Abstract:
:Two boys developed rhythmic involuntary movements in the extremities on one side of the body after febrile illness. They also showed behavioral disturbances. In both patients, serum antistreptolysin-O and antistreptokinase titers were elevated in acute illness and decreased a few months later. One patient showed tremorous movement, and the other choreiform movement. In the former, a surface EMG showed short-duration (30 to 60 ms), highly frequent (6 to 8 Hz) and synchronous discharges of multiple muscles, including the antagonists, suggesting myoclonic jerk. In the latter, a surface EMG showed long-duration (0.5 to 1 s), repetitive (about 0.5 Hz) and synchronous or asynchronous discharges of the antagonists, suggesting choreoathetosis. In both patients, giant somatosensory evoked potentials and high-voltage slow EEG activities were observed predominantly in the hemisphere contralateral to the involuntary movement. In the myoclonic patient, long-latency EMG responses were enhanced and cortical potentials preceding the myoclonus were present by jerk-locked back averaging technique. The present data suggest that unilateral rhythmic involuntary movements occur secondary to streptococcal infection. The pathophysiology of the involuntary movements may be associated with sensorimotor cortex hyperexcitability.
journal_name
Neuropediatricsjournal_title
Neuropediatricsauthors
Maegaki Y,Akaboshi S,Inagaki M,Takeshita Kdoi
10.1055/s-2000-7476subject
Has Abstractpub_date
2000-04-01 00:00:00pages
70-4issue
2eissn
0174-304Xissn
1439-1899journal_volume
31pub_type
杂志文章相关文献
NEUROPEDIATRICS文献大全abstract::Incidence of sudden death in Rett syndrome is greater than that of the general population and cardiac electrical instability is a prime suspect cause. Our study shows that girls with Rett syndrome had significantly lower heart rate variability (marker of autonomic disarray) and longer corrected QT intervals compared w...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973480
更新日期:1999-06-01 00:00:00
abstract::The response of kidney and bone to parathyroid extract (PTE) was investigated in 8 epileptic children on long-term treatment with primidone in combination with phenytoin or other anticonvulsant drugs. The results indicate a dissociation between normal and cyclic AMP excretion and disturbed renal handling of phosphate ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1059588
更新日期:1982-02-01 00:00:00
abstract::Etiological factors and long-term prognosis were studied in 562 cases with convulsive disorders in the first year of life; 114 (20.3%) were prenatal, 114 (20.3%) perinatal, 24 (4.3%) postnatal, and 257 cases (45.7%) were cryptogenic. The remaining 53 (9.4%) patients were doubtful cases. The mortality before six years ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1059584
更新日期:1983-11-01 00:00:00
abstract::Two distinct disorders with elevated urinary excretion of 2-hydroxyglutaric acid are known: L-2-hydroxyglutaric aciduria and D-2-hydroxyglutaric aciduria. This paper presents clinical and biochemical studies in three patients and unsuccessful prenatal diagnosis in one case with combined D-2- and L-2-hydroxyglutaric ac...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2000-7497
更新日期:2000-06-01 00:00:00
abstract::Two patients with insular and striatal postnatal scar had epileptic spasms (ES) that were asymmetrical and the only seizure type, whereas none of the usual ictal symptoms of insular seizures occurred. Ictal electroencephalography (EEG) showed the high-amplitude slow-wave characteristic of ES. Vigabatrin remained effic...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0040-1702226
更新日期:2020-08-01 00:00:00
abstract::Gene expression in the central nervous system is highly region-specific. We tested the hypothesis that certain developmental biomarkers could be detected in the whole brain or in cortical, subcortical or cerebellar structures. Brain gene expressions of male and female rats at birth, 3 days, and 10 days of age were mea...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0028-1105923
更新日期:2008-08-01 00:00:00
abstract::Activin A is a multifunctional growth and differentiation factor belonging to the transforming growth factor β (TGF-β) family. Growing evidence indicates its role as a neurotrophic factor and regulator of synaptic transmission as well as its functional importance in several types of cerebral injury. We recently descri...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0035-1547345
更新日期:2015-04-01 00:00:00
abstract::Intracerebral hemorrhage (ICH) is the most frequent complication in postnatal development of preterm infants. The purpose of the present work is the statistical evaluation of seven standard paraclinical parameters and their association to the development of ICH. Clinical records of 265 preterm infants with gestational...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0038-1677515
更新日期:2019-04-01 00:00:00
abstract::A Japanese girl showed deterioration in development from the age of 13 months. At the age of 16 months, there were mild spastic diplegia, increase in cerebrospinal fluid protein to 61.5 mg/dl and deficient galactosylceramidase I. Magnetic resonance imaging (MRI) demonstrated a high signal intensity with increased T2 i...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1052476
更新日期:1987-08-01 00:00:00
abstract::In order to determine the relationship between visual functions and neonatal cerebral ultrasound, neurological examinations and cognitive development, a prospective longitudinal study was conducted in 69 high-risk very-low-birthweight children. Visual development was studied at 1 and 2.6 years of corrected age by asse...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071533
更新日期:1993-06-01 00:00:00
abstract:BACKGROUND:In Rett syndrome the autonomic nervous system is abnormal at various levels, from the central to the peripheral nervous system. A role for serotoninergic dysfunction has been suggested. OBJECTIVES:The aim of our study was to evaluate the relation between cardiac dysautonomia (expressed by means of heart rat...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2004-815789
更新日期:2004-02-01 00:00:00
abstract::Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder associated with typical clinical and imaging features such as bilateral symmetrical polymicrogyria, either exclusively or mainly affecting the perisylvian region of the brain. We present a girl with the typical clinical picture of a CBPS ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0040-1710588
更新日期:2020-12-01 00:00:00
abstract::Congenital or acquired disorders of hemostasis can cause thrombotic events in adults as well as in children. Recently, acute neurologic complications such as hemiparesis or transitory ischemic attacks (TIA) have been reported in patients with different disorders of hemostasis. In addition, the interaction between anio...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1071573
更新日期:1994-02-01 00:00:00
abstract::The clinical and some laboratory details of three children who had severe neurological sequelae after either infection with Bordetella pertussis or immunisation with diphtheria, tetanus and pertussis vaccine and oral polio vaccine are reported. Each of these patients had had a recent or concurrent viral illness. The s...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1059668
更新日期:1981-11-01 00:00:00
abstract::Positron Emission Tomography (PET) with 2-deoxy-2 [18F]-fluoro-D-glucose provides a measure of functional brain activity, particularly in the dendritic field. In CLN3 (juvenile neuronal ceroid lipofuscinosis or juvenile Batten disease, with fingerprint inclusions) hypometabolism slowly spreads from calcarine to anteri...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2007-973675
更新日期:1997-02-01 00:00:00
abstract::Two children with biotinidase deficiency presented with seizures at 2 months of age. The first child had a fluctuating course with continual developmental progress and cessation of seizures despite symptoms of chronic neurologic dysfunction until he was diagnosed at 17 months. The second child had a progressive course...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071543
更新日期:1993-08-01 00:00:00
abstract:BACKGROUND:Sleep is known to improve the yield of EEG recording in children but is often difficult to obtain. In order to evaluate the efficacy and to test the practicability of oral melatonin in obtaining sleep for EEG recording, we studied its use in 70 children. RESULTS:Sleep was obtained in 56 children (80%) with ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0030-1267920
更新日期:2010-08-01 00:00:00
abstract::134 preterm infants were investigated in their ability to coordinate sucking and breathing. Of those infants who did not coordinate, 79% showed immature cardiorespiratory control whereas of those who suck and breathe simultaneously only 12% had poor cardiorespiratory control. This finding suggests that in infants with...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071449
更新日期:1990-02-01 00:00:00
abstract::We report on a child with Chudley-McCullough syndrome and re-evaluate the spectrum of imaging findings (in 15 previously reported patients) which appear to be variable and, to some extent, ambiguous in the literature. Magnetic resonance imaging of the brain revealed asymmetric colpocephaly with agenesis of the spleniu...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-0032-1307451
更新日期:2012-02-01 00:00:00
abstract::Data coming from a prospective multidisciplinary study with repeated examinations of children with fetal alcohol syndrome (FAS) are reported. These patients underwent pediatric, neurological and psychiatric assessment, EEG-recordings and psychological testing. After a period of 3-4 years various subgroups of these chi...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1052428
更新日期:1987-02-01 00:00:00
abstract::Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features: no presence of se...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1071490
更新日期:1990-11-01 00:00:00
abstract::Epidermal nevus syndrome (ENS) is a congenital neurocutaneous disorder characterized by linear nevus with a significant involvement of the nervous, ophthalmological and skeletal systems. Clinical manifestations of ENS include neurological features such as mental retardation, seizures, and movement disorders which are ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2000-7457
更新日期:2000-08-01 00:00:00
abstract:AIM: Tonic tics (TTs) are a part of a clinical picture of Gilles de la Tourette syndrome (GTS) and manifest themselves as sustained and isometric contraction of a muscle group devoid of the movement effect or accompanied by only slight visible motion. The aim of this study was to evaluate the prevalence and phenomenolo...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0040-1722689
更新日期:2021-01-14 00:00:00
abstract:BACKGROUND:Hereditary spastic paraplegia is a neurodegenerative phenotype characterized by a progressive loss of corticospinal motor tract function. In a majority of affected individuals the pathogenesis remains undetermined. METHODS:We identified a series of patients with a phenotype of nonsyndromic spastic paraplegi...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0034-1389161
更新日期:2014-12-01 00:00:00
abstract::Primary degeneration of the granular layer of the cerebellum is an autosomal recessive disorder exhibiting characteristic clinical features: hypotonia, strabismus, delayed motor development, nonprogressive ataxia, delayed language development with dysarthria and mental retardation. We studied fourteen children, seven ...
journal_title:Neuropediatrics
pub_type: 杂志文章,评审
doi:10.1055/s-2008-1073020
更新日期:1994-08-01 00:00:00
abstract::Forty-four patients aged from one month to 16 years suffering from arterial stroke were carefully studied for any hereditary and acquired risk factors for stroke. No physiologic anticoagulant deficiency or antiphospholipid syndrome was found. Two patients had mitochondrial disease (MELAS). Six patients had migraineous...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2008-1073026
更新日期:1994-10-01 00:00:00
abstract::Alexander disease (AD) is a leukodystrophy caused by heterozygous mutations in the gene encoding the glial fibrillary acidic protein (GFAP). Currently, de novo heterozygous missense mutations in the GFAP gene are identified in over 95% of patients with AD. However, patients with biopsy-proven AD have been reported in ...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0037-1608921
更新日期:2018-04-01 00:00:00
abstract::Mutations of the SCN2A gene have originally been described in association with benign familial neonatal-infantile seizures (BFNIS). Recently, single patients with more severe phenotypes and persisting epileptic encephalopathies have been recognized. We report the case of a girl with severe infantile onset epileptic en...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0034-1372302
更新日期:2014-08-01 00:00:00
abstract::Hashimoto's encephalopathy (HE) is an acute or subacute relapsing disorder usually affecting euthyroid patients with evidence of autoimmune thyroiditis. The neurological manifestations are non-specific, with subacute cognitive impairment, movement disorders, generalized seizures, focal neurological symptoms such as st...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-2005-865712
更新日期:2005-06-01 00:00:00
abstract::Purpose In periventricular leukomalacia (PVL), apparent diffusion coefficient (ADC) reduction, normally shown as dark stripe in the peritrigonal (PT) white matter, may be incomplete. We assessed the PT dark stripe to differentiate between PVL patients and control subjects. Patients and Methods We reviewed the magnetic...
journal_title:Neuropediatrics
pub_type: 杂志文章
doi:10.1055/s-0036-1597613
更新日期:2017-04-01 00:00:00