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Volume 30, Issue 4
Displaying 1-16 of 16 articles from this issue
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[in Japanese]1998 Volume 30 Issue 4 Pages 282
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (121K) -
Takahito Wada, Mihoko Nakamura, Yuko Matsushita, Michiko Yamada, Sumim ...1998 Volume 30 Issue 4 Pages 283-289
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSX-linked α-thalassemia/mental retardation syndrome (ATR-X), which was first reported by Wilkie, et al. in 1991, is a disorder with severe mental retardation, characteristic facial appearance, genital abnormalities, and mild form of α-thalassemia.
At present, about 50 cases have been reported in the world, but few in Japan. We report 3 cases of this disorder in 2 families. All cases prefer a peculiar posture and show unique movements, such as self-induced vomiting or self-hanging, which can be diagnostic. ATR-X should be considered as a differential diagnosis in all male patients with severe mental retardation.View full abstractDownload PDF (6229K) -
Kimio Minagawa, Seika Yanai1998 Volume 30 Issue 4 Pages 290-294
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMidazolam infusion was tried as the treatment for 48 episodes of refractory status epilepticus or a cluster of seizures in 16 children. The mean age of patients was 3.5 years (range, 1 month to 18 years). Nine children had epilepsy, one purulent meningitis, one encephalitis, one acute cerebral infarction, and the remaining four had acute phase of hypoxic ischemic encephalopathy. The type of the seizure was a generalized tonic clonic seizure (including a partial seizure secondarily generalized) in 41 episodes, a tonic seizure in 3, an atypical absence in 1, and a complex partial seizure in 3, respectively.
All patients received intravenous midazolam at 0.15 mg/kg as bolus, followed by a continuous infusion at 0.1-0.15 mg/kg/hr initially. The dose was increased gradually up to 0.3 mg/kg/hr until the complete control of seizures was achieved.
Fourty-one of the 48 episodes of seizures were controlled within 30 minutes after the initiation of midazolam therapy. The mean infusion rate of midazolam required was 0.22 mg/kg/hr. The mean duration of the treatment was 4.1 days.
None of the patients had serious changes in the blood pressure or respiratory status attributable to the use of midazolam.
In conclusion, midazolam infusion is an effective and safe therapeutic approach for the management of childhood status epilepticus.View full abstractDownload PDF (715K) -
Kozaburo Hiramatsu, Kimiko Baba1998 Volume 30 Issue 4 Pages 295-299
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTo study the effects of brain atrophy on cerebral palsy with severe motor and intellectual disability, cerebral CT examinations were conducted over a period of 12.7±5.0 years on 14 patients with cerebral palsy. The ratios of the cortical, subarachnoid space and ventricular space areas to the inner skull space were determined. The initial CT was conducted at an average age of 18.9±5.0 and indicated dilatation of the subarachnoid space and ventricles. The subsequent CT studies disclosed that brain atrophy had developed during a mean period of 12.7±5.0 years. Dilatation of the subarachnoid space was particulary prominent. Brain atrophy with dilatation of the subarachnoid space may be characteristic of severe motor and intellectual disability.View full abstractDownload PDF (3818K) -
Chuan-Yu Wang, Hisashi Kawashima, Takeshi Takami, Naoto Yamada, Tasuku ...1998 Volume 30 Issue 4 Pages 300-306
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a 13-year-old boy diagnosed as multiple sclerosis associated with narcolepsy. He had suffered from retrobulbar optic neuritis at the age of 11 years which was improved gradually by prednisolone. Four months later he was admitted because of excessive somnolence. The diagnosis of narcolepsy was made based on hypnagogic hallucination, sleep paralysis, changes of personality and the sleep onsets REM (SOREM). The elevation of anti-measles antibody and a positive oligoclonal band in CSF, low density areas in the bilateral internal capsule on CT scan, and high signal areas in the same region on T2-weighted MRI confirmed the diagnosis of multiple sclerosis. An abnormal arousal response occurred occassionally in sleep stage 2 and 4, which started with electrical silence followed by a period with irregular high voltage slow waves and repetitive focal spike train. Those clinical symptoms and abnormalities of biochemical and electrophysiological studies normalized after treatment with prednisolone. However, abnormalities on MRI showed no improvement even after long term administration of prednisolone (2.5 mg/day).View full abstractDownload PDF (4442K) -
Rie Shibata, Isao Kusakawa, Matsuko Ozawa, Tatsuo Ohya, Ikuya Nonaka1998 Volume 30 Issue 4 Pages 307-311
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMany patients with a severe infantile form of congenital myopathies have respiratory and feeding difficulties from early infancy. We experienced a male patient who required an artificial ventilation soon after birth and showed marked generalized muscle weakness involving the facial muscles. He had skull deformity and mild mental retardation at the age of one year and 10 months.
The first muscle biopsy at the age of 1 month showed small caliber fibers and an increase in the number of undifferentiated type 2C fibers, indicating muscle fiber immaturity. Since type 1 fibers were smaller than type 2 fibers, he was tentatively diagnosed as having congenital fiber type disproportion myopathy. The muscle biopsied findings improved significantly in the second biopsy at 1 year of age which showed type 1 fiber predominance but no cytoarchitectural abnormalities. Accordingly, he was diagnosed as having congenital myopathy with type 1 fiber predominance.
A severe neonatal form of congenital myopathies may show striking muscle fiber immaturity in the early infancy. This may later develop into characteristic morphologic findings such as the formation of nemalines and cores, and abnormal fiber type distribution.View full abstractDownload PDF (5901K) -
Miyuki Ushida, Kuniaki Fukuda, Shouichi Endo, Takashige Okada1998 Volume 30 Issue 4 Pages 312-316
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe report a 6-year-old girl with Japanese B encephalatis.
The initial symptoms were high fever, headache and vomiting. On the second day of illness, she developed hemiconvulsion and was admitted to our hospital.
Physical examination demonstrated a stiff neck. C-reactive protein elevated to 22.7 mg/dl. CSF examination showed a marked increase in the cell count (10, 896/3 mm3). During the course of the treatment, she showed transient hemiparesis and dysphagia, followed by akinetic mutism lasting for about a month. The patient was left with severe cognitive and memory impairment and complex partial seizures but no motor dysfunction. Japanese B encephalitis was diagnosed by means of serological examination. Magnetic resonance imaging revealed cystic lesions in the medial and posterior thalamus and substantia nigra and severe atrophy of the hippocampus. Despite the involvement of substantia nigra, the patient had no parkinsonism. The cognitive impairment may in part be explained by the lesions in the medial and posterior thalamus.View full abstractDownload PDF (7402K) -
Jun Tohyama, Yoshihiro Naganuma, Seiko Shirane, Makoto Uchiyama1998 Volume 30 Issue 4 Pages 317-322
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a Japanese girl with the Sanfilippo syndrome type C. She was born to healthy parents married consanguineously. She began to deteriorate and became disoriented at the age of 6 year and 8 month. She also developed sleep problems and dysphagia. Physical examination revealed short stature, slightly coarse facial features, contracture of the PIP joints and hypertrophy of the tonsils. There was neither hepatomegaly nor corneal clouding. Laboratory examination demonstrated an increase in urinary excretion of glycosaminoglycan. Electrophoresis of the urinary glycosaminoglycans indicated that heparan sulfate was the predominant component. Enzymatic assay using her skin fibroblasts demonstrated a complete deficiency of acetyl-CoA: a-glucosaminide N-acetyltransferase activity. Low dose erythromycin alleviated hypertrophy of her tonsils, thereby improving dysphagiaView full abstractDownload PDF (6513K) -
Yukinori Maeoka, Yoshihiro Maegaki1998 Volume 30 Issue 4 Pages 323-327
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA 13-month-old girl developed opsoclonus-myoclonus syndrome in association with neuroblastoma. She showed irritability, hyperhidrosis and a bad temper. Serum and urinary vanilmandelic acid, homovanilic acid and catecholamines were elevated at the same time. Chest CT demonstrated the presence of neuroblastoma in the paravertebral region. Cranial CT and MRI revealed no abnormal findings. Brainstem auditory-evoked potentials and short latency somatosensory evoked potentials were normal, whereas blink reflex showed long duration and high amplitude of late components (R2 and R2') indicating hyperexcitability of the interneurons in the lower brainstem. These findings improved gradually as opsoclonus disappeared. Her neurologic symptoms resolved completely within 2 years after the resection of the tumor. The hyperexcitability of the blink reflex may indicate the hyperactivity of the neurons in the brainstem reticular formation responsible for the abnormal saccadic eye movements (opsoclonus), which could be caused by the oversecretion of dopamine or by the supersensitivity of dopaminergic receptors.View full abstractDownload PDF (3191K) -
Masahiro Itoh, Keiko Hayakawa1998 Volume 30 Issue 4 Pages 328-333
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA previously healthy 7-year-old girl suffered from oral herpetic gingivostomatitis. After four days, oral administration of acyclovir (ACV), 1, 000 mg in five divided doses, was started. She became irritable and intermittently delirious next day. Laboratory tests of blood and cerebrospinal fluid (CSF) were within normal limits and EEG showed no abnormality. Brain CT and MRI demonstrated mild asymmetry of the lateral ventricle. SPECT showed low perfusion area in the bilateral thalamus. The titer of IgG and IgM against herpes simplex virus (HSV) was increased in the serum but not in the CSF. A PCR study of HSV DNA was negative in the CSF. ACV was discontinued after 5 days' administration and she recovered from neurological disorders within 48 hours.
ACV neurotoxicity is self-limiting and dose-dependent. It may be distinguished from herpes simplex encephalitis by the lack of fever, headache, focal neurologic signs, and abnormal CSF and neuroradiological findings. However, when the differentiation should be difficult, we believe that the administration of ACV can be carefully continued because the potential fatality of HSE is high while ACV neurotoxicity is reversible.View full abstractDownload PDF (7816K) -
Natsue Nakamoto, Toyoaki Nakayama, Satoshi Kudo, Manabu Tanaka, Yasuko ...1998 Volume 30 Issue 4 Pages 334-338
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe reported a 5- year- old boy with acute encephalitis due to suspected herpes simplex infection, who developed confusion, agitation and insomnia during intravenous administration of acyclovir. He recovered from these neuro-psychiatric symptoms two days after the cessation of acyclovir. The same symptoms recurred two days after its re- administration and resolved on the next day of the second cessation of the drug. Electroencephalogram (EEG) showed periodic lateralized epileptiform discharges (PLEDs) on hospital day 16, which disappeared on hospital day 27, suggesting that neurotoxicity of acyclovir may induce PLEDs.
Althogh acyclovir is useful for the treatment of herpes simplex and varicella-zoster virus infections, we have to pay attention to its neurotoxicity.View full abstractDownload PDF (2524K) -
Takashi Hayashi, Takashi Ichiyama, Miki Nishikawa, Susumu Furukawa1998 Volume 30 Issue 4 Pages 339-345
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe experienced a 4-year-11-month old girl with right side dominant spastic diplegia caused by preterm birth and neonatal asphyxia. She tended to use the left hand but showed mirror writing on both hands, which was aggravated by correction of handedness to the right. Magnetic resonance T2-weighted imaging revealed a localized high intensity lesion in the white matter of her left parietal lobe, caused by hypoxic insult and suggested a damage of spatial orientation center. Wechsler Preschool and Primary Scale of Intelligence showed a total IQ of 76, VIQ of 86 and PIQ of 74, revealing borderline intelligence with insufficient visual perception. Frostig visual perception test revealed lower scores of position in space. Spatial confusion caused by a lesion in the left parietal lobe might have induced the mirror writing of this case. Forced correction to use the right hand with damaged left parietal lobe may aggravate spatial confusion and consequently worsen mirror writing.View full abstractDownload PDF (4193K) -
Soukun Fuwa, Tatsurou Kaminaga, Masaaki Kobayashi, Yasushi Sasaki, Shi ...1998 Volume 30 Issue 4 Pages 346-349
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS1H-NMR spectroscopy was performed on a 6-month-old boy with pachygyria. A MRI study demonstrated an abnormally thick cortex localized in the right occipital lobe. 1H-NMR spectrums were collected from the lesion and the contralateral cortex that appeared normal on MRI. The N-acetylaspartate (NAA)/Cre (creatine) ratio was makedly lower in the abnormal cortex than in the contralateral cortex and the occipital cortex. NAA localizes to neurons, axons, dendrites and synaptic connections and increases with maturation of neurons. Its decrease is considered to represent the decrease in the number of these structures and/or disturbance of neuronal maturation. We conculde that NAA/Cre can be an important index that reflects the pathogenesis of pachygyria.View full abstractDownload PDF (2801K) -
[in Japanese]1998 Volume 30 Issue 4 Pages 350-351
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1998 Volume 30 Issue 4 Pages 352-353
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese]1998 Volume 30 Issue 4 Pages 358-363
Published: July 01, 1998
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (823K)
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