NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 46, Issue 6
Displaying 1-11 of 11 articles from this issue
  • Akiyoshi Kakita
    2014 Volume 46 Issue 6 Pages 413-417
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      I retrospectively reviewed the histopathologic features of surgical specimens taken consecutively from 600 patients with intractable epilepsy, and showed the scope of variation in lesions responsible for epileptogenesis. The patients were divided into three groups on the basis of age at seizure onset: 94 patients with infantile onset (before 1 year of age), 307 patients with juvenile onset (between 1 and 12 years of age), and 199 patients with adolescent/adult onset (at 13 years of age or beyond). In the infant group, seizure duration was significantly shorter than in the other groups, and malformations caused by abnormalities of cortical development, including focal cortical dysplasia (FCD) type IIa/b, tuberous sclerosis, hemimegalencephaly, and polymicrogyria were predominant, whereas in the juvenile and adolescent/adult groups, other lesions such as hippocampal sclerosis (HS), tumors, FCD type I, and vascular lesions were frequently observed. FCD type IIIa was noted in nearly half of patient with HS in both juvenile and adolescent/adult groups. The causative tumors included dysembryoplastic neuroepithelial tumors, gangliogliomas, astrocytomas, and other glioneuronal and glial tumors. Thus, various histopathological entities and types, showing clear predominance depending on the age at seizure onset, were observed in patients with epilepsy. These features appear to provide information on the pathomechanisms of the lesions and their clinical relevance in affected patients.
    Download PDF (2267K)
  • Kana Ogawa, Yukiko Mogami, Hiromitsu Toshikawa, Tomokazu Kimizu, Sadam ...
    2014 Volume 46 Issue 6 Pages 419-423
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: We evaluated the relationship between MRI findings and clinical features in patients with hypothalamic hamartoma (HH). Methods: We retrospectively reviewed MRI and clinical data (mental retardation, precocious puberty, behavioral problems, and epilepsy) in six patients (3 males and 3 females, ages 12 to 26) with HH. Based on the MRI classification by Arita, HH was classified into two types: parahypothalamic (P) and intrahypothalamic (I). Results: Only one patient was classified as having P-type HH and five were classified as having I-type HH. The patient with P-type HH (diameter 21 mm) showed precocious puberty and mild behavioral problems, but did not developed epilepsy. On the other hand, all patients with I-type HH (diameter 10-32 mm, median 17 mm) developed epilepsy and behavioral problems. Except for one patient, who had the smallest sized HH, I-type four patients developed mental retardation and precocious puberty. Among patients with I-type HH, the size of the tumor was inversely correlated with the age at epilepsy onset and with the degree of mental retardation (DQ/IQ). Conclusion: Our data suggested that the MRI classification by Arita, when combined with tumor size, might be helpful in predicting the clinical manifestations in patients with HH.
    Download PDF (570K)
  • Tomoka Kobayashi, Masumi Inagaki, Hiroko Yamazaki, Yosuke Kita, Makiko ...
    2014 Volume 46 Issue 6 Pages 424-428
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: Developmental dyslexia (DD) is a neurodevelopmental disorder that is characterized by difficulties with accurate and/or fluent word recognition and by poor spelling and decoding abilities. The magnocellular deficit theory is one of several hypotheses that have been proposed to explain the pathophysiology of DD. In this study, we investigated magnocellular system dysfunction in Japanese dyslexic children. Methods: Subjects were 19 dyslexic children (DD group) and 19 aged-matched healthy children (TD group). They were aged between 7 and 16 years. Reversed patterns of black and white sinusoidal gratings generated at a low spatial frequency, high reversal frequency of 7.5 Hz, and low contrasts were used specifically to stimulate the magnocellular system. We recorded visual evoked potentials (VEP) from the occipital area and examined their relationship with reading and naming tasks, such as the time to read hiragana characters, rapid automatized naming of pictured objects, and phonological manipulation. Results: Compared to the TD group, the DD group showed a significantly lower peak amplitude of VEPs through the complex demodulation method. Structural equation modeling showed that VEP peak amplitudes were related to the rapid automatized naming of pictured objects, and better rapid automatized naming resulted in higher reading skills. There was no correlation between VEP findings and the capacity for phonological manipulation. Conclusions: VEPs in response to the magnocellular system are useful for understanding the pathophysiology of DD. Single phonological deficit may not be sufficient to cause DD.
    Download PDF (383K)
  • Harumi Azuma, Ikuko Mohri, Masaya Tachibana, Yuko Ohno, Masako Taniike
    2014 Volume 46 Issue 6 Pages 429-437
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: To investigate the factors that affect the developmental trajectory in autism spectrum disorders (ASD) by means of a questionnaire-based retrospective analysis. Method: This study included 292 consecutive Japanese children with ASD who visited the pediatric developmental clinic at the Osaka University Hospital. Questionnaires were completed by caregivers on their first visit and were used for obtaining demographic information as well as nurturing environment and history of supportive intervention. Caregivers also fulfilled out The Infant Behavior Checklist (revised, in Japanese), and The Children's Behavior Questionnaire (revised, in Japanese). The scores for autistic traits during infancy and at present were compared in order to evaluate the developmental trajectory. Results: Early intervention may attenuate ASD symptoms, whereas unfavorable domestic conditions, any psychiatric or neurological disorder in a family member, and epilepsy as a co-morbidity may aggravate these symptoms. In addition, impaired social interaction among all other ASD symptoms was specifically improved by early intervention, even if such intervention was not tailored to ASD. Conclusion: Early intervention and family support are important for children with ASD to promote their social development.
    Download PDF (459K)
  • Shigeaki Hamada, Seishi Shimakawa, Shigeko Satomura, Etsuo Naito, Tosh ...
    2014 Volume 46 Issue 6 Pages 439-442
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      We report a 21-year-old male patient with dentatorubral-pallidoluysian atrophy (DRPLA) showing progressive myoclonus epilepsy (PME), who responded to levetiracetam (LEV) at an initial dose of 1,000 mg/day. The patient developed epilepsy at the age of 10 years, and also showed intellectual regression. Various antiepileptic drugs showed no effects on generalized tonic seizures, tonic-clonic seizures, and myoclonus. Addition of LEV (1,000 mg/day) led to the reduction of myoclonus and tonic-clonic seizures, and improved the EEG and sleep-wake rhythm. He had a better appetite and gain weight. It is suggested that LEV may improve quality of life in patients with DRPLA, in addition to reducing the frequency of epileptic seizures.
    Download PDF (1240K)
  • Seiji Watanabe, Yoshinori Okumura, Hideo Aiba
    2014 Volume 46 Issue 6 Pages 443-446
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      We experienced a case of acute encephalitis with refractory, repetitive partial seizures (AERRPS) found in an 8-year-old boy. Convulsive status epilepticus developed at the onset, which was intractable to the treatment with intravenous thiopental sodium even at the maximum dose of 9 mg/kg/hr. Since the adverse effect developed, thiopental sodium was discontinued. Phenobarbital (PB) was administrated at a very high daily dose up to 80 mg/kg, reaching serum trough level of 250 μg/ml, which was markedly effective to the treatment. Because seizures reappeared during tapering the dosage of PB, potassium bromide (KBr) at a daily dose of 80 mg/kg was additionally administrated. PB was successfully tapered into a daily dose of 20 mg/kg with a trough serum level around 80 μg/ml. He recovered in motor functions, but had disturbance of memory and apneic seizures. A very-high-dose PB therapy in an early period may be helpful for the treatment of intractable convulsive status epilepticus.
    Download PDF (427K)
feedback
Top