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Volume 34, Issue 4
Displaying 1-14 of 14 articles from this issue
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[in Japanese]2002 Volume 34 Issue 4 Pages 294
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (255K) -
Chisako Fukuda, Yutaka Tomita, Yoshihiro Maegaki2002 Volume 34 Issue 4 Pages 295-299
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSMedian nerve short-latency somatosensory evoked potentials (MN-SSEPs) were recorded from the scalp to assess frontal lobe functions in children. Forty-nine patients, aged between three and fifty years old underwent 87 examinations. They were devided into four groups: A (3 to 4 years old); B (5 to 6 years old); C (7 to 15 years old) and D (20 to 50 years old). Stimulus electrodes were placed near the wrist on the median nerve. The study was done mostly in the waking state.
To evaluate the independence of the frontal lobe from the parietal lobe we correlated the frontal P22 and parietal N20, and compared the frontal N30 with parietal negative waves. The intervals of the peak latency differed between P22 and N20 by - 1.5 to 1.5 msec. Significant differences were noted between groups A and D, and between C and D. N30 and negative parietal waves resembled each other in children 3 to 4 years of age, but became different from 5 years old. They were dissimilar after 6 years old. Therefore, N30 waves were useful for evaluating the frontal elements in children over 5 years of age.View full abstractDownload PDF (2589K) -
Developmental Changes of Visual P300 Event-Related PotentialsYoshimi Sata, Masumi Inagaki, Seiko Shirane, Makiko Kaga2002 Volume 34 Issue 4 Pages 300-306
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSIn order to evaluate developmental change of visual perception, the P300 event-related potentials (ERPs) of visual oddball task were recorded in 34 healthy volunteers ranging from 7 to 37 years of age. The latency and amplitude of visual P300 in response to the Japanese ideogram stimuli (a pair of familiar Kanji characters or unfamiliar Kanji characters) and a pair of meaningless complicated figures were measured. Visual P300 was dominant at parietal area in almost all subjects. There was a significant difference of P300 latency among the three tasks. Reaction time to the both kind of Kanji tasks were significantly shorter than those to the complicated figure task. P300 latencies to the familiar Kanji, unfamiliar Kanji and figure stimuli decreased until 25.8, 26.9 and 29.4 years of age, respectively, and regression analysis revealed that a positive quadratic function could be fitted to the data. Around 9 years of age, the P300 latency/age slope was largest in the unfamiliar Kanji task. These findings suggest that visual P300 development depends on both the complexity of the tasks and specificity of the stimuli, which might reflect the variety in visual information processing.View full abstractDownload PDF (1140K) -
Michiro Iiyama, Tasuku Miyajima, Akinori Hoshika2002 Volume 34 Issue 4 Pages 307-312
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe evaluated developmental change of the reaction time of the Moro reflex from birth to three months of age using a three-dimensional motion analysis system (ToMoCo-VM: Tousou system).
The reaction time was the shortest in one-month-old babies and became successively prolonged in two and threemonth-old babies, showing a U-shaped pattern.
The three phase of the Moro reflex, as defined by McGraw, changed with advancing age.
The reaction time changed monthly. At the same monthly age, the reaction time was the same irrespective of the phase.View full abstractDownload PDF (2815K) -
II. Assessment of Gene Analyses for the Diagnosis of Developmental Disorders in JapanToshihiro Horiguchi, Makiko Kaga, Masumi Inagaki2002 Volume 34 Issue 4 Pages 313-317
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe authors assessed the present status of gene analysis utilized in Japan. Physicians completed a mail-in questionnaire with their comments regarding the types of gene analyses they used, the perceived effectiveness of the analyses, and how they conducted genetic counselling for the patients. Among the 76 physicians who responded, gene analysis was most often conducted (n=16, 75.0%) for muscle dystrophies, and considered to be effective by most of them (χ2 (df=2) =11.99, p=0.003). Many factors including the age of physicians, location of their facilities, and experience in child neurology were related to the utilization of gene analyses. Future studies based on ethical guidelines should establish a network between specialists and facilities and encourage medical approach to developmental disorders.View full abstractDownload PDF (1087K) -
Gaku Yamanaka, Kanako Goto, Yukiko K Hayashi, Tasuku Miyajima, Akinori ...2002 Volume 34 Issue 4 Pages 318-324
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSFacioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant form of muscular dystrophy characterized by progressive weakness and wasting of the facial, shoulder-girdle and upper arm muscles. The gene locus for FSHD is mapped to the subtelomeric region of chromosome 4q35, in which smaller EcoRI fragments (10 to 33kb) are detected in most families by Southern blot analysis. The purpose of this study is to clarify the frequency and clinical/genetical features of early-onset FSHD in Japanese patients with 4q35-FSHD. In a series of 231 patients from 145 families with 4q35-FSHD, there were 31 patients (13.4%; male: female=12: 19) of 29 families (20%) with early-onset FSHD, 16 of whom were sporadic. Genetic analysis revealed that they had significantly smaller sized EcoRI fragments (range, 10 to 23kb; mean 14.1kb) than the other patients (range, 12 to 33kb; mean 19.6kb) (p<0.001, U-test). All patients with the smallest EcoRI fragments (10 to 11kb) were sporadic cases with early onset. Mental retardation (10/11) and epilepsy (4/11) was often observed in them, but not in the other patients. In early-onset FSHD, tongue muscle involvement (8/31) and swallowing disturbance (2/31), which has been regarded as exclusion criteria of FSHD, were also present. The onset of gait disturbance was significantly earlier (mean age=11.9) in early-onset FSHD compared to the other group (mean age=28.7). All patients with early-onset FSHD showed gait disturbance before 28 years of age. In conclusion, Japanese early-onset FSHD patients tend to have large gene deletions on chromosome 4q35, and show severe and variable phenotypes.View full abstractDownload PDF (905K) -
Toshio Saito, Yuko Yamamoto, Susumu Shinno2002 Volume 34 Issue 4 Pages 326-329
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSTo determine whether soluble adhesion molecules are affected in muscular dystrophy, we measured serum levels of creatine kinase (CK), soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble intercellular adhesion molecule-1 (sICAM-1), soluble (s) E-selectin, and fibrin and fibrinogen degradation products (FDP) in 25 patients with Duchenne muscular dystrophy (DMD), 7 with Becker muscular dystrophy, 7 with Fukuyama type congenital muscular dystrophy, 6 with myotonic dystrophy (MyD), and 5 with spinal muscular atrophy (SMA) type 2, and also serum sVCAM-1, sICAM-1, and sE-selectin in 9 healthy controls. The levels of sVCAM-1 in the patients with DMD were 367.0-852.0ng/ml (552.8±23.1) and significantly elevated than those in the patients with MyD, SMA type 2, and controls. The levels of sICAM-1 and sE-selectin in the patients with muscular dystrophy were 0.2-376.0ng/ml and 17.9-119.0ng/ml, respectively. They were also elevated than those in the patients with SMA type 2 and controls, but not significantly. The levels of sVCAM-1 and sE-selectin in the patients with DMD significantly correlated with age. There was no correlation between the levels of soluble adhesion molecules and those of CK or FDP in any groups. These changes of soluble adhesion molecules may reflect the process of muscle destruction and endothelial cell activation in muscular dystrophy.View full abstractDownload PDF (664K) -
Keisuke Sato, Yuka Ishikawa, Yukitoshi Ishikawa, Tatsuro Izumi, Minoru ...2002 Volume 34 Issue 4 Pages 330-335
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe studied the clinical courses of Fukuyama type congenital muscular dystrophy (FCMD) based on a longterm follow-up of 7 patients. Their peak motor function varied from controlling the head incompletely to standing with support. Six patients had epilepsy, which in some cases was diagnosed with difficulty because of changing seizure types and multiple joint contracture. Six patients underwent endotracheal intubation, and in 5 of them long-term mechanical ventilation began at the age of 15 to 20 years. Three of them underwent trachestomy intermittent positive pressure ventilation (TIPPV), whereas recently 2 patients were treated successfully with nasal intermittent positive pressure ventilation (NIPPV). Mechanical in-exsufflation (MI-E) was effective in clearing airway secretion of 4 patients. With disease progression, the motor, mental, nutritional, cardiorespiratory and swallowing functions of FCMD patients should be assessed to ensure adequate management by NIPPV and MI-E.View full abstractDownload PDF (4859K) -
[in Japanese], [in Japanese]2002 Volume 34 Issue 4 Pages 336-342
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
Seiji Kimura, Yoshiaki Saito, Kaori Kaneko, Atsuo Nezu2002 Volume 34 Issue 4 Pages 343-346
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe report here a 25-year-old girl with Friedreich's ataxia (FA) who showed slowly progressive ataxia, deep sensory disturbance and loss of large myelinated fiber in the sural nerve. There was no evidence of cerebellar atrophy or abnormal values of vitamin E, albumin, CK, and γ-globulin in the serum. Except for mild mental retardation, her clinical and laboratory findings were consistent with those of FA. However, she had no abnormal GAA trinucleotide repeat expansion on chromosome 9q13, unlike typical FA patients in Europe. Her cardiac muscle is not involved instead of 20 years have passed since her ataxia developed. She is considered to belong to a specific type of FA which lacks cardiac muscle involvement and abnormal gene encoding frataxin.View full abstractDownload PDF (2667K) -
Atsushi Kamei, Makoto Sasaki, Manami Akasaka, Shoichi Chida2002 Volume 34 Issue 4 Pages 348-352
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSCharacteristic pathologic changes of cranial computed tomography (CT) and magnetic resonance imaging (MRI) have never been reported in “Alice in Wonderland” syndrome (AIWS) caused by Epstein-Barr (EB) virus infection. We present here a 10-year-old girl with AIWS with an abnormal MR finding. During the course of serologically confirmed EB virus encephalopathy, she had distortion of the body image, visual hallucinations and depersonalization characteristic of AIWS. MRI demonstrated transient T2 prolongation and swelling of the cerebral cortex, especially at the bilateral temporal lobes, bilateral cingulate gyrus, right upper frontal gyrus, bilateral caudate nucleus, and bilateral putamen, whereas CT showed no abnormalities. Transient MRI lesions were occasionally reported in patients with EB virus encephalopathy/encephalitis who presented visual illusions and psychotic reactions, although the diagnosis of AIWS was not described. We consider that any patient with symptoms of AIWS should have MRI because the abnormal MRI findings may disappear in a short period.View full abstractDownload PDF (3227K) -
Mika Mochizuki, Shinichiro Hamano, Sakiko Oshima, Takahiro Nara, Manab ...2002 Volume 34 Issue 4 Pages 353-356
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe described a child who developed paroxysmal abnormal eye movement. At the age of 12 months, she had a high fever and a febrile seizure. On the next day she showed tonic upward deviation of the eyes for 1 to 2 seconds, and downward saccades on attempted downward gaze. The upward deviation was repeated for a period of 2 to 3 hours, and disappeared during sleep. The administration of L-dopa was not effective. The symptoms subsided gradually over 10 months without other neurological impairment. She walked alone at 1 year and 6 months. At the beginning of her walking she showed truncal ataxia, but it gradually disappeared and her development was normal at the age of 2 years and 6 months. These abnormal eye movements and another symptoms were similar to paroxysmal tonic upgaze of childhood (PTU) that has been first described by Ouvrier and Billson (1988) as intermittent upward deviations of eyes. In Japan there was only one report of this syndrome with periventricular leukomalacia and hypomyelination.View full abstractDownload PDF (2422K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2002 Volume 34 Issue 4 Pages 357-359
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese]2002 Volume 34 Issue 4 Pages 366-372
Published: July 01, 2002
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (1431K)
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