NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
Volume 15, Issue 6
Displaying 1-9 of 9 articles from this issue
  • [in Japanese]
    1983 Volume 15 Issue 6 Pages 466
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Download PDF (127K)
  • Keiko Shishikura, Ryoji Umezu, Masayuki Aoyama, Yoshito Hirayama, Haru ...
    1983 Volume 15 Issue 6 Pages 467-477
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Abnormal polysaccharide accumulation was found in the muscle biopsied from a patient of progressive myoclonus epilepsy (PME) (Lafora body form), whose elder brother had died at the age of 16 years and diagnosed as PME (Lafora body form) by autopsy. Muscle biopsy was performed at the age of 13 and 18 years. Light microscopy of the muscle revealed PAS positive stippling and type grouping, while electron micloscopy revealed only an increase of glycogen granules. The second muscle biopsy revealed abundant fibers strongly positive for PAS and phosphorylase activities. Acid phosphatase and DAB peroxidase activitise were negative. In an electron microscopic study, abnormal accumulation of small particles which were demonstrated as polysaccharides because of positive reaction for the methenamine silver stain was found. These particles were finer and smaller than normal glycogen particles, some of which formed a beads like arrangement. No distinct filamentous material like the Lafora body was found. Liver biopsy was also performed at the age of 13 years which showed strongly PAS positive cells by the light microscopy and an increase of glycogen granules and dilatation of smooth endoplasmic reticulum by the electron microscopy. These results might suggest the possibility that PME (Lafora body form) is a systemic metabolic disorder with an error of glycogen synthesis.
    Download PDF (24238K)
  • Hitoshi Onodera, Goro Takada, Keiya Tada
    1983 Volume 15 Issue 6 Pages 478-486
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The localization of intravenously injected liposome-entrapped or unentrapped 3H-β-galactosidase in various tissues of rats was studied by microautoradiography. The appearance of microautoradiographic silver grains, that is, the uptake of the 3H-enzyme was observed in all of the tissues studied, such as the liver, kidneys, spleen, lungs, heart, muscle and brain from both groups of rats. The most active organ in taking up both forms of the 3H-enzyme was the liver, where Kupffer cells were supposed to be most responsivle for it. The uptake of both forms of the enzyme was also observed in the brain, which indicate that the administered enzyme could pass through the blood-brain barrier. Moreover, the uptake of the 3H-enzyme in the brain was revealed to be greater in the gray matter than in the white matter. The possibility of the enzyme replacement therapy towards the lysosomal storage diseases involving the central nervous system was suggested.
    Download PDF (13999K)
  • Tadashi Tsuchida, Mitsunori Fukuda, Akimichi Ichikawa, Ryuichi Tanaka
    1983 Volume 15 Issue 6 Pages 487-496
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The long-term results of 129 pediatric patients with hydrocephalus treated at the Department of Neurosurgery of Niigata University Hospital between 1960 and 1976 were studied according to the causes and severity of hydrocephalus. The following diagnostic categories were used:(1) congenital simple hydrocephalus (36 cases);(2) hydrocephalus with dysraphism (41 cases);(3) complicated hydrocephalus with porencephaly, arachnoid cyst or hydroencephalodysplasia (7 cases);(4) postmeningitic hydrocephalus (32 cases);(5) hydrocephalus due to aqueduct stenosis (3 cases).
    Fifty- eight patients survived, 53 were dead, and the remaining 18 were missed during the follow-up period. Overall mortality was 41.1%. Life expectancy was the worst in group 3 and the best in group 5. Mental and physical development of the survivals was the worst in group 4. Two thirds of living patients except group 4 were found to be leading useful lives with educable mental and fairly good physical states. Mental state was well related to the ultimate thickness of the frontal cerebral mantle. Thirteen of 15 patients of slight hydrocephalus whose cerebral mantles measured more than 3 cm, 20 of 30 moderate hydrocephalus of 1 cm to 3 cm, and 1 of 12 severe hydrocephalus less than 1 cm were in educable mental state, respectively. Seven of 17 patients survived for more than 15 years after initial treatments were employed.
    Download PDF (3756K)
  • Kazuie Iinuma, Kuniaki Narisawa, Hiraku Tateda, Kikuo Suzuki, Keiya Ta ...
    1983 Volume 15 Issue 6 Pages 497-502
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Serial EEG examinations of a schoolboy with phenylketonuria (PKU) were studied. The patient had been treated with a low phenylalanine (phe) diet since 3 months of age. The serum level of phe had been kept between 10 and 20 mg/dl. All his EEGs were normal before school age. After the age of 7, his serum phe level increased to over 20 mg/dl due to his eating a school lunch. Three months after he started having school lunches, his EEG showed localized spikes in the left mid-temporal and central regions, but the waking background consisted of symmetrical, regular and reactive 10.3 Hz alpha activity. After his phe intake was once more restricted, the phe serum level dropped to below 20 mg/dl. Three months after the restricted diet was reinstituted, the EEG showed no epileptiform activities and 10.3 Hz alpha activity in the waking background remained. The patient has never had convulsive attacks, even while the EEG was abnormal. But he was restless and unable to concentrate when the phe level was high.
    In this patient, the EEG did not show background abnormality, but was epileptogenic. Epileptogenic change in EEG may relate to high level of serum phe, because it disappeared after reinstitution of the dietary treatment. A level of 20 mg/d/ of serum phe seems to be critical in this case in terms of EEG abnormality.
    The age of termination of dietary treatment of PKU is generally suggested to be about 4 to 6 years, but even after 6 years of age it should be carefully considered whether dietary treatment should be discontinued or not. The serial EEG observations seem to be helpful in deciding on discontinuation of the treatment.
    Download PDF (757K)
  • Their Development in Normal Infants
    Hidetoshi Tachibana
    1983 Volume 15 Issue 6 Pages 503-506
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    The rolling-over movement of the body of an infant from the supine to abdominal position in infants involves a number of righting reflexes, especially those of the head. Therefore, the development of the righting reflexes acting on the head was studied in relation with that of rolling-over of the body in 33 normal infants.The infants were seen once every month from 1 month through 12 months of age, the developmental history including that of rolling-over was taken, and the righting reflexes acting on the head were examined. The infant to be examined was held at bilateral axillae with the hands of the examiner while either 1) at the vertically suspended position or 2) at the sitting position. The body of the infant was inclined 30° to either side.
    The righting reflex acting on the head was judged positive when the head was brought to the upright position while the body was inclined. The righting reflex acting on the head on suspension became positive at 3 to 8 months of age, coinciding with rolling-over of the body. The righting reflex acting on the head at the sitting position became positive a few months earlier.
    Five infants who developed one-sided righting reflex acting on the head on suspension rolled over to the opposite side. When they acquired righting reflex acting on the head on both sides a few months later, they also could roll over on both sides.
    These findings indicate that rolling-over of the body is closely related to the righting reflex acting on the head on suspension, and that one-sided rolling-over results from asymmetrical development of the labyrinthine or optical righting reflex.
    Download PDF (424K)
  • Kazuyo Kuzume, Hideo Nagao, Mitsugi Takahashi, Takehiko Morimoto, Nozo ...
    1983 Volume 15 Issue 6 Pages 507-511
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    Pseudoachondroplasia is a short-limbed dwarfism with marked ligamentous laxity. Dwarfism is not recognized until after the second year of life, and the body proportion resembles achondroplasia. However, the head and face are normal. In some patients cervical myelopathy caused by atlanto-axial dislocation is revealed.
    A 14-year-old boy with pseudoachondroplasia associated with chronic cervical cord myelopathy was reported. He was admitted to Ehime University Hospital for evaluation and treatment of short-limbed dwarfism, complete paraplegia and respiratory failure. Roentgenographically, fragmentation and irregular changes of the epiphyses in the extremities, hypoplasia of vertebrae and odontoid process, and atlanto-axial dislocation were seen.
    Surgical treatment of atlanto-axial dislocation was performed. However, no improvement of neurologicaland respiratory signs was obtained.
    This report indicates the importance of early diagnosis and treatment of atlanto-axial dislocation.
    Download PDF (5107K)
  • Hiroki Takakura, Shigeto Kasagi, Sachio Takashima
    1983 Volume 15 Issue 6 Pages 512-518
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A 11-month-old boy was a product of the first pregnancy of a 40-year-old mother who had used a betamethasone, bronchodilators and antihistaminics during pregnancy. His birth weight was 2, 280 g. There was not birth asphyxia. He smiled and controlled the head at 3 months of age. Internal strabismus and visual impairment were noticed at 4 months. He creeped on the prone position at 8 months. There were no epileptic fits.
    On neurological examination at 11 months, there were abnormal eyeball movements and spastic paraplegia. There were no paresis of upper extremities. He could vocalize sound, but no intelligible words. Parachute reflex was not present.
    Plain CT showed symmetrical, large and well-defined low density areas in the parieto-temporal regions which appeared to communicate with subarachnoid space at the occipital horn. Defect of septum pellucidum was also demonstrated.
    EEG showed localized irregular polyspike and wave complexes independently in the bilateral parietooccipital areas. Visual evoked potential was not elicited. Auditory brain stem responses showed normal latency of wave V. Short latency somatosensory evoked potentials showed normal latency of each waves except relatively low amplitude of N1 compared with normal controls.
    These electrophysiological findings suggests that clefts transverse the optic radiations and involve a part of somatosensory and somatomotor areas, but the sensory function in the brain stem and thalamus are normal.
    Download PDF (6453K)
  • Naofumi Ichiba
    1983 Volume 15 Issue 6 Pages 519-525
    Published: November 01, 1983
    Released on J-STAGE: August 10, 2011
    JOURNAL FREE ACCESS
    A 9-year-old girl was reported, who presented many neuropsychological symptoms including peculiar unilateral spatial agnosia following anoxic encephalopathy due to carbon monoxide poisoning. After recovering from the comatose state which continued 8 days intermittently, she developed unilateral spatial agnosia, agraphia, apraxia, acalculia and right-left disorientation. Among these, the most interesting symptom was unilateral spatial agnosia. Although there were no disturbance of both visual acuity and visual fields, she showed the left-sided spatial agnosia on the reading and right-sided spatial agnosia on the simulation of movement. This report was the first that the side of spatial agnosia changed according to contents of stimulation at the same period. On the examinations of CT scan and EEG, it was suggested a demyelinated change of cerebral white matter which was considerd as a main lesion of carbon monoxide poisoning.
    Considering that the lesion of cerebral white matter includes the corpus callosum in carbon monoxide poisoning, it was suggested that the unilateral spatial agnosia was due to the interhemispheric disconnexion syndrome.
    Download PDF (3970K)
feedback
Top