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Volume 23, Issue 1
Displaying 1-23 of 23 articles from this issue
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[in Japanese]1991 Volume 23 Issue 1 Pages 3
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (140K) -
Hisaharu Suzuki, Masao Aihara, Kenji Sugai1991 Volume 23 Issue 1 Pages 4-8
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe studied all severely retarded children in a defined area of Japan. The area is a small and rural prefecture with the total population of 880, 000. The population of children aged 6-14 years was 102.000. The minibe r of severely retarded children (those who neither walk nor have IQs over 35) was 50. The prevalence rate was 0.49/1, 000 and its 95% confidence interval was 0.36-0.65. The rate was significantly lower than that has been reported 20 years ago from another prefecture in Japan. Among the cases, children with very severe retardation (those who are bedridden with their IQs less than 20) accounted for 66% and children who were institutionalized for permanent care accounted for 46%. Association of several disabilities was as follows: cerebral palsy 62%, epilepsy 82%, decreased visual acuity 42%, respiratory distress 16%, and feeding difficulty 32%. The causes of prenatal origin accounted for about two -thirds of the total causes .
We concluded that the prevalence rate of severely retarded children is b ecoming lower in Japan and that the prenatal factors play an important role in the pathogenesis of severe retardView full abstractDownload PDF (665K) -
Makiko Kaga, Hisaharu Suzuki, Sui Sone, Kimitaka Kaga, Atsushi Araki, ...1991 Volume 23 Issue 1 Pages 9-14
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSNine patients ranged between 5 months and 35 years (means 14 years) of age with multiple and severs disabilities were comparatively studied, on extratympanic recorded compound action potential (AP) of elec. trocochleography and auditory brainstem response (ABR). Both ABR and AP were obvious in 3 patients with clear auditory behavioral responses. The ABR was not recorded to high intensity click stimuli in six patients without visible auditory behavioral responses. Four of the 6 cases showed undetectable AP and the 2 rest of the 6 cases showed abnormal but discernible AP. In two cases with history of the anoxic encephalopathy, AP was recorded but ABR was not detected in one case, and in the other, neither AP nor ABR was recorded. At least two different levels of lesions could be differentiated in cases without detectable ABR by using extratympanic recorded cochleography in these kinds of severely handicapped patientsView full abstractDownload PDF (593K) -
Shinji Yamada, Yutaka Tomita1991 Volume 23 Issue 1 Pages 15-19
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe studied juvenile ocular myasthenia gravis (MG) with special reference to evoked EMG in orbicularis oculi muscle. The subjects consisted of 5 cases aged 1-4 years and 3 cases over 10 years. Young children were examined during drug-induced sleep. Stimulation was delivered to preauricular facial nerve, and M waves were recorded from ipsilateral orbicularis oculi muscle. Examinations were also carried out in thenar or hypothenar muscles by stimulating the median or ulnar nerves respectively.
On Harvey-Masland test, 6 out of 8 cases showed waning of more than 10%. On M-wave recovery cycle, 3 out of 6 cases examined showed decrement during stimulation interval of 100 msec to 500 msec. In 2 of these, the peak of recovery cycle curve rose over 100 % during stimulation interval of 30 to 60 msec on a usual dose treatment of anti-cholinesterase, while on prednisolone treatment this peak did not increase over 100% and the pattern of recovery cycle changed to almost normal. On post-tetanic cycle study, only 1 out of 4 cases examined showed post-tetanic facilitation and exhaustion. The response of the belly-tendon in the hand showed no abnormality.
Although there still remains technical difficulty in the examination of evoked EMG in orbicularis oculi muscle, this examination is very useful in diagnosis, evaluation and understanding of pathophysiology of MG.View full abstractDownload PDF (635K) -
Seiji Kimura1991 Volume 23 Issue 1 Pages 20-25
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSUltrastructural pathology on sweat gland epithelium was studied in various neurodegenerative disorders ; neuronal ceroid-lipofuscinosis (NCL), Lafora disease, mucopolysaccharaidosis, GM1 gangliosidosis, Nieman- Pick disease, Fabry disease, Krabbe disease and metachromatic leukodystrophy (MLD). Every disease had its own chracteristic inclusions in sweat galnd epithelium. Curvilinear profiles and fingerprint patterns were seen in NCL, but there were no morphological differences among late infantile, early juvenile and juvenile types. On the other hand, the granular matrix was characteristic of the infantile type. The presence of specific inclusions in a 23-year-old female carrier with Fabry disease indicated that a skin biopsy was one of the useful methods to detect a female carrier. In MLD and Krabbe disease, there were disease specific inclusions in sweat gland epithelium. These results indicate that the sweat glands should be investigated when a skin biopsy is performed for the diagnosis of neurodegenerative diseases.View full abstractDownload PDF (7481K) -
Chieko Shimomura, Tetsuo Matsuzaka, Eiichiro Koide, Setsuko Kinoshita, ...1991 Volume 23 Issue 1 Pages 26-31
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe heart rate variability, modulated by autonomic nervous system, has been reported to decrease in depression of the central nervous system, especially of the brainstem. To assess the brainstem dysfunction, we analyzed R-R interval values (the intervals between R waves of ECG) in 9 children (7 in comatose children and 2 in central apnea) by spectral analysis using an autoregressive model. The findings of spectral analysis were compared with those of auditory brainstem response (ABR). In comatose children with the brainstem dysfunction, the reduction of the total power was apparent before the appearance of ABR abnormality. In central apnea due to the dysfunction of respiratory center in brainstem, only the respiratory component decreased without the reduction of the total power or ABR abnormality. Spectral analysis of the heart rate variability is a useful means for assessment of the brainstem function.View full abstractDownload PDF (688K) -
Valproate Induced Hyperammonemia and Hypocarnitinemia-Kiyoshi Matsui, Hiroko Iwamoto, Noriyuki Ohtsuki, Takuya Kobayashi, Sh ...1991 Volume 23 Issue 1 Pages 32-38
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSBlood ammonia and serum free carnitine were measured in 49 severely handicapped epileptic patients treated with or without valproate. DL-or L-carnitine were administered to patients treated with valproate, and the effects of carnitine supplemention were evaluated. Furthermore we analyzed the relationship between serum free carnitine and nutrition.
In patients treated with valproate, blood ammonia statistically increased, and serum free carnitine concentration statistically decreased. Free carnitine was low in tube-fed patients, as compared with that in oralfed patients. Carnitine therapy was successful in improvement of hyperammonemia and hypocarnitinemia.
It is concluded that hypocarnitinemia was caused not only by valproate therapy, but also by tube-feeding. Carnitine supplementation therapy is important to both hyperammonemia and hypocarnitinemia. But the long term effect of carnitine therapy remains to be studied further.View full abstractDownload PDF (683K) -
Serial Axial and Sagittal Section Studies -Hideo Nagao, Takehiko Morimoto, Nozomi Sano, Mitsugi Takahashi, Hirona ...1991 Volume 23 Issue 1 Pages 39-43
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMagnetic resonance imaging of skeletal muscles in thirteen patients with Duchenne muscular dystrophy was performed to estimate pathological changes. Serial axial and sagittal sections of the right lower extremity were recorded.
In the early stage, the Ti values of gastrocnemius and soleus muscles were slightly lower than the control values, and in the late stage, the values were much lower in all muscles examined. In sagittal sections, the gastrocnemius muscle in the early stage showed a high density area at the distal region adjacent to soleus muscle, and the soleus muscle showed a high density area adjacent to the gestrocnemius muscle. In serial axial sections, high density areas of the anterior and posterior tibialis muscles appeared first at their proximal and peripheral regions.
It was concluded that the sequence of appearance of pathological changes was different not only amongindividual muscles but also among various regions of each muscle ; the high density changes appeared first at myotendon junctions.View full abstractDownload PDF (3345K) -
Takeo Kubota, Masaru Tatsuno1991 Volume 23 Issue 1 Pages 44-49
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSFlow velocities in the anterior cerebral artery (ACA) and the internal cerebral vein (ICV) were studied by a Doppler ultrasound technique. A longitudinal study was undertaken on 59 newborn infants during the first month of life. The newborn infants were classified into 3 groups: 22 term infants, 21 preterm infants, and 16 very low birth weight infants.
A rapid linear increase of flow velocities was found in the ACA in the first month of life with higher velocities in neonates of higher birth weight. The index of resistance was not significantly different among the 3 groups. A linear increase of flow velocities was also found in the ICV in the same period. The velocities in the ICV were correlated with those in the ACA.
These data suggest that the increasing velocities probably reflect the increasing cerebral blood flow during the first month of life.View full abstractDownload PDF (2069K) -
Masahiko Kimura, Masako Kato, Kunio Yoshino1991 Volume 23 Issue 1 Pages 50-56
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThyriod function tests were studied in 105 severely disabled children. Fifty- four cases (53%) showed following abnormalities. Serum TSH concentration was increased in 2 cases. Both serum T4 and T3 levels were decreased in 18 cases (17%). Only the serum T4 level was decreased in 25 cases (24%). Only the serum T3 level was decreased in 10 cases (9.5%).Two patients showed primary hypothyroidism. Two patients (monozygotic twins) were suspected to have subclinical hypothalmic- pituitary hypothyroidism caused by septo- optic- dysplasia. Abnormal thyroid functions were caused by anticonvulsants in most patients. The serum T4 level was correlated with the number of anticonvulsants, but not correlated with motor performance (daily activity) or feeding function. Four patients who had low T4 level or low T4 and T3 levels were received L-thyroxine supplementation, but no cli- nical improvement was observed. The serum TSH concentration was decreased and the TRH test showedh yporeaction in all of these cases. The low T3 level and normal T4 level were not related to anticonvulsant administration. Two of these patients were in poor nutritional condition, resulting in so called low T3 syn-d romView full abstractDownload PDF (734K) -
Shinichiro Hamano, Takahiro Nara, Hidetsugu Nozaki, Kiyomi Fukushima, ...1991 Volume 23 Issue 1 Pages 58-64
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSCrossed cerebellar diaschisis (CCD) in twenty five children with hemiplegia were studied using single photon emission computed tomography (SPECT) with N- isopropyl- p-I-123- iocloamphetamine. Seven of twenty-five patients had cerebral palsy, and the others were impaired by acquired brain injury between ten months and fourteen years of age. CCD was demonstrated in five patients (20%), who were impaired by acquired brain injury after seven years of age. CCD could never be detected in patients with cerebral palsy. Ipsilateral cerebellar diaschisis was also demonstrated in two patients with cerebral palsy and three with ear-ly acquired brain injury before three years of age.
It is suggested that diaschisis presents itself as a different form in a contralateral and ipsilateral cerebellum before three years of age from a form which presents after seven years of age.View full abstractDownload PDF (9129K) -
Masahito Miyazaki, Takahiko Saijo, Kenji Mori, Masanobu Tayama, Etuo N ...1991 Volume 23 Issue 1 Pages 65-70
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe report a 14-year-old boy with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) who presented repeated episodes of abdominal pain and vomiting since the age of 8 years. In addition, he developed strokelike episodes with myoclonic seizures and transient hemiplegia on three occasions. At the age of 14-1/12-years, he also developed epilepsia partialis continua persisting for 10 days, which was associated with myoclonic seizures synchronized with spike discharges at the right central area. Laboratory examination disclosed increased levels of lactate and pyruvate in serum and CSF and low density areas in the bilateral temporal regions on CT scan. Muscle biopsy showed scattered ragged- red fibers. The enzyme activities (pyruvate dehydrogenase complex, pyruvate carboxylase, phosphoenol pyruvate carboxykinase, and cytochrome c oxidase) and the rates of decarboxylation of [3-14C] pyruvate in cultured skin fibroblasts were within normal ranges.View full abstractDownload PDF (3342K) -
Akashi Ishikawa, Takako Okayasu, Kazuo Miyasaka, Naoki Fukushima, Aiko ...1991 Volume 23 Issue 1 Pages 71-74
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a mild variant case of maple syrup urine disease. He was unable to walk alone at 2 years of age and developed seizures and intermittent ataxia at 5 years of age. Activity of 1-14C-leucine decarboxylase in fibroblasts revealed 40% of normal activity in the boy and 90% in the mother. MRI showed hypomyelination of white matter and mild atrophy of brain stem and cerebellum. Dietary treatment was not effective for ataxia and brain atrophy.View full abstractDownload PDF (3528K) -
Kazuo Hatakeyama, Masao Aihara, Akira Shimizu, Norihiko Uchida, Takesh ...1991 Volume 23 Issue 1 Pages 76-80
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSHigher cerebral dysfunctions such as aphasia, apraxia and agnosia have seldom been reported in multiple sclerosis (MS). 12 year-old right-handed boy felt unsteadiness of the body and headache for several days. Two months later, he had the same episode and complained of visual disturbance, and weakness and sensory disturbance on the face and the extremities. Additionally, he showed amnestic aphasia, acalculia, ideomotor apraxia, finger agnosia and right-left disorientation. Cerebrospinal fluid examinations revealed increases IgG, myelin basic protein and neuron specific enolase (11%, 25 ng/m/ and 28.8 ng/m/, respectively). X-ray CT scan and MRI-CT examinations revealed sclerotic lesions on the left parietal white matter and the right midbrain. The diagnosis was made as MS. He was treated with m-PSL (methyl-prednisolone) pulse therapy for three weeks and consecutively treated with PSL for four weeks. He recovered gradually, but visual disturbance and facial palsy remained. After seven months MRI-CT showed a high signal intensity on the left parietal white matter in spite of the disappearance of the lesion on X-ray CT scan.
We suggest that these higher cerebral dysfunctions may result from the lesion of the left parietal white matter which produces a disconnection between each cortical area.View full abstractDownload PDF (4397K) -
Yukitoshi Ishikawa, Keiji Kameda, Minoru Okabe, Tomihiro Imai, Masato ...1991 Volume 23 Issue 1 Pages 81-86
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 9-year-old girl with type I hyperprolinemia, who also had photogenic epilepsy, was reported. She showed epileptic discharges and the regression in speech and motor activities, since 7 years of age. Her plasma proline levels were 3 to 4 times higher than control levels. In urine, iminoglycinuria appeared, when plasma proline value exceeded 0.80 mM. The proline oxidase activity of the liver tissues obtained by biopsy in the patient was about 23.5%, compared to that of controls. In spite of the restriction of proline and protein intake, she showed progressive speech and motor retardation.View full abstractDownload PDF (627K) -
Masako Watanabe, Tateki Fujiwara, Hitoshi Nakamura, Tateki Morikawa, K ...1991 Volume 23 Issue 1 Pages 87-92
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported 3 cases with subacute sclerosing panencephalitis (SSPE) showing recurrent and involuntary motor phenomena. Their electroclinical correlates were analyzed using CCTV-EEG.
Clinically, they slanted their trunks and/or heads, and dropped their upper limbs momentarily only when their antigravity muscles were suitably contracting (sitting, standing, or stretching out their arms). Electrographically, the motor phenomena corresponded with the occurrence of periodic synchronous discharges (PSD) (in one-to-one manner). EMG potentials associated with the muscle contraction subsided at the very moment. These motor phenomena observed in patients with SSPE were discussed in relation to asterexis.View full abstractDownload PDF (4303K) -
Hisaharu Suzuki, Yoshito Hirayama, Satoru Hirano, Ritsuko Takahashi, I ...1991 Volume 23 Issue 1 Pages 93-97
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 13-year-old girl who had severe brain damage due to unknown prenatal cause presented rhabdomyolysis triggered by a mild viral infection. Her muscle biopsy revealed mild variation in fiber size and type 2 fiber atrophy without excess lipid storage. Biochemical analysis of the biopsied material showed decreased carnitine palmitoyltransferase (CPT) activity (15% of the control). Serum and urinary carnitine levels were normal. Skeletal muscle CT scanning showed multiple low density spots. The patient was diagnosed as having CPT deficiency. She recovered from rhabdomyolysis without renal failure after a month with conservative therapy. CPT deficiency is usually found in young healthy persons. This is the first case report of CPT deficiency which presented severe psychomotor retardation since neonatal period.View full abstractDownload PDF (5364K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1991 Volume 23 Issue 1 Pages 98-100
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese]1991 Volume 23 Issue 1 Pages 100-102
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]1991 Volume 23 Issue 1 Pages 102-104
Published: January 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 1 Pages 104-106
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese]1991 Volume 23 Issue 1 Pages 106-109
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese]1991 Volume 23 Issue 1 Pages 110-111
Published: January 01, 1991
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (276K)
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