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Volume 17, Issue 5
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[in Japanese]1985 Volume 17 Issue 5 Pages 390
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (152K) -
Tateo Sugimoto, Hitoshi Okazaki, Toko Hara, Man Woo, Naoki Nishida, Ak ...1985 Volume 17 Issue 5 Pages 391-397
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe have experienced 10 patients with herpes simplex encephalitis (HSE) in childhood during the last 7 years. The diagnosis of HSE was made either by a significant increase in the virus antibody in 7 patients or by a viral isolation in 3. The outcome of these patients was poor. Three patients died, 6 survived with severe neurological sequelae and one had a secondary epilepsy without motor disturbance. In the present series, we investigated retrospectively the diagnostic value of early symptoms, EEG, CT and serological studies.
Early symptoms were convulsions in 8 cases, particularly of unilateral type in 6 of them. Cerebrospinal fluid findings in the early period were normal in half of the patients. EEG within one week from the onset showed lateralized temporal spikes and periodicity in 4, and CT of the same period demonstrated localized abnormalities in the unilateral temporal region in 2 of 7 cases. The early diagnosis of HSE by EEG and CT inchildhood was insufficient. Therefore, we reported in this retrospective study that the determination of the ELISA IgG in cerebrospinal fluid and IgM in serum to herpes simplex virus would establish the early diagnosis of HSE within 3-5 days from the onset of the disease.View full abstractDownload PDF (894K) -
Masaru Tatsuno, Susumu Sunaga1985 Volume 17 Issue 5 Pages 398-404
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSValproic acid (VPA) has been widely used for the treatment of convulsive disorders in infants, but there has been concern that VPA administration may interfere with normal brain growth. Effects of VPA on brain growth were studied in young Sprague-Dawley rats. In order to eliminate nutritional factors first-day rats were subjected to implantation of intragastric cannules, through which they received a milk formula for 7 days. Either 75mg/kg or 200mg/kg VPA, or physiological saline was injected daily intraperitoneally during the first week of life. No differences in body weight was seen between control and VPA treated groups. However, VPA treated groups showed decreases not only in the cerebellar weight but also in the levels of protein, DNA, RNA and cholesterol, with the greatest reductions in the 200mg/kg/day group. In addition, VPA retarded cerebellar growth to a greater extent than cerebral growth. Our data indicated that administration of valproic acid during early life might have adverse consequences on brain development.View full abstractDownload PDF (869K) -
Katsuya Yamamoto, Yoichi Goto, Kazuie Iinuma, Kikuo Suzuki, Tasuke Kon ...1985 Volume 17 Issue 5 Pages 405-411
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSHuman lymphoblastoid interferon (interferon-α) was administered intrathecally to two patients with subacute sclerosing panencephalitis (SSPE) as a therapeutic trial and its effects were evaluated clinically, electroencephalographically and immunologically. Inosiplex was administered together to both cases and transfer factor to one of them.
Case 1, a ten-year-old boy, was at the second stage of Jabbour's classification on admission and deteriorated gradually in spite of administration of interferon over five months. No significant beneficial effects were found.
Case 2, a ten-year-old girl, was at the second stage on admission. Although interferon was started immediately after admission, her neurological symptomes worsened during the first two months. However, the patient showed a marked clinical improvement during the further interferon therapy. No significant improvement was recognized in the serial electroencephalograms. Measles antibody titers in serum and cerebrospinal fluid were parallel to the clinical course. In this case, natural-killer cell activity against Vero cells infected with SSPE virus (Niigata 1 strain) was found to be extremely decresed but it was enhanced by in vitro interferon treatment.
No evident side effects were recognized except for transient elevation of body temperature for several hours after the i. t. interferon.
The clinical improvement in Case 2 cannot be attributed solely to the effects of interferon itself at present, because of the possibility of a fluctuating course and a spontaneous improvement of SSPE. But, there might be a possibility that interferon played an important role in improvement of clinical features in the second patient. In addition, a decrease of natural-killer cell activity found in Case 2 might be responsible in part for the pathogenesis of the disease and give a rationale to interferon therapy to it.View full abstractDownload PDF (829K) -
Hiroshi Suzuki, Yasuji Inamo, Narumi Michihiro, Yukihiko Fujita, Shuic ...1985 Volume 17 Issue 5 Pages 412-419
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTo investigate the immunological process involved in the pathogenesis of epilepsy, we performed renal biopsies and EEG recordings on 3 cases of immune complex glomerulonephritis and speculated on brain lesions associated with renal lesions.
The case of nephrotic purpura nephrites (Case 1) showed high voltage slow waves on EEG, which were exacerbated by injection of a high dose gamma globulin with Fc segment. But this paroxysmal discharge disappeared after pulse therapy without clinical attacks. Cases of post-streptcoccal subacute glomerulonephritis (Case 2) and membrano-proliferative glomerulonephritis (Case 3) showed only slowing of the background activity. The histological lesion was found mainly in the mesangial region in Case 1 but the basement membrane was invloved markedly in Cases 2 and 3.
In Cases 1 and 2 with treatment at the early period of the illness, the amount of urinary protein decreased gradually. But Case 3 receiving immunological treatment later in the course of illness showed the increase of the amount of urinary protein by the treatment.
Based on preceding 3 cases, we speculated the possibility that the concept of immune complex explain the pathogenesis of paroxysmal discharge and clinical attacks and the variable effects of gamma globulin with Fc segment on epilepsy as well as on nephritis.View full abstractDownload PDF (3756K) -
Junko Tanaka, Sachiko Onoe, Jiro Abe, Tetuzo Tagawa, Jiro Ono, Naoyuki ...1985 Volume 17 Issue 5 Pages 420-425
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSRepetitive supramaximal stimulation (RSS) testings of the ulnar or median nerve were performed on 35 children with ocular myasthenia. Serum titers of antiacetylcholine receptor antibody were also determined.
Sixteen patients (46%) had the abnormal decremental response to RSS even in clinically uninvolved muscles. However, none of these 16 patients developed generalized myasthenia. The duration of the drug therapy in these patients was 4.7±3.5 years (mean±SD), while that in patients with the normal response to RSS was 2.6±2.5 years. Fourteen (88%) of 16 patients who had the abnormal response received steroid therapy, while 13 (68%) of 19 patients with the normal response were treated with the steroid hormone. No difference was seen in the remission rate between patients with the abnormal response (75%) and those with the normal response (79%). However, patients with the abnormal response tended to have a higher recurrence rate of myasthenic signs, a longer duration of therapy, and slightly higher serum titers of antiacetylcholine receptor antibody.
Our results suggested that in juvenile ocular myasthenia the positive abnormal decremental response to RSS in clinically uninvolved muscles correlated with the clinical response to the drug therapy as well as higher titers of serum antiacetylcholine receptor antibody.View full abstractDownload PDF (647K) -
Tatsuro Izumi, Hiromi Utsumi, Makiko Osawa, Yukio Fukuyama1985 Volume 17 Issue 5 Pages 426-430
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA clinico-biochemical study was undertaken to determine whether Ca-hopantenate plays a role in the development of acute encephalopathy. The patients were divided into eight group: those treated with Ca-hopantenate only (group 1); Ca-hopantenate and other drugs (group 2); Ca-hopantenate, phenobarbital and other antiepileptic drugs (group 3); Ca-hopantenate and VPA (group 4); VPA only (group 5); VPA and other antiepileptic drugs (group 6), common drugs (group 7) and without medication (group 8). The ammonia values of the blood were slightly higher in groups 4 and 6 than those in other groups, but the differences between these mean values in eight groups were not statistically significant. Further investigations were necessary to determine the pathogenesis of increased blood ammonia values in patients treated with Ca-hopantenate and VPA. The possibility of polypharmaceutic effects was not completely excluded in these patients. The values of free carnitine, pantothenic acid and CoA were not reduced in the blood of the patients under the treatment with Ca-hopantenate.
It was not possible to obtain the results indicating that Ca-hopantenate was a possible cause of acute encephalopathy.View full abstractDownload PDF (619K) -
Fumio Hayakawa, Naoki Yamamoto, Kozaburo Aso, Kazuyo Inokuma, Etsuko T ...1985 Volume 17 Issue 5 Pages 431-437
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSAlthough the Kearns-Shy syndrome (KSS) is now recognized as one of disorders of mitochondrial dysfunction characterized by external ophthalmoplegia, retinal pigmentary degeneration, and heart block. cas having full-blown symptoms are rare. We recently examined four children with KSS. One case had a family history of KSS. Ptosis, external ophthalmoplegia, and neurosensory hearing loss were observed in all cases. So-called “atypical pigmentary degeneration of the retina” was demonstrated in three. Failure of cardiac conduction was demonstrated in two patients ; in one an epicardiac ventricular demand pacemaker was in-serted. Short stature and mild muscular weakness were seen in all cases. Cerebellar signs such as tremor were detected in two cases. Endocrine disorders were seen in two cases ; one presented growth hormone deficiency, and the other insulin dependent diabetes mellitus. Elevated CSF protein levels were demonstrated in all cases, but serum lactate and pyruvate levels were normal except for one case. EEGs were abnormal in three patients, all showing dysrhythmic basic rhythm. Intelligence was low normal in all cases. Computed tomographic scans demonstrated diffuse low density changes in the white matter of one case. In three cases “ragged red fibers” were shown in muscle biopsy specimens, and the electron microscopic examination showed some stractural abnormalities of mitochondria in three cases. Large mitochondria with proliferation of cristae were prominent, but paracrystalline arrangement of cristae was scarsely observed. The appearance of characteristic features of KSS might depend upon the clinical stage of this disorder, but different clinical and biochemical features might indicate heterogeneity of this syndrome.View full abstractDownload PDF (5029K) -
A Case of Turner Syndrome with Renal Hypertension: Fibromuscular Dysplasia in Cerebro-Renal ArteriesShota Miyake, Junichi Arai, Michiko Hayashi, Hiroko Iwamoto, Kenichi S ...1985 Volume 17 Issue 5 Pages 438-442
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 6-year-9-month-old girl with Turner syndrome who suffered from fibromuscular dysplasia and renal hypertension was reported. She was born at 38 weeks' gestation, and weighed 2, 845 g. Her developmental milestones were mildly delayed. At the age of 6 years she was attacked by sudden paresis of right extremities. The CT scan on the 5 day revealed low density at the anterolateral region of the left cerebral hemisphere, and EEG showed focal low voltage at the same region. Left internal carotid artery showed alternating zones of widening and narrowing of the arterial lumen (string of beads). There was stenosis at a branch of the left middle cerebral artery. The left renal artery had two branches and showed marked narrowing at the beginning. Aortic stenosis was also recognized. The characteristic face and multiple anomalies were pointed out, and the diagnosis of mosaic Turner syndrome (45, X/47, XXX) was established by the chromosomal analysis. From angiographic findings, the authors concluded that the symptoms and signs of this patient were originally caused by the systemic disease of arteries, “fibromuscular dysplasia”.View full abstractDownload PDF (6310K) -
Akiko Terauchi, Ikuko Kuroda, Tetsuo Mori, Takafumi Matsuoka1985 Volume 17 Issue 5 Pages 443-447
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 10-year-old boy with generalized muscular atrophy and chronic heart failure was reported. Family history revealed a male with unknown cardiopathy and a female with scoliosis on the paternal side. His early motor development was retarded because of muscle hypotonia, and the patient started walking at 2 years 7 month of age. Gradually he developed kyphoscoliosis and tiptoe walking due to shorting of bilateral Achilles tendons. Cardiomegaly and A-V block were first noticed at age 5. He was admitted to the hospital for the treatment of chronic heart failure. Physical examination revealed generalized atrophy, especially of scapulohumeroperoneal distribution, myopathic face, and decreased deep tendon reflexes. Intelligence was normal. Serum creatine kinase was 140-270 units/L (normal controles 30-80). EMG showed both myogenic and neurogenic paterns. Muscle fibers were relatively well preserved with slightly increased connective tissue and scattering small fibers in the biopsied specimen of rectus femoris muscle. Fiber type proportion was normal as demonstrated by ATPase staining. Brain CT and EEG were normal. ECG revealed A-V block (first grade) and IRBBB. Remarkable enlargement of left and right atrial cavities were shown by ultrasonic echocardiography. These findings established the diagnosis of Emery-Dreifuss type muscular dystrophy.View full abstractDownload PDF (4913K) -
Takebumi Onda, Kiyoshi Ogawa, Shigeo Yokoi, Koumei Kumagai, Kihei Maek ...1985 Volume 17 Issue 5 Pages 448-452
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIn 1949 Robertson reported a case of mega cisterna magna (MCM) for the first time, but only a few reports about MCM have been published. Recently, Adam, Alker and others reported 20 MCM cases.
A five-month-old mentally retarded girl, who had a large hemangioma on the left lateral aspect of her face, visited our hospital and was diagnosed as MCM by means of brain X-ray, CT and NMR-CT.
This is probably the first reported case of MCM with the NMR-CT imaging technique.View full abstractDownload PDF (7225K) -
Kouzou Mutoh, Kwang-Myon Kim, Teizou Tomizawa, Tatsuo Takao, Takehiko ...1985 Volume 17 Issue 5 Pages 453-458
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSAn 11-year-old boy with left spastic hemiplegia and learning difficulty was reported. Changes of his character appeared first, followed by atrophy of the left upper and lower extremities. Exaggerated deep tendon reflexes and positive Babinski sign were present on the left extremities, but sensory disturbances and ataxia were absent. There was no denervation pattern in EMG. Brain CT revealed a high density area in the right basal ganglia and enlarged right lateral ventricle without shift of the midline.
This high density area on CT gradually became conspicuous within 6 months without a mass effect or shift of the midline. CSF cytology was negative and stereotaxic biopsy revealed two cell pattern germinoma of basal ganglia.View full abstractDownload PDF (5582K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1985 Volume 17 Issue 5 Pages 459-460
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese]1985 Volume 17 Issue 5 Pages 460-462
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1985 Volume 17 Issue 5 Pages 462-464
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese]1985 Volume 17 Issue 5 Pages 465
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (104K) -
[in Japanese]1985 Volume 17 Issue 5 Pages 466-469
Published: 1985
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (719K)
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