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Volume 29, Issue 5
Displaying 1-14 of 14 articles from this issue
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[in Japanese]1997 Volume 29 Issue 5 Pages 360
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (145K) -
Hidee Arai, Michiko Yamada, Shota Miyake, Sumimasa Yamashita, Hiroko I ...1997 Volume 29 Issue 5 Pages 361-366
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported two patients with severe motor and intellectual disabilities syndrome, who were born to mothers having inhaled organic solvents during pregnancy. They had microcephaly, cerebral palsy, mental retardation, seizures, growth failure and minor craniofacial anomalies, variable growth deficiency including a small midface, narrow bifrontal diameter, low-set ears, thin upper lips and micrognathia. Patient 1, a male, died at 8 years and 8 months. The autopsy of his brain revealed marked cereberal atrophy and destruction of bilateral temporal lobes with ventricular enlargements. Microscopic examination revealed migration disorders with polymicrogria at the remaining cerebrum and the cerebellum as well as very thin white matter. Much hemosiderin was found around ventricles, suggesting recurrent minimal bleedings which led to more brain atrophy. Patient 2, a 5 months old male infant, had infantile spasms. On CT and MRI, he had bilateral temporal lobe defect, which might be due to the infarction of bilateral middle cereberal arteries at the prenatal period. These clinical findings are similar to those of other embryopathies, caused by alcohol, phenytoin and other agents. Hersh et al. reported five cases of toluene embryopathy in 1985 and 1988, but they did not report such central nurvous system abnormalties. The pathogenesis of toluene embryopathy remains to be solved, but our cases suggested the possible teratogenesis of toluene.View full abstractDownload PDF (6799K) -
Michio Hiratani, Toshio Munesue, Katsuyuki Terai, Shinichi Haruki1997 Volume 29 Issue 5 Pages 367-372
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe syndrome of water intoxication, resulting from dilutional hyponatremia and characterized by lethalgy, confusion, seizures, and coma was seen in two autistic boys living in the institution for mentally retarded children. Patient 1, a 19 year-old autistic boy showed loss of attention, inactiveness, sleepiness and delirium and then followed by overbreathing, severe vomiting and finally convulsive seizures several times, or coma, since October 1985. In August 1988, he was admitted with generalized tonic clonic convulsion associated with frequent vomiting. EEG showed diffuse spike and wave complex with slow background activity. Laboratory data showed inappropriately high serum ADH level (8.5 pg/ml), low sodium concentration (121 mOsm/l), serum osmolality (237 mOsm/l) which was lower than urine osmolality (334 mOsm/l), and remarkable body weight gain (8.5 kg). He was diagnosed as water intoxication due to compulsive water drinking and SIADH. Diminished GH secretion to insulin-induced hypoglycemia and exaggerated prolactin response to LHRH stimulation suggested a hypothalamic lesion. Patient 2, a 17 year-old autistic boy, showed essentially the same symptoms and laboratory data as Patient 1, except that he had no epileptic discharge in EEG, and curious GH response to insulin-induced hypoglycemia. A remarkable daily body weight change suggested excessive water drinking and a possible episodic release of ADH. With mild water restriction, this became smaller. Since Patient 1 had epileptic attacks several times without hyponatremia and his EEG showed epileptic discharges, he was diagnosed as having epilepsy. Patient 2 has been seizure-free until now. Abnormality of hypothalamic or pituitary defects and polydipsia and possibility of water intoxication should always be considered when an autistic patients shows recurrent epileptic attacks or episodic strange behaviors with hyponatremia.View full abstractDownload PDF (635K) -
Satoshi Araki, Masaharu Hayashi, Masahiro Itoh, Kiyoko Kurata, Hideaki ...1997 Volume 29 Issue 5 Pages 373-377
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSStatus marmoratus (ST) of the basal ganglia and thalamus has been observed in cases of perinatal hypoxic ischemic encephalopathy and rarely of postnatal encephalopathy. In occasional cases, the cerebral cortex is also involved. Here, we reported a 22-year-old male with a sequela of postnatal anoxic encephalopathy. He had been bedridden with severe mental retardation since he suffered anoxic encephalopathy at 3 months of age. ST was found in the cerebral cortex as well as basal ganglia and thalamus. In addition to ST, the cerebral cortex and white matter were remarkably devastated. We also performed a preliminary immunohistochemical study to investigate the process of ST formation, using antibodies against glial fibrillary acidic protein (GFAP), myelin basic protein (MBP) and synaptophysin. MBP-positive abnormal myelinated fibers were identified in the ST areas, which were not colocalized with GFAP-immunopositivity. Synaptophysin was positive in the areas of ST, which were not stained with Holzer method. These results suggest that the myelination occurring in the early infancy damage of basal ganglia and cerebral cortex could be implicated in the formation of ST.View full abstractDownload PDF (6947K) -
Yoshiaki Saito, Kenji Sugai, Masayuki Sasaki, Satoru Hirano, Shigeru H ...1997 Volume 29 Issue 5 Pages 378-383
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA four-year old boy with congenital hypomyelination neuropathy (CHMN) had mental retardation and a tic disorder, the latter commencing at 2 years of age.The diagnosis of CHMN was confirmed by electron microscopy of his biopsied sural nerve which showed loss of large myelinated fibers, decreased density of myelinated fibers, many naked axons and atypical onion bulb formation. MR imaging showed type I Chiari malformation in the absence of clinical signs attributable to it. Auditory brainstem response (ABR) showed delayed wave I peak latency, prolonged I-III interpeak latency, broad wave II duration and normal III-V interpeak latency, suggesting abnormal peripheral and normal central myelination. The association of CHMN with mental retardation, Chiari malformation and tic disorder has never been reported, the significance of which remained unclear.View full abstractDownload PDF (5815K) -
Takahito Wada, Yuri Shimazaki, Sumimasa Yamashita, Michiko Yamada, Hir ...1997 Volume 29 Issue 5 Pages 384-389
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a 9-year-old girl with idiopathic spinal epidural hematoma. She complained of her back and neck pain only during night for a few days, followed by days with no symptoms (a few days remission of her symptoms). This episode repeated, and she was suspected to have a psychogenic reaction. However, 28 days later after the first symptoms appeared, paralysis of lower extremities, anesthesia and urinary bladder dysfunction (neurogenic bladder) appeared. Spinal MRI showed a spinal epidural hematoma at the C 5-Th 2 level. An emergency operation of laminectomy and evacuation of the hematoma was done, and she has recovered gradually. We diagnosed her as having idiopathic spinal epidural hematoma. Excerbations of symptoms during nights were thought to be due to an elevation of venous pressure by lying, and transient remissions were due to intermittent small hemorrhages.View full abstractDownload PDF (3994K) -
Atsushi Nishimura, Hirotaka Yamazaki, Tatsuo Fuchigami, Yukihiko Fujit ...1997 Volume 29 Issue 5 Pages 390-395
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe described a 12-year-old girl with systemic lupus erythematosus (SLE) associated with myasthenia gravis (MG). She has had absence seizures from 6 years old. She admitted to our hospital at 12 years of age because of absence seizures and dyspnea. The diagnosis of SLE was made on the basis of convulsion, arthritis, pleurisy, and positive antinuclear factor and was started therapy with prednisolone. The clinical course was complicated by the appearance of dysphagia and hoarseness. On the basis of positive Tensilon test and a high level of serum anti-acetylcholine receptor antibody, we made a diagnosis of the systemic type of MG. Her condition was improved by methylpredonisolone pulse therapy and y-globulin therapy after plasmapheresis. The association of early-onset SLE with MG is rare.View full abstractDownload PDF (2892K) -
Atsushi Nishimura, Tatsuo Fuchigami, Hiroyuki Izumi, Osami Okubo, Shig ...1997 Volume 29 Issue 5 Pages 396-400
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a rare case of early-onset acute disseminated encephalomyelitis (ADEM). After a nonspecific upper respiratory infection, a 14-month-old boy developed oculomotor nerve paralysis, nystagmus, intention tremor and ataxic gait. MRI showed extensive symmetric high signal lesions in the bilateral cerebellar and cerebral white matters. We made the diagnosis of ADEM on the basis of the high CSF myelin basic protein, clinical course, symptoms and MRI findings. Following the administration of steoid hormone, his clinical symptoms rapidly improved.View full abstractDownload PDF (3363K) -
Megumu Tojo1997 Volume 29 Issue 5 Pages 401-405
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA case of severe motor and intellectual disabilities syndrome after being nearly drowned was reported. He could walk at the age of one year. But his condition became to a bedridden state by the hypoxic brain damage due to near drowning at one year and 5 months old. At one year and 8 months, he was treated by the “Ueda” method for physical therapy to cerebral palsy (CP). Before the treatment he showed decorticate posture and opisthotonic posture. Six weeks after the treatment, the severity of spasticity was remarkably decreased. He could take hip flexion posture and relaxed posture easily. His status remarkably improved. The effectiveness of the “Ueda” method to different spasticity from CP was also confirmed.View full abstractDownload PDF (5117K) -
Makoto Ueno, Tatsuya Koeda, Yoshihiro Maegaki1997 Volume 29 Issue 5 Pages 406-410
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a 9-year-old boy who had a complex partialseizure for the first time, 4 years and 7 months after an episode of theophylline-associated convulsive status. An ictal EEG showed abnormal theta wave burst which showed no superficial focus, implying existence of deep focus in the brain. MRI revealed left thalamic infarction and hippocampal sclerosis. We regarded these abnormalities as the sequelae of theophylline-associated convulsive status.View full abstractDownload PDF (7044K) -
[in Japanese], [in Japanese], [in Japanese]1997 Volume 29 Issue 5 Pages 411-412
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese]1997 Volume 29 Issue 5 Pages 414-415
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (3311K) -
[in Japanese]1997 Volume 29 Issue 5 Pages 416-417
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (233K) -
[in Japanese], [in Japanese]1997 Volume 29 Issue 5 Pages 420-427
Published: September 01, 1997
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (1137K)
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