Journal of the Japan Epilepsy Society
Online ISSN : 1347-5509
Print ISSN : 0912-0890
ISSN-L : 0912-0890
Volume 28, Issue 3
Displaying 1-7 of 7 articles from this issue
Editorial
Original Articles
  • Satsuki Watanabe, Shin Tanaka, Keiko Hino, Keiko Hara, Masako Watanabe
    2011 Volume 28 Issue 3 Pages 393-400
    Published: 2011
    Released on J-STAGE: February 01, 2011
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    Epilepsy and syncope are two main causes of transient loss of consciousness. It is important to distinguish epilepsy from syncope. However, we recognized that there were many cases of syncope that had been diagnosed as epilepsy. We need to evaluate these cases and discuss causes of misdiagnosis. This paper reports ten syncope cases which had been misdiagnosed as epilepsy in other clinics and hospitals. Nine cases have triggers and seven cases have prodromal symptoms that are characteristic of syncope. Five patients had involuntary movements while they lost their consciousness. We suspect that convulsive syncope may not be recognized some physicians. The EEGs did not have the epileptiform discharges in all cases. We could not examine all EEGs taken in other hospitals. We must observe triggers, prodromal symptoms and concomitant symptoms carefully and identify the existence of convulsive syncope to prevent misdiagnosis.
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  • Eri Takeshita, Eiji Nakagawa, Asako Arai, Yoshiaki Saito, Hirofumi Kom ...
    2011 Volume 28 Issue 3 Pages 401-408
    Published: 2011
    Released on J-STAGE: February 01, 2011
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    To evaluate the effect of surgical intervention on behavioral problems and mood disorders in children with intractable epilepsy, we examined 21 children using the Child Behavior Checklist (CBCL) before and after surgery, considering the classification of the seizure outcome and type of epilepsy. CBCL for children aged 2 to 3 years was used for 5 subjects, of which 4 became seizure free, T scores of CBCL showed improvement in the subscales "Development" and "Attention/Concentration" in these 4 subjects. The 5th case with persistent seizures showed T scores worse than those before surgery. The other 16 cases were evaluated using CBCL for children aged 4 to 18 years. Of these, 12 became seizure free, and T scores showed significant improvement in the subscales "Thought problems", "Attention problems", "Delinquent behavior", "Aggressive behavior", "Externalizing" and "Total problems". In the remaining 4 cases with residual seizures, T scores for "Externalizing" and "Total problems" improved. Subscale scores were improved significantly for "Externalizing" and "Total problems" in temporal lobe epilepsy patients and for "Total problems" in parietal lobe epilepsy patients. Our results suggest that surgical intervention of intractable epilepsy is helpful for improving not only control of seizures but also behavioral problems.
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Case Reports
  • Tsunenori Hirayama, Yoshiko Takeda, Harumi Saijo, Mayumi Iwata-Okada, ...
    2011 Volume 28 Issue 3 Pages 409-415
    Published: 2011
    Released on J-STAGE: February 01, 2011
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    It is commonly known carbamazepine (CBZ) induced hyponatremia and/or syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but the mechanism is not clear. We reported a male-case of Septo-optic dysplasia with CBZ induced severe SIADH. He has a severe mental retardation and eight years old on-set epilepsy. Inter-ictal EEG showed spike and wave complex at the area around of right frontal and antero-temporal in eleven years old age. He has had generalized tonic clonic seizures sometimes in a year, and often seizures like myoclonia. He was treated by valproate (VPA) and clonazepam (CZP), from thirteen years old age added CBZ, and his medication changed to VPA, CBZ, zonisamide (ZNS), clobazam (CLB). He entered our hospital at 23 years old age for hyponatremia (118mEq/L). We diagnosed SIADH from increase of urinary sodium and the level of serum ADH. He has had a secondary sexual characteristic. We performed cranial MRI and LH-RH test, diagnosed septo-optic dysplasia with hypothalamic hypogonadism. We decreased and stopped the medication of CBZ and VPA, so he does not have had vomiting and/or hyponatremia until now. It was suggested that abnormalities of function and/or construction of hypothalamus and/or pituitary grand was the risk factor of CBZ induced severe SIADH.
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  • Nami Soga, Manami Akasaka, Atsushi Kamei, Makoto Sasaki, Masahiro Endo ...
    2011 Volume 28 Issue 3 Pages 416-421
    Published: 2011
    Released on J-STAGE: February 01, 2011
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    We encountered an infant with bleeding tendency and hypofibrinogenemia (45.4mg/dl) after combined therapy with valproic acid and adrenocorticotropic hormone (ACTH) for relapse of West syndrome. Plasma fibrinogen concentrations were measured in 3 subsequent infants with West syndrome who required ACTH therapy in addition to valproic acid. All infants showed transient mild hypofibrinogenemia (122.3-139.0mg/dl, reference value 170-405mg/dl) during ACTH therapy, but none displayed clinical signs of bleeding tendency. We recommend monitoring plasma fibrinogen concentrations when combined therapy with valproic acid and ACTH is performed, and discontinuing the drug or reducing the dosage when fibrinogen is below hemostatic levels or bleeding tendencies are recognized.
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  • Susumu Miyake, Takafumi Sugimine, Jun Sato, Chie Endo
    2011 Volume 28 Issue 3 Pages 422-426
    Published: 2011
    Released on J-STAGE: February 01, 2011
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    We report a 12-year-old boy with chronic stage of acute encephalitis with refractory, repetitive partial seizures (AERRPS). At the age of 1 year and 8 months, he was admitted to our hospital because of fever and repetitive convulsions. His consciousness was disturbed (Japan coma scaleII-20). After admission, generalized clonic convulsions and left or right hemiconvulsions with vomiting and apnea frequently occurred. Continuous midazolam infusion and intermittent intravenous pentobarbital injection were done. On cerebrospinal fluid (CSF) examination, cell count was 22/3. His whole blood cell counts and laboratory tests were normal. EEG showed diffuse high voltage slow waves. CT and MR images of his brain showed no abnormality. Therefore, we diagnosed him as having AERRPS. In the course of his disesase, NT antibody to HHV7 elevated, and viral DNA of HHV7 was detected in his plasma. HHV7 infection seemed to be related to the onset of his AERRPS. Convulsions occurred 6-12 times per month during the chronic phase of AERRPS although the intractable convulsions had been treated with CBZ, PHT, VPA, KBr, and DZP. After administering TPM, 300mg/day (6mg/kg/day), convulsions almost completely disappeared. Our report suggests that TPM might be effective for convulsions even in the chronic phase of AERRPS.
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  • Shunsuke Takagi, Tatsuya Nagai, Satoshi Saito, Masuhiro Sakata, Yutaka ...
    2011 Volume 28 Issue 3 Pages 427-433
    Published: 2011
    Released on J-STAGE: February 01, 2011
    JOURNAL RESTRICTED ACCESS
    Unverricht-Lundborg disease (ULD), a type of progressive myoclonus epilepsy, is an autosomal recessive disorder with point mutation in gene encoding Cystatine B. It is well known that ULD is so similar to focal epilepsy or other epilepsy syndromes because of its slow progression that it leads to a misdiagnosis, and also known that ULD is worsened by phenytoin (PHT) treatment. The gene encoding Cystatine B is the only gene known to be associated with ULD.
    We found a patient who had characteristic symptoms as high amplitude evoked potentials, hyper photosensitivity and frequent myoclonus at morning. We diagnosed her as ULD by clinical features, although she didn't have genetic abnormality, like an expansion of a 12-nucleotide repeats often expressed in ULD patients. She had been misdiagnosed as focal epilepsy and received PHT treatment for more than 10 years until we diagnosed her as ULD. Changing PHT to clonazepam and piracetam, betterment of her living conditions was observed. It is suggested that we should be careful in ULD diagnosis as well as initial choice of anti epileptic drug. And we should know the features of ULD enough.
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