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Volume 8, Issue 6
Displaying 1-10 of 10 articles from this issue
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Kazuyoshi WATANABE, Kimiko HARA, Katsuhiko IWASE1976 Volume 8 Issue 6 Pages 432-440
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSSerial neurological, electroencephalographic and cerebral evoked response studies were per formed in a case of globoid cell leucodystrophy from 9 months through 6 years of age. The diagnosis was confirmed by a cerebral biopsy and later by an assay of galactocerebroside βA-galactosidase in leucocytes.
Tendon reflexes were rather exaggerated even in the late stage of illness, except for Achilles reflexes which were depressed and later aboli shed.
The results of nerve conduction studies indica ted the presense of a severe sensorimotor poly neuropathy.
There was no optic atrophy even in the late stage.
Tonic reflexes such as tonic neck and tonic labyrinthine reflexes were hyperactive in the stage II of Hagberg.
At 10 months of age, the EEG showed a normal basic rhythm during wakefullness, while sigma rhythms were poorly defined and few in numberduring sleep.
At 14 months of age, the basic rhythm showed a disorganization and slowing, with focal sharp and slow wave discharges in the left central area and localized superimposed fast activity during sleep. Sigma rhythms were absent.
The record showed a further disorganization of the basic rhythm over the next year.
The record taken at 2 years and 3 months disclosed a marked slow wave dysrhythmia with multifocal asynchronous sharp waves or spikes. Over the next 3 years, there was a gradual reduction of the amplitude of slow waves and a decrease of epileptiform discharges, although the record did not become isolectric even at 6 years of age.
Marked superimposed fast activity was seen at 3 years and 2 months and a definite asymmetry was noted at 5 years and 2 months.
Definite visual and auditory evoked responses were observed even at 6 years of age, although there were delayed latencies.View full abstractDownload PDF (1719K) -
Norishige YOSHIKAWA, Motoko MIZOJIRI, Sumiyoshi YOKOYAMA, Yorihiko MOR ...1976 Volume 8 Issue 6 Pages 441-446
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSSeven cases of “moyamoya” disease were studied with regard to sex, age of onset, family and past history, clinical signs and symptoms, carotid angiograms and EEG. A boy was followed up for 6 years with repeated carotid angiography and EEG.
The disease appeared before 5 years of age in most cases. Successive carotid angiograms showed progressive cerebral vascular changes in a case. This fact suggests that congenital vascular abnormalities in the brain may have been aggravated by postnatal factors.
Prolonged slow wave formation in EEG after hyperventilation were of diagnostic value in this disease.
Five cases were diagnosed at the stage 3 of cartotid angiographic changes (Suzuki's classification). Clinical signs and symptoms got worse at the stages 5 and 6. The severity of clinical symptoms seemed to be related to the cerebral blood flow and the extent of the angiographic changes.View full abstractDownload PDF (2927K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1976 Volume 8 Issue 6 Pages 447-453
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSIn the present study the initial therapy for infantile spasms was evaluated. Three groups of patients on different therapeutic methods were compared: group A, on ACTH-Z in a dose of 0.25mg a day, group B, on ketogenic diet in a ratio of 2-3: 1 of Woodyatt index combined with ACTH-Z, and group C, on sodium-dipropylacetate (DPA) in a dose of 30-50 mg/kg a day combined with ACTH-Z. Treatment was continued for 10 to 14 days. Whenobjective disappearance of jerks and hypsarhythmia was observed, the treatment was evaluated as effective. Results were as follows.
1) 19 of 33 cases in group A, 3 of 7 cases in group B and 7 of 8 cases in group C were evaluated as effective.
2) DPA administration with ACTH-Z was evaluated as effective in 3 of 4 cases with subdural effusion or congenital cerebral defects.
3) The subdural-peritoneal shunting operation was evaluated as effective in only 1 of 3 cases of infantile spasms with bilateral subdural effusion.View full abstractDownload PDF (3126K) -
Hisao MIURA, Kimio MINAGAWA, Jiro YAGI, Yuzuru KATO, Tsugio KANEKO1976 Volume 8 Issue 6 Pages 455-462
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSA preliminary study on the plasma concentration of carbamazepine (Tegretol) in children was performed as related with the oral dosage.
Forty children at the pediatric seizure clinic of a university hospital (group A, aged 1-14, mean 8years) and 26 institutionalized both mentally and physically severely handicapped epileptic patients (group B, aged 5-23, mean 13 years) were selectedfor the study.
Plasma levels of carbamazepine, phenobarbital, etc. were determined by the modification of Kupferberg's. GLC methods.
In both groups A and B plasma carbamazepine Concentrations inμg/ml, plotted against dosage in mg/kg/day, showed great individual variation.
However, there was a definite tendency that the patients taking carbamazepine, but not phenobarbital or primidone, showed high plasma level/dosage ratio ofcarbamazepine; while the carbamazepine plasma level, or plasma level/dosage ratio, was low in those on both carbamazepine and barbiturates, especially in those with high phenobarbital concentration above potentially toxic level.
These probable drug interaction may have resulted in the differences of calculated values in both groups shown below.
In group A, plasma levels (3.58±2.43μg/ml) to dosage (12.5±3.27mg/kg/day) ratios of carbamazepine were 0.28±0.18; and group B presented 1.83±1.38μg/ml, 13.10±7.19 mg/kg/day and 0.15±0.11, respectively.View full abstractDownload PDF (1071K) -
Akira HORI1976 Volume 8 Issue 6 Pages 463-468
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSFetal external granular layer cells were observed in human embryos, fetuses and neonates and were discussed from the view point of the developmental pathology. They appear physiologically in the 3rd-4th month of gestation and disappear rapidly after the 8th month till birth, while they remain in certain sites such as “carrefour insulo-temporo-hippocampique” during the neonatal period. They seem to differentiate into astrocytes, which come to participate with “membrana limitans gliaesuperficialis.” In certain instances they appeared in the ectopic glial nests in “carrefour ” as abberations, where they might differentiate into ganglion cells.View full abstractDownload PDF (5686K) -
Keiichi MOROOKA, Hironori MAKINO, Masakazu IKEDA, Mitsuyoshi TAKATA, K ...1976 Volume 8 Issue 6 Pages 469-480
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSNeurological, neuro-ophthalmologic and neurootologic studies were performed on sixty-eight institutionalized children with cerebral palsy. Convergent squint was noted in 6 patients, 4 of them were born with low birth weight.
Disturbance of upward gaze was seen in 20% of the athetotic type patients, but only one had both upward and downward gaze palsy. Spontaneous and gaze nystagmus was found more often in the spastic type patients.
The incidence of non-gaze nystagmus under Frenzel's spectacles was 28% and was not different between the spastic and athetotic types. Electronystagmograph (ENG) recording showed spontaneous nystagmus in 83% of the patients, which was more common in the spastic type, while gaze nystagmus was predominant in the athetotic type.
The optokinetic nystagmus and the eye tracking test (ETT) were abnormal in the majority of the patient with athetotic movements.
The observation by the naked-eye detected almost the same number of the patients with the optokinetic nystagmus as compared to that by the ENG, but ENG revealed much more ETT changes.View full abstractDownload PDF (1828K) -
A Case of Peculiar Progressive Neurogenic Muscular Atrophy Accompanied with Various Associated SignsNoboru KAWANO, Naoko MIYAUCHI, Junko HARADA, Yukio FUKUYAMA1976 Volume 8 Issue 6 Pages 481-491
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSA fourteen-year-old boy suffered from distal dominant neurogenic muscle atrophy since his eight years old of age. In addition to the rarity of distal dominant chronic spinal muscle atrophy in this young age period, a variety of associated symptoms were observed such as muscle cramps, localized muscle bulbs (“boule musculaire”), slight sensory disturbances, marked growth failure and emaciation, intractable diarrhea, pig mentation of skin and gingiva, delayed maturation of secondary sexual characters, and mental retardation.
Muscle cramps and bulbs are rarely described in a very limited number of cases with progressive muscular dystrophy and spinal muscle atrophy.
The literatures of the description of cases showing these and other related phenomena such as myotonia, muscle contracture, stiffness, or spasms was extensively reviewed, and diagnostic and pathogenetic implications of these signs were discussed.View full abstractDownload PDF (8663K) -
Hitoshi OGINO, Sumiyoshi YOKOYAMA, Soichi KODAMA, Kunihiko OSAKA, Nori ...1976 Volume 8 Issue 6 Pages 492-501
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSTwo male infants with systemic tortuosity and lengthening of the arteries were reported. Both cases had convulsive seizures and psychomotor retardation.
Clinical analysis of these cases was performed.
1) Cerebral angiogram and abdominal aortogram revealed systemic tortuosity and lengthening of the arteries.
2) Serum copper and ceruloplasmin were found to be extremely low.
3) Biopsy of the artery done in the first cases failed to show any marked abnormality. Clinical analysis, signs and symptoms of these cases resembled those of kinky hair disease, but these cases had no kinky hair, metaphyseal supurring, or osteoporosis.
Syphilis, arteriosclerosis, hypertension, mucopolysaccharidosis, and amino acid metabolism disorders were ruled out.
Pathophysiology of arterial abnormality (hemo dynamic), signs and symptoms of central nervous system (biochemical) and their relationship to biometal (copper, zinc, molybdenum, iron) metabolism were discussed by review of literatures.View full abstractDownload PDF (7860K) -
Kihei Maekawa1976 Volume 8 Issue 6 Pages 503-505
Published: November 01, 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSDownload PDF (476K) -
1976 Volume 8 Issue 6 Pages 511
Published: 1976
Released on J-STAGE: May 24, 2011
JOURNAL FREE ACCESSDownload PDF (40K)
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