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Volume 44, Issue 1
Displaying 1-17 of 17 articles from this issue
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[in Japanese]2012 Volume 44 Issue 1 Pages 2
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSDownload PDF (141K)
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Ichiro Kuki, Hisashi Kawawaki, Shin Okazaki, Takeshi Inoue, Megumi Nuk ...2012 Volume 44 Issue 1 Pages 5-12
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSThis study examined the usefulness of 123I-iomazenil SPECT (IMZ-SPECT), a type of brain scintigram that focuses on the central benzodiazepine receptor in order to determine its distribution and the function of inhibitory neurons. IMZ-SPECT has been used for the detection of epileptogenic foci, especially when surgical intervention is considered. Interictal study by IMZ-SPECT is widely available at numerous institutions and its usefulness has been confirmed in patients with not only focal cortical dysplasia and hippocampal sclerosis, but also tuberous sclerosis and neuronal migration disorders, even when magnetic resonance image fails to demonstrate any abnormal findings. When interpreting scintigrams, the developmental dynamic change of the central benzodiazepine receptor in childhood and the duration of the benzodiazepine exposure period should be taken into consideration. It is expected that IMZ-SPECT will be used in various neurological disorders other than epilepsy in the future allow medical services to be provided based on findings in the inhibitory synaptic system obtained with IMZ-SPECT.View full abstractDownload PDF (4094K)
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Natsuko Hashimoto, Kuriko Kagitani-Shimono, Koji Tominaga, Yukihiro Ki ...2012 Volume 44 Issue 1 Pages 13-18
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSBotulinum toxin A (BTX-A) therapy has been approved as a first-line therapy for spastic torticollis. However it has been suggested as that its use in patients with respiratory distress should be decided cautiously.
We treated 5 patients with abnormal posture, cervical hypertonia and obstructive respiratory distress by BTX-A, and analyzed its efficacy for respiratory distress by their Tsui score and respiratory status after BTX-A therapy. All 5 patients clinically had some degree of dysphagia before BTX-A therapy.
Cervical hypertonia and induced abnormal posture were improved in all patients. The youngest patient could control muscle tone after only 2 doses of BTX-A and subsequently maintained a good condition without additional BTX-A.
BTX-A therapy can decrease torsion and hyperextension of the upper respiratory tract by reducing cervical hypertonia. Consequently, it may improve respiratory status. On the other hand, mild dysphagia and excessive salivation was noted in one patient for each symptom.
It is safe to avoid BTX-A invasion to the anterior muscle of neck and rapid changes in the swallowing pattern.View full abstractDownload PDF (411K) -
Tatsuya Fukasawa, Hiroyuki Yamamoto, Tetsuo Kubota2012 Volume 44 Issue 1 Pages 19-24
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSIt is important to predict the neurological prognoses of preterm infants as part of their normal follow-up. Previous reports have shown that conventional magnetic resonance imaging (MRI) and electroencephalography are useful in predicting neurological prognoses. Diffusion tensor imaging (DTI) is a relatively new method of evaluating the central nervous system (CNS) that can detect abnormalities quantitatively. We compared DTI at term-equivalent age in two extremely-low-birth-weight infants diagnosed with periventricular leukomalacia (PVL) with conventional MRI and DTI in three control extremely-low-birth-weight infants. DTI was analyzed using the free software, “Volume-one” and “dTVII SR.” We compared the fractional anisotropy (FA) values at the corpus callosum, posterior limbs of the internal capsule, cerebral peduncle, and corticospinal tract using manual region of interest (ROI) analysis, and at the commissural fibers and corticospinal tract using tract-specific analysis. The FA values were lower in patients with PVL than in control infants at all measurement points, except the commissural fibers on tract-specific analysis. These measurement points showed no abnormality using conventional MRI. This suggests that DTI can detect CNS abnormalities that cannot be detected with conventional MRI. However, our sample size was very small and we examined only cases in which PVL was detected with conventional MRI. Further study is necessary to examine the correlation of DTI findings and neurological prognoses in infants who have no abnormality on conventional MRI.View full abstractDownload PDF (925K) -
Survival Analysis for Patients with Severe Motor and Intellectual Disabilities Following TracheotomyKoichi Maruyama, Hirokazu Kurahashi, Motomasa Suzuki, Kiyokuni Miura, ...2012 Volume 44 Issue 1 Pages 25-28
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSTo investigate the survival rate and causes of death in patients with severe motor and intellectual disabilities (SMIDs) that necessitated tracheotomy, we retrospectively analyzed 90 patients who underwent tracheotomy between 1990 and 2009. Indications for tracheotomy in these patients were upper airway obstruction (44 patients), recurrent aspiration pneumonia (28 patients), retained secretions (23 patients), prolonged mechanical ventilation (18 patients), chronic respiratory failure (9 patients), central respiratory failure (5 patients), and gastroesophageal reflux (8 patients). Most of the patients underwent tracheotomy at the age of 0-5 years or 10-19 years. As of April 1, 2010, 28 patients had died. The survival rate was 0.91 at 1 year, 0.74 at 5 years, 0.59 at 10 years, 0.54 at 15 years, and 0.40 at 19 years after tracheotomy. Massive tracheal bleeding due to development of tracheo-innominate artery fistulas occurred in 5 patients, and 4 of them died. They were thirteen years of age or older when they underwent tracheotomy, and developed fistulas after 2 weeks or later. In contrast, 7 patients at high risk for fistula formation, including those that had developed severe tracheomalacia associated with granulation or warning hemorrhages, underwent preventive resection of the innominate artery, and all of them had survived. It is important to regularly evaluate patients with SMIDs who have undergone tracheotomy by using bronchofiberscopy to identify risk factors for tracheo-innominate artery fistulas, a preventable cause of death.View full abstractDownload PDF (275K) -
Masato Kaneko, Akira Uno, Noriko Haruhara, Noriko Awaya2012 Volume 44 Issue 1 Pages 29-34
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSWe investigated the usability and limitations of Rapid Automatized Naming (RAN) results in 6-year-old Japanese preschool children to estimate whether reading difficulties will be encountered after school entry. We administered a RAN task to 1,001 preschool children. Then after they had entered school, we performed follow-up surveys yearly to assess their reading performance when these children were in the first, second, third and fourth grades. Also, we examined Hiragana non-words and Kanji words at each time point to detect the children who were having difficulty with reading Hiragana and Kanji. Results by Receiver Operating Characteristic analysis showed that the RAN result in 6-year-old preschool children was predictive of Kanji reading difficulty in the lower grades of elementary school, especially in the second grade with a probability of 0.86, and the area under the curve showed a probability of 0.84 in the third grade. These results suggested that the RAN task was useful as a screening tool.View full abstractDownload PDF (3260K) -
Yoshihiro Watanabe, Megumi Tsuji, Kiyoko Sameshima, Takahito Wada, Miz ...2012 Volume 44 Issue 1 Pages 35-40
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSWe report 12 cases of acute encephalopathy associated with influenza H1N1-2009 treated according to Japanese guideline (2009). In all 12 cases, electroencephalogram presented diffuse or localized high-amplitude slow waves. Brain CT and MRI showed abnormalities in 4 and 6 cases, respectively. We used hypothermia therapy for 5 patients. One patient showed impairment in short term memory, while the rest of the patients showed no sequelae. These 12 cases presented here suggest the early recognition and therapy according to the newly proposed guideline may reduce severe sequelae and mortality by acute encephalopathy associated with influenza H1N1-2009.View full abstractDownload PDF (1373K) -
Nobuko Sugiura, Satoru Konuki, Koichi Hirano, Hiroshi Ozawa2012 Volume 44 Issue 1 Pages 41-44
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSTo measure current public awareness about epilepsy, questionnaires were sent to the following non-doctor groups: 150 parents of pediatric outpatients (control parents), 26 parents of pediatric neurology outpatients who were treated with anticonvulsants (epilepsy parents), 38 elementary school teachers, 42 teachers of disabled children, 28 nurses, and 46 medical students. Analysis of replies revealed that the majority of control parents accepted epilepsy as a disease that is “mostly hereditarily acquired, and needing lifelong medication as it is incurable.” Significantly more epilepsy parents replied “even though epilepsy is well controlled, women receiving epilepsy treatment should avoid pregnancy and delivery during treatment.” Many control parents, schoolteachers and medical students failed to correctly answer general questions about treatment of epilepsy. The results of our survey call attention to the need for further public educational programs to understand epilepsy.View full abstractDownload PDF (245K) -
Toshiya Yamashita, Takashi Hayashi2012 Volume 44 Issue 1 Pages 45-49
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSGuidelines for the diagnosis of reading disorders in elementary school students were published recently in Japan. On the basis of these guidelines, we administrated reading test batteries to 43 Japanese junior high-school students from grade two. The reading test consisted of single sounds, single words, and single sentences. We evaluated the reading speed and the number of reading errors made by the test takers; their performance was compared with the normal value for elementary school students in grade six, as stated in the guidelines. The reading ability of the junior high-school students was not higher than that of the elementary school students. Seven students (16.3%) were found to have reading difficulties (RD group) and they met the criterion for diagnosis of reading disorder as per the guidelines. Three students had difficulties in reading single sounds and single words, but they faced no problems when reading single sentences. It was supposed that the strategies used by the students for reading sentences may have differed from those used for reading single sounds or single words. No significant differences were found between the RD and non-RD group students on scores of scholastic self-evaluation, self-esteem, and depressive symptoms. Therefore, reading difficulty did not directly influence the level of self-evaluation or depression.View full abstractDownload PDF (313K)
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Tomohiro Kumada, Ryuichi Nishii, Tatsuya Higashi, Tomoko Miyajima, Noz ...2012 Volume 44 Issue 1 Pages 50-54
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSThe ketogenic diet (KD) is a high-fat, low-protein, low-carbohydrate diet which is effective in the treatment of medically refractory epilepsy. Several theories have been proposed to explain the mechanism underlying the anticonvulsant efficacy of the KD, however, the precise anticonvulsant mechanism of the KD is still unknown. We speculated the mechanism underlying the effect of the KD in patients with intractable epilepsy, based on the results of the [11C] flumazenil (FMZ) -positron emission tomography (PET) study.
A patient developed frontal lobe epilepsy at the age of 2 years. At the age of 4 years 11 months, she was admitted to our hospital for the initiation of a KD. At the time of admission, she had several epileptic attacks each day: frequent postural tonic seizures, hypermotor seizures, head nodding, and intermittent loss of consciousness (non-convulsive status epilepticus). MR imaging showed no abnormal signal intensity in the brain. With the KD, the seizure frequency reduced dramatically on the fifth day. Interictal [11C] FMZ-PET was performed before and 2 months after the initiation of the KD.
Before the KD, the [11C] FMZ-PET images and [11C] FMZ-PET binding potential (BP) images showed extremely low accumulation of FMZ throughout the cerebral cortex. Two months after the initiation of the KD, significantly increased binding potential of [11C] FMZ was observed, implying the increased binding potential of the benzodiazepine receptors, probably due to the anticonvulsant effect of the KD.
These PET findings suggested that KD may control seizures by directly or indirectly increasing the binding potential of the benzodiazepine receptors.View full abstractDownload PDF (1984K) -
Yu Ishida, Tasuku Miyajima, Masaru Shimura, Shinichiro Morichi, Yasuyu ...2012 Volume 44 Issue 1 Pages 55-59
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSCongenital cytomegalovirus (CMV) infection occurs frequently in neonates. However, there are no screening tests or definitive treatments for this infection in Japan. We report a case of a 21-day-old Japanese boy with congenital CMV infection. He was referred to our hospital for treatment of congenital bilateral deafness. Brain magnetic resonance imaging (MRI) revealed cortical dysplasia of the temporal poles, enlarged ventricles, and areas of abnormal intensity in the white matter. He was given a diagnosis of congenital CMV infection based on the detection of CMV DNA in his urine and the umbilical cord. After the administration of valganciclovir, no CMV DNA was detected in his serum, and brain MRI and electroencephalogram findings, motor development, and deafness improved. Further investigation is needed to establish a screening test and treatment for congenital CMV infection in Japan.View full abstractDownload PDF (1012K) -
Mieko Yoshioka, Masako Isaka2012 Volume 44 Issue 1 Pages 60-65
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSThis follow-up study evaluated cognitive and language development in Asperger's disorder (AD) patients diagnosed at the age of 5 to 6 years, with initial complaints of delays in motor or language development in infancy. We evaluated 12 patients (10 males and 2 females) using two intelligence tests: Kyoto Scale of Psychological Development 2001 (K-scale) for those under 6 years, and WISC-III for those over 5-6 years. The cognitive-adaptive area (C-A) of the former test was compared to the performance IQ (PIQ) of the latter test, and the language-social area (L-S) of the former to verbal IQ (VIQ) of the latter. The mean age at the first examination was 3.2 years (range: 2.1-4.6 years), and the average age at follow-up was 7.7 years (range: 5.3-12.3 years). The average length of follow-up from the initial visit was 5.6 years (range: 3.3-8.6 years). During follow-up, the PIQ, VIQ and full scale DQ or IQ (F-DQ/IQ) improved with age. Average scores of the 12 patients at the first examination and last follow-up evaluated by K-scale were: C-A: 70.6 (first) and 84.5 (last), L-S: 64.8 (first) and 85.8 (last), and F-DQ: 68.5 (first) and 84.8 (last).
Compared to those with AD, 12 autistic patients with the Kanner type (10 males and 2 females) who visited our clinic during almost the same period, and belonged to almost the same age group, showed average scores at the first examination and last follow-up of: C-A: 61.9 (first) and 43.3 (last), L-S: 43.0 (first) and 43.4 (last), and F-DQ: 60.2 (first) and 44.5 (last).
From these observations, it is apparent that AD patients also showed language delay during infancy, but they improved rapidly between the ages of 4-6 years. This developmental spurt was not seen in autistic patients with the Kanner type.View full abstractDownload PDF (346K) -
Yasuko Nakamura, Hiroshi Matsumoto2012 Volume 44 Issue 1 Pages 66-68
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSWe herein present a case of 6-year-old female demonstrating atypical Ramsay-Hunt syndrome. She presented with an earache, severe vertigo, and vomiting at onset, and thereafter, herpes zoster oticus appeared. No facial nerve palsy was seen. She was vaccinated for varicella and had no past history of apparent varicella infection. The patient showed positive IgG and negative IgM serum antibodies for varicella-zoster virus (VZV). An analysis of VZV-DNA from the patient's ear lesion using alleric discrimination real-time PCR identified a wild-type strain of VZV. We diagnosed her to have atypical Ramsay-Hunt syndrome caused by reactivation of the VZV. Aciclovir and prednisolone were administered, and she recovered completely. This case indicates that Ramsay-Hunt syndrome could be caused by a VZV infection after vaccination, even though its frequency is low. Ramsay-Hunt syndrome may thus be considered as one of causes of sudden onset vertigo in children.View full abstractDownload PDF (219K) -
Tatsuharu Sato, Hideo Kuniba, Mitsuhiro Matsuo, Tetsuo Matsuzaka, Hiro ...2012 Volume 44 Issue 1 Pages 69-72
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSDrug-induced hypersensitivity syndrome (DIHS) is a rare but severe multiorgan disorder. The reactivation of human herpesvirus-6 (HHV-6) and other human herpesviruses has been reported to be associated with its pathogenesis. We herein report a case of 14-year-old female who developed DIHS during the treatment with lamotrigine, a novel antiepileptic drug. She initially presented with fever, skin rash, cervical lymphadenopathy, leukocytosis with eosinophilia and atypical lymphocytosis, liver dysfunction and hypogammaglobulinemia. Discontinuation of the drug and administration of prednisolone led to improvement; however, tapering of prednisolone and administration of midazolam and ketamine thereafter triggered clinical deterioration. She subsequently developed hyperthyroidism followed by hypothyroidism. Herpesviral loads were determined in her peripheral blood by real-time PCR during the course of the treatment, and sequential reactivation of Epstein-Barr virus (EBV), HHV-6 and cytomegalovirus was demonstrated. EBV viremia was detected throughout the course, except for a short period when HHV-6 viremia was at the peak. HHV-6 viremia developed after the secondary deterioration. Cytomegalovirus viremia appeared transiently before the hyperthyroidic state reversed and became hypothyroidic. Although this syndrome should be regarded as a systemic reaction induced by a complex interplay among herpesviruses and the immune responses against viral infections and drugs, it remains unknown how such a sequential reactivation is related to the pathogenesis of the condition.View full abstractDownload PDF (1406K)
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[in Japanese], [in Japanese], [in Japanese], [in Japanese]2012 Volume 44 Issue 1 Pages 73-74
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESS
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2012 Volume 44 Issue 1 Pages 82-83
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSDownload PDF (284K)
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[in Japanese]2012 Volume 44 Issue 1 Pages 84-85
Published: 2012
Released on J-STAGE: December 25, 2014
JOURNAL FREE ACCESSDownload PDF (609K)
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