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Volume 37, Issue 4
Displaying 1-17 of 17 articles from this issue
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[in Japanese]2005 Volume 37 Issue 4 Pages 278
Published: July 01, 2005
Released on J-STAGE: December 15, 2011
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[in Japanese], [in Japanese]2005 Volume 37 Issue 4 Pages 279-280
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
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Toshio Saito, Susumu Shinno2005 Volume 37 Issue 4 Pages 281-286
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSPatients with Duchenne muscular dystrophy (DMD) and severe congestive heart failure (CHF) frequently feel mental anguish due to severe mental and physical restriction. Since therapy is not efficacious enough, their quality of life is often disturbed during the terminal stage. We retrospectively evaluated the treatment and care for 11 cases of DMD with severe CHF in our hospital. All cases had unrest and anxiety, which weresuccessfully treated with benzodiazepines and haloperidol. In many cases, patients' families craved for patients' comfort without mental and physical pain. Nine cases resulted in death and 2 cases survived. We also sent a questionnaire to doctors of muscular dystrophy wards of 27 Japanese national hospital, inquiring about therapy protocol, monitoring system, intravenous nutrition, limitation of feeding/recreation/visitors, management of pain/anxiety/sedation, and cardiopulmonary resuscitation (CPR). Sixty-eight doctors answered the questionnaire. Forty-sevendoctors (69%) had the experience to treat DMD patients with severe CHF. The majority of them monitored electrocardiography, SpO2 and blood pressure. About a half adopted intravenous nutrition. If recovery was expected, limitation of feeding/recreation/visitors was based mainly on discussion with the patients and their families. If recovery was impossible, the limitation was decided according to their wishes. Nonsteroidal anti-inflammatory drugs were properly used for pain, and minor and major tranquilizers for sedation. Morphine was also used. Only one doctor adopted positive CPR, while the others answered “do not CPR” or “do CPR according to the wish of patients' families”. The burdento patients and their families during treatment is unavoidable but should be reduced as much as possible. Medical staffs should ask themselves about the problems to support the families as well as patients repetitively.View full abstractDownload PDF (755K) -
[in Japanese]2005 Volume 37 Issue 4 Pages 287
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
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[in Japanese]2005 Volume 37 Issue 4 Pages 287a
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
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[in Japanese]2005 Volume 37 Issue 4 Pages 288
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSDownload PDF (163K) -
[in Japanese]2005 Volume 37 Issue 4 Pages 288a
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
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Mana Kurihara, Yoichiro Nakae, Toshitaka Kohagizawa, Yoshikatsu Eto2005 Volume 37 Issue 4 Pages 289-292
Published: July 01, 2005
Released on J-STAGE: December 15, 2011
JOURNAL FREE ACCESSWe studied single photon emission computed tomography (SPECT) of 15 children withacute encephalopathy after more than 1 year from the onset, using technetium-99 m-L, L-ethyl cystinatedimer (99mTc-ECD) and a threedementional stereotaxic region of interest template. Regional cerebral blood flowwas evaluated and divided in three groups according to the severity of disability: absent or mild, moderate, and severe. There was no abnormality on SPECT in the patients without disability or with mild disability. Diffuse hypoperfusion was shown in the groups with moderate and severe disability. The patients with severe disability showed hypoperfusion in the pericallosal, frontal and central areas which was more pronounced than in the patients with moderate disability.View full abstractDownload PDF (536K) -
Kiyokuni Miura, Yoshiko Suzuki, Toshiyuki Kumagai, Chiemi Hayakawa, Ak ...2005 Volume 37 Issue 4 Pages 293-298
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSWe discuss here the indication and complications of tracheostomy performed in 57 home-care pateints with severe motor and intellectual disabilities (SMID) during the past 13 years at our hospital. Thirty-five cases underwent tracheostomy following emergency endotracheal intubation for acute respiratory failure. Recently, the number of cases without preceding endotracheal intubation have increased. Many patients underwenttracheostomy at the age of 1 to 4 years and 10 to 14 years. The quality of life (QOL) of almost all the patients without preceding intubation markedly improved, as well as that of their families, and they were able to return to home. The most decisive reason for tracheostomy was secretions and recurrent aspiration pneumonia in 8 patients, gastroesophageal reflux in 4 and upper airway obstructions in 3. Several complications of tracheostomy were observed: tracheal granulations in 9 patients, tracheal malacia in 8, and tracheoinnominate artery fistula in 5. Among 8 patients with tracheal malacia, bleeding from the tracheoinnominate artery fistula occurred in 3. In 7 patients, self-madelong tracheostomy tubes were necessary for the initial management of the tracheal malacia or tracheal granulations. Subsequently, made-to-order long tracheostomy tubes were used in three of these patients. In 12 patients, improvedendotracheal T-tube with the tip sealed on the vocal cord side was used to prevent aspiration. Home-care SMID patients with respiratory disturbance require tracheostomy timely performed, followed by careful observation to preventpostoperative complications.View full abstractDownload PDF (889K) -
Toshihiro Horiguchi, Makiko Kaga, Masumi Inagaki2005 Volume 37 Issue 4 Pages 301-306
Published: July 01, 2005
Released on J-STAGE: December 15, 2011
JOURNAL FREE ACCESSWe assessed the utilization of diagnostic analyses for fragile X syndrome by a mail-in questionnaire on 1) the number of patients analyzed and diagnosed with the syndrome in the past year, 2) types of diagnostic analyses used, 3) clinical features that made physicians to decide analyses, 4) purpose of analyses, and 5) informed consent for analyses. Facilities for the mentally handicapped, as well as hospitals and physicians specialized in genetics, completed our questionnaire. Among 101 responders, total of 543 cases underwent analyses. Nine cases (including 3 cases over 20 years old) were finally diagnosed in a year. The rate of positive findings was 0.6% for chromosomal analyses, and 8% for gene analyses. Physicians dicided to make analyses based on clinical features such as mental retardation, characteristic face, and autistic features, in order to find the cause (s) of the subjects' condition. For gene analyses, more than a half of physicians obtained a form of informed consent. Specialists should have interest in this syndrome because the analyses identified new adult cases. Establishment of a guideline for diagnosis of this syndrome requires gene analyses based on evidence and informed consent.View full abstractDownload PDF (787K) -
Toyoko Kanda, Keiko Murayama, Izumi Kondo, Eiji Kitazumi, Kan Takahash ...2005 Volume 37 Issue 4 Pages 307-316
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSAlthough the most common cause of death in patients with severe motor and intellectual disabilities (SMID) is aspiration pneumonia, there are no criteria to detect aspiration. We have been making an evaluation tool to detect aspiration in patients with SMID easily, in cooperation with the project of comprehensive study of disability, social health, and welfare, supported by the Ministry of Health, Labor and Welfare, Japan. Here we studied the reliability and accuracy of the 2001 edition of the “questionnaires for functions of eating and swallowing” and “estimation chart for the possibility of aspiration” in 20 patients with risk of aspiration (13 with cerebral palsy, six with acquired cerebral palsy and one with muscle disease). Family members or nursing staffs taking care of them answered the questionnaires. A pediatrician and an occupational therapist or speech therapist checked the chart.
Reliability was checked by the agreement between the estimation of the two professionals, and accuracy was determined by comparing the results of the chart and videofluolography. The results showed good reliability (κ 0.63) and accuracy (κ 0.47).
Based on these results and additional analysis of the check items, the new edition of the chart was established.View full abstractDownload PDF (1164K) -
Sachiko Onoe2005 Volume 37 Issue 4 Pages 317-321
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSNeurocysticercosis is common in Asia, Africa and South America including Mexico. A diagnosis of neurocysticercosis was made neuroradiologically in an 8-year-old Mexican girl, who had suffered from learning difficulties for a year, and subsequently developed headache, vomiting, gait disturbance, visual hallucination, apraxia and euphoric state for 4 months. Plain CT demonstrated numerous cystic lesions with or without scolex in addition to calcified lesions. The lesions were seen throughout the whole brain, although they were relatively scarce in the cerebellum and the lateral ventricular system. Although edema was prominent around the bilateral ventricles, neither ventricular dilatation, midline shift nor inflammatory reaction around the cysts was detected. The patient was treated with albendazole for a month and with steroid for two months. Headache and vomiting disappeared in the first week of treatment but other symptoms persisted, with fluctuation. CT after 2 months of treatment showed dilatations of the third and lateral ventricles. Despite massive infection in the brain, the clinical picture at onset was mild and compatible with nonencephalitic cerebral cysticercosis. The symptoms might be caused by the progressive hydrocephalus due to cerebrospinal fluid blockage, the prognosis of which is predicted to be poor.View full abstractDownload PDF (3483K) -
Kentaro Okamoto, Mitsumasa Fukuda2005 Volume 37 Issue 4 Pages 323-326
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSA 2-day-old boy had hemi-convulsion and diagnosed as having neonatal infarction. Brain MRI was performed on the day after the day of onset. Abnormal finding were noted on diffusion weighted imaging, but not on T1 and T2 weighted imaging.
During the first few days of life, it is difficult to demonstrate neonatal brain infarction by T1 and T2 weighted imaging. Diffusion weighted imaging is useful for the early diagnosis of neonatal infarction.View full abstractDownload PDF (2147K) -
Yoichiro Nakae, Mana Kurihara, Toshitaka Kohagizawa, Yoshikatsu Etoh2005 Volume 37 Issue 4 Pages 327-331
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSA 3-year-old girl developed right hemiplegia with acute onset, followed by generalized convulsion, choreic movement, and severe motor deterioration. She became bed-ridden a few months after the onset. Intravenous cyclophosphamide pulse therapy resulted in a remarkable improvement of her clinical symptoms. At age of six, she has recovered markedly with the residual symptom of minimum dysarthria. Autoimmune mechanism similar to the antiphospholipid syndrome was considered to be the pathogenesis of this case.View full abstractDownload PDF (5205K) -
Naoko Wada, Keiko Murayama, Tatsuyuki Kaneko, Eiji Kitazumi2005 Volume 37 Issue 4 Pages 332-336
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSAn intrapulmonary percussive ventilator (IPV) improves airway clearance and lung function, and is useful for wide variety of respiratory disorders, such as cystic fibrosis, chronic obstructive pulmonary disease, aspiration pneumonia, and neuromuscular diseases. However, there are few reports on IPV usein patients with severe neurological impairment, scoliosis and thoracic deformity. They have poor mobility of the rib cage and difficulty in sputum expectoration. The use of IPV significantly improved persistent consolidation shown by chest computed tomography (CT) in one of such patients. The patient was a 33-year-old woman with severe spastic quadriplegia and tracheostomy and she was dependent on mechanical ventilation because of chronic restrictive respiratory failure. After fever and mild hypoxemia for one day, chest CT revealed consolidation of the left lower lobe. An IPV-I ventilator was used for 15 min once a week, with a stroke frequency of 250-300 cycles/min and pressure of 22 PSI. Mechanical ventilation was withheld during the IPV therapy. Chest physiotherapy was also done. According to the worsening of the consolidation on chest CT, the frequency of IPV was changed to once a day at day 23 and then to twice a day. Chest CT at day 44 showed further improvement. In patients with severe motor and intellectual disabilities, it is sometimes difficult to control progressive deterioration of pulmonary function and persistent atelectasis even with tracheostomy, mechanical ventilation, and conventional physiotherapy. Our results indicate that IPV may improve respiratory function and the quality of life in such patients.View full abstractDownload PDF (5020K) -
Norikazu Shimizu, Masaoki Mizutani, Tsugutoshi Aoki2005 Volume 37 Issue 4 Pages 337-341
Published: July 01, 2005
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSCrigler-Najjar syndrome type I is an autosomal recessive disorder with severe unconjugated hyperbilirubinemia, caused by the complete absence of bilirubin uridine 5'-diphosphate-glucuronosyltransferase (UGT) activity. The authors reported a 24-year-old male with this syndrome. He had severe icterus from the age of 4 days, and was diagnosed as having Crigler-Najjar syndrome type I at 51 days after birth. Despite repeated phototherapy, his serum bilirubin was increased. When bilirubin encephalopathy occurred at the age of 16 years, the serum bilirubin level was 47 mg/100 ml. EEG showed diffuse and continuous high voltage slow waves. He was treated with bilirubin adsorption, which reduced the serum bilirubin level to 10-20 mg/100 ml, with disappearance of the EEG abnormality. Subsequent liver transplantation resulted in improvement of neurological signs and symptoms, and recovery of his mental function.View full abstractDownload PDF (549K) -
[in Japanese], [in Japanese], [in Japanese]2005 Volume 37 Issue 4 Pages 348-355
Published: July 01, 2005
Released on J-STAGE: December 15, 2011
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