NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Search
Advanced search
OR
Browse
Search
Volume 41, Issue 6
Displaying 1-15 of 15 articles from this issue
Show all abstracts
    • |<
    • <
    • 1
    • >
    • >|
  • Epigenetic Mechanisms Regulating Neural Cell Fate Determination
    Kinichi Nakashima, Jun Kohyama, Masakazu Namihira, Fred H Gage, Hideyu ...
    2009 Volume 41 Issue 6 Pages 411-414
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.411
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      Neural stem/progenitor cells (NSCs/NPCs) give rise to neurons, astrocytes, and oligodendrocytes. It has become apparent that intracellular epigenetic modification including DNA methylation, in concert with extracellular cues such as cytokine signaling, is deeply involved in specifiying the fate of NSCs/NPCs by defining cell-type specific gene expression. However, it is still unclear how differentiated neural cells retain their specific attributes by repressing cellular properties characteristic of other lineages. In previous work, we have shown that methyl-CpG binding protein transcriptional repressors (MBDs), which were expressed predominantly in neurons in the central nervous system, inhibited astrocyte-specific gene expression by binding to highly methylated regions of their target genes. Here we report that oligodendrocytes, which do not express MBDs, can transdifferentiate into astrocytes both in vitro (cytokine stimulation) and in vivo (ischemic injury) through the activation of the JAK/STAT signaling pathway. These findings suggest that differentiation plasticity in neural cells is regulated by cell-intrinsic epigenetic mechanisms in collaboration with ambient cell-extrinsic cues.
    View full abstract
    Download PDF (376K)
  • Neurological Findings in Severe Congenital Neutropenia with HAX1 Mutations
    Nobutsune Ishikawa, Masao Kobayashi
    2009 Volume 41 Issue 6 Pages 415-419
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.415
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      HAX1 is an anti-apoptotic factor with multiple functions that controls the integrity of the inner mitochondrial membrane potential and interacts with various viruses and cellular proteins. We have already reported that severe congenital neutropenia (SCN) with HAX1 mutations produces neurological symptoms. In this report, we studied the correlation between the neurological symptoms and genetic mutations in all reported cases of HAX1-deficient SCN, including our five cases. Twelve of the 40 patients with HAX-1-deficient SCN had cognitive impairment and ten of these 12 patients suffered from epilepsy. Based on transcription, HAX1 has two isoforms: isoforms a and b. Neurological symptoms were found in HAX1-deficient patients with mutations in the HAX1 gene affecting both transcript variants, while they were not found in those affecting isoform a only. These results suggest that impairment of both of HAX1 isoforms leads to neurological dysfunction.
    View full abstract
    Download PDF (239K)
  • A Study of Cognitive and Behavioral Development in Pre-School and School Children with High Functioning Pervasive Developmental Disorder
    Yoshimi Tsuda, Toshiaki Hashimoto, Kenji Mori, Mio Nishimura, Aya Fuku ...
    2009 Volume 41 Issue 6 Pages 420-425
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.420
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      It has been reported that school-aged children with high functioning pervasive developmental disorder (HFPDD) have numerous difficulties in their school class.
      We used three psychological tests to investigate whether there is a relationship between intelligence and cognitive, behavioral development in children with HFPDD. The three tests used were an intelligence test (WIPPSI, WISC-III), the P-F (Picture Frustration) study, and behavioral assessment by their parents. In the P-F study, 60% of 23 children with HFPDD showed a GCR% (Group Conformity Rating) above or below the standard. There was no relationship between GCR% and IQ. In the behavioral assessment by their parents, over 50% of 40 children with HFPDD showed maladaptive behaviors. The high VIQ group showed more maladaptive behaviors than the low VIQ group. These findings suggest that school-aged children with H-FPDD need educational treatment for social deficits and maladaptive behaviors.
    View full abstract
    Download PDF (274K)
  • Standard Value and Developmental Changes in the Indices of Interference Effect in the Modified Stroop Test
    Toshimi Hirasawa, Satoshi Sanada, Masafumi Yanagihara, Yasuko Tsushima ...
    2009 Volume 41 Issue 6 Pages 426-430
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.426
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      The Stroop test was originally invented by Stroop to measure selective attention and cognitive flexibility and various versions of this test have been developed by many other researchers. Since the Stroop test requires the examinee's sustained efforts, it is not readily applicable to children with developmental disorders. In order to overcome this weakness, a modified Stroop test by reducing the total number of stimulations from 300 to 72 was proposed for clinical use. This study was performed to obtain the standard value of the modified Stroop test, and also to clarify the developmental changes in indices of interference effect. Two hundred eighty one normal children and adults, ranging from 6 to 20 years of age were examined. A simple regression analysis was performed to examine the relation between age and the score of indices such as Incongruent Color Naming (ICN), ICN － Color Naming (CN), ICN/CN. The results from this analysis showed significant age-related changes. Subjects between sixteen and seventeen showed the best score in each index. These findings suggest that a brain region and/or functional system of late maturation might participate in the execution of the interference task.
    View full abstract
    Download PDF (580K)
  • Waiting-for-Instruction Behavior as Depressive Symptom in Mentally Retarded Autistic Children and Adolescents and Its Treatment
    Hiroyuki Yokoyama, Mieko Hirose, Chieko Nara, Keisuke Wakusawa, Yuki K ...
    2009 Volume 41 Issue 6 Pages 431-435
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.431
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      We have seen 9 moderately to severely mentally-retarded autistic children and adolescents who waited for small-step instructions to perform previously acquired daily life activities (called “waiting-for-instruction” behavior). None of these patients were capable of expressing their depressive mood. All cases were considered to meet the criteria for major depressive episode described in DSM-IV. The “waiting-for-instruction” behavior was suggested to be a diagnostic key for depressive state in mentally retarded children and adolescents. GAF scales for depressive symptoms including the “waiting-for-instruction” behavior improved in 7 of these 9 cases with fluvoxamine. Risperidone and valproate sodium were useful for these symptoms in patients who were not responsive to fluvoxamine. Therefore, there is a possibility that they met the criteria for bipolar II disorder in DSM-IV.
    View full abstract
    Download PDF (277K)
  • Clinical Assessment of the Effect of Switch from Immediate-Release to Extended-Release Methylphenidate in 181 Patients with Isolated attention dificit/hyperactivity disorder (AD/HD) or AD/HD Associated with Pervasive developmental disorder
    Michiko Sugama, Asayo Ishizaki
    2009 Volume 41 Issue 6 Pages 436-441
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.436
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      We switched medication from conventional immediate-release preparations of methylphenidate to extended-release tablets (an osmotic release oral system) in 165 of 181 cases with attention deficit/hyperactivity disorder (AD/HD), in accordance with the revised indications for these tablets. We investigated the types of developmental disorders, doses of the drug, efficacy, adverse effects, concomitant medication, other relevant problems, and so on, prior to switching the medications. The most common types of developmental disorders were AD/HD with symptoms of pervasive developmental disorder (PDD) and PDD with symptoms of AD/HD. The efficacy evaluation revealed that the extended release tablets had efficacy equivalent to or greater than that of the immediate release preparation. The efficacy rate was 82.7% in our patients. However, cases with difficulty ingesting the tablets, requiring small doses, and those over 18 years of age still seen in the Pediatrics department, remained as problematic issues which require further consideration.
    View full abstract
    Download PDF (251K)
  • Criteria for Differential Diagnosis of Epilepsy and Long QT syndrome Presenting with Seizures and EEG Abnormalities
    Ayumi Yamamoto, Go Yamamoto, Masaharu Ohfu, Kunihiro Hamamoto, Sawa Ya ...
    2009 Volume 41 Issue 6 Pages 442-446
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.442
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      We report a 13-year-old girl with congenital long QT syndrome (LQTS) who developed a cluster of generalized tonic clonic seizures with post-ictal EEG abnormality. The provisional diagnosis was epilepsy. However, ECG monitoring showed torsade de pointes, and thus the final diagnosis was LQTS. Although LQTS can be potentially misdiagnosed as epilepsy when it presents with seizures, it is important to differentiate LQTS from epilepsy because patients with LQTS are at risk of sudden death. We reviewed 11 previously reported cases with LQTS and EEG abnormalities who were initially diagnosed as epilepsy. We emphasized the importance of the following five criteria in the differentiation of LQTS from epilepsy: 1) awareness that LQT2 and LQT3 can cause life-threatening arrhythmia at rest or during sleep, 2) examination of arterial pulse during seizures, 3) monitoring ECG during EEG recording, 4) careful establishment of the correct diagnosis taking into consideration the interictal EEG findings, and 5) reconsidering the possibility of cardiac origin when the attacks cannot be controlled even by therapeutic levels of antiepileptic drugs in the blood.
    View full abstract
    Download PDF (1565K)
  • Fulminating Meningitis Caused by Haemophilus Influenzae with Rapid Progression of Severe Brain Edema Similar to Acute Encephalopathy
    Kentaro Shirai, Keisuke Nakajima, Akimitsu Watanabe, Yutaka Kawano, Hi ...
    2009 Volume 41 Issue 6 Pages 447-451
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.447
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      We report a 4-year-old boy with fulminating meningitis caused by Haemophilus influenzae (Hib). He suddenly developed fever, vomiting and then somnolence. As bacterial meningitis was suspected, treatment with antibiotics was started at 12 hours after the onset. However, there was arapid progression of severe brain edema and brain hernia, leading to clinical brain death. His clinical course and neuroradiological findings mimicked those in patients with acute encephalopathy, with cytokine profiles in cerebrospinal fluid demonstrating a marked increase of inflammatory cytokines. From a review of the literature, fulminating Hib meningitis may be classified into two disease types: DIC plus multiple organ failure and acute brain swelling types. The present case belongs to the latter type, in which cytokine storm seems to play an important pathogenic role.
    View full abstract
    Download PDF (476K)
  • Two Patients with Congenital Insensitivity to Pain with Anhidrosis Showing Marked Prolongation of Central Conduction Time in Short Latency Somatosensory Evoked Potential
    Ryuta Tanaka, Kenji Sugai, Yoshinao Fujikawa, Hirofumi Komaki, Eiji Na ...
    2009 Volume 41 Issue 6 Pages 452-456
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.452
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      We examined the evoked potentials in 2 patients, a 6-month-old girl and a 3-year-old boy, with congenital insensitivity to pain with anhidrosis (CIPA). While auditory brainstem response (ABR) in both patients showed normal latencies, flash visual evoked potential (F-VEP) revealed delayed latency of wave IV (P100), and short latency somatosensory evoked potential (SSEP) demonstrated marked prolongation of the central conduction time (CCT; N13-N20 interval). The boy had West syndrome and his prolonged CCT might have been influenced by abnormal cortical activities. The girl did not have epilepsy and the abnormalities of her F-VEP and SSEP might have been caused by the developmental deficit of the central nervous system associated with the pathogenesis of CIPA.
    View full abstract
    Download PDF (832K)
  • Reappraisal of Vitamin B6 Therapy for West Syndrome
    Yuki Imai, Harumi Yoshinaga, Yumiko Ishizaki, Yoshiaki Watanabe, Yoko ...
    2009 Volume 41 Issue 6 Pages 457-461
    Published: 2009
    Released on J-STAGE: May 11, 2016
    DOIhttps://doi.org/10.11251/ojjscn.41.457
    JOURNAL FREE ACCESS
    Show abstractHide abstract
      Vitamin B6 (VB6) is used frequently as one of the first-choice drug for the treatment of West syndrome (WS) in Japan. We report 2 cases of symptomatic WS who had a good response to readministration of VB6.
      Case 1 was a 3-year-old girl diagnosed as having severe hydroencephalus. She developed WS at the age of 9 months. She was treated with ACTH, but relapsed when she was 1 year old. Despite treatment with various conventional drugs and second ACTH therapy, her seizures were not suppressed. We reviewed past treatment records in another hospital, and found that VB6 treatment was stopped because her EEG did not improve within a week. We then retried VB6 therapy when she was 3 years and 6 months old, and as a result she became seizure-free and hypsarrhythmia disappeared on EEG within a month.
      Case 2 was a boy with severe cerebral palsy who was diagnosed as having WS at the age of 9 months. His MRI revealed bilateral subdural hematoma. Treatment was started with VB6, and he became seizure-free within several days. But 7 days after starting VB6, treatment was stopped because of the side effects. VPA was started instead, but his EEG showed gradual worsening. Then, we added a smaller dose of VB6 to VPA. His EEG improved dramatically, and his seizures have been controlled without any side effects.
      The present cases indicate the possible clinical usefulness of successful VB6 retrials even in older patients with severe organic lesions, by means of combined therapy with other drug, and alternative design of the dosing. However, we suspect there might be many cases in which the efficacy of VB6 therapy has not been properly assessed due to the short observation period and/or side effects.
    View full abstract
    Download PDF (1404K)
    • |<
    • <
    • 1
    • >
    • >|
feedback
 Top