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Iwasaki M.,Tohoku University | Uematsu M.,Tohoku University | Sato Y.,Tohoku University | Nakayama T.,Tohoku University | And 6 more authors.
Journal of Neurosurgery: Pediatrics | Year: 2012

Object. Corpus callosotomy is usually intended to alleviate - not to achieve total control of - epileptic seizures. A few patients experience complete seizure control after callosotomy, but the associated clinical factors are unknown. The object of this study was to investigate clinical factors associated with long-term seizure remission after total corpus callosotomy in patients with infantile or early childhood onset epilepsy. Methods. Thirteen consecutive patients with infantile or early childhood onset epilepsy underwent 1-stage total corpus callosotomy for alleviation of seizures. Their age at surgery ranged from 1 year and 5 months to 24 years (median 7 years). Eleven patients had West syndrome at the onset of disease, and the other 2 had Lennox-Gastaut syndrome. All patients suffered from spasms, axial tonic seizures, or atonic seizures. Six patients had proven etiology of epilepsy, including tuberous sclerosis, polymicrogyria, trauma, and Smith-Magenis syndrome. The association between postoperative seizure freedom and preoperative factors including age at surgery, no MRI abnormalities, proven etiology, and focal electroencephalographic epileptiform discharges was examined. Results. Postoperative seizure freedom was achieved in 4 of 13 patients for a minimum of 12 months. All 4 patients had no MRI abnormalities and no identified etiology. None of the 8 patients with MRI abnormality, 6 patients with known etiology of epilepsy, or 4 patients aged older than 10 years at surgery achieved seizure freedom. Two of the 7 patients with focal electroencephalographic abnormalities became seizure free. Absence of MRI abnormalities was significantly associated with postoperative seizure freedom (p < 0.01). Conclusions. Complete seizure remission is achieved after total corpus callosotomy in a subgroup of patients with intractable epilepsy following West syndrome or Lennox-Gastaut syndrome. One-stage total corpus callosotomy at a young age may provide a higher rate of seizure freedom, especially for patients with no MRI abnormalities and no identified etiology of epilepsy.

Zhao Y.-J.,Liaoning Medical University | Wang H.,Liaoning Medical University | Liu X.,Liaoning Medical University | Sun M.,Liaoning Medical University | Kazuhiro H.,Takuto Rehabilitation Center for Children
Molecular Medicine Reports | Year: 2012

The aim of this study was to characterize the protective effects of glutamine (Gln) on brain cells undergoing experimental endotoxemia induced by lipopolysaccharide (LPS) injection. Young rats were injected with LPS or control, and a subset of LPS-injected rats were pretreated with Gln. Electron microscopy and immunohistochemistry were used to visualize apoptosis and to determine distribution and expression of nuclear factor-κB (NF-κB), heat shock protein 70 (HSP70), platelet-derived growth factor-B (PDGF-B) and PDGF receptor-β (PDGFR-β). The levels of HSP70, PDGF-B and PDGFR-β in the rat brain were comparatively analyzed by western blotting. In a rat brain model of endotoxemia, Gln decreases the magnitude of apoptosis, upregulates the expression of HSP70 and inhibits the translocation of NF-κB from the cytoplasm to the nucleus. Gln upregulates PDGF-B and PDGFR-β expression in early and advanced sepsis. PDGF-B and PDGFR-β upregulation in the cerebral cortex are likely neuroprotective effects of Gln. We found that Gln is capable of regulating the immunological defense of local brain tissue, which provides a theoretical basis for using Gln to prevent and treat encephalopathy.

Tanaka S.,Takuto Rehabilitation Center for Children | Tanaka S.,Tohoku University
Brain and Development | Year: 2013

Relative to their numbers, more than twice the number of disabled children fell victim to the Great East Japan Earthquake than did normal people. It was important to find out needs and provide support, as the needs of disabled children vulnerable to the disaster, such as a shortage of diapers of the right size for disabled children in the affected areas, were not given priority. In addition, the role of coordinators to spread word of who needed what and where, and linking this to specific support, was important. Regions and authorities need to determine how disabled children are to be evacuated in a disaster. Each household should prepare, as disaster prevention measures, their own private power generator and carry medical information for oral or other medicine. Each region should prepare, as a local disaster measure, welfare evacuation areas for disabled children. One thing that was felt acutely in this recent disaster is that disaster preparations and manuals need to be revised from the point of view of welfare, and that the most reliable people were those who, whether as assisters or the assisted, were involved with the disabled on a daily basis from before the disaster. The existence of disabled children as a familiar part of society, and supporting agencies networking based around the children as part of normal operations, plays a very large part. Raising children as part of their local communities is the biggest factor in saving them from disasters. © 2012 The Japanese Society of Child Neurology.

Sugiura M.,Tohoku University | Sassa Y.,Tohoku University | Jeong H.,Tohoku University | Wakusawa K.,Tohoku University | And 6 more authors.
Human Brain Mapping | Year: 2012

The concept of "social self" is often described as a representation of the self-reflected in the eyes or minds of others. Although the appearance of one's own face has substantial social significance for humans, neuroimaging studies have failed to link self-face recognition and the likely neural substrate of the social self, the medial prefrontal cortex (MPFC). We assumed that the social self is recruited during self-face recognition under a rich social context where multiple other faces are available for comparison of social values. Using functional magnetic resonance imaging (fMRI), we examined the modulation of neural responses to the faces of the self and of a close friend in a social context. We identified an enhanced response in the ventral MPFC and right occipitoparietal sulcus in the social context specifically for the self-face. Neural response in the right lateral parietal and inferior temporal cortices, previously claimed as self-face-specific, was unaffected for the self-face but unexpectedly enhanced for the friend's face in the social context. Self-face-specific activation in the pars triangularis of the inferior frontal gyrus, and self-face-specific reduction of activation in the left middle temporal gyrus and the right supramarginal gyrus, replicating a previous finding, were not subject to such modulation. Our results thus demonstrated the recruitment of a social self during self-face recognition in the social context. At least three brain networks for self-face-specific activation may be dissociated by different patterns of response-modulation in the social context, suggesting multiple dynamic self-other representations in the human brain. © 2011 Wiley Periodicals, Inc.

Miyatake S.,Yokohama City University | Osaka H.,Clinical Research Institute | Shiina M.,Yokohama City University | Takanashi J.-I.,Kameda Medical Center | And 11 more authors.
Neurology | Year: 2014

Objective: We performed whole-exome sequencing analysis of patients with genetically unsolved hypomyelinating leukoencephalopathies, identifying 8 patients with TUBB4A mutations and allowing the phenotypic spectrum of TUBB4A mutations to be investigated. Methods: Fourteen patients with hypomyelinating leukoencephalopathies, 7 clinically diagnosed with hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC), and 7 with unclassified hypomyelinating leukoencephalopathy, were analyzed by whole-exome sequencing. The effect of the mutations on microtubule assembly was examined by mapping altered amino acids onto 3-dimensional models of the ab-tubulin heterodimer. Results: Six heterozygous missense mutations in TUBB4A, 5 of which are novel, were identified in 8 patients (6/7 patients with H-ABC [the remaining patient is an atypical case] and 2/7 patients with unclassified hypomyelinating leukoencephalopathy). In 4 cases with parental samples available, the mutations occurred de novo. Analysis of 3-dimensional models revealed that the p.Glu410Lys mutation, identified in patients with unclassified hypomyelinating leukoencephalopathy, directly impairs motor protein and/or microtubule- associated protein interactions with microtubules, whereas the other mutations affect longitudinal interactions for maintaining ab-tubulin structure, suggesting different mechanisms in tubulin function impairment. In patients with the p.Glu410Lys mutation, basal ganglia atrophy was unobserved or minimal although extrapyramidal features were detected, suggesting its functional impairment. Conclusions: TUBB4A mutations cause typical H-ABC. Furthermore, TUBB4A mutations associate cases of unclassified hypomyelinating leukoencephalopathies with morphologically retained but functionally impaired basal ganglia, suggesting that TUBB4A-related hypomyelinating leukoencephalopathies encompass a broader clinical spectrum than previously expected. Extrapyramidal findings may be a key for consideration of TUBB4A mutations in hypomyelinating leukoencephalopathies. © 2014 American Academy of Neurology.

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