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Volume 16, Issue 1
Displaying 1-12 of 12 articles from this issue
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[in Japanese]1984 Volume 16 Issue 1 Pages 2-3
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (212K) -
Sachiko Onoe, Jiro Abe, Tetuzo Tagawa, Jiro Ono, Takashi Mimaki, Hyaku ...1984 Volume 16 Issue 1 Pages 4-11
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSAcetylcholine receptor antibody (AchR-Ab) has been recognized to play a vital role in the pathogenesis of myasthenia gravis. In this study, we examined serum AchR-Ab levels and prognosis. Forty-three patients were followed for 7 months to 12.5 years. Patients were grouped by Fukuyama's classification; 36 patients of ocular type, 4 patients of generalized type. and 3 patients of bulbar type. Assay for AchR-Ab was performed as described by Almon (1976) using detergent-extracted acetylcholine receptor from the rat denervated muscle as antigen. Ten patients of ocular type were treated with anticholinergic drugs only.
Most of these patients had serum AchR-Ab levels lower than 0.2 pmol/ml throughout the course of our study and had no ocular symptoms for 0.8 to 7.3 years. AchR-Ab was assayed for 11 in of 36 patients with ocular type before steroid therapy. All these patients showed a reduction in AchR-Ab levels 6 months after the steroid therapy. Six of these 11 patients experienced prolonged asymptomatic periods (1.1 to 5.7 years). Three of 4 patients with generalized type revealed higher levels of AchR-Ab compared with those of ocular type. Their serum AchR-Ab levels once fell down 6 months after the steroid therapy, but their AchR-Ab levels fluctuated during treatment. Three patients of bulbar type showed a reduction in AchR-Ab following the steroid therapy.
Our results suggest that there seems to be a relationship between AchR-Ab levels and clinical severity during drug therapy.View full abstractDownload PDF (1089K) -
Analysis of 21 CasesKyoichi Murayama, Shozo Nakazawa1984 Volume 16 Issue 1 Pages 12-20
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSCerebrovascular diseases have been considered very rare in children, but there has been a gradual increase of the cases detected by CT scans. Twenty-one of 221 in patients of pediatric ages (9.5%) were found to have cerebrovascular diseases. In this survey were excluded the cases with intracranial bleeding caused by vitamin K deficiency and other types of bleeding tendency. The diagnosis of the following diseases was established; arteriovenous malformations (12 cases), spontaneous intracerebral hemorrhages (5 cases), aneurysms (2 cases), moyamoya disease (1 case) and intracranial arterial occlusion (1 case).View full abstractDownload PDF (6124K) -
Sadayasu Akai, Kimitaka Kaga, Yoshisato Tanaka1984 Volume 16 Issue 1 Pages 21-26
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe investigated the auditory and vestibular function of 18 deaf children who suffered from meningitis and visitedt he Departmento f Oto-Rhino-Laryngologyo f Teikyo University Hospital during the period from 1975 to 1981. The following results were obtained.
1) The hearing loss in our patients was severe.
2) The articulation and language were deteriorated after the onset of hearing loss in 17 patients except for one who suffered at the age of 9 years. Speech deterioration was recovered in a two-year-old boy who received speech therapy as early as two months after the onset of the disease.
3) The damped rotation test revealed hypofunction in 8 patients, afunction in 2 patients and normal function in 2 patients. The optokinetic pattern test revealed normal pattern in 5 patients. The caloric test revealed no response in 2 patients. These results suggested disorders of the vestibular in these patients.
All patients had disturbance of equilibrium; ataxic gait and impaired head control due to loss of vestibular function. But these symptoms disappered later in all patients. Meningitis should be considered to have complication of auditory and vestibular disorders. The early diagnosis and training of such disorders are expected.View full abstractDownload PDF (871K) -
Nariaki Ikeda, Akihisa Mitsudome, Hiroko Ogata, Masaharu Ohfu1984 Volume 16 Issue 1 Pages 27-31
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe platelet function and platelet count were studied in 28 epileptic children who were treated only with sodium valproate (VPA). Fifteen patients showed some platelet dysfunction; abnormalities of clotretraction in 2, platelet adhesiveness 9, ristocetin-induced aggregation 6, and ADP-induced aggregation 15. In 5 out of 15 patients, the secondary aggregation was inhibited. No relation between platelet dysfunction and serum VPA level was recognized. Clinically only 2 patients showed hemorrhagic diathesis; epistaxis 1, and prolonged bleeding after tooth extraction 1. One case who had accidental overdosis of VPA (serum VPA level: 110μg/ml) showed remarkable platelet dysfunction, but 40 days later the platelet function became normal.View full abstractDownload PDF (553K) -
Shigehiro Oka, Yoshio Igarashi, Kuniaki Narisawa, Keiya Tada1984 Volume 16 Issue 1 Pages 32-37
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of propionic acidemia revealed hyperammonemia and lethargy by the administration of sodium valproate (VPA) ten months after birth is reported. Prior to administration of VPA, the patient was a floppy infant of retarded growth with hypothermia, neutropenia, thrombopenia and convulsion but without ketosis, vomiting, lethargy and hyperammonemia. Hyperglycinemia was only transient. Subsequently she developed lethargy, vomiting and hyperammonemia, which needed differentiation from the side effect of. VPA. The propionyl CoA carboxylase activity level in the cultured skin fibroblasts in this patient was reduced. The diagnosis of propionic acidemia was established.
Administration of leucine-isoleucine-valine-methionine-threonine-glycine free milk and low protein weaning food produced improvement, though very gradually, of the growth. Control of convulsion was also regained.View full abstractDownload PDF (726K) -
Dystrophy (Subtype IV?): A Case ReportTatsuya Ishikawa, Kuniki Shimizu, Atsuko Awaya, Yoshio Inoue1984 Volume 16 Issue 1 Pages 38-46
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe described a sporadic female case with congenital muscular dystrophy (CMD).
The disease started between four and eight months of age and progressed slowly. She was unable to roll over till 7 months old, and could not creep or stand up without support in infancy. She began to walk at 13 months of age, kept waddling and never ran. In addition, facial muscle involvement became evident and joint contractures developed in the course of 4 years.
She had muscle weakness predominant at the pelvic girdle, slight hypotonia and absent patellar tendon reflexes. Pseudohypertrophyw as noted in the four limbs, b ut prominenti n the calves. The Tanaka-Binet test gave an IQ of about 70. The serum creatine phosphokinase was about 20 times higher than the normal upper limit and electromyographys howed a myogenicp attern. A muscle biopsyr evealed pathologicalc hanges of progressive muscular dystrophy. The karyotype was that of a normal female with 46 XX.
Clinical features of this patient were similar to those of the patient with atypical Fukuyama type CMD (FCMD) s, ubtype N describedb y Fukuyama et al. in 1981.S ince this case showed more mild involvemento f intelligence, e arly ambulation, m ore localizedd istribution of pseudohypertrophya nd pelvic girdle predominance in weakness, we consider that this case might be a variant form of subtype N of FCMDView full abstractDownload PDF (8502K) -
Takaharu Yamamoto, Norio Kamiya, Meiko Hachiya, Seiji Hirata, Tsutomu ...1984 Volume 16 Issue 1 Pages 47-53
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 5-year-old boy with nemaline myopathy was reported. He was admitted to our hospital because of severe respiratory and cardiac failure. He became unable to be weaned from a respirator.
He was born after 42 weeks gestation, weighed 3, 400 g and was mildly asphyxiated. His parents were unrelated and there was no family history of neuromuscular disorders. Since birth he was hypotonic and developmental milestones were delayed. He walked at 1 year. He got ap with Gowers' maneuver. Five months before admission, he entered a kindergarten where he had to practice marching every day. Five days prior to admission, his face became pale. On the day before admission, marked edema of the eyelids and the legs appeared. Next morning he became lethargic with generalized cyanosis and respiratory difficulty, and was referred to our hospital. Physical examination revealed a moderate and slender boy with higharched palate and mild scoliosis. The lungs were clear but galloping rhythms were audible. Liver and spleen were not palpable and tendon jerks were negative. The chest x-ray film showed cardiomegaly (CTR 63%) and EKG showed RVH. PaCO2 was 83 Torr, ESR was 0.5 mm/hr but serum levels of GOT, GPT, LDH, AL-P and CPK were normal. The movement of diaphragm was markedly diminished at x-ray examination. The rectus femoris muscle was biopsied and a moderate variation in muscle fiber size was observed on HE staining. Modified Gomori-Trichrome staining showed many muscle fibers containing nemaline rods. Type 1 fibers were markedly small in caliber on NADH staining and routine ATPase reaction. Serum carnitine level was normal.
He had been hospitalized for 18 months because he required mechanical ventilation during sleep.View full abstractDownload PDF (4250K) -
Mariko Okamoto, Hideo Nagao, Hiroshi Matsuda, Saburo Sakaki1984 Volume 16 Issue 1 Pages 54-60
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of a 8-month-old girl with herpes simplex encephalitis was reported. She was admitted for convulsions on June 23, 1981. She developed fever on June 17, and the next day, she had a convulsion on her right leg, which became generalized subsequently. On admission she was comatose and there was no meningeal sign. The deep tendon reflexes were not observed. Cerebrospinal fluid (CSF) was normal and an electroencephalogram revealed periodic sharp waves. Computerized tomography showed a high density area in the left parietal region and streak linear enhancement was demonstrated. To decrease the intracranial pressure, a craniotomy was performed. The brain was hyperemic and a biopsy was done for virologic studies. She was diagnosed as herpes simplex encephalitis clinically, and adenine arabinoside therapy was started after the biopsy.
The smeared brain biopsy specimen was submitted to immunofluorescence staining with FITC-anti herpes simplex virus (HSV) and revealed positive staining for the HSV antigen. For the virus isolation, the homogenized biopsy specimens were inoculated into 4 strains of cultured cells (Vero etc). All of the inoculums showed the typical cytopathic effect of HSV. A neutralization test with type-specific anti sera demonstrated that all of the isolates were HSV type 1. This assignment was confirmed by the host range specificity of HSV type 1 and type 2 toward Vero cells and chicken embryo cells. The herpes simplex complement fixation titer in the serum was 1: 32 on June 23 and more than 1: 4096 on July 6, and in the CSF, it rose from less than 1 1 on June 26 to more than 1: 64 on July 1.
This is the first case in Japan of a diagnosis made by HSV isolation from a biopsy specimenView full abstractDownload PDF (5176K) -
Yasuo Muramatsu, Hideomi Ota, Yukikatsu Ochiai, Yoshikatsu Eto, Satoru ...1984 Volume 16 Issue 1 Pages 61-67
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMenkes' kinky hair disease is a disorder of copper metabolism with clinical features of motor and mental retardation, convulsive seizures and characteristic hair deformities. We followed CT findings in a case of kinky hair disease.
The male infant weighting 3, 240g at birth after 37 weeks' gestation, developed convulsions at 3 months of age, suffered from pneumonia at 8 months and was admitted to our hospital because of frequent convulsions. On admission, he had rough, short, brown and curly hair. Serum copper and serum ceruloplasmin levels were low, and there was no response to oral loading of copper sulfate. CT scan revealed enlargement of ventricles, atrophy of cerebral hemispheres and spinal cord at 4 months, and subdural high density effusion at 9 months. A remarkable subdural hematoma that shows higher density than that of the effusions on CT scan appeared at 10 month, which was removed surgically. Xanthochromic effusion and subdural hematoma with septum were found during the opreation.
It was concluded that these changes of CT scans were caused by reccurent minimal hemorrhage, and these hemorrhage was caused by rupture of bridging vein due to fragility of the veins and progressive brain atrophy.View full abstractDownload PDF (10800K) -
Masaru Tatsuno, Michiko Hayashi, Hiroko Iwamoto, Yoshiro Sasaki, Masam ...1984 Volume 16 Issue 1 Pages 68-75
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSAn autopsy case of Leigh's encephalopathy with wide lesions of central nervous system was reported.
The elder brother, also retarded mentally and physically, had infantile spasms and died of unknown cause at age 1. The CT findings and the clinical course were quite similar to this reported case.
This patient had developmental retardation, and frequent tonic spasms since 1.5 month of age. His EEG showed the suppression-burst pattern. His clinical findings were consistent with so-called “early infantile epileptic encephalopathy with burst-suppression”. His CT scans showed symmetrical low-density areas in the basal ganglia, thalamus, cerebral cortex and cerebellum. His condition gradually deteriorated, and he died of respiratory difficulties at 4.5 month of age.
Autopsy revealed spongy necrosis of the thalamus, basal ganglia with typical histological features of Leigh's encephalopathy. Similar necrotic lesions had developed in the cerebral cortex, cerebellum and mamillary body. Enzyme studies of autopsied liver including pyruvate carboxylase and pyruvate dehydrogenase complex were all within normal limits. The urinary inhibitor of thiamine pyrophosphate-adenosine triphosphate phosphotransferase was also negative.
We concluded that clinical symptoms of so-called “early infantile epileptic encephalopathy with burstsuppression” of this case were caused by the wide lesions of central nervous system due to Leigh's encephalopathy.View full abstractDownload PDF (8401K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]1984 Volume 16 Issue 1 Pages 76-78
Published: 1984
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS
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