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Volume 45, Issue 1
Displaying 1-18 of 18 articles from this issue
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  • Severe Motor and Intellectual Disabilities and Brain Death in Children
    Masayuki Sasaki
    2013 Volume 45 Issue 1 Pages 5-10
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.5
    JOURNAL FREE ACCESS
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      Japan has established a unique medical and social care system for both children with chronic severe motor and intellectual disabilities (SMID) and their family members. Many kinds of specialist, including doctors (Pediatrician, Neurologist, Surgeon, Orthopedist, Rehabilitation, etc.), nurses, physical therapists, medical social workers, and special school teachers, are working with children with SMID. There are many special facilities, for example, special hospitals, special schools, or special day services or nursery systems for children with SMID. Consequently, the number of children with extreme SMID who have severe respiratory insufficiency (tracheostomy and/or ventilator care) and require tube feeding who stay at home is increasing in Japan. The medical and social care system for children with SMID aims to draw possibilities from them and to support them to keep their bodies and lives safe and stable, even if there is no cure. This system contains the character of palliative medicine for children with extreme SMID. Family members of children with SMID and many specialists working with such children should know each child's present condition precisely, and should accept their actual situation and natural course in the future. The most important purpose of this unique medical and social care is that children with SMID and their family members can live a satisfactory life with minimal pain or worry. I hope that they have no regrets about their lives. The length of a life is not so important. Extreme SMID and “brain death” are completely different concepts. I hope that we can create a safe and comfortable society for all individuals in the near future.
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  • Survey for Assessing How Duchenne Muscular Dystrophy is Explained to Children with the Disorder
    Haruo Fujino, Toshio Saito, Osamu Imura, Tsuyoshi Matsumura, Susumu Sh ...
    2013 Volume 45 Issue 1 Pages 11-16
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.11
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      Objective: There are many difficulties in disclosing Duchenne muscular dystrophy (DMD) to children with the disorder. The purpose of this study was to assess the explanation of DMD given to affected children by child neurologist.
      Methods: The questionnaire was mailed to board-certified child neurologists of the Japanese Society of Child Neurology. The questionnaire consisted of questions on how physicians explained the condition to children with DMD (their patterns of explanation) and their attitude towards the children while explaining the disease.
      Results: We received 311 replies. The contents of physicians' explanations were categorized and correspondence analysis revealed “medical support” (explanation about the symptoms, prognosis, medical responses) and “humanistic support” (telling purpose in life, patient group introduction). Parents' understanding of the disease, acceptance, and trust relationships were considered important factors for disease explanation by the physicians. Physicians agreed with the need of clinical psychologist and other psychological professionals when they tell their diagnosis, and agreed with telling the diagnosis to a DMD child reached a certain age.
      Conclusions: It was revealed that physicians' explanation were largely categorized into two groups, and the important factors for disease explanation and physicians' attitudes towards disclosure of the diagnosis. This information will help in explaining the disease to children with DMD.
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  • Carnitine Deficiency with Valproate Sodium Therapy
    —the Difference by Normal Diet and Enteral Nutrition—
    Akira Kato
    2013 Volume 45 Issue 1 Pages 17-20
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.17
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      Objective: Valproate sodium (VPA) treatment is known to reduce blood carnitine levels; however, some enteral nutrition formulas contain carnitine. The objective of this study was to verify the hypothesis that blood carnitine levels are lower after VPA treatment in patients on enteral nutrition than in those on normal diet.
      Methods: Forty-five epilepsy patients under medical treatment in our hospital were classified according to their alimentation type into normal diet group (n=31) and 14 on enteral nutrition group (n=14). The differences in the blood free carnitine levels between the two groups were evaluated.
      Results: Plasma free carnitine levels were below the normal value in 15 of 31 patients on normal diet and 13 of 14 patients on enteral nutrition. There was a significant reduction of the levels of carnitine in the enteral nutrition group (p<0.001).
      Conclusions: Although normal diet contains sufficient amount of carnitine, most enteral nutrition formulas lack carnitine; and hence, blood carnitine levels in patients on enteral nutrition after VPA treatment is low. It is therefore suggested that carnitine supplemented diet is necessary for patients treated with VPA, especially for those who receive enteral nutrition.
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  • The Relationship between the Severity of Periventricular Leukomalacia and the Fractional Anisotropy Values of Diffusion Tensor Imaging
    Toshihiko Suzuki, Hiroyuki Kidokoro, Tetsuo Kubota, Jun Natsume, Tamik ...
    2013 Volume 45 Issue 1 Pages 21-25
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.21
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      Objective: We performed diffusion tensor imaging (DTI) in children with periventricular leukomalacia (PVL) to quantify the relationship between the fractional anisotropy (FA) values of DTI and the severity of PVL.
      Methods: In this study, we performed DTI in 16 children (seven males, nine females) with PVL. To evaluate the FA values, we used region-of-interest (ROI) measurements and tractography-based measurements. We classified the patients into two groups based on the severity of the magnetic resonance imaging (MRI) findings: the mild group had white matter injury limited to a triangular zone around the lateral ventricle (n=9) and the severe group had it extended forward (n=7). Then, we performed ROI measurements for these two groups to evaluate the FA values. We also divided the patients into two groups based on their motor ability: those that could (n=10) and could not (n=6) stand. We used tractography-based measurements to evaluate the FA values. To reduce the bias caused by age, we divided the patients into two groups: those younger than 3 years and those 3 years of age and older. All data were analyzed using the Mann-Whitney U-test, and p<0.05 was considered statistically significant.
      Results: In the ROI measurements, regardless of age, the severe group showed a more significant FA reduction in the white matter of the parietal and occipital lobes, including the middle/posterior part of the centrum ovale, superior longitudinal fasciculus, arcuate fasciculus, and thalamic radiation. In the tractography-based measurements, regardless of age, the measured FA values were significantly lower in the group that could not stand.
      Conclusions: This study suggested that the measured FA values could be used to evaluate the severity of PVL quantitatively, and that DTI provides much more information for understanding the pathophysiology of PVL, as compared with conventional MRI.
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  • Effectiveness of a Modified Parent Training of Smaller Groups and Shorter Schedules for Children with Pervasive Developmental Disorders
    Hiroko Okuno, Toshisaburo Nagai, Ikuko Mohri, Arika Yoshizaki, Tomoka ...
    2013 Volume 45 Issue 1 Pages 26-32
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.26
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      Objective: We have previously reported the modified parent training of smaller groups and shorter schedules (PTSS). In this study, we applied PTSS to the mothers of children with pervasive developmental disorder (PDD) and revealed the new evidence for its effectiveness.
      Methods: The participants were 30 mothers of children with PDD aged from 4.2 to 9.6 years. The effectiveness of PTSS was assessed with the confidence degree questionnaire (CDQ) and the child behavior checklist (CBCL), before and after each PTSS course. The recorded interviews were qualitatively analyzed using the KJ (Kawakita Jiro) methods.
      Results: Average CDQ scores were clearly improved as previously reported. In addition, the CBCL total T-score was significantly improved, which was not observed in the previous study. About KJ methods, six factors that change of mothers recognition were identified.
      Conclusions: Our findings provides additional evidence for the usefulness of PTSS for children with PDD.
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  • Parent Training for Parents of Children with Attention Deficit/Hyperactivity Disorder (AD/HD) Does not Improve the Behavior of Children with Both High-Functioning Pervasive Developmental Disorder and AD/HD
    Yayoi Tomizawa, Yoshinori Sato, Hiroyuki Yokoyama
    2013 Volume 45 Issue 1 Pages 33-37
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.33
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      Objective: Parent training (PT) has been developed as psychotherapy for parents who have children with attention deficit/hyperactivity disorder (AD/HD). We examined PT for parents who have a child diagnosed with a combination of high functioning pervasive developmental disorders (HFPDD) and AD/HD.
      Method: PT was used on parents with children with HFPDD+AD/HD and with children with AD/HD. The effects of PT were evaluated by the behaviors of children and the well-being of parents indicated by The Home Situation Questionnaire and the Subjective Well-Being Inventory, respectively.
      Results: PT did not improve the behaviors of children with HFPDD+AD/HD. PT did not improve the well-being of parents as indicated by the Subjective Well-Being Inventory. In contrast, PT did improve the behaviors of children with AD/HD and their well-being.
      Conclusions: This study indicated that PT developed for AD/HD may not be applied to parents with a child diagnosed with a combination of HFPDD and AD/HD. A new strategy is expected for parents who have a child with HFPDD+AD/HD.
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  • The Relationship between the Gross Motor Function and the Causes of Death in Persons with Severe Motor and Intellectual Disabilities
    Kuniaki Fukuda, Yosinobu Nakagawa
    2013 Volume 45 Issue 1 Pages 38-43
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.38
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      Objective: We investigated the relationship between the gross motor function and the causes of death and complications in persons with severe motor and intellectual disabilities (SMID).
      Methods: The causes of death, complications and ages of the 777 patients who had been admitted to the wards for persons with SMID in institutions involved in the national hospital organization and died from 1999 to 2008, were reported. Of these patients, 679 patients had data available. The patients were divided into 4 groups; group A (479 cases who could not roll over), group B (31 cases who could roll over but could not sit up), group C (53 cases who could sit up but could not stand up), and group D (22 cases who could stand up but could not walk without support).
      Results: The frequency of swallowing disturbance in group A was significantly higher than that in the other groups, while the frequency of gastroesophageal reflux was significantly lower in group C than in groups A and B. The percentage of tube feeding in group A was higher than that in the other groups. The differenc between groups A and C was statistically significant. The frequency of tracheotomy in group B was significantly lower than in group A and there were no cases of tracheotomy in groups C and D. Concerning the causes of death, the percentages of respiratory disorders in groups A, B, C, D were 52.4, 32.3, 35.8, 31.8, respectively. The mean age of death in group A was about 10 years younger than in the other groups.
      Conclusions: The gross motor function predicted the likelihood of respiratory and digestive systems complications and was related to life expectancy.
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  • A Case of X-linked α-Thalassemia/Mental Retardation (ATR-X) Syndrome with Repeated Apnea Attacks Due to Laryngomalacia
    Yuko Ebishima, Takako Misaki, Kenji Owa, Takehiko Okuno, Takahito Wada ...
    2013 Volume 45 Issue 1 Pages 44-48
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.44
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      We report a case of X-linked α-thalassemia/mental retardation syndrome (ATR-X) with repeated apnea attacks dating from the patient's 12th year. We initially diagnosed them as obstructive apnea due to upper pharyngeal stenosis and laryngomalacia by polysomnography and laryngo-fiberscopy. However, reevaluation after one and a half years revealed that the boy had central and mixed apnea, as well as obstructive apnea. To date, few reports have been published on the causes of apnea attacks in ATR-X patients. We clinicians should therefore consider laryngomalacia as one cause of apnea attacks in ATR-X patients, and choose the appropriate therapy for a pattern of apnea that can change during its clinical course.
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  • Cyclophosphamide Pulse Therapy Effective for Childhood-Onset Refractory Multiple Sclerosis: a Case Report
    Tae Ikeda, Kuriko Kagitani-Shimono, Yoshiko Iwatani, Yukihiro Kitai, M ...
    2013 Volume 45 Issue 1 Pages 49-52
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.49
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      We report a case of a 15-year-old girl with relapsing-remitting multiple sclerosis (MS) who received cyclophosphamide pulse therapy. At the age of 5 years, she displayed symptoms such as headache and unconsciousness after varicella infection as the first episode of MS. She had been treated with methylprednisolone pulse therapy, intravenous immunoglobulin, interferon-β1b, and azathioprine. However, she had relapsed 12 times by the age of 15 years. At this time, she showed weakness and severe paralysis of her left leg, and even 1 month after methylprednisolone pulse therapy, she still had gait impairment and showed gadolinium-enhanced lesion on brain magnetic resonance imaging. We then started cyclophosphamide pulse therapy (600 mg/m2) once a month for 12 months combined with interferon-β1a. She had no serious side effects and she could walk again after 4 months on cyclophosphamide treatment. She has been free from relapse for 2 years and 8 months until the present time. Although only a few studies have indicated the efficacy of cyclophosphamide pulse therapy for childhood MS, we consider careful use of cyclophosphamide could be one of the options for refractory childhood MS.
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  • An 8-Year-Old Boy with Anti-NMDA Receptor Encephalitis, Successfully Treated with Cyclophosphamide
    Tadahiro Mitani, Yoshimitsu Ohtsuka, Kei Yamamoto, Yoshihiro Watanabe, ...
    2013 Volume 45 Issue 1 Pages 53-57
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.53
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      We report on an 8-year-old boy with non-paraneoplastic anti-NMDA receptor (NMDAR) encephalitis, who presented with psychotic symptoms and involuntary movement following an intractable seizure. His serum and CSF tested positive for anti-NMDAR antibodies. He received an initial immunotherapy consisting of methylprednisolone pulse therapy (mPSL) and intravenous immunoglobulin therapy (IVIg), without any clinical improvement. He had three cycles of monthly cyclophosphamide pulse therapy (500 mg/m2), and his clinical condition started to improve gradually two weeks after the first cycle, without any side effects. Six months after onset, he tested normal upon standard neurological examination. Cyclophosphamide therapy should be considered for children with anti-NMDAR encephalitis, as well as mPSL and IVIg.
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  • ACTH Therapy for Two Cases of Epileptic Spasms without Hypsarrhythmia
    Masahiro Ishii, Masayuki Shimono, Ayako Senju, Tomofumi Fukuda, Naoki ...
    2013 Volume 45 Issue 1 Pages 58-61
    Published: 2013
    Released on J-STAGE: March 03, 2014
    DOIhttps://doi.org/10.11251/ojjscn.45.58
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      We present two patients with epileptic spasms without hypsarrhythmia (ESWoH) who were successfully treated with ACTH. One patient was an 18-month-old boy who had normal development for the first 16 months of his life. Thereafter, he experienced sudden flexion of the axial muscles, resulting in head nodings, or epileptic spasms (ES). EEG tracings showed generalized polyspikes that appeared frequently during sleep without associated hypsarrhythmia. We were thus able to diagnose him with ESWoH and treated him with ACTH, which resolved his seizures. Another patient was a 7-year-old boy. At 18 months of age, the patient began to sufferdil from sudden ES. Anti-epileptic drugs were administered individually and in combination but were proved ineffective. Like the first case, this child's EEG tracing showed generalized polyspikes during sleep without associated hypsarrhythmia. We diagnosed him with ESWoH and treated him with ACTH. His ES disappeared soon after the initiation of treatment. These two cases demonstrate the difficulty of treating ESWoH only with standard anti-epileptic drugs, we emphasize the importance of early treatment with ACTH.
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