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Volume 48, Issue 1
Displaying 1-16 of 16 articles from this issue
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  • The rewards of working with medicine for patients with severe motor and intellectual disabilities
    Kiyokuni Miura
    2016 Volume 48 Issue 1 Pages 5-9
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.5
    JOURNAL FREE ACCESS
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      I think that medicine for patients with severe motor and intellectual disabilities (SMID) should aim to help them and their families to promote a better quality of life in their communities by building multidisciplinary networks. Below, I mention four personal opinions on why I find working with medicine for SMID patients attractive: 1. Sharing in the joy experienced by SMID patients and their family members as they learn to cope with SMID and grow as people. When I see how patients who are affected by SMID and their family members grow and lead fulfilling lives, I feel that my work has been richly rewarded. 2. The joy of being considered reliable and appreciated. I feel highly rewarded when I earn the trust of the family members, and I get a word of gratitude from them. 3. Enjoying the experience of working with ingenious and team medicine. I experience a feeling of accomplishment after engaging in intense discussions on successful preventive innominate transections. 4. The opportunity to make a social contribution. I believe that I can contribute to making our world more livable to a greater number of people. To spread awareness about the attractiveness of SMID medicine, I provide SMID medicine education at Nagoya University. My lectures and training are attended by patients with SMID and their families. I also hope that many young doctors become interested in SMID medicine and find it an enjoyable experience.
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  • Hypnotic effects of ramelteon during electroencephalography
    Hiroto Akaike, Eisuke Kondo, Mina Kono, Atsushi Kato
    2016 Volume 48 Issue 1 Pages 10-13
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.10
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      Objective: The efficacy of ramelteon, a sleep agent thought to induce natural sleep through its actions on the melatonin receptors 1 and 2, was evaluated during electroencephalography (EEG). Methods: We retrospectively analyzed the data from 862 EEG sessions in the electronic medical records of 523 patients (mean age, 8.9±6.4 years; range, 0-38 years) who underwent EEGs in the Department of Pediatrics of Kawasaki Medical School Hospital. We compared the sleep induction statuses of patients not treated with hypnotics and those treated with the following hypnotics: ramelteon, triclofos sodium, and/or chloral hydrate. Results: The success rates of sleep induction in the different groups were as follows: 440/513 (85.7%) in the hypnotic-free, 60/63 (95.2%) in the ramelteon, 209/217 (96.3%) in the triclofos sodium, 36/37 (97.3%) in the chloral hydrate, and 29/32 (90.6%) in the triclofos sodium plus chloral hydrate group. The incidence of adverse reactions was 0/63 (0%) in the ramelteon, 104/199 (52.2%) in the triclofos sodium, 12/31 (38.7%) in the chloral hydrate, and 20/30 (66.7%) in the triclofos sodium plus chloral hydrate group. The mean sleep latency in the ramelteon group was 22.3±12.8 min (range, 5-60 min). Conclusions: Ramelteon induced sleep significantly more compared to the control group. The incidence of adverse reactions after treatment with ramelteon was significantly lower than after treatment with any of the other hypnotics. These results suggest that ramelteon is useful as a hypnotic during EEG.
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  • Incidence of cerebral palsy in Himeji over a 25-year period
    Keiko Koterazawa, Yuka Okada, Hiroyoshi Miyata
    2016 Volume 48 Issue 1 Pages 14-19
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.14
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      Objective: To investigate epidemiological and clinical trends of cases of cerebral palsy between 1983 and 2007 in Himeji, Hyogo, Japan. Methods: A retrospective chart review of all children diagnosed with cerebral palsy and evaluated for complications at the authors’ clinic in the targeted 25-year period was conducted. Collected data were analyzed and compared across five 5-year blocks (Ⅰ: 1983-1987, Ⅱ: 1988-1992, Ⅲ: 1993-1997, Ⅳ: 1998-2002, Ⅴ: 2003-2007). Results: The incidence of cerebral palsy per 1000 live births increased until block Ⅳ (Ⅰ: 1.4, Ⅱ: 2.0, Ⅲ: 2.2, Ⅳ: 2.9) and then dropped to 2.0 in block Ⅴ. The most frequent cause of cerebral palsy was periventricular leukomalacia, which was common in preterm births. The frequencies of twins and triplets were highest in block Ⅳ, contributing to the elevated incidence of cerebral palsy in this block. In terms of complication severity, mild cases increased during the study period while the percentage of severe cases remained high, thus resulting in a polarized distribution of severity in block Ⅴ. Conclusions: The features of cerebral palsy were observed to change during the study period, most likely as functions of the recent progress in perinatal.
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  • Safety and efficacy of surgical closure of the larynx for recurrent aspiration pneumonia in persons with severe motor and intellectual disabilities: a comparative study with tracheoesophageal diversion
    Sakiko Oshima, Yukikatsu Ochiai, Masamichi Ariga, Mika Hayakawa, Masam ...
    2016 Volume 48 Issue 1 Pages 20-24
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.20
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      Objective: We retrospectively investigated the efficacy and complications of surgical closure of the larynx (SCL) for recurrent aspiration pneumonia in comparison with tracheoesophageal diversion. Methods: The subjects were persons with severe motor and intellectual disabilities (SMID) who had undergone surgery for recurrent aspiration pneumonia between 1994 and 2011: A 8 SCL patients group and a 16 tracheoesophageal diversion patients group. We investigated two groups the lower respiratory infection incidence, length of hospital stay for the surgery, postoperative complications, and rate of cannula withdrawal, by reviewing medical records. Results: Both the SCL and the tracheoesophageal diversion group showed a reduction in the incidence of infection after surgery, indicating that the efficacy of SCL was equivalent to that of tracheoesophageal diversion in preventing aspiration pneumonea. The SCL group showed a reduction in the length of hospital stay and an increased rate of cannula withdrawal as compared with the tracheoesophageal diversion group. Conclusion: The efficacy of SCL was equivalent to that of tracheoesophageal diversion in preventing aspiration for SMID. We consider SLC to have potential for reducing the burden on patients.
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  • Respiratory complications in children with Chiari malformation type Ⅱ associated with myelomeningocele
    Koichi Mizuguchi, Nobuhito Morota, Masaya Kubota
    2016 Volume 48 Issue 1 Pages 25-28
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.25
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      Background: Management of life-threatening respiratory complications of Chiari malformation type Ⅱ (CM Ⅱ) is important in patients with myelomeningocele (MMC). The objective of this study is to determine the clinical features and outcome of respiratory complications in MMC. Methods: The study was a retrospective chart review of 50 patients with MMC who were treated from birth between 2002 and 2013 at the National Center for Child Health and Development, Tokyo Japan. Respiratory complications were divided into three types; upper airway obstruction, sleep-disordered breathing (SDB) and prolonged expiratory apnea with cyanosis (PEAC). SDB was further divided into two subtypes: sleep apnea type and central hypoventilation type. Results: Twelve (24%) of the 50 MMC patients had respiratory complications. Among them, most had at least two types of complications; six had upper airway obstruction, 10 had SDB, and 10 had PEAC. The respiratory complications appeared during the first six months in most patients. Surgical decompression was performed 11 of the patients during 10 and 60 days after respiratory symptoms appeared; of which, four required invasive respiratory support in spite of decompression surgery. Three patients with central hypoventilation type SDB required ventilator support with tracheostomy, and one with upper airway obstruction needed tracheostomy. In the patients with PEAC, the frequency of apneic spells decreased over time. There was no death in the patients with respiratory complications of MMC. Conclusions: In addition to surgical decompression for CM Ⅱ, management of respiratory complications may improve mortality outcome. Such screening should be performed in patient with MMC particularly in the first six months.
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  • Successful treatment with chloramphenicol in four pediatric cases of intractable bacterial meningitis
    Kayo Morita, Yu-ichi Abe, Atsushi Itano, Ikuma Musha, Takeshi Koga, Ta ...
    2016 Volume 48 Issue 1 Pages 29-33
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.29
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      Chloramphenicol (CP) is recently one of the rarely-used antibiotics. In this study, we present four patients with intractable bacterial meningitis, who were successfully treated with CP and discuss the therapeutic indications of CP in these pediatric cases. The patients were diagnosed as bacterial meningitis at the ages ranging from 2 months to 1 year and 4 months. The causative organisms found in three of the patients were H. influenzae and in the fourth patient, S. pneumoniae. According to the microbial sensitivity tests, these organisms were highly sensitive to antibiotics including ceftriaxone, meropenem and/or panipenem/betamipron. Treatment with these antibiotics was initially effective; however, recurrences of meningitis appeared in all patients. Administration of CP (100 mg/kg/day) started between the 11th and the 58th days, and was continued for 9 days up to 19 days. Their fever had disappeared within four days after the administration of CP, and it was confirmed that all patients completely recovered from meningitis. Two of the patients developed a mild degree of anemia, but soon recovered after the discontinuation of CP. None of them had neurological sequela. We recommend CP as one of the choices for the treatment of intractable bacterial meningitis.
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  • A case of X-linked myotubular myopathy with chylothorax
    Taku Oishi, Tetsuya Sato, Kenshi Matsushita, Tomoki Takechi, Nobuyuki ...
    2016 Volume 48 Issue 1 Pages 34-36
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.34
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      We report a case of X-linked myotubular myopathy with chylothorax. A male infant weighing 2,114 g was born to a mother whose pregnancy was complicated with polyhydramnios from gestational week 32. At gestational week 37, emergent caesarian section was performed due to membrane rupture followed by fetal bradycardia. Ventilatory support was necessary because the neonate showed severe birth asphyxia accompanied by hypotonia and dyspnea. He also showed a respiratory complication of chylothorax at 10 days old; therefore, thoracic drainage was performed. Congenital chylothorax associated with congenital myotonic dystrophy (CMD) has been described in a number of past reports. Specific findings of congenital myotubular myopathy and partial CMD, such as peripheral halo of muscle fibers, were demonstrated in biopsied muscle, and mutation of the myotubularin (MTM1) gene was identified. Tracheostomy was performed at 5 months old because of prolonged ventilatory support and severe dysphagia. The infant was able to be discharged at 17 months old. Congenital chylothorax might be associated with congenital myotubular myopathies such as CMD.
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  • Enteral nutrition ameliorated superior mesenteric artery syndrome in a patient with Duchenne muscular dystrophy
    Fumihito Nozaki, Tomohiro Kumada, Minoru Shibata, Anri Hayashi, Ikuko ...
    2016 Volume 48 Issue 1 Pages 37-40
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.37
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      The abdominal complications of Duchenne muscular dystrophy (DMD) include acute gastric dilatation, superior mesenteric artery (SMA) syndrome, ileus and constipation. We report herein a patient with DMD in whom SMA syndrome was successfully treated with enteral tube nutrition. The patient was a 16-year-old boy diagnosed with DMD at 2 years. Steroid therapy was started at 5 years, and he was unable to walk and was wheelchair-bound at 11 years. Lordoscoliosis progressed after the age of 14 years. Noninvasive mechanical ventilation was introduced due to respiratory impairment at 15 years. During 8 months with respiratory impairment, his body weight decreased from 40.3 kg to 33.4 kg. He was referred to our hospital for vomiting and hematemesis. Radiographic studies indicated a diagnosis of SMA syndrome. Enteral nutrition with a nasojejunal tube successfully treated SMA syndrome for 5 months and his body weight increased from 32.7 kg to 36.1 kg. Gastrostomy was subsequently performed and no recurrence was evident. SMA syndrome is caused by compression of the third part of the duodenum at the angle between the aorta and SMA. The conditions for duodenal vascular compression are weight loss resulting in depletion of the retroperitoneal fat and progressive lordosis. The reasons for SMA syndrome with our patient were weight loss and progressive lordoscoliosis. A conservative approach with enteral nutrition promoted weight gain, increasing retroperitoneal fat. Enteral nutrition should be considered for the treatment of SMA syndrome as a complication of DMD.
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  • A child case of surfer’s myelopathy
    Nobuyoshi Sugiyama, Jun-ichi Yokoyama, Mariko Ikegami, Shin-ichi Matsu ...
    2016 Volume 48 Issue 1 Pages 41-44
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.41
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      We report a case of so-called “surfer’s myelopathy” involving a 15-year-old girl. Preceding episodes of vaccination and infection were absent. She experienced back pain during a surfing class for beginners, followed by weakness in both legs, which progressed to paraperesis. Then, 2 days later, sensory disturbance and bladder/rectal disturbance also developed. Spinal MRI revealed a ischemic lesion compatible with anterior spinal cord syndrome. A combination of methylprednisolone pulse therapy and high-dose intravenous immunoglobulin therapy was effective leaving no residual dysfunction. The acute onset during the surfing class and characteristic MRI findings led to the diagnosis of surfer’s myelopathy. Although rarely reported, warnings are warranted to prevent surfer’s myelopathy and avoid the progressive deterioration of neurological dysfunction.
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  • Hashimoto’s encephalopathy presenting with vertigo and muscle weakness in a male pediatric patient
    Hiroe Ueno, Chizuru Nishizato, Tomoyuki Shimazu, Hiziri Watanabe, Tomo ...
    2016 Volume 48 Issue 1 Pages 45-47
    Published: 2016
    Released on J-STAGE: March 26, 2016
    DOIhttps://doi.org/10.11251/ojjscn.48.45
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      Hashimoto’s encephalopathy is an anti-thyroid antibody-positive autoimmune encephalopathy. We herein report the case of a 13-year-old male patient with subacute vertigo, muscle weakness in the extremities and gait disturbance who was diagnosed with Hashimoto’s encephalopathy. He showed no severe impairment of consciousness and no seizures, and there were no abnormalities on the brain MRI. However, epileptic spike and wave complexes were observed on an electroencephalogram, and a decline in blood flow was diffusely observed on brain SPECT (single photon emission computed tomography). His thyroid function was normal, but he was positive for anti-thyroid antibodies, such as anti-TPO (thyroid peroxidase) antibodies. He was also positive for serum anti-NAE (NH2-terminal alpha-enolase) antibodies. Systemic corticosteroid therapy and high-dose intravenous immunoglobulin therapy were effective, greatly improving his quality of life.
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