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1.
Health Phys ; 119(2): 163-175, 2020 08.
Article in English | MEDLINE | ID: mdl-31913861

ABSTRACT

In the event of a radiological incident, the release of fission products into the surrounding environment and the ensuing external contamination present a challenge for triage assessment by emergency response personnel. Reference exposure rate and skin dose rate calibration data for emergency response personnel are currently lacking for cases where receptors are externally contaminated with fission products. Simulations were conducted to compute reference exposure rate coefficients and skin dose rate coefficients from photon-emitting fission products of radiological concern. To accomplish this task, simplified mathematical skin phantoms were created using surface area and height specifications from International Commission on Radiological Protection Publication 89. Simulations were conducted using Monte Carlo radiation transport code using newborn, 1-y-old, 5-y-old, 10-y-old, 15-y-old, and adult phantoms for 22 photon-emitting radionuclides. Exposure rate coefficient data were employed in a case study simulating the radionuclide inventory for a 17 × 17 Westinghouse pressurized water reactor, following three burn-up cycles at 14,600 MWd per metric ton of uranium. The decay times following the final cycle represent the relative activity fractions over a period of 0.5-30 d. The resulting data can be used as calibration standards for triage efforts in emergency response protocols.


Subject(s)
Radiation Exposure/prevention & control , Radiation Protection/methods , Adolescent , Adult , Calibration , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Kinetics , Male , Models, Statistical , Monte Carlo Method , Phantoms, Imaging , Photons , Radiation Dosage , Radiation Protection/statistics & numerical data , Radioactive Hazard Release , Risk Assessment , Skin
2.
Sci Adv ; 4(12): eaat3702, 2018 12.
Article in English | MEDLINE | ID: mdl-30585286

ABSTRACT

Prevailing theories link prefrontal cortex (PFC) maturation to the development of declarative memory. However, the precise spatiotemporal correlates of memory formation in the developing brain are not known. We provide rare intracranial evidence that the spatiotemporal propagation of frontal activity supports memory formation in children. Seventeen subjects (6.2 to 19.4 years) studied visual scenes in preparation for a recognition memory test while undergoing direct cortical monitoring. Earlier PFC activity predicted greater accuracy, and subsecond deviations in activity flow between subregions predicted memory formation. Activity flow between inferior and precentral sites was refined during adolescence, partially explaining gains in memory. In contrast, middle frontal activity predicted memory independent of age. These findings show with subsecond temporal precision that the developing PFC links scene perception and memory formation and underscore the role of the PFC in supporting memory development.


Subject(s)
Brain Mapping/methods , Brain/physiology , Memory/physiology , Prefrontal Cortex/physiology , Adolescent , Child , Electroencephalography , Female , Humans , Male , Photic Stimulation , Reaction Time , Young Adult
4.
Prog Neurobiol ; 98(3): 302-15, 2012 Sep.
Article in English | MEDLINE | ID: mdl-22480752

ABSTRACT

Epilepsy is one of the most frequent neurological diseases. In focal medically refractory epilepsies, successful surgical treatment largely depends on the identification of epileptogenic zone. High-frequency oscillations (HFOs) between 80 and 500Hz, which can be recorded with EEG, may be novel markers of the epileptogenic zone. This review discusses the clinical importance of HFOs as markers of epileptogenicity and their application in different types of epilepsies. HFOs are clearly linked to the seizure onset zone, and the surgical removal of regions generating them correlates with a seizure free post-surgical outcome. Moreover, HFOs reflect the seizure-generating capability of the underlying tissue, since they are more frequent after the reduction of antiepileptic drugs. They can be successfully used in pediatric epilepsies such as epileptic spasms and help to understand the generation of this specific type of seizures. While mostly recorded on intracranial EEGs, new studies suggest that identification of HFOs on scalp EEG or magnetoencephalography (MEG) is possible as well. Thus not only patients with refractory epilepsies and invasive recordings but all patients might profit from the analysis of HFOs. Despite these promising results, the analysis of HFOs is not a routine clinical procedure; most results are derived from relatively small cohorts of patients and many aspects are not yet fully understood. Thus the review concludes that even if HFOs are promising biomarkers of epileptic tissue, there are still uncertainties about mechanisms of generation, methods of analysis, and clinical applicability. Large multicenter prospective studies are needed prior to widespread clinical application.


Subject(s)
Biological Clocks , Electroencephalography/methods , Epilepsy/diagnosis , Epilepsy/physiopathology , Hippocampus/physiopathology , Models, Neurological , Nerve Net/physiopathology , Humans
5.
Rev Sci Instrum ; 83(2): 02B113, 2012 Feb.
Article in English | MEDLINE | ID: mdl-22380270

ABSTRACT

Electron density measurements of a large-scaled negative ion source were carried out with a surface wave probe. By comparison of the electron densities determined with the surface wave probe and a Langmuir probe, it was confirmed that the surface wave probe is highly available for diagnostic of the electron density in H(-) ion sources. In addition, it was found that the ratio of the electron density to the H(-) ion density dramatically decreases with increase of a bias voltage and the H(-) ions become dominant negative particles at the bias voltage of more than 6 V.

6.
Rev Sci Instrum ; 83(2): 02B116, 2012 Feb.
Article in English | MEDLINE | ID: mdl-22380273

ABSTRACT

We report on the characteristics of the electronegative plasma in a large-scale hydrogen negative ion (H(-)) source. The measurement has been made with a time-resolved Langmuir probe installed in the beam extraction region. The H(-) density is monitored with a cavity ring-down system to identify the electrons in the negative charges. The electron-saturation current decreases rapidly after starting to seed Cs, and ion-ion plasma is observed in the extraction region. The H(-) density steps down during the beam extraction and the electron density jumps up correspondingly. The time integral of the decreasing H(-) charge density agrees well with the electron charge collected with the probe. The agreement of the charges is interpreted to indicate that the H(-) density decreasing at the beam extraction is compensated by the electrons diffusing from the driver region. In the plasmas with very low electron density, the pre-sheath of the extraction field penetrates deeply inside the plasmas. That is because the shielding length in those plasmas is longer than that in the usual electron-ion plasmas, and furthermore the electrons are suppressed to diffuse to the extraction region due to the strong magnetic field.

7.
Rev Sci Instrum ; 81(2): 02B116, 2010 Feb.
Article in English | MEDLINE | ID: mdl-20192422

ABSTRACT

An additional beamline, BL5, equipped with four positive ion sources will be installed on Large Helical Device (LHD) in 2010. The performance of an ion source which generates 80 keV deuterium and 60 keV hydrogen beams was investigated. The structure of the ion source is based on that of a BL4 ion source on LHD. The main differences between the ion sources for the BL4 and BL5 are the acceleration voltages and the materials of plasma electrodes: copper and molybdenum, respectively. The molybdenum plasma electrode for BL5 has better performance than the copper plasma electrode of BL4. The integrated performance of the ion source for BL5 reached a value equivalent to approximately 58 A in the beam current of hydrogen positive ion at 60 keV in the beam energy.

8.
Rev Sci Instrum ; 81(2): 02B117, 2010 Feb.
Article in English | MEDLINE | ID: mdl-20192423

ABSTRACT

Characteristics of multibeamlets are investigated by means of beamlet monitoring technique. The beamlets are extracted from an accelerator with multislot grounded grid and the profiles are observed as infrared images of temperature distributions on a cold isostatic pressed graphite plate exposed by H-beamlets. The optimal horizontal and vertical divergence angles of single beamlet are estimated at 4.1 and 6.1 mrad, respectively.

9.
AJNR Am J Neuroradiol ; 30(10): 1963-70, 2009 Nov.
Article in English | MEDLINE | ID: mdl-19661173

ABSTRACT

BACKGROUND AND PURPOSE: Several studies have questioned the traditional belief that the corticospinal tract (CST) arises exclusively from the precentral gyrus and passes through the anterior half of the posterior limb of the internal capsule (PLIC) in humans; however, no direct evidence existed from structural imaging, and developmental aspects of CST origin have not been clarified. We used diffusion tensor imaging (DTI) tractography to test the hypotheses that CST can originate from both pre- and postcentral gyri and is located posteriorly in the PLIC, and we also determined how age, sex, or handedness affected these locations. MATERIALS AND METHODS: Forty-two healthy children (2.6-17.5 years of age; 20 girls) underwent DTI. Subsequently, tractography was performed on the basis of fiber assignment by continuous tracking (FACT) algorithm and brute force approach, with a fractional anisotropy (FA) threshold of <0.2 and an angle threshold of >50 degrees . The CST was isolated by using a knowledge-based region-of-interest approach, and its cortical origin and location on the PLIC was determined. RESULTS: DTI revealed that the CST originated from both pre- and postcentral gyri in 71.4% of hemispheres, from the precentral gyrus only in 19%, and from the postcentral gyrus only in 7.1%. The overall distribution was similar in both hemispheres. However, children with CST originating from both pre- and postcentral gyri were older (mean, 11.1 years of age) than those with precentral origin (mean, 5.8 years of age) or postcentral origin (mean, 7.8 years of age) only (P = .00003). The center of the CST was localized at 65% of the length (from its anterior margin) of the PLIC, and the CST occupied 26.5% of its anteroposterior length. There was a significant positive correlation between age and FA of the CST (r = 0.49; P = .002). The volume of the precentral portion of the left CST was significantly higher than that of its postcentral portion (P = .01) and that of the right CST (P = .0002). The pattern of cortical origin of CST, its location at the level of PLIC, and its volume and FA were unaffected by sex or handedness. CONCLUSIONS: The CST most frequently originates from both pre- and postcentral gyri, especially in older children, and is typically centered approximately two thirds of the distance from the anterior margin of the PLIC and occupies about a quarter of its anteroposterior length. In young children, the CST can often be seen originating exclusively from the precentral gyrus by DTI.


Subject(s)
Diffusion Magnetic Resonance Imaging/methods , Motor Cortex/anatomy & histology , Motor Cortex/growth & development , Pyramidal Tracts/anatomy & histology , Pyramidal Tracts/growth & development , Adolescent , Age Factors , Algorithms , Child , Child, Preschool , Diffusion Magnetic Resonance Imaging/standards , Efferent Pathways/anatomy & histology , Efferent Pathways/growth & development , Female , Functional Laterality , Humans , Internal Capsule/anatomy & histology , Internal Capsule/growth & development , Male , Reference Values , Sex Factors
10.
Rev Sci Instrum ; 79(2 Pt 2): 02C105, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18315231

ABSTRACT

In the large area negative ion source for the LHD negative-ion-(H(-))-based neutral beam system, (I) we used the spectrometer to measure caesium lines in the source plasma during beam shots. (II) With Doppler-shifted measurements, the H(alpha) line at three different locations along the beam as well as the spectrum profile for cases of different plasma grid areas. (III) Caesium deposition monitor with a high speed shutter was tested to measure the weight of the deposited Cs layer. In the observation, cleaner spectra of Doppler-shifted H(alpha) line with only a small level of background light were obtained at a new observation port which viewed the blueshifted light in the drift region after the accelerator of a LHD ion source. Both the amounts of Cs I (852 nm, neutral Cs(0)) and Cs II (522 nm, Cs(+)) in the source plasma light rose sharply when beam acceleration began, and continued rising during a 10 s pulse. It was thought that this was because the cesium was evaporated/sputtered from the source back plate by the back-streaming positive ions. Cs deposition rate to the crystal sensor measured by adjusting the shutter open time was evaluated to be 2.9 nanograms/s cm(2) for preliminary testing. More neutral Cs tended to be evolved in the source after arc discharge. Much Cs could be consumed in a high rate-pulsed operation (such as LHD source).

11.
Rev Sci Instrum ; 79(2 Pt 2): 02C107, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18315233

ABSTRACT

The beam profiles, port-through, rates and injection powers obtained with an improved accelerator with the multislot grounded grid are described. The accelerator has a combination of a steering grid with racetrack shaped aperture and multislot grounded grid to improve the beam optics. The optimal beam optics is obtained at the voltage ratio of 16.5-16.8, and the profiles are well fit by superposing multibeamlets with the divergent angles of 5.0 and 7.2 mrad along the direction parallel to the long and short axes of the slots of grounded grid. By adopting the racetrack shaped steering grid, the port-through rate increases from 34% to 38%, and the maximum injection power reaches 6 MW/187 keV.

12.
Biomed Pharmacother ; 59 Suppl 1: S236-8, 2005 Oct.
Article in English | MEDLINE | ID: mdl-16275501

ABSTRACT

In order to evaluate the EEG of patients with epilepsy, chaos analysis was performed for the subdural EEG time series data. The chaos attractor was reconstructed in the phase space and the correlation dimension. KS entropy calculated from the Lyapunov exponents was evaluated. Before the seizure attack, the KS entropy showed a lower value when compared with the time series data recorded during healthy condition. The results of our study suggest that it is possible to predict the seizure attack by the chaos analysis of the EEG signal. Further, we aim at developing an automatic control system for predicting a seizure attack by the use of local cooling of the focus with Peltier elements.


Subject(s)
Electroencephalography/statistics & numerical data , Epilepsy/drug therapy , Epilepsy/physiopathology , Nonlinear Dynamics , Seizures/drug therapy , Seizures/physiopathology , Algorithms , Chronobiology Phenomena , Entropy , Humans , Infant , Male , Predictive Value of Tests
13.
Ultramicroscopy ; 102(1): 13-21, 2004 Dec.
Article in English | MEDLINE | ID: mdl-15556696

ABSTRACT

Reductions in bright-field (BF) scanning transmission electron microscopy (STEM) and high-angle annular dark-field (HAADF) STEM image calculations with the aid of Bloch wave symmetry are discussed under assumptions that an absorption potential is written by a local potential and a zero-order Laue zone lies parallel to the crystal surface. Translational symmetry allows us to take only partial incident beams in the first Brillouin zone instead of enormous number of partial incident beams in a large convergent disk. Two dimensional point group confines partial incident beams to an irreducible area in addition to factoring a dispersion matrix into noninteracting submatrices on a high symmetry line using the projection operator. The drastic reductions in computing time and memory enable us to readily calculate various BF STEM and HAADF STEM images. The validity and accuracy are demonstrated in comparisons with high resolution experimental BF STEM and HAADF STEM images.


Subject(s)
Imaging, Three-Dimensional/methods , Microscopy, Electron, Scanning Transmission , Models, Theoretical , Algorithms
14.
Acta Crystallogr A ; 60(Pt 6): 591-7, 2004 Nov.
Article in English | MEDLINE | ID: mdl-15507742

ABSTRACT

Atomic resolution low-angle bright-field (LABF) scanning transmission electron-microscope (STEM) images and high-angle bright-field (HABF) STEM images of [011]-orientated Si have been experimentally obtained together with high-angle annular dark-field (HAADF) STEM images. The contrast formation mechanisms of the LABF STEM and HABF STEM images are examined in comparison with HAADF STEM images. The HABF STEM images independent of defocus and thickness have spatial resolution comparable with HAADF STEM images, and are shown to be given as a simple convolution under the non-dispersion approximation of localized Bloch waves.

15.
Neurology ; 61(3): 395-7, 2003 Aug 12.
Article in English | MEDLINE | ID: mdl-12913207

ABSTRACT

Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy underwent hemispherectomy, and pathology showed the typical findings of Rasmussen encephalitis, suggesting that these two conditions may share common etiologic factors.


Subject(s)
Encephalitis/complications , Encephalitis/diagnosis , Facial Hemiatrophy/complications , Facial Hemiatrophy/diagnosis , Child , Disease Progression , Encephalitis/surgery , Epilepsia Partialis Continua/etiology , Fluorodeoxyglucose F18 , Frontal Lobe/pathology , Frontal Lobe/surgery , Gliosis/etiology , Gliosis/pathology , Humans , Magnetic Resonance Imaging , Male , Parietal Lobe/pathology , Parietal Lobe/surgery , Tomography, Emission-Computed
16.
Neurology ; 60(6): 960-8, 2003 Mar 25.
Article in English | MEDLINE | ID: mdl-12654960

ABSTRACT

BACKGROUND: In children with tuberous sclerosis, the PET tracer alpha[11C]methyl-L-tryptophan (AMT) has been shown to be selectively taken up by epileptogenic tubers, thus allowing differentiation from nonepileptogenic tubers in the interictal state. OBJECTIVE: To determine whether cortical areas showing increased AMT uptake in children without tuberous sclerosis complex with intractable neocortical epilepsy indicate the epileptogenic zone, and to assess the relative contributions of AMT and 2-deoxy-2[18F]fluoro-D-glucose (FDG) PET abnormalities to the localization of epileptogenic cortical regions. METHODS: Areas of increased AMT and decreased FDG uptake were marked objectively as regions with abnormal asymmetry using an in-house written software in 27 children who underwent comprehensive evaluation for resective epilepsy surgery. The marked PET abnormalities were compared to the locations of scalp and subdural EEG epileptiform abnormalities, as well as histology and surgical outcome. RESULTS: Focal cortical increases of AMT uptake were found in 15 patients. The lobar sensitivity (39.0%) of AMT PET for seizure onset was lower, but its specificity (100%) was higher (p < 0.0001) than that of hypometabolism on FDG PET (sensitivity 73.2%, specificity 62.7%). AMT PET abnormalities were smaller than corresponding FDG PET hypometabolic regions (p = 0.002), and increased AMT uptake occurred in two patients with nonlocalizing FDG PET. Histologically verified cortical developmental malformations were associated with increased AMT uptake (p = 0.044). Subdural electrodes adjacent to the area of increased AMT uptake were most often involved in seizure onset. CONCLUSIONS: Focal increase of cortical AMT uptake in children is less sensitive but more specific for the lobe of seizure onset than corresponding FDG PET hypometabolism, and it is often associated with epileptogenic cortical developmental malformations. AMT PET can assist placement of subdural electrodes even when MRI and FDG PET fail to provide adequate localizing information. Cortical areas adjacent to increased AMT uptake should be carefully addressed by intracranial EEG because these regions often show a high degree of epileptogenicity.


Subject(s)
Epilepsy/diagnostic imaging , Neocortex/diagnostic imaging , Tomography, Emission-Computed , Tryptophan/analogs & derivatives , Adolescent , Child , Child, Preschool , Electroencephalography , Energy Metabolism , Epilepsy/pathology , Epilepsy/surgery , Female , Fluorodeoxyglucose F18 , Humans , Infant , Magnetic Resonance Imaging , Male , Neocortex/metabolism , Neocortex/pathology , Neocortex/surgery , Radiopharmaceuticals , Sensitivity and Specificity , Serotonin/metabolism , Treatment Outcome
17.
Brain Dev ; 23(7): 668-76, 2001 Nov.
Article in English | MEDLINE | ID: mdl-11701275

ABSTRACT

The discovery of focal or multifocal cortical lesions using magnetic resonance imaging (MRI) and positron emission tomography (PET) scanning in the majority of infants with West syndrome has led to a surgical approach in the treatment of some patients with intractable infantile spasms. The locations of these lesions should be concordant with localization of focal ictal and/or interictal electroencephalographic (EEG) abnormalities prior to proceeding with cortical resection. When a single lesion is present on the MRI or PET, and there is good correlation with EEG localization, surgical treatment is generally quite favorable in terms of both seizure control and cognitive development. Interictal glucose metabolism PET scans in children with intractable cryptogenic infantile spasms show unifocal cortical hypometabolism in about 20% of cases. In the majority, however, multifocal asymmetric hypometabolism is suggestive of multifocal underlying lesions, possibly multifocal cortical dysplasia. When the pattern of glucose hypometabolism is symmetric, a lesional etiology is less likely, thus neurometabolic or neurogenetic disorders should be considered. Therefore, the pattern of glucose hypometabolism on PET in infants with intractable cryptogenic spasms is a useful guide to decide whether a medical or surgical approach should be undertaken. In order to achieve the best cognitive outcome with surgery, it is important to resect the entire 'nociferous' area rather than just the seizure focus. Our research with new PET imaging probes has attempted to provide a comprehensive evaluation of the epileptogenic zone including the 'nociferous' cortex. We have used [(11)C]flumazenil (FMZ), which labels gamma aminobutyric acid(A) (GABA(A)) receptors, and have found this to be particularly useful in showing: (i) decreased receptor binding with medial temporal involvement thus indicating resection of medial temporal structures, (ii) the peri-lesional epileptogenic zone surrounding MRI lesions, (iii) the seizure onset zone in MRI-negative cases, and (iv) potential secondary epileptic foci. Another recently developed PET probe, alpha[(11)C]methyl-L-tryptophan (AMT) which is a precursor for the serotonin and the kynurenine metabolism pathways, is capable of differentiating between epileptogenic and non-epileptogenic tubers in patients with tuberous sclerosis complex and intractable epilepsy (including infantile spasms). Subsequently, we have applied AMT PET in patients with multifocal cortical dysplasia to determine the predominant seizure focus, and the results have been promising with regard to seizure control but not cognitive development. Thus, the introduction of newer more specific PET probes for epilepsy has led to improved and more accurate localization of seizure foci that should ultimately improve outcome of epilepsy surgery in West syndrome.


Subject(s)
Spasms, Infantile/surgery , Electroencephalography , Humans , Infant , Neurosurgical Procedures/methods , Patient Selection , Spasms, Infantile/diagnostic imaging , Tomography, Emission-Computed
18.
Neurology ; 57(7): 1269-77, 2001 Oct 09.
Article in English | MEDLINE | ID: mdl-11591847

ABSTRACT

OBJECTIVE: To examine the relationship between autism and epilepsy in relation to structural and functional brain abnormalities in children with tuberous sclerosis complex (TSC). METHODS: Children with TSC and intractable epilepsy underwent MRI as well as PET scans with 2-deoxy-2-[(18)F]fluoro-D-glucose (FDG) and alpha-[(11)C]methyl-L-tryptophan (AMT). Based on the results of Autism Diagnostic Interview-Revised, Gilliam Autism Rating Scale, and overall adaptive behavioral composite (OABC) from Vineland Adaptive Behavior Scale, subjects were divided into three groups: autistic (OABC < 70; n = 9), mentally-retarded nonautistic (OABC < 70; n = 9), and relatively normal intelligence (OABC > or = 70; n = 8). RESULTS: PET studies showed that the autistic group had decreased glucose metabolism in the lateral temporal gyri bilaterally, increased glucose metabolism in the deep cerebellar nuclei bilaterally, and increased AMT uptake in the caudate nuclei bilaterally, compared to the mentally-retarded nonautistic group. In addition, a history of infantile spasms and glucose hypometabolism in the lateral temporal gyri were both significantly associated with communication disturbance. Glucose hypermetabolism in the deep cerebellar nuclei and increased AMT uptake in the caudate nuclei were both related to stereotypical behaviors and impaired social interaction, as well as communication disturbance. CONCLUSIONS: These results suggest that generalized epilepsy in early life and functional deficits in the temporal neocortices may be associated with communication delays, and that functional imbalance in subcortical circuits may be associated with stereotypical behaviors and impaired social interaction in children with TSC.


Subject(s)
Autistic Disorder/etiology , Autistic Disorder/physiopathology , Cerebellar Nuclei/physiopathology , Cerebral Cortex/physiopathology , Tryptophan/analogs & derivatives , Tuberous Sclerosis/complications , Tuberous Sclerosis/physiopathology , Adolescent , Autistic Disorder/diagnostic imaging , Carbon Radioisotopes , Cerebellar Nuclei/metabolism , Cerebral Cortex/metabolism , Child , Child, Preschool , Female , Fluorodeoxyglucose F18 , Glucose/metabolism , Humans , Infant , Male , Radiopharmaceuticals , Spasms, Infantile/diagnostic imaging , Spasms, Infantile/etiology , Spasms, Infantile/physiopathology , Tomography, Emission-Computed , Tuberous Sclerosis/diagnostic imaging
19.
Neurology ; 57(2): 189-95, 2001 Jul 24.
Article in English | MEDLINE | ID: mdl-11468301

ABSTRACT

OBJECTIVE: To determine whether the extent and degree of glucose hypometabolism defined by PET correlate with seizure characteristics, cognitive function, and interictal EEG abnormalities in children with unilateral cerebral involvement of Sturge-Weber syndrome (SWS). METHODS: 2-Deoxy-2[18F]fluoro-D-glucose (FDG) PET was performed in 13 children (age range 0.7 to 15.1 years; five boys, eight girls) with unilateral SWS. Based on asymmetries between homologous cortical areas in FDG PET images, cortical areas of mildly (10% to 20% decrease), and severely (>20% decrease) asymmetric cortical metabolism were defined. These areas were normalized to the size of the ipsilateral hemisphere and correlated with clinical seizure characteristics, full-scale IQ, and interictal EEG abnormalities. RESULTS: Both seizure frequency (p = 0.027) and lifetime number of seizures (p = 0.017) showed a positive correlation with the area (expressed as the percentage of cortical area of ipsilateral hemisphere) of mildly asymmetric cortical metabolism. Patients with higher IQ had a shorter duration of epilepsy (p = 0.044) and a larger area of severely asymmetric cortical metabolism (p = 0.044). Patients with bilateral interictal EEG abnormalities had larger lifetime number of seizures (p = 0.042), lower IQ (p = 0.024), and smaller area of severely asymmetric cortical metabolism (p = 0.019) than those with only ipsilateral EEG abnormalities. CONCLUSIONS: Association of severely asymmetric cortical metabolism with relatively preserved cognitive function in SWS suggests that functional reorganization occurs more readily when cortex is severely rather than mildly damaged. Therefore, the area of mildly asymmetric cortical metabolism may exert a nociferous effect on the remaining of the brain. Thus, the extent and degree of glucose asymmetry detected by PET are sensitive markers of seizure severity and cognitive decline in SWS.


Subject(s)
Brain/diagnostic imaging , Fluorodeoxyglucose F18 , Sturge-Weber Syndrome/diagnostic imaging , Sturge-Weber Syndrome/physiopathology , Tomography, Emission-Computed , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male
20.
J Child Neurol ; 16(5): 357-63, 2001 May.
Article in English | MEDLINE | ID: mdl-11392521

ABSTRACT

We investigated the vasoactive intestinal peptide receptor type 2 (VIPR2) gene as a candidate gene for autism. We searched for mutations in the VIPR2 gene in autistic individuals, and 10 novel polymorphisms were identified. Three polymorphisms in the upstream region were studied in detail, and there was no significant difference in the frequencies between the autistic group (n = 14) and unrelated controls (n = 52). The distribution of the genotypes in two of the three polymorphisms differed somewhat between autistic subjects with gastrointestinal problems and those without. Moreover, there was a trend showing a correlation between the genotypes for the third polymorphism and the severity of stereotypical behavior as ranked by the Gilliam Autism Rating Scale. These preliminary results suggest that VIPR2 may have a role in gastrointestinal symptoms and stereotypical behaviors in autism, although a larger collection of samples suitable for transmission disequilibrium tests is necessary to validate the results.


Subject(s)
Autistic Disorder/genetics , Gene Expression/genetics , Polymorphism, Genetic/genetics , Promoter Regions, Genetic/genetics , Receptors, Vasoactive Intestinal Peptide/genetics , Child , Child, Preschool , Exons/genetics , Female , Gastrointestinal Diseases/genetics , Gene Frequency/genetics , Genotype , Humans , Male , Polymerase Chain Reaction
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