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Volume 23, Issue 4
Displaying 1-19 of 19 articles from this issue
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Shota Miyake, Miyabi Tanaka, Kiyoshi Matsui, Tazuko Miyagawa, Sumimasa ...1991 Volume 23 Issue 4 Pages 329-335
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTwo hundred and thirty-seven children have died who were seen in the Division of Neurology, Kanagawa Children's Medical Center from 1975 to 1989. They corresponded to about 11 % of the total death in the hospital.
A total of 128 cases of 237 deaths (54.0%) were suffering from epilepsies, most of whom were also complicated with other CNS disorders. The causes of death were pneumonia, respiratory failure and suffocation in 60%, whereas status epilepticus or convulsions only in 14 %. About 40% of victims died at home orin an emergency room without effective resuscitation.
Mortality was considered high in infantile spasms (16 out of 146, 11%) and in severe myoclonic epilepsy in infancy (4 out of 8, 50%). It was note worthy that 3 cases of severe myoclonic epilepsy in infancy died of status epilepticus and 2 died at home.
Sixteen cases whose chief problems were partial epilepsies and severe myoclonic epilepsy in infancy, died of status epilepticus in 10 cases, convulsions after tonsillectomy, etc. Causes of death of these16 cases were related to convulsions except in two cases whose causes were pneumonia in one and unknown in the other. About a half of them (7 cases) died at home.
Adequate managements appeared necessary to prevent accidental deaths of children with epilepsies.View full abstractDownload PDF (812K) -
Evaluation by MRIKazuko Noma, Hiroshi Tamai, Seiichi Shimada, Masahisa Funato1991 Volume 23 Issue 4 Pages 336-341
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe prognostic significance of cerebral myelination was evaluated with magnetic resonance imaging (MRI) in very low birth weight infants. Myelination was graded in two specified sites, optic radiation and corpus callosum, based on the stages of normal term babies and healthy premature infants. The subjects were 30 preterm infants weighing less than 1, 500 gm at birth. MRI was performed at 4 to 7 months (corrected age). The normal myelination stage was seen in 18 cases, while a delayed stage was noticed in 12 cases. In the normal myelination group, only 1 case (6%) had handicaps. In the delayed myelination group, 8 cases (67%) had handicaps. Our results showed that delayed myelination was closely related to a poor prognosis. We believe that MRI would be a very good imaging modality for predicting the outcome of very low birth weight infants, particularly in terms of evaluation of myelination.View full abstractDownload PDF (6310K) -
Mariko Yuge, Toyoko Kanda, Yuriko Yamori, Hiroshi Fukase1991 Volume 23 Issue 4 Pages 342-347
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe studied the correlation between motor developmental processes and prognosis of locomotion at the age of 4 years, among 15 spastic diplegic children (S) and 21 athetotic children (A), who started treatment under 1 year of age. The children of each type were divided into two groups according to the ability of locomotion at the age of 4 years; S-I: 6 walkers, S-II: 9 crawlers, A-I: 11 walkers and A-10 crawlers. We compared the average corrected ages when the subjects attained the abilities to roll over, creep, sit with hands, crawl, sit up, cruise with support, stand up, and walk alone, and made a statistical analysis by t test among 4 groups. Also we compared the cumulative frequency percentages for the ages about the locomotions mentioned above. The S-I group attained all of the abilities of locomotions significantly earlier than the S-II group. The A-I group could crawl and sit up significantly earlier than the S-II group. The A-I group could crawl and sit up significantly earlier than the A-II group. The S-I group began to creep and sit with hands significantly earlier than the A-I group. We found no significant differences between the S-II group and the A- II group in motor developmental processes.
These studies revealed that it would be possible to expect the prognosis of ambulation at the age of 4 years according to the early motor developmental processes among the spastic diplegic children. But it was suspected that it would be difficult to differentiate walkers from crawlers until crawling and sitting up were attained among the athetotic children.View full abstractDownload PDF (706K) -
Akiko Kimura1991 Volume 23 Issue 4 Pages 348-354
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSUsing an ambulatory cassette EEG system (Oxford Medilog 9000), polysomnography was performed in 25 cases of children with epilepsy and 25 normal children. The results revealed decreases in REM sleep and REM density in intractable epilepsy. An increase of slow waves was observed during REM sleep in severe myoclonic epilepsy in infancy or its adjacent group, and this result was more clearly demonstrated by an EEG auto analysis with fast Fourier transform.
This above ambulatory cassette EEG system will be utilized more easily for polysomnography, and contribute to the research not only of the pathophysiology of epilepsy but also of the sleep mechanism itself.View full abstractDownload PDF (741K) -
Ikuyo Eguchi, Masutomo Miyao, Takamichi Yamagata, Hideo Shimoizumi, Sa ...1991 Volume 23 Issue 4 Pages 355-361
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported computed cranial tomography (CCT), magnetic resonance imaging (MRI) and brain echo imaging in Japanese B encephalitis. The result were assessed in comparison with the clinical feature of the disease.
CCT of the three patients showed low density in thalamus and basal ganglia. In two, the lesions were detected in the acute phase, and changed to high density in the chronic phase. Their prognosis was poor, psychomotor delay and paresis persisted. Among the previously reported sixteen patients of Japanese B encephalitis in Japan, the four developed thalamic lesions on CCT and the prognosis was poor in all patients. Brain echo detected the lesions in the acute phase before CCT visualised them clearly.
MRI demonstrated thalamic hemosiderin deposits and calcification. These findings were compatible with the pathological findings, or past hemorrhage and organization. The distribution of the lesion were closely connected with the prognosis.View full abstractDownload PDF (5741K) -
Takashi Hayashi, Takashi Ichiyama, Harumi Tanaka, Hidetoshi Tachibana1991 Volume 23 Issue 4 Pages 362-365
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe rolling-over of the body is one of the developmental mile-stones. Several infants have the laterality of rolling-over in early state and the dominant side in later state. The abilateral occipital flattening is thought to be caused by head positioning in the neonatal period and early infancy. Asymmetrical development of rolling-over was studied in relation with head positioning, relating skull occipital flattening.
We examined 2919 infants aged 7 months with the questionnaire about the side of skull occipital flattening and the initial turning side of rolling-over. 1975 infants had the dominant side of rolling-over; the left side 45.4%, and the right side 47.7%. 761 infants had one-sided occipital flattening, left-side 41.3%, and rightside 54.8%. 520 infants had dominant-sided rolling-over accompanied with one-sided occipital flattening. The positive relationship between the side of rolling-over and the side of occipital flattening could not be proved by chi-squre statistical analysis.
These findings suggest that laterality of rolling-over, one of asymmetrical motor developments is not related to the head positioning in early infancy.View full abstractDownload PDF (442K) -
Nanae Fujimoto, Katsuo Sugita, Hiroo Niimi, Yutaka Takeuchi1991 Volume 23 Issue 4 Pages 366-373
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe investigated two children with cystic periventricular leukomalacia, which we had detected by ultrasonography (US) during the neonatal period, with magnetic resonance imaging (MRI) of the brain at 4, 9, and 15 or 19 corrected months of age. Both inversion-recovery (IR) and T2-weighted spin-echo (SE) sequences were used for this study. We observed cysts involving the periventricular white matter at the region adjacent to bilateral frontal horns in case 1 and adjacent to the posterior horns of the lateral ventricles in case 2. MRI at 4 months showed the irregularity of the ventricular wall and delayed myelination, compared to that of an age-matched control. The T2-weighted SE and IR imaging at 9 months demonstrated abnormally increased signal in the white matter. These findings were more evident at the 3rd MRI examination. The location of end-stage periventricular leukomalacia lesions were consistent with the distribution of cystic lesions seen on US. MRI at sagital sections was useful for the detection of main periventricular leukomalacia lesions. A possibility exists that MRI might be useful in detecting subtle lesions of periventricular leukomalacia in which we cannot find by US.View full abstractDownload PDF (12533K) -
Kiyoshi Matsui, Michiko Yamada, Hiroko Iwamoto, Miyabi Tanaka, Tazuko ...1991 Volume 23 Issue 4 Pages 374-379
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe evalation of four patients with Cockayne syndrome (CS) by computed tomography (CT) and magnetic resonance imaging (MRI) is reported. All patients had characteristic clinical manifestations of CS. In a special respect, we found hyperopia in two patients and previous habitual abortions in two maternal histories. Extrapyramidal signs were seen in one patient. Three patients are type 1 CS (case 1, 3, 4) and one patient (case 2) is type 2 CS (congenital form). The cranial CT in two patients (case 1, 2) revealed prominent calcifications in basal ganglia, dentate nucleus and hemispheric white matter. While CT showed vagal calcifications in basal ganglia in other two patients (case 3, 4), T2-weighted MRI revealed obvious low intensity area in putamen and caudate nucleus, and high intensity area in the white matter. Sagittal section revealed atrophic changes of cerebellar vermis and brain stem. Thus it seems that MRI may be useful diagnostic adjunct in CS patients.View full abstractDownload PDF (5685K) -
Yasuo Sunaga, Takashi Fujinaga, Hiroshi Tamura1991 Volume 23 Issue 4 Pages 380-383
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 4-year-old boy was admitted to our hospital because of dyspnea and muscle-weakness. His developmental milestones were delayed. His face was long with opened mouth. He spoke with nasal voice. He had proximally dominant muscle weakness and his deep tendon reflexes were absent. Laboratory examination revealed normal serum creatine kinase level and a myopathic EMG pattern. Blood gas analysis revealed metabolic acidosis with PH 7.35, PCO2 55.4 mmHg, PO2 62.4 mmHg, BE 3.0, probably from the metabolic acidosis by respiratory muscle weakness. In the biopsied left biceps brachii muscle, there were scattered fibers with nemaline bodies and abnormal fiber type distribution; type 1, 2 A, 2 B and 2 C fibers comprised 7%, 70%, 21% and 2% respectively. Small type 1 fibers and type 1 fiber predominance are the characteristic and common histochemical findings in nemaline myopathy. Accordingly, type 2 fiber predominance in the present patient is a unique, rare phenomenon. The finding might result from a preferential loss of type 1 motoneurons or muscle fiber type transformation from type 1 to type 2 fibers due to a certain abnormal neuronal influence on developing muscles.View full abstractDownload PDF (4561K) -
Toshiyuki Yamamoto, Hitoshi Kawahara, Masumi Inagaki, Shigeru Ohta1991 Volume 23 Issue 4 Pages 384-388
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA two-year-old symptomatic carrier of Duchenne muscular dystro phy (DMD) confirmed by dystrophin immunohistochemical study was reported. She had mild proximal muscular weakness and elevated serum creatine kinase (CK) level. There were no family members of DMD. CT examination revealed low density areas in the muscles similar to that seen in the early stage of DMD. Biopsied specimen of muscle showed myopathic changes with necrotic and regenerating fibers. The immunohistochemical study using an antiserum against dystrophin showed the mosaic expression of the surface membrane, with positive and negative patches. Accordingly, she was strongly suggested to be a DMD carrier. This case shows that dystrophin immunohistochemistry is useful for diagnosis of a DMD carrier without affected family members.View full abstractDownload PDF (5972K) -
Masako Ando, Katsuo Sugita, Michiko Makino, Mana Haraki, Kanshi Minami ...1991 Volume 23 Issue 4 Pages 389-394
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a female infant with early myoclonic encephalopathy (EME). She was diagnosed on the basis of clinical and laboratory features including electroencephalographic and magnetic resonance image (MRI) findings. Frequent erratic myoclonic seizures appeared since 28 days after birth and EEG showed a typical suppression-burst pattern. We administered a high-dose pyridoxal phosphate, thyrotropin-releasing hormone analogue (TRH), and then ACTH, but could not control the seizures at all. With seizure types, we observed the change from erratic myoclonus to tonic spasms in series, with concomitant EEG change to hypsarhythmia at the age of 6 months. Cranial MRI revealed delayed myelination in the white matter but no brain malformation. We administered ACTH to her again and succeeded partially in the decrease of the seizure frequency, and significantly in the improvement of EEG findings. It is supposed that the responsiveness to ACTH treatment changed with age as the seizure patterns developed from erratic myoclonus to tonic spasm.View full abstractDownload PDF (2752K) -
Chieko Akiyama, Hisaharu Suzuki, Ikuya Nonaka1991 Volume 23 Issue 4 Pages 395-399
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe report an 18-year-old female with facioscapulohumeral dystrophy (FSHD), who had sensorineural deafness, retinal vessel abnormality, mental retardation, and epilepsy. She had infantile spasms at 6 months of age. Muscle atrophy and weakness of facial muscles were first noticed at 3 years of age. From 10 years of age, she had rapidly progressive generalized muscle weakness especially of facial, neck and truncal muscles with marked lordosis. Although mental retardation is commonly complicated with FSHD, infantile spasms or epilepsy has never been reported. Not only mental retardation but epilepsy may be one of the central nervous system symptoms in a systemic disorder, FSHD.View full abstractDownload PDF (4428K) -
Kazuhiro Hashimoto, Masako Ando, Yoshikazu Kida, Yutaka Takeuchi1991 Volume 23 Issue 4 Pages 401-404
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe changes of cerebral blood flow velocity (CBFV) and pulsatility index (PI) in anterior cerebral artery were examined by cranial color Doppler ultrasonography while a patient recovered from hypocarbia. As arterial PCO2 was elevated, PI was decreased and color imaging of CBF from basilar artery to anterior cerebral artery were improved in saggital view. Hypocarbia is considered to induce the constriction of the cerebral vessels and the decrease of CBF in premature infants.View full abstractDownload PDF (2889K) -
Junichi Oki, Junichi Itoh, Yuichi Kusunoki, Kazuhiko Cho1991 Volume 23 Issue 4 Pages 405-410
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA two-year-old, right-handed girl was admitted to our hospital with a history of partial seizures. The first seizure, conjugated eye movements and head turning to the right, occurred at the age of 24 months. Later, she suffered from several seizures daily at the age of 34 months. At the age of 35 months, she was admitted to our hospital. On admission her neurological examination was normal. EEG showed a left parietal spike focus. A computed tomographic scan showed a small hyperdense area in the left parietal lobe without contrast enhancement. She was treated with carbamazepine (CBZ: 6 mg/kg/day). When seizures occurred several times within an hour, intravenous or rectal administration of diazepam (DZP: 0.3 mg/kg/dose) was added. However, she complained of sleepiness. The seizures occurred more frequently than before, 50 to 80 times daily, and became secondarily generalized. We thought that the exacerbation of the seizures resulted from the somnolence caused by DZP and CBZ. Consequently, these drugs were discontinued, and phenytoin was begun. She has since been free of seizures for two years. Approximately one year after the discontinuance of DZP and CBZ, heterotopic gray matter and abnormal gyri involving the left parieto-temporal lobe were found by magnetic resonance imaging (MRI). MRI is useful for detecting small heterotopic gray matter. To summarize this case, one should consider the possibility that excessive polytherapy induces seizures, particularly in patients with structural brain abnormalities such as heterotopic gray matter.View full abstractDownload PDF (5116K) -
Mihoko Matsuzaki, Tatsuro Izumi, Keiko Ebato, Haruko Suzuki, Keiko Shi ...1991 Volume 23 Issue 4 Pages 411-416
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes, in which a pituitary growth hormone (GH) secretion deficiency of hypothalamic origin was revealed through neuro-endocrinological examinations, was described.
The case was a 10-year-old girl, who had been suffering from generalized tonic seizures since age 5, four episodes of alternating hemiplegia since age 6, stunted growth since age 7, and simple partial motor seizures as well as gelastic seizures since age 8. Marked elevation of lactate and pyruvate in both serum and CSF, abundant ragged red fibers in biopsied muscles, and low density areas in the left occipital lobe and bilateral globus pallidus in addition to diffuse brain atrophy on CT scan and MRI of the head were demonstrated, although the activities of muscle enzymes complex I-IV were within normal ranges.
Pituitary GH secretion was deficient under the loadings with insulin, L-DOPA, sleep, and a single growth hormone releasing factor (GRF) administration, but normal GH response was registered under the repetitive stimulation with GRF. Activities of other hormonal axes were normal.
It is likely that short stature commonly observed in MELAS patients is due to hypothalamic dysfunction, which might be brought out by chronic ischemia and energy deficiency of the diencephalon based upon mitochondrial abnormality of that region. It is likely that gelastic seizure in this case is due to hypothalamic dysfuction.View full abstractDownload PDF (3446K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1991 Volume 23 Issue 4 Pages 417-419
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese]1991 Volume 23 Issue 4 Pages 419-421
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1991 Volume 23 Issue 4 Pages 421-423
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1991 Volume 23 Issue 4 Pages 424-426
Published: July 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS
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