OBJECTIVE: To investigate gray matter concentration changes in the brains of narcoleptic patients. MATERIALS AND METHODS: Twenty-nine narcoleptic patient with cataplexy and 29 age and sex-matched normal subjects (mean age, 31 years old) underwent volumetric MRIs. The MRIs were spatially normalized to a standard T1 template and subdivided into gray matter, white matter, and cerebrospinal fluid (CSF). These segmented images were then smoothed using a 12-mm full width at half maximum (FWHM) isotropic Gaussian kernel. An optimized voxel-based morphometry protocol was used to analyze brain tissue concentrations using SPM2 (statistical parametric mapping). A one-way analysis of variance was applied to the concentration analysis of gray matter images. RESULTS: Narcoleptics with cataplexy showed reduced gray matter concentration in bilateral thalami, left gyrus rectus, bilateral frontopolar gyri, bilateral short insular gyri, bilateral superior frontal gyri, and right superior temporal and left inferior temporal gyri compared to normal subjects (uncorrected p < 0.001). Furthermore, small volume correction revealed gray matter concentration reduction in bilateral nuclei accumbens, hypothalami, and thalami (false discovery rate corrected p < 0.05). CONCLUSION: Gray matter concentration reductions were observed in brain regions related to excessive daytime sleepiness, cognition, attention, and memory in narcoleptics with cataplexy.
Adult ; Analysis of Variance ; Brain/pathology/*radionuclide imaging ; Case-Control Studies ; Cataplexy/radionuclide imaging ; Female ; Humans ; Image Processing, Computer-Assisted ; Magnetic Resonance Imaging ; Male ; Narcolepsy/pathology/*radionuclide imaging ; Polysomnography

PURPOSE: To investigate the general characteristics of glucose metabolism distribution and the functional deficit in the brain of children with developmental language delay (DLD), we compared functional neuroradiological studies such as positron emission tomography (PET) of a patient group of DLD children and a control group of attention- deficit hyperactivity disorder (ADHD) children. PATIENTS AND METHODS: Seventeen DLD children and 10 ADHD children under 10 years of age were recruited and divided into separate groups consisting of children less than 5 years of age or between 5 and 10 years of age. The PET findings of 4 DLD children and 6 control children whose ages ranged from 5 to 10 years were compared by Statistical Parametric Mapping (SPM) analysis. RESULTS: All of the DLD children revealed grossly normal findings in brain MRIs, however, 87.5% of them showed grossly abnormal findings in their PET studies. Abnormal findings were most frequent in the thalamus. The patient group showed significantly decreased glucose metabolism in both frontal, temporal and right parietal areas (p < 0.005) and significantly increased metabolism in both occipital areas (p < 0.05) as compared to the control group. CONCLUSION: This study reveals that DLD children may show abnormal findings on functional neuroradiological studies, even though structural neuroradiological studies such as a brain MRI do not show any abnormal findings. Frequent abnormal findings on functional neuroradiological studies of DLD children, especially in the subcortical area, suggests that further research with quantitative assessments of functional neuroradiological studies recruiting more DLD children and age-matched normal controls could be helpful for understanding the pathophysiology of DLD and other disorders confined to the developmental disorder spectrum.
Attention Deficit Disorder with Hyperactivity/metabolism/*pathology ; Basal Ganglia/abnormalities/metabolism/radionuclide imaging ; Brain/*abnormalities/metabolism/radionuclide imaging ; Caudate Nucleus/abnormalities/metabolism/radionuclide imaging ; Cerebellum/abnormalities/metabolism/radionuclide imaging ; Child ; Child, Preschool ; Glucose/metabolism ; Humans ; Language Development Disorders/metabolism/*pathology ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; Thalamus/abnormalities/metabolism/radionuclide imaging

This study examined the effectiveness of Holmium-166 (Ho-166) chitosan complex therapy for a malignant glioma. Cultured C6 glioma cells (100, 000 in 5microliter) were injected into the caudate/putamen of 200 - 250 gram Wistar rats. Five days later, a Ho-166 chitosan complex was injected into the same site of the glioma injection. Four injection doses were administered: the control group received PBS 10microliter, group 1 received an injection of 100micro Ci (10microliter), group 2 received an injection of 50microCi (5microliter), and group 3 received an injection of 10micro Ci (1microliter). The average tumor volume for each group was 1.385 mm3 for the control group, 0.036 mm3 for group 1, 0.104 mm3 for group 2, and 0.111 mm3 for group 3. Compared with the control group, the size of the tumors in groups 1, 2 and 3 was reduced by an average of 97.4%, 92.5% and 91.9%, respectively. The Kaplan-Meier survival curve of group 2 was the longest, followed by groups 3, group 1 and the control. The mean survival was 22.8, 59, 60, and 44.6 days for the control group and groups 3, 2 and 1, respectively. H-E staining revealed that group 2 yielded the best results in the destruction of the malignant glioma. TUNEL staining and immunohistochemical studies indicated apoptotic features. The Ho-166 chitosan complex proved to be effective in destroying the malignant glioma.
Animals ; Brachytherapy ; Brain Neoplasms/mortality/pathology/*radionuclide imaging ; Cell Line, Tumor ; Chitin/analogs & derivatives/*pharmacology ; Disease Models, Animal ; Glioma/mortality/pathology/*radionuclide imaging ; Holmium/*pharmacology ; Radioisotopes/*pharmacology ; Rats ; Rats, Wistar ; Research Support, Non-U.S. Gov't

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

Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of endocrine and non-endocrine tumors. There are also a considerable number of atypical MEN1 syndrome. In this case, a 68-yr-old woman was referred to the Department of Endocrinology for hypercalcemia. Five years ago, she had diagnosed as primary hyperaldosteronism and now newly diagnosed as parathyroid hyperplasia with laboratory and pathologic findings. Hurthle-cell thyroid cancer was also resected during the parathyroid exploration and small meningioma was found on brain MRI. Her general condition has markedly improved and her adrenal mass and meningioma are being closely observed now. We could find the loss of heterozygosity of the MEN1 locus in parathyroid glands, suggesting a MEN1-related tumor, but not a germline mutation. Considering a variety of phenotypic expression and a limitation of current molecular analysis, periodic follow up will be needed in patients with a MEN1-like phenotype.
Aged ; Base Sequence ; Brain/radionuclide imaging ; Female ; Humans ; Hyperaldosteronism/complications/*diagnosis ; Hyperparathyroidism, Primary/*diagnosis/etiology/pathology ; Loss of Heterozygosity ; Magnetic Resonance Imaging ; Meningeal Neoplasms/complications/*diagnosis/radionuclide imaging ; Meningioma/complications/*diagnosis/radionuclide imaging ; Mutation ; Parathyroid Glands/pathology ; Proto-Oncogene Proteins/genetics/metabolism ; Sequence Analysis, DNA ; Thyroid Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

Few studies have included magnetoencephalography (MEG) when assessing the diagnostic value of presurgical modalities in a nonlesional epilepsy population. Here, we compare single photon emission computed tomography (SPECT), positron emission tomography (PET), video-EEG (VEEG), and MEG, with intracranial EEG (iEEG) to determine the value of individual modalities to surgical decisions. We analyzed 23 adult epilepsy patients with no abnormal MRI findings who had undergone surgical resection. Localization of individual presurgical tests was determined for hemispheric and lobar locations based on visual analysis. Each localization result was compared with the ictal onset zone (IOZ) defined by using iEEG. The highest to the lowest hemispheric concordance rates were MEG (83%) > ictal VEEG (78%) > PET (70%) > ictal SPECT (57%). The highest to lowest lobar concordance rates were ictal VEEG = MEG (65%) > PET (57%) > ictal SPECT (52%). Statistical analysis showed MEG to have a higher hemispheric concordance than that of ictal SPECT (P = 0.031). We analyzed the effects of MEG clustered-area resection on surgical outcome. Patients who had resection of MEG clusters showed a better surgical outcome than those without such resection (P = 0.038). It is suggested that MEG-based localization had the highest concordance with the iEEG-defined IOZ. Furthermore, MEG cluster resection has prognostic significance in predicting surgical outcome.
Adolescent ; Adult ; Brain/*pathology/surgery ; *Brain Mapping ; Electroencephalography ; Epilepsy/*radionuclide imaging/surgery ; Female ; Humans ; Magnetic Resonance Imaging ; *Magnetoencephalography ; Male ; Middle Aged ; Positron-Emission Tomography ; Preoperative Care ; Tomography, Emission-Computed, Single-Photon ; Video Recording

Few studies have included magnetoencephalography (MEG) when assessing the diagnostic value of presurgical modalities in a nonlesional epilepsy population. Here, we compare single photon emission computed tomography (SPECT), positron emission tomography (PET), video-EEG (VEEG), and MEG, with intracranial EEG (iEEG) to determine the value of individual modalities to surgical decisions. We analyzed 23 adult epilepsy patients with no abnormal MRI findings who had undergone surgical resection. Localization of individual presurgical tests was determined for hemispheric and lobar locations based on visual analysis. Each localization result was compared with the ictal onset zone (IOZ) defined by using iEEG. The highest to the lowest hemispheric concordance rates were MEG (83%) > ictal VEEG (78%) > PET (70%) > ictal SPECT (57%). The highest to lowest lobar concordance rates were ictal VEEG = MEG (65%) > PET (57%) > ictal SPECT (52%). Statistical analysis showed MEG to have a higher hemispheric concordance than that of ictal SPECT (P = 0.031). We analyzed the effects of MEG clustered-area resection on surgical outcome. Patients who had resection of MEG clusters showed a better surgical outcome than those without such resection (P = 0.038). It is suggested that MEG-based localization had the highest concordance with the iEEG-defined IOZ. Furthermore, MEG cluster resection has prognostic significance in predicting surgical outcome.
Adolescent ; Adult ; Brain/*pathology/surgery ; *Brain Mapping ; Electroencephalography ; Epilepsy/*radionuclide imaging/surgery ; Female ; Humans ; Magnetic Resonance Imaging ; *Magnetoencephalography ; Male ; Middle Aged ; Positron-Emission Tomography ; Preoperative Care ; Tomography, Emission-Computed, Single-Photon ; Video Recording

We report a 52-yr-old Korean woman with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) whose diagnosis was confirmed by skin biopsy and the presence of a novel mutation in the NOTCH3 gene. The patient's clinical features were rather unusual in that 1) clinical presentations were only two episodes of stroke and mild dementia unaccompanied by mood disturbances or migraine, and 2) there was no family history. Brain MRI showed T2 hyperintensities in both temporal pole areas in line with the recent suggestion by O'Sullivan et al. that the abnormality could be a radiologic marker of CADASIL. FDG-PET also showed a hypometabolism in the temporal pole areas with an abnormal finding on MRI in addition to the hypometabolism in cortical and subcortical regions. We could learn from this case that CADASIL may be included in the differential diagnoses in patients with vascular dementia associated with a small vessel disease, even in the absence of a family history, especially when there are no known stroke risk factors and when the MRI shows T2 hyperintensity in the temporal pole regions.
Amino Acid Substitution ; Biopsy ; Brain/pathology ; Codon/genetics ; Dementia, Multi-Infarct/diagnosis ; Dementia, Multi-Infarct/genetics* ; Dementia, Multi-Infarct/pathology ; Dementia, Multi-Infarct/radionuclide imaging ; Female ; Human ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Mutation, Missense* ; Neuropsychological Tests ; Point Mutation* ; Proto-Oncogene Proteins/genetics* ; Skin/pathology ; Tomography, Emission-Computed