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Volume 29, Issue 1
Displaying 1-14 of 14 articles from this issue
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[in Japanese]1997 Volume 29 Issue 1 Pages 2
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
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[in Japanese], [in Japanese]1997 Volume 29 Issue 1 Pages 3-12
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
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Kazuya Honke, Masaki Hasui, Nobuhiko Takano1997 Volume 29 Issue 1 Pages 13-18
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIt has been reported that serum levels of ketone bodies often elevate in patients with Duchenne muscular dystrophy (DMD). The cause of this abnormal metabolism, however, has not been elucidated. In this study, serum levels of ketone bodies were measured in the early morning in patients with DMD, congenital muscular dystrophy and cerebral palsy. Serum free fatty acids (FFA), glucose, and thickness of subcutaneous fat were also measured. Ketone bodies elevated in most DMD patients, but not elevated in patients with the other disorders. Hyperketonemia in the DMD patients was accompanied by elevation of the FFA level, but no correlation was seen between hyperketonemia and the creatine kinase level or the severity of muscle involvement. No differences were found between DMD patients and others in the levels of FFA, glucose and thickness of subcutaneous fat. These results indicate that DMD patients are more prone to ketosis than the others. The effect of biotin administration (2 mg/day) on hyperketonemia also was investigated 11 patients with DMD. The levels of total ketone bodies decreased by biotin administration for 2 weeks. These data suggest that the utilization of FFA and ketone bodies may be impaired in DMD patients. Biotin treatment will be useful for improving the abnormal metabolism of ketone bodies in DMD patients.View full abstractDownload PDF (599K) -
nalysis of Psychomotor Development and MRI Findings of the Brain in Sixteen Congenitally Hydrocephalic PatientsJunichi Shimada, Masakazu Miyajima, Kiyoshi Sato, Takuji Yamamoto, Haj ...1997 Volume 29 Issue 1 Pages 19-26
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSSixteen patients out of 58 children with congenital hydrocephalus were retrospectively investigated in terms of their psychomotor development and morphological findings of the brain by magnetic resonance imaging (MRI), and morphological backgrounds of the brain in patients affected by “intractable hydrocephalus” were intended to be clarified. The 16 patients were primarily diagnosed as having aqueductal stenosis or communicating hydrocephalus during the era before introduction of MRI.
On the basis of the rate of head growth, sixteen patients were divided into three groups; group-1: overt neonatal hydrocephalus (5 cases); group-2: rapidly progressive hydrocephalus with normal head circumference at birth (5 cases); and group-3: slowly progressive hydrocephalus with its onset after the neonatal period (6 cases). Psychomotor development of the 16 cases was analyzed by using various methods including the Tanaka-Binet intelligence test and WISC-R. Four cases in group-1 and all cases in group-2 were found to have IQ or DQ below 50, while the other remaining cases were found to have IQ of 82 in group-1 (one case) and IQ ranging from 67 to 113 in group-3. MRI performed in those 9 cases with IQ below 50 revealed various morphological abnormalities of the brain. Those abnormalities were characterized by disturbance of organogenesis and/or histogenesis of the brain dating from the first 20 weeks of fetal life.
It is strongly suggested that in the pathogenesis of intractable hydrocephalus developmental malformation of the brain occurring during the first 20 weeks of gestation are involved. Functional and morphological impairments of the brain possibly caused by progression of fetal hydrocephalus and delay of CSF diversion before and after birth seem important in the pathogenesis of intractable hydrocephalus.View full abstractDownload PDF (6365K) -
Hisaharu Suzuki1997 Volume 29 Issue 1 Pages 27-32
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSGestational age, birth weight, degree of motor impairment, and causes of brain damage were analized in 68 patients with cerebral palsy born and identified in a community. This community is located in the suburban Tokyo with about 480, 000 inhabitants. In the period of 1985- 1991, it had 34, 289 live births (annual birth rate 1.0%) and all the births were recorded at public health centers. Sixty- eight patients with cerebral palsy were identified with the incidence rate of 2.0/1, 000 birth.
Twenty out of 68 cases (29%) were born before 37 weeks of pregnancy and 27 out of 68 cases (40%) were born with birth weight less than 2, 500 gm. Patients with full term gestational weeks had severe motor impairment compared to preterm cases. Brain damages occurring in the prenatal period played a major role as the cause of cerebral palsy in the full-term-born group, and brain damage occurring in perinatal period played a major role in the preterm-born group.
We conclude that to lessen new cases of cerebral palsy, we need two starategies ; prevention of brain damages in the prenatal period, and prevention of preterm births.View full abstractDownload PDF (804K) -
Keiko Koterazawa, Makoto Nabetani, Hiroyoshi Miyata, Satoshi Takada, Y ...1997 Volume 29 Issue 1 Pages 33-38
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe volume of the white matter in 5 cases of cerebral palsy (CP) of term delivery without hypoxic episodes with MRI findings of periventricular leukomalacia (PVL), was compared with that of 12 CP cases in preterm delivery manifesting typical MRI findings of PVL. It was significantly decreased in the latter. The volume for the full-term CP cases was the same as that for children born at term and developing normally. These findings suggest that the grade of perinatal brain damage is different between the full-term and preterm CP cases, and that the intrauterine timing of fetal circulatory disturbance is different.View full abstractDownload PDF (5403K) -
Yoshimi Sata, Masao Aihara, Kazuo Hatakeyama, Yoh-ichi Hinohara, Yuhko ...1997 Volume 29 Issue 1 Pages 39-44
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIntravenous drip infusions of lidocaine (IDIL; 1-5 mg/kg/h) were performed in ten patients with intractable seizures. The medication was very effective in five patients, whose seizures disappeared immediately after the treatment of IDIL. In four patients, the medication proved to be effective judging from decreased incidence of seizures. In one patient with intractable seizures, the therapy was not effective. In nine patients with the effective medication, five had generalized seizures and four had partial seizures.
Side effects were observed in four patients. Two patients had muscle hypotonia, one had visual and auditory hallucination and another had bradycardia. These symptoms completely disappeared after the ceasing of IDIL. Serum concentrations of lidocaine at the appearance of the side effects ranged from 1.8 to 4.7ug/ml, although the toxicity level is more than 5.0μg/ml for arrhythmic adult patients.
These findings suggest that the serum toxic level of lidocaine in children is different from that in adults, and that careful observation and appropriate management for the children with lidocaine therapy should be necessary, even though the serum level of lidocaine ranges within the therapeutic level.View full abstractDownload PDF (784K) -
Yuhko Kamiya, Masao Aihara, Kazuo Hatakeyama, Yoh-ichi Hinohara, Chika ...1997 Volume 29 Issue 1 Pages 45-50
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSClinical and electroencephalographic (EEG) studies were performed in nine patients with cerebral cortical dysplasia (CD). Interictal single photon emission computed tomography (SPECT) using 99mTchexamethyl propylene amine oxime were studied in four patients. A patient with hemimegalencephaly and agyria had poor outcome in both developmental and epileptic aspects. The prognoses of clinical seizures were generally related to the severity, location and size of CD. The size of the lesion was not always correlated with the clinical seizure outcome. Four patients had focal pachygyria. Clinical pictures were diverse in these patients. One patient underwent callosotomy for the control of intractable seizures. The others had no clinical seizures despite of the appearance of paradoxical discharges in the area of pachygyria. The distribution of CD detected by MRI did not always correlate with that of paradoxical discharges in EEG and/or hypoperfusional areas seen in SPECT. These findings suggest that a detailed neuroimaging study is useful to elucidate the epileptogenesis in patients with CD, and that all the cortical abnormalities in patients with intractable epilepsy are not detected by MRI.View full abstractDownload PDF (2730K) -
Chitose Tanaka, Yoshihiro Maegaki1997 Volume 29 Issue 1 Pages 51-54
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 13-year-old boy with juvenile-onset acid a glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO, 54.1 mmHg) and hypercapnia (PCO2 72.3mmHg), and spirometry showed significantly decreased vital capacity (%VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial P02 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid a glucosidase deficiency.View full abstractDownload PDF (2328K) -
Tsunenori Hirayama, Takehisa Fujita, Kazuhiko Matsuoka, Kazuhiko Shiro ...1997 Volume 29 Issue 1 Pages 55-60
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe 11-year-old girl had hypertension and generalized tonic-clonic convulsions during the steroid pulse therapy. Her cranial T2- weighed MRI showed small areas of high signal intensity in the right occipital lobe. Her neurological findings were normal. After 2 months of steroid pulse therapy, multiple high signal intensities were demonstrated on T2- weighed MRI. After 5 months of the therapy, however, only a small T2-weighed MRI spot of high signal intensity in the white matter was observed, and the multiple lesions disappeared. On follow up, she did not show any neurological defects. It is postulated that not only steroid pulse therapy-induced hypertension but also SLE- induced angiopathy caused such flexible lesions. According to the literature, these lesions can be considered to be edema around small vessels. Such MRI findings have been reported in adult patients with SLE, but to our knowledge there have not been reports on children with SLE.View full abstractDownload PDF (8121K) -
Masaki Hasui, Chikako Saito, Takeshi Kasama, Takao Taki, Akihiro Yachi ...1997 Volume 29 Issue 1 Pages 61-66
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported the decreased level of cholesterol as well as the elevated levels of 7-and 8-dehydrocholesterol in the serum and erythrocytes of a Japanese patient with Smith-Lemli-Opitz syndrome. These findings suggested that the detection of these precursors of cholesterol synthesis should become an important biochemical parameter for this syndrome in which clinical features are not always obvious.View full abstractDownload PDF (2733K) -
Naomi Kubo, Naoya Itokazu, Shinobu Inoue1997 Volume 29 Issue 1 Pages 67-72
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA female case of isolated ACTH deficiency associated with neuromuscular symptoms was reported. Although her initial development was delayed with perinatal troubles, developmental catch up was seen. The patient complained of general fatigue with weakness and poor school performance at the age of eleven. Muscle weakness predominant to the proximal portion and mental dullness were suspected from neurological examination. Her laboratory data were as follows; mild elevation of CK in serum, subclinical thyroidal dysfunction, abnormal electrocardiogram, slow wave activity on electroencephalogram, delayed nerve conduction velocity, and localized hypoperfusion of cerebral blood flow on single photon emission computed tomogram. Histological examination of muscle biopsy demonstrated only mild fiber size variation. During two years' follow-up, her intelligence quotient fell down, while muscle weakness did not progress significantly. At the age of fourteen, a low level of plasma ACTH was pointed out by chance and a definitive diagnosis was obtained by endocrinological examinations; no response of ACTH and cortisol on insulin stimulation, delayed response of cortisol on continuous ACTH stimulation, and no response of plasma ACTH on corticotropin releasing hormone stimulation. Other signs of adrenocortical insufficiency, such as hypoglycemia and abnormal serum electrolytes, were not observed during the clinical course. This case suggested that isolated ACTH deficiency should be considered for differential diagnosis of neuromuscular disorders.View full abstractDownload PDF (6607K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1997 Volume 29 Issue 1 Pages 73-75
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese]1997 Volume 29 Issue 1 Pages 76-77
Published: January 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (1347K)
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