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Volume 35, Issue 4
Displaying 1-14 of 14 articles from this issue
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[in Japanese]2003 Volume 35 Issue 4 Pages 284
Published: July 01, 2003
Released on J-STAGE: December 15, 2011
JOURNAL FREE ACCESSDownload PDF (176K) -
Yoshimitsu Fukushima2003 Volume 35 Issue 4 Pages 285-291
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSGenetic counseling provides medical and genetic information of the disease including its natural history, recurrence risk, availability and usefulness of genetic testing, as well as psycho-social support. In the field of pediatric neurology, the majority of genetic counseling seems to be a simple risk estimation of the next child and unrelated to ethical issues. However, in some cases requiring prenatal diagnosis or presymptomatic testing, we have to address serious ethical issues. Genetic counseling should be provided by an educated medical doctor at a suitable genetics clinic. In Japan, we have “Japanese Board of Medical Genetics, Clinical Geneticist” as an education system of clinical genetics for medical doctors. Pediatric neurologists should know the special issues of genetic information and contact with clinical geneticists in selected cases.View full abstractDownload PDF (1049K) -
Megumu Tojo, Hatsumi Nitta2003 Volume 35 Issue 4 Pages 292-296
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSOne hundred and sixteen children with an acquired damage to the central nervous system (CNS) during the last decade were studied to determine the causes and sequelae and to find strategies for prevention
The etiology was CNS infections and related disorders in 46 cases (39.4%), non-infectious diseases (epileptic, hypoxic etc.) in 16 cases (13.8%), iatrogenic brain injury in 15 cases (12.9%), accident-related brain damage in 31 cases (26.7%), and vascular disorders in 8 cases (6.9%). Since infections and accidents accounted for as much as 66.4%, prevention of their sequelae is important. Of 37 patients (31.9%) with severe motor and intellectual disabilities syndrome (SMIDS), 18 were due to infectious diseases. SMIDS cases accounted for 39.1% of the infectious diseases group, 25% of the non-infectious diseases group, 40% of the iatrogenic group, 25.8% of the accidentrelated group and 12.5% of the vascular group. The prevalence rate of acquired CNS damage was estimated to be 0.5/103/year and that of SMIDS due to acquired damage to be 0.16/10 3/year. A monitoring system for patients with an acquired brain damage and for their treatment is important.View full abstractDownload PDF (1975K) -
Estimated Prevalence Rate in Various FacilitiesToshihiro Horiguchi, Makiko Kaga, Masumi Inagaki2003 Volume 35 Issue 4 Pages 297-303
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSWe assessed the present status of chromosome and gene analyses utilized in Japan for the diagnosis of fragile X syndrome. Nursery facilities for the mentally handicapped and hospitals completed a mail-in-questionnaire including information on the number of patients diagnosed with the syndrome, the types of diagnostic analyses used, and the need for such analyses. Among 517 responders, ninety-five (18.4%, more frequently in hospitals) reportedly conducted chromosome or gene analyses, which established the diagnosis of fragile X syndrome in 56 patients, accounting for 2.2% of patients who underwent the analyses, and 0.13% of the patients included in this study. Each responder's experience with chromosome or gene analyses were related to his/her demand for them. Some facilities that had not utilized these analyses requested information on them as well as access to them. A larger number of specialists interested in diagnostic screening are essential to establish a diagnosis system of fragile X syndrome.View full abstractDownload PDF (992K) -
Shinichiro Hamano, Manabu Tanaka, Mika Mochizuki, Nobuyoshi Sugiyama, ...2003 Volume 35 Issue 4 Pages 304-309
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSWe investigated the efficacy and safety of midazolam given intravenously for the treatment of status epilepticus in children. Patients received one to three bolus injections of midazolam (0.15 to 0.40 mg/kg) until seizures disappeared completely. In other patients, continuous infusion of midazolam (0.06 to 0.48 mg/kg/hr) followed a bolus of midazolam or other anti-epileptic agents. The dose was increased every 15 minutes until the seizures were brought under control. The etiology of 62 episodes of status epilepticus was epilepsy in 43, acute encephalopathy/encephalitis in 11, febrile seizures in 7 and hypoxic encephalopathy in 1. The age of the patients ranged from 0.2 to 18.4 years (average: 3.7 years). Bolus injections were administered in 53 episodes. The average loading dose was 0.35±0.22 mg/kg (range 0.15 to 0.90 mg/kg). Of the 42 episodes in which seizures disappeared, 13 required no further continuous infusions. Of the remaining 29 in which continuous infusion was done at the rate of 0.06 to 0.60 mg/kg/hr (mean 0.30 mg/kg/hr), 21 ended in cessation of the seizures. The duration of continuous infusions ranged from 4 to 288 hours (average 49.0 hours). In 6 episodes there were adverse effects: transient hypoxemia (5) and agitation during withdrawal (1). No intervention was needed except oxygenation by mask for less than 72 hours. Midazolam is effective and safe, and can be used as the first line drug in the treatment of status epilepticus in children.View full abstractDownload PDF (816K) -
Mitsuru Kashiwagi, Takuya Tanabe, Mototada Shichiri, Hiroshi Tamai2003 Volume 35 Issue 4 Pages 310-315
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSChildren who present delirium associated with high fever may develop with encephalitis or encephalopathy, especially in influenza infection. The aim of this study is to differentiate the patients with the central nervous infection or with the parasomnias from benign transient delirium in patients who visit the emergency room complaining of illusions.
Ten patients aged from 2 to 7 years were enrolled in this study. There were 2 patients with central nervous infection, one with encephalo-myelitis due to mycoplasma infection and one with acute necrotizing encephalopathy due to influenza infection. The remaining 8 patients had benign delirium associated with high fever which disappeared in a self-limiting manner. Three patients had a febrile seizure (FS) and 4 patients had family history of FS. The points to differentiate the delirium with parasomnias from benign type is fearful expression, positive past history, autonomic nerve symptoms. Delirium consisted of visual hallucination, and occurred in association with sleep except in the patients with encephalopathy who became delirious when they were awake. Abnormal neurological findings such as meningeal signs and disturbed consciousness, appearance of delirium in the waking state, and marked slowing in the EEG background activity were considered to be warning factors useful in differentiating the benign type from the delirium with central nervous infection.View full abstractDownload PDF (723K) -
Yosikazu Nakamura, Kazuie Iinuma, Eiji Oka, Kenji Nihei2003 Volume 35 Issue 4 Pages 316-320
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSIn 1999, clinical data of 125 patients with subacute sclerosing panencephalitis (SSPE) were obtained by the Research Committee from local prefectural governments. The data were made by physicians treating the patients, and were submitted to the governments when the patients applied for the aid. By analyzing the data, we observedthe epidemiologic features of the disease in Japan, and discussed the availability of the data as the source of epidemiologic researches. Of the 125 patients, 66 were males and 59 were females. The distribution of age at onset had a peak in 5-14 years of age with the average of 10.3 years. Among the 109 cases in which the time of infection was obvious, more than 80% suffered from measles before 2 years of age, in agreement with the hypothesis that measles infection in young age is a risk factor of SSPE. The interval between measles and the onset of SSPE was between 5 and10 years in most cases, with average of 8.8 years, median of 4.3 years, ranging from 2 months to 23.6 years. Because the data contain some problems, we have to observe the epidemiologic features of SSPE in Japan based on multiple data sources including this one, considering their advantages and disadvantages.View full abstractDownload PDF (589K) -
Akemi Tomoda, Keiko Nomura, Seiji Shiraishi, Teruhisa Miike, Akinobu H ...2003 Volume 35 Issue 4 Pages 321-326
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSA survey was made on 10 patients with subacute sclerosing panencephalitis (SSPE) during the last four years from the viewpoint of clinical safety of ribavirin therapy in Japan. Although the age of onset, latent period, and effec-tiveness were variable among the cases, they were treated safely with intraventricular ribavirin combined with high doses of interferon (IFN). Their clinical stages on admission ranged from the first to fourth of Jabbour's classification, and those on the beginning of ribavirin therapy also ranged from the first to fourth. Seven of them were first treated with intraventri-cular IFN-α monotherapy, however, the combination of intraventricular IFN-α and ribavirin was started after clini-cal worsening. Although 5 patients showed slow progression, seven responded to the therapy with clinical improve-ment or decreased measles antibody titers in the CSF. Especially, one patient showed improvement to Jabbour's first stage and no further progression during the following 3 years. Ribavirin therapy caused no severe effects. Lip swelling (50%), sleepiness (40%), and headache (30%) were noted. Although we were unable to perform a double-blinded clinical study for ethical reasons, our results suggest that treatment with intraventricular ribavirin and high doses of IFN may be effective for SSPE. Early administration of intraventricular ribavirin and IFN might be considered especially to IFN in non-responders. To establish this combi-nation therapy as a safe and effective treatment of SSPE, further studies are necessary on the role of ribavirin in the pathogenesis as well as its effects in the central nervous system.View full abstractDownload PDF (810K) -
High-dose Intravenous Immunoglobulin Therapy in a Child Case of Bickerstaff's Brainstem EncephalitisNobuyoshi Sugiyama, Shinichiro Hamano, Michiaki Koga2003 Volume 35 Issue 4 Pages 327-330
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSWe report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT 1 a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset.
Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barre syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.View full abstractDownload PDF (541K) -
Mika Matsui, Akemi Tomoda, Yoshinobu Otani, Teruhisa Miike, Yoshifumi ...2003 Volume 35 Issue 4 Pages 331-335
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSTwo adolescents with complex regional pain syndrome (CRPS) were treated safely and effectively by spinal cord stimulation (SCS). They complained of intractable pain resistant to conservative therapies. Whereas continuous epidural anesthesia temporarily reduced pain, SCS was more effective in alleviating chronic severe pain and improving the quality of life. With careful selection of patients, SCS therapy might be recommended even in young cases.View full abstractDownload PDF (4709K) -
Miki Inutsuka, Tatsuya Ogino, Harumi Yoshinaga, Yoko Ohtsuka, Eiji Oka2003 Volume 35 Issue 4 Pages 336-341
Published: July 01, 2003
Released on J-STAGE: December 15, 2011
JOURNAL FREE ACCESSWe report a 4-year-old boy with ictal fear as his primary epileptic manifestation. Following an arrest of motion, the boy started to scream and struggle with an expression of horror on his face. Oral automatisms appeared around the end of the seizure. Complex visual and gustatory hallucinations and pain in the left leg were also observed. Ictal and interictal scalp EEGs revealed epileptic discharges in bilateral frontal regions. Ictal SPECT (99 mTc-HMPAO) showed hyperperfusion in right medial temporal area. These findings suggest that ictal fear associated with other ictal manifestations such as various hallucinations and oral automatisms resulted from rapid spread of epileptic discharges from frontal lobes to the right anterior temporal region.View full abstractDownload PDF (3607K) -
Tomohiro Kumada, Kozo Mutoh, Masakazu Sakaguchi, Keiji Murata2003 Volume 35 Issue 4 Pages 342-346
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESSAn 11-year-old boy gradually developed headache, vomiting and diplopia over a period of 1 month. Repeated examinations of head CT scan revealed an arachnoid cyst in the right middle cranial fossa and bilateral subdural effusion of enlarging size. Papilledema was absent on admission, but it became evident after 1 week, and lumbar puncture disclosed very high pressure (800 mmH2O) of the cerebrospinal fluid. Fenestration of the cyst to the basal cisterns quickly alleviated his symptoms of intracranial hypertension as well as the bilateral subdural effusion on CT. Macroscopically, there was a small tear on the wall of the arachnoid cyst, and it probably served as a communication valve with the subdural space. Since he had no history of head trauma in the past few months, the reason of the tear formation was unclear. Intracranial arachnoid cyst is a relatively common congenital malformation of usually benign and non-pathogenic nature. However, it may occasionally cause non-traumatic subdural effusion and intracranial hypertension.View full abstractDownload PDF (5590K) -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...2003 Volume 35 Issue 4 Pages 347-349
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese]2003 Volume 35 Issue 4 Pages 349-352
Published: July 01, 2003
Released on J-STAGE: December 12, 2011
JOURNAL FREE ACCESS
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