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Yukuo Konishi, Giovanni Cioni, Heinz F.R. Prechtl
1993 Volume 25 Issue 1 Pages
3-8
Published: January 01, 1993
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The development of posture during the prenatal period was studied longitudinally in 12 matched pairs of low-risk and high-risk preterm infants. The gestational age of the infants in both groups ranged from 28 to 36 weeks. The two members of each pair had the same gestational age at birth and the video recordings were performed at the same postmenstrual age. They were filmed more than three times during the prenatal period, each video recording lasting 1 h. Time of occurrence and duration of each posture were analyzed from replay of the video recordings. No age-specific preference posture described in the literature was found from birth until the term age between two groups. There was no age-related tendency in duration and frequency of postures between two groups. There was a clear-cut dominant head position to the right side from 31 weeks onward. Asymmetrical tonic neck postures (ATN) were very inconsistently present before the term age. And there was no significant difference in the occurrence of ATN postures between two groups. We could not find any specific abnormal postures in the high-risk group. Our study suggested that posture in preterm infants is not a good indicator of brain damage in the prenatal period.
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Mitsuko Ishii, Junichi Takanashi, Katsuo Sugita, Arata Suzuki, Michiyo ...
1993 Volume 25 Issue 1 Pages
9-15
Published: January 01, 1993
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We performed serial magnetic resonance imaging (MRI) toward 4 male children with possible Pelizaeus- Merzbacher disease (PMD). They were clinically suspected as PMD on the basis of typical neurological, electrophysiological and MRI examinations. MRI revealed that myelinated fibers were localized in brainstem, cerebellum, internal capsule, optic radiation and proximal corona radiata in case 1, while myelinated fibers were almost absent in case 2-4. On repeated MRI investigation, we found no further extension of myelination in case 1 and the absence of myelination in case 2-4. This study demonstrated that myelination in the brain is arrested immediately after birth in case 1, while no myelination developed before birth in case 2-4. It was speculated that MRI in case 1 and case 2-4 might correspond to neuropathologic findings of classical and connatal form as reported in the literature.
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Hisaharu Suzuki, Ayako Iso, Mitsuru Ishikawa
1993 Volume 25 Issue 1 Pages
16-20
Published: January 01, 1993
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We investigated the incidence rates of cerebral palsy, severe mental and motor retardation, and Down syndrome in the City of Kokubunji in suburban Tokyo with a total population of about 100, 000. The number of liveborn babies during 1985-1989 was 5, 475. The number of children with cerebral palsy (CP) was 11 and the incidence rate was 2.01/1, 000, which was equal to the rates in reports from several countries and was higher than those of the previous reports from Japan. Two out of 10 patients with CP had mild motor handicaps and were expected to “outgrow” their handicaps. The number of children with “severe mental and motor retardation”(SMMR) was 6 and the incidence rate was 1.10/1, 000. Prenatal brain damage played a major role in the pathogenesis of CP and SMMR. The number of children with Down syndrome (DS) was 11 and the incidence rate was 2.01/1, 000 which was higher than the previous rates. Incidence rates of CP, SMMR, and DS still remain high and further strategy to prevent pediatric neurological diseases is necessary.
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Sawa Yasumoto, Akihisa Mitsudome, Hiroko Ogata, Masaharu Ohfu
1993 Volume 25 Issue 1 Pages
21-25
Published: January 01, 1993
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An electrophysiological study of Bell's palsy in children was reported. Direct response to facial nerve stimulation was tested in 28 patients. Of these, 17 patients were further tested for electrically elicited blink reflex. The reduction in amplitude of direct response on affected side was characteristic of Bell's palsy. The direct response was evoked on affected as well as nonaffected side. The amplitude of the direct response on the affected side was expressed as percentage of that on the nonaffected side (ratio of M response amplitude: RMA). 13 patients showed incomplete recovery after 3 months, and RMA of the 11 patients of them were reduced below 30%. In the patients showing good clinical recovery within 2 months, RMA returned to normal promptly. In the blink reflex, R1 R2 components were found to be persistent on the first examination, suggesting a satisfactory functional outcome in Bell's palsy. As these electrophysiological methods are conventional assessing facial nerve function, it seemed to be valuable for the follow up of children with Bell's palsy.
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Asayo Ishizaki, Masaya Kubota, Noboru Fueki, Masako Shinozaki, Kiyoko ...
1993 Volume 25 Issue 1 Pages
26-32
Published: January 01, 1993
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A long-term obsevation has been made in 58 patients (30 males and 28 females) with severe sequelae of neonatal anoxic encephalopathy. They aged from 8 months to 65 years. All of them had motor disturbances and profound mental retardation. Motor function was improved in 4 patients with aging. In contrast, motor activity deteriorated in 11 cases, of which 4 showed a mental regression. Among them, patients who had originally better motor ability than sitting were likely to deteriorate by uncontrollable epilepsy and/or excessive administration of anticonvulsants. Regression of the patients with worse motor ability like bedridden appeared to attributable hypertonia of muscles and bodily deformation.
Fifteen cases showed an exacerbation of general condition which originated predominantly to respiratory distress. Twelve patients died including 6 exacerbated cases. Exacerbation or death may have occurred frequently in specific periods of infancy, adolescence and youth with the patients who showed very low motor function such as bedridden and no locomotion.
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Hirokuni Negishi, Yokei Lee, Masamitsu Nishino, Kyoko Itoh, Shinji Kaw ...
1993 Volume 25 Issue 1 Pages
33-39
Published: January 01, 1993
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Auditory brainstem response (ABR) was recorded in 41 full-term newborn infants with intracranial hemorrhages (ICH; 10 infants with and 31 infants without neurological sequela) and the data were compared with those obtained in normal full-term newborn infants as controls.
The wave-I peak latency was significantly prolonged in the non-sequela group than in the control group and in the sequela group than in the non-sequela group. The wave-III and wave-V peak latencies were significantly prolonged in the ICH group (sequela and non-sequela groups) than in the control group but did not significantly differ between the sequela and non-sequela group. The wave I-V interpeak latency did not significantly differ among the three groups.
Among 10 infants in the sequela group, 9 had a V/I amplitude ratio (the amplitude of wave-I divided by the amplitude of wave-V) of less than 1.0. This suggests that the V/I amplitude ratio is of prognostic value in ICH infants.
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Hitoshi Yamamoto, Bunsei Egawa, Kumiko Horiguchi, Akiko Kaku, Kaneo Ya ...
1993 Volume 25 Issue 1 Pages
40-44
Published: January 01, 1993
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Changes in cerebrospinal fluid (CSF) neurotransmitter metabolite concentrations in 32 neurologically normal children aged 0 month to 7 years were analyzed to assess maturation of the central nervous system and compared to CSF from adults. Significant inverse correlations with aging were observed for tryptophan, 5-hydroxyindoleacetic acid, kynurenine, tyrosine, L-dihydroxyphenylalanine, dopamine, homovanillic acid and 3-methoxy-4-hydroxyphenylglycol concentrations. There were no significant differences with aging in CSF serotonin or 3-hydroxykynurenine concentrations. These findings demonstrated that changes in major CSF neurotransmitters occurred with aging during childhood. The importance of age-matched controls in studies of CSF neurotransmitter metabolites in the developing brain was emphasized.
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[in Japanese], [in Japanese], [in Japanese], [in Japanese]
1993 Volume 25 Issue 1 Pages
45-51
Published: January 01, 1993
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Masaharu Hayashi, Takeshi Hasegawa, Keiko Hayakawa, Yoshihide Iwakawa
1993 Volume 25 Issue 1 Pages
53-58
Published: January 01, 1993
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We examined long-term neurological outcome of 12 patients, whose first febrile seizures had occurred during exanthema subitum (ES) and who had been treated with continuous daily administration of anticonvulsant drugs.
Six of 12 children had a family history of febrile or afebrile convulsions and in a sister of one patient, athetoid palsy followed the status of afebrile convulsion (AC) during ES.
Twelve cases were clinically classified into four groups;(A) 2 patients in whom ACs were repeated as soon as the fever of ES had dropped, (B) 6 patients in whom frequent recurrences of febrile convulsion followed ES, showing epileptic EEG abnormalities, (C) 1 patient who had suffered from later epilepsy, (D) 3 patients who showed complex type of febrile convulsion only during ES without any EEG abnormalities.
Except one patient in group (A), who showed residual hemiparesis on the left, 11 cases demonstrated normal psychomotor development, and in 5 of all 12 cases, the treatment with anti-convulsant drugs could be discontinued. Unless severe neurological sequelae are complicated, long-term prognosis is not so poor in children whose first febrile convulsion occurred during ES.
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Tomoko Miyajima, Eiji Kurihara, Yoshihiko Mizuno, Kimiko Tamagawa, Kaz ...
1993 Volume 25 Issue 1 Pages
59-64
Published: January 01, 1993
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Prognosis of 175 psychoneurologically normal children with the onset of epilepsy after 18 months of age were studied. The remission rate of partial epilepsy (81 of 107 cases, 76%) was higher than that of generalized epilepsy (34 of 56 cases, 61%). Prognoses for patients with sleep epilepsy (52 of 62 cases, 84%) were better than those for patients with waking epilepsy (63 of 101 cases, 62%). Most (95%) of the children with sleep epilepsy had partial epilepsy, including benign childhood epilepsy with centrotemporal spike (BECT, 36 cases) and partial epilepsy other than BECT (23 cases). Benign courses were not limited to BECT patients. Generalized waking epilepsy other than absence had the lowest remission rate. Eleven children exhibited mild mental retardation on last examination. Most of them had atypical absence and/or myoclonic seizures with the onset of seizure before 3 years of age. It appeared important to classify the epileptic children with regard to time of seizure occurrence as well as seizure type to determine their prognoses.
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Yasuo Sunaga, Takayuki Ohtsuka, Takashi Fujinaga
1993 Volume 25 Issue 1 Pages
65-69
Published: January 01, 1993
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A 3-year-old girl with Down syndrome was admitted to our hospital, associated with hypotonia and dyspnea. She presented frog-posture and shallow breathing. The blood gas analysis revealed hypercapnic acidosis with pH 7.371, P0
2 74.6 mmHg, PCO
2 52.6 mmHg, and BE 3.5. The cervical X-ray films with flexion and extension of head showed anterior dislocation of the atlanto-axial articulation. Magnetic resonance imaging clearly showed a severe cord compression between C
1 and C
2.
In children, spinal cord compression induced by dislocation of the atlanto-axial articulation is very rare. However, we should take into consideration of this insidious risk associated often with Down syndrome.
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Yukie Watanabe, Yukitoshi Ishikawa, Shuji Wakai, Nobutada Tachi, Shunz ...
1993 Volume 25 Issue 1 Pages
70-75
Published: January 01, 1993
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We report a 19-year-old girl with multiple sclerosis (MS) who had chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). At the age of 9 (1981), she was diagnosed as having Devic disease, including optic neuritis and transverse myelitis. For two years after the onset of the disease, she suffered from three relapses of MS and was treated with prednisone. At the age of 11, she noticed muscle weakness and sensory disturbance of four limbs. On examination, all tendon reflexes were absent, CSF protein was elevated, and motor nerve conduction velocity of the ulnar nerves was markedly slowed. Sural nerve biopsy showed decreased density of myelinated fibers and de-re-myelinated fibers with onion-bulb formations. Between 11 and 19 years, she had a number of episodes of the central nervous system (CNS) and peripheral nervous system (PNS) deficits with partial or complete recovery by oral administration of prednisone. The clinical course and laboratory data are consistent with MS associated with CIDP. Such our case of MS with CIDP in childhood is very rare. Patients with MS and CIDP may represent a subpopulation who had a common pathogenetic factor for both CNS and PNS demyelination.
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Makoto Nabetani, Shuichi Tsuneishi, Masaaki Kugo, Hideki Konishi, Take ...
1993 Volume 25 Issue 1 Pages
76-80
Published: January 01, 1993
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A 2-month- old girl had focal tonic convulsions. Brain CT showed no abnormalities on admission. Three weeks later she got a severe epileptic status. T1-weighted MRI demonstrated low intensity areas in the right occipital and left frontal regions, and enhanced CT demonstrated low density areas in the same region. An increase of CMV antibody titer in serum suspected CMV infection associated with brain infarction. Then γ-globulin was given in addition to PB, CBZ, and VPA, resulting in supression of seizures. At 9 months of age, she had right tonic herniconvulsions. An increase of CMV IgM antibody titer showed reactivation of CMV infection. Acyclovir and γ-globulin were given, and her seizures were controlled. However, she showed progressive motor disability with spastic muscle tonus. CT and MRI showed a severe progressive atrophy of the cerebrum and brain stem.
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Akihiko Tateno, Keiichiro Morisawa, Niroku Koya, Yoshimi Fujioka, Ken ...
1993 Volume 25 Issue 1 Pages
81-86
Published: January 01, 1993
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This report described a patient with degenerative type of progressive myoclonus epilepsy (PME), who showed slowly progressive deterioration of the central nervous system; intellectual impairment, dysarthria, and involuntary movements, particularly action myoclonus and dystonia.
The patient was a 19-year-old woman who had no hereditary factors. At the age of 4, she developed action myoclonus in the upper limbs bilaterally. Her condition became gradually worse, and at the age of 15, she was admitted to our hospital because of involuntary movement in the upper limbs. First physical examination revealed mild mental retardation, action myoclonus, dystonia, and delayed adolescence. As giant SEP characteristic of PME and Ramsay Hunt syndrome was found, she was tentatively diagnosed as having Ramsay Hunt syndrome without epilepsy, and delayed adolescence.
Now, she is 19 years old, and unable to walk alone because of involuntary movements and paralysis. But she has not developed epilepsy. As she has not been compatible with progressive myoclonus epilepsy (PME) and progressive myoclonic ataxia (PMA) classified by Marseille Consensus Group, she has been diagnosed as having an atypical PME syndrome.
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[in Japanese], [in Japanese]
1993 Volume 25 Issue 1 Pages
88-90
Published: January 01, 1993
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[in Japanese], [in Japanese]
1993 Volume 25 Issue 1 Pages
90-92
Published: January 01, 1993
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[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...
1993 Volume 25 Issue 1 Pages
92-94
Published: January 01, 1993
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[in Japanese], [in Japanese]
1993 Volume 25 Issue 1 Pages
95
Published: January 01, 1993
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[in Japanese], [in Japanese]
1993 Volume 25 Issue 1 Pages
97-98
Published: January 01, 1993
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