tumor in the bilateral frontal lobes. After obtaining informed consent, bradykinin and carboplatin were infused through a microcatheter at the left A1 portion under general anesthesia. By dose escalation of bradykinin, the enhanced lesion in the bilateral frontal lobes diminished on magnetic resonance imaging after 3 trials with 3-week intervals, regardless of new lesions outside of the treated area. No neurological or physiological complication including myelosuppression was noted. Bradykinin-enhancing chemotherapy appeared to be effective and safe for malignant glioma. Because it was able to increase drug delivery to the tumor, it was possible to reduce the size of the dose of chemotherapeutic agent, which resulted in minimum complication.

Diagnostic usefulness of diffusion-weighted magnetic resonance imaging in influenza-associated acute encephalopathy or encephalitis.

A magnetic resonance imaging (MRI) study was performed for a 20-month-old girl with an influenza type A infection who presented acute encephalopathy. Conventional MRI performed 8 days after the onset of encephalopathy, including T1-weighted, T2-weighted, and fluid-attenuated inversion recovery imaging, revealed only vague lesions in the right frontal, temporal, and parietal lobes. In contrast, diffusion-weighted imaging (DWI) then demonstrated the lesions much more intensively. On the 26th day, the lesions previously observed on DWI had become less discernible. The hyperintensity observed on DWI might reflect cytotoxic edema. Thus, DWI may be useful for evaluation of acute influenzal encephalopathy/encephalitis.

Correlation between proton magnetic resonance spectroscopic lactate measurements and vascular reactivity in chronic occlusive cerebrovascular disease: a comparison with positron emission tomography.

The purpose of this study is to investigate the correlation between lactate levels and cerebral vascular reactivity (VR) in regions outside an area of chronic cerebral infarction. Multivoxel proton magnetic resonance spectroscopy ((1)H-MRS) and positron emission tomography (PET) were performed in 11 patients who suffered chronic cerebral infarction. Of these 11 patients, 4 were examined before and after bypass surgery. Two regions-of-interests (ROIs) were placed outside the area of chronic infarction. One ROI was placed within a control region on the contralateral side. A lactate peak area was obtained in all ROIs. An N-acetyl aspartate (NAA) peak area was obtained in the ROI within the control region. The ratio of the lactate peak area and NAA peak area (Lct/NAA) was calculated for normalization of the lactate level, and was found to be 0.13 +/- 0. 10 (range, 0 to 0.43). The VR was recorded at 13.3 +/- 20.7% (range, - 44.3 to 68.9%), utilizing PET and administering acetazolamide. A significant negative correlation was observed between the Lct/NAA ratio and VR (r = - 0.709, p < 0.0001). These results suggest that lactate levels and VR are closely related in regions outside areas of chronic cerebral infarction.

Ketoacidosis accompanied by epileptic seizures in a patient with diabetes mellitus and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS).

We herein report a rare case of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and diabetes mellitus with ketoacidosis. An 18-year-old female patient was diagnosed to have diabetes mellitus and insulin therapy was thereafter initiated. At 26 years of age, she was hospitalized for diabetic ketoacidosis, soon followed by a loss of consciousness, left-sided dysmetria, and ataxic speech. MELAS was diagnosed because of the presence of ragged red fibers in a muscle biopsy. At 33 years of age, she was admitted to our hospital because of ketoacidosis and partial status epilepticus. A blood gas examination revealed as follows; arterial pH, 6.88; bicarbonate, 2.1 mmol/l; base excess - 29.8 mmol/l. The serum level of glucose had also increased to 30 mmol/l. The serum levels of lactate and B-hydroxybutyrate were elevated to 11.4 mmol/l and 1,990 micromol/l, respectively. Ketoacidosis improved by fluid replacement and continuous intravenous insulin infusion. A brain MRI demonstrated hyperintensity areas on FLAIR images in the bilateral temporal lobes and the cerebellum. A proton MRS demonstrated the abnormal lactate accumulation in the bilateral temporal and occipital lobes. Since epileptic seizures are rare in patients with diabetic ketoacidosis, such seizures may indicate the existence of MELAS syndrome.

Subependymoma of the lateral ventricles.

Four subependymomas of the lateral ventricle were reviewed with regard to clinical presentation, neuroimaging features, treatment, histopathological features, and long-term follow-up. There were two male and two female patients ranging in age from 27 to 60 years (mean 48.3 years). While two patients presented with symptoms and signs of raised intracranial pressure, two others were found incidentally during neuroimaging investigations to have intraventricular tumors. Neuroimaging characteristics of these tumors included no paraventricular extension, iso- or hypodensity with minimal enhancement on computerized tomography (CT), or iso- or hypointensity on T1-weighted and hyperintensity on T2-weighted magnetic resonance images (MRI). The usual finding on MRI was of no or scarce contrast enhancement, but one case showed heterogeneous enhancement. Three patients underwent total resection of the tumor and one underwent partial resection. No patients received postoperative radiation therapy. All patients have been doing well 4.8 to 15.4 years (mean 8.8 years), after surgery. Although there are no absolutely specific features to distinguish these tumors from other intraventricular tumors preoperatively, subependymoma should be kept in mind for differential diagnosis, as this tumor may safely be removed without sacrificing contiguous normal tissue and with good long-term results.

[Occipital encephalocele and epilepsy].

We report the incidence of epilepsy in 7 patients with occipital encephalocele (meningocele: 6 cases, meningoencephalocystocele: 1 case). Two cases had epilepsy and a case without the history of epileptic seizure had an epileptic pattern on the EEG. All these 3 cases had cortical dysplasia(CD) such as schizencephaly and subcortical gray matter, and two of them were associated with mental retardation. The location of the prominent CD was correlated with the EEG abnormalities and clinical epileptic pattern in each case. Associated hydrocephalus and placement of VP shunt had no significant influence on the occurrence of the epilepsy. Thus, complicated CD is thought to be epileptogenic in patients with occipital encephalocele.