NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 46, Issue 4
Displaying 1-15 of 15 articles from this issue
  • Toru Okanisni, Kouji Iida, Ayako Ochi, Hiroshi Otsubo
    2014 Volume 46 Issue 4 Pages 257-263
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Tuberous sclerosis complex (TSC) has been known to present a high incidence (60-90%) of epilepsy due to cortical tubers. In 50 to 80%of the epilepsy patients with TSC, epileptic seizures become resistant against anti-epileptic medications. The multiple cortical tubers are considered to promote the wide epileptic network in a part of TSC patients. The extensive and disrupted epileptic network with the multiple tubers and abnormal cortices are highly correlated with their complicated epileptogenesis. In a subset of TSC patients, the distribution of epileptogenic zone can propagate from a single focus at the beginning of seizure history, to bilateral and multiple foci during the clinical course. We found that the laterality of spikes during rapid-eye-movement (REM) sleep despite of diffuse and multifocal spikes during non-REM sleep on the long-term video EEG monitoring related with the laterality of the epileptogenic hemisphere. Furthermore, the pattern of distribution of equivalent current dipoles on magnetoencephalography (MEG) can be applied to identify epileptogenic zone among multiple cortical tubers.
      This paper presents roles of pediatric neurologists to which patients are candidate for surgical treatments and when is the best to offer surgery; using the long-term video EEG and MEG.
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  • Mana Kurihara, Atsushi Shishido, Manabu Yoshihashi, Hiroyuki Fujita, T ...
    2014 Volume 46 Issue 4 Pages 265-269
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: Reports of the prognosis of hypoxic encephalopathy in children have not been common. We investigated the prognoses in 35 children with hypoxic encephalopathy at more than one year from the onset. Methods: The average age of onset was 5 years 8 months, and the present age was 12 years 6 months in all cases. The medical records were reviewed, and the clinical courses during the acute stage, the state of sequelae were investigated. Results: The etiologies were drowning 12 cases, asphyxia in 6, heart diseases in 10, respiratory diseases in 2, cardiac arrest in 3, etc. Each etiology showed age-related characterisitics. All cases showed consciousness loss levels of triple-digits on the Japan coma scale. The sequelae comprised physical disabilities in 28 cases, mental disabilities in 30, epilepsy in 16, higher brain dysfunction in 12 especially visiospacial disturbance, etc. The onset of epilepsy was mainly within 3 months after onset of the hypoxic encephalopathy. The types of epileptic seizures were focal seizures in 14 cases and generalized seizures in 12. On average, 2.1 antiepileptic drugs, such as carbamazepine and valproate were prescribed. EEG, brain MRI and brain SPECT showed an extensive range of abnormalities. Severe disabilities depended on the following factors: ①the etiologies such as asphyxia, congenital heart diseases, cardiac arrest, ②the age of onset under 2 years or over 13 years, ③long and severe consciousness loss during the acute stage. Conclusion: Though the sequelae of hypoxic encephalopathy were similar to those of acute encephalopathy, the former was more serious than the latter.
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  • Tatsuya Koeda, Ayumi Seki, Daisuke Tanaka, Hitoshi Uchiyama
    2014 Volume 46 Issue 4 Pages 270-274
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: The present study examined the effectiveness of response to intervention (RTI) for the early detection and intervention for specific reading disorder. Method: RTIs were applied to seventy-seven first graders (36 boys and 41 girls) for the early detection and intervention of specific reading disorders. The outcomes were examined when the children reached the third grade. Results: Reading difficulties were detected in four children at the beginning of the first grade. Decoding training and vocabulary learning were conducted with these children as interventions for reading difficulties. Three children exhibited improvements in reading difficulties at the end of the first grade. The fourth child responded poorly and was diagnosed with specific reading disorder. The assessment of outcomes at the third grade showed that this child still had reading difficulties, whereas no other child displayed symptoms for being diagnosed with specific reading disorder. Conclusion: RTI is effective for the early detection and intervention of specific reading disorder.
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  • Asako Horino, Masashi Shiomi, Takeshi Inoue, Megumi Nukui, Ichiro Kuki ...
    2014 Volume 46 Issue 4 Pages 275-280
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: We examined the clinical course and the prognosis of patients with anti-NMDAR encephalitis. Methods: We retrospectively evaluated the patients who has distinctive clinical features as anti-NMDAR encephalitis based on their medical records. Results: There were two male and four female patients with anti-NMDAR encephalitis. They were aged between 13 and 16 years. One of the six, 14 years female patient was negative for anti-NMDAR antibody. All four female patients with anti-NMDAR encephalitis had an ovarian tumor. Neurocognitive dysfunction and epilepsy remained in one female patient with right temporal lobe lesion and one male patient with celebellar abnormalities had mild mental impairment. In three patients including two patients who were examined abdominal MRI for the first time after recovery from the encephalitis, overian tumors became apparent during follow-up. In one of other patients, overian tumors had a tendency to increase in size after recovery. Conclusion: Sequellae were seen in two cases that have abnormalities in brain MRI. As to ovarian tumor, it was considered to be necessary to checkup pelvic MRI for at least four years after the onset of encephalitis.
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  • Kenji Mori, Tatsuo Mori, Aya Goji, Hiromichi Ito, Yoshihiro Toda, Emik ...
    2014 Volume 46 Issue 4 Pages 281-286
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Objective: To examine the hemodynamic activities in the frontal lobe, children with autistic disorder and matched controls underwent near-infrared spectroscopy (NIRS) while imitating emotional facial expressions. Methods: The subjects consisted of 10 boys with autistic disorder without mental retardation (9~14 years) and 10 normally developing boys (9~14 years). The concentrations of oxyhemoglobin (oxy-Hb) were measured with frontal probes using a 34-channel NIRS machine while the subjects imitated emotional facial expressions. Results: The increments in the concentration of oxy-Hb in the pars opercularis of the inferior frontal gyrus in autistic subjects were significantly lower than those in the controls. However, the concentrations of oxy-Hb in this area were significantly elevated in autistic subjects after they were trained to imitate emotional facial expressions. The increments in the concentration of oxy-Hb in this area in autistic subjects were positively correlated with the scores on a test of labeling emotional facial expressions. Conclusions: The pars opercularis of the inferior frontal gyrus is an important component of the mirror neuron system. The present results suggest that mirror neurons could be activated by repeated imitation in children with autistic disorder.
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  • Keitaro Yamada, Tomokazu Kimizu, Sadami Kimura, Tae Ikeda, Yukiko Moga ...
    2014 Volume 46 Issue 4 Pages 287-289
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      A 3-year-old boy developed left-sided convergent strabismus one week after upper respiratory infection. All examinations, including analysis of cerebrospinal fluid, a tensilon test, and brain MRI, were negative. He was diagnosed with idiopathic sixth nerve palsy. His symptom resolved gradually with vitamin B12, and remitted completely three months after onset. At the age of 6years, he experienced recurrence of left-sided sixth nerve palsy. After vitamin B12 failed, his symptom responded markedly to intravenous steroid pulse therapy starting on day 26 after relapse. He has been symptom-free for three years since the second remission. Steroid therapy might be effective, and should be considered in children with idiopathic sixth nerve palsy who do not show spontaneous remission.
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  • Toshihiro Suzuki, Yuji Hashimoto, Satoshi Anzai, Kasumi Nagasawa
    2014 Volume 46 Issue 4 Pages 290-296
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      We presented a case of an 8-year-old boy with Guillain-Barré syndrome characterized by severe intractable pain in the soma and lower extremities, which appeared 2 weeks after a febrile cold. At his first visit to our hospital, he could not stand or walk because of the severe pain, and muscle weakness and absence of deep tendon reflexes were observed. Guillain-Barré syndrome was diagnosed on the basis of cerebrospinal fluid study results, nerve conduction velocity, and spinal cord magnetic resonance imaging. His pain was scored as a five on a six-point visual analog scale, and it persisted despite routine supportive therapy. The pain was successfully controlled with parenterally infused fentanyl. It is suspected that opioid analgesics are useful for severe pain control in patients with Guillain-Barré syndrome.
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  • Hisako Hayashi, Masao Kawatani, Genrei Ohta, Hiroshi Kometani, Yusei O ...
    2014 Volume 46 Issue 4 Pages 297-300
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      We report a case of 12-year-old girl with Graves' disease who had presented with deterioration in physical and scholastic performances since 10 years of age. She had an episode of atonic seizure and difficulty in speech. Brain MRI revealed formation of moyamoya vessels and a lesion suggestive of ischemic changes in the left frontal lobe. Because of uncontrollable thyrotoxicosis with anti-thyroid drug, she received a subtotal thyroidectomy. Two months later, she received a shunt operation between left superficial temporal artery and middle cerebral artery. The postoperative arterial spin-labeling MR imaging demonstrated an improvement of brain perfusion in left frontal lobe compared with the preoperative one, and provided comparable results of angiography and acetazolamide-challenged 15O-gas PET. Thus, arterial spin-labeling MR imaging seems useful for follow-up evaluation of brain perfusion in qusai-moyamoya disease.
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  • Yohane Miyata, Makiko Shimazaki, Yumiko Komatsu, Yukiko Nakamura, Akir ...
    2014 Volume 46 Issue 4 Pages 301-306
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      An 11-year-old boy, who had no remarkable past history, exhibited disorientation and abnormal behavior lasting for several hours. Continuous ictal discharges on his EEG lead to the diagnosis of nonconvulsive status epilepticus (NCSE). The administration of diazepam instantly resulted in the cessation of ictal discharges, associated with clinical recovery. Interictal spikes distributed in frontal lobes are sporadically seen, suggesting frontal lobe as an epileptic focus. After starting medication, he showed excellent clinical course without recurrence of seizure or neurological sequelae. Although NCSE is generally suggestive of poor prognosis, some subtypes of NCSE, such as partial status epilepticus and absence status epilepticus, are not always associated with adverse outcome. The present case suggests that epileptic patients who present NCSE at onset and lack interictal neurological impairments might have good outcome.
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  • Ayumi Yoshimura, Tetsuya Kibe, Kenji Yokochi
    2014 Volume 46 Issue 4 Pages 307-310
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Os odontoideum is a separate ossicle from the odontoid process from the body of the axis by a variable transverse gap. A boy with cerebral palsy probably due to prematurity and kernicterus, was treated with botulinum toxin for continuous dystonic movements at the age of 3.5 years. Although botulinum toxin appeared to be remarkably effective for relaxing hypertonia, abnormal frequent anterior flexion of the neck remained. Because of feeding difficulty and frequent aspiration episodes, additional botulinum toxin therapy was discontinued. His condition seemed to be stable and he could walk with support at age 7. However, at age 8, he presented with decreased movement of the extremities and bilateral ankle clonus. Radiographic examination of the cervical spine revealed cystic lesion and os odontoideum. With cervical posterior fixation, the patient made a good recovery. Although athetoid cerebral palsy displays an increased risk of cervical myelopathy, os odontoideum is rare in early childhood. The frequent dynamic stress of the neck due to an unbalanced, persistently contracted state and sudden collapse, possibly attributed to botulinum toxin therapy, might have led to atlantoaxial instability and os odontoideum.
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  • Toshino Motojima, Katsuo Sugita, Taku Omata, Katsunori Fujii
    2014 Volume 46 Issue 4 Pages 311-314
    Published: 2014
    Released on J-STAGE: December 25, 2014
    JOURNAL FREE ACCESS
      Cockayne syndrome (CS) is a rare hereditary disease, characterized by profound postnatal brain and somatic growth failure and by the degeneration of multiple tissues resulting in cachexia, dementia, and premature aging. This syndrome is often associated with renal dysfunction, which usually correlates with the patient's prognosis. In the present study, we evaluated the longitudinal changes in serum creatinine and serum cystatin C levels in three patients with CS to examine whether these markers can help detect renal disorders at the earlier stages. The serum creatinine level in these CS patients gradually exceeded the reference level from5 to 7 years of age, after correcting for body length. The cystatin C level of the CS patients increased to above the reference level while their estimated glomerular filtration rate remained within stage 2 or 3. Thus, we conclude that the serum creatinine level, following correction by body length, is very useful for the evaluation of renal function in CS. Moreover, the appropriate estimation of renal function facilities the administration of suitable medication, thus avoiding some harmful effects on the kidney.
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