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Biomedical subjects

Yung-Jung Chen

Publications and source records attributed to Yung-Jung Chen.

6 recordsLinked to original sources

Clinical characteristics and prognostic factors of postencephalitic epilepsy in children.

The goal of this study was to clarify the clinical characteristics and prognostic factors of childhood postencephalitic epilepsy. Forty-four patients (20 boys and 24 girls; age range 21 months to 17 years, mean age 8.1 +/- 4.6 years) with postencephalitic epilepsy were selected from the 798 epileptic children treated and followed up at our hospital between 1993 and 2003. The clinical data included clinical features, electroencephalograms (EEGs), and neuroimages, all reviewed and analyzed retrospectively. Based on their post-treatment seizure outcomes, the children were divided into favorable (n = 20) and poor outcome groups (n = 24). Between the two groups, the age at encephalitis, cerebrospinal fluid findings, and seizure type were comparable. Factors indicating a poor prognosis for these patients during the acute phase of encephalitis were (1) status epilepticus occurring as the first seizure (P < .005), (2) slow background activity (P < .001) and multifocal spike discharges on EEGs (P < .01), and (3) herpes simplex viral encephalitis (P < .01). Our findings indicated that patients with status epilepticus and multifocal spikes on EEG during acute encephalitis have an increased risk of developing intractable epilepsy. To improve the outcome of postencephalitic epilepsy, intervention must occur earlier in the encephalitis stage.

Action Potentials↗

Sensory evoked potentials in infants with Down syndrome.

AIM: To investigate the sensory functions of the peripheral to central pathways in infants with Down syndrome (DS) by sensory evoked potentials. METHODS: Fifty-five infants, 30 DS infants and 25 controls, were examined by multimodal evoked potentials, including brainstem auditory evoked potentials (BAEP), visual evoked potentials (VEP) and short-latency somatosensory evoked potentials (SSEP). RESULTS: No obvious difference was found in the peak latencies between the two groups for BAEP. Nine children with DS showed abnormal BAEP; six had hearing loss and three had prolonged wave I latencies. For VEP, the peak latencies of P(2) and N(2) were significantly longer and the amplitudes were smaller in the DS group than in the control group. Of the 30 infants with DS, five had significantly prolonged P(2) latencies and two had lower amplitudes. In SSEP, the mean latencies of N(20) and the interpeak latencies of N(13)-N(20) of the infants with DS showed apparent prolongation compared to the controls. Seven of 30 (23.3%) DS patients had prolonged N(20) latencies. CONCLUSION: Our results indicate that various sensory deficits occur in patients with DS during the first year of life.

Analysis of Variance↗

The auditory performance in children using cochlear implants: effects of mental function.

OBJECTIVE: Mental function is considered to affect the post-operative outcomes of deaf children with cochlear implants. The purpose of this study is to evaluate the effect of mental function on the auditory performance in children with cochlear implants. METHODS: In a retrospective review of data, 26 pre-lingual deafened children received pre-operative evaluation of mental function and were divided into normal and retarded groups. Categories of auditory performance scale (CAP) was conducted at 1-year intervals after implantation. ANCOVA was used for statistic analysis. RESULTS: The average scores of auditory performance in normal group (n=14) were 3.93 (S.D. 1.07) and 5.86 (S.D. 0.95) at 1- and 2-year post-implantation. While the average scores in the retarded group were 2.5 (S.D. 1.51) and 4.17 (S.D. 1.85), both groups demonstrated obvious improvement in speech perception (F 103.12, P<0.001) during the first 2 post-operative years. The auditory performance in the normal group was significantly superior to the retarded group (F 8.67, P<0.01). However, the interaction between the duration of the device use and mental status showed no significant difference in the auditory perception performance (F 1.575, P=0.222). CONCLUSIONS: The results revealed the mental function plays as one of the predictive parameters of auditory performance in profoundly hearing impaired children after cochlear implantation.

Child↗

Cardiopulmonary manifestations of fulminant enterovirus 71 infection.

BACKGROUND: The pathogenesis of acute pulmonary edema and cardiac collapse after enterovirus 71 (EV71) infection are not completely understood. OBJECTIVE: To determine the hemodynamic features and the mechanism of pulmonary edema (PE) after EV71 infection by direct intracardiac monitoring. DESIGN: Prospective clinical and laboratory study at a tertiary medical center. PARTICIPANTS: Five consecutive infants, ages 2 to 13 months, with EV71 infection-proved by viral isolation in 4 and antibody in 1-with PE were enrolled. The clinical characteristics were systemically assessed. Hemodynamic profiles were determined every 4 hours by simultaneously implanted pulmonary arterial and central venous catheters during the acute stage. RESULTS: Magnetic resonance imaging revealed that all 5 infants had brainstem lesions. All patients had tachycardia and hyperthermia. Transient systolic hypertension was noted in 1 patient, and 1 presented with hypotension. Pulmonary artery pressure in all 5 infants was normal or mildly elevated (26-31 mm Hg), and central venous pressure ranged from 10 to 22 mm Hg. Pulmonary artery occlusion pressures were normal or slightly elevated (13-16 mm Hg). Systemic and pulmonary vascular resistances were transiently increased in only 1 patient. The stroke volume index decreased to 15.3 to 35.7 mL/M2 (normal: 30-60 mL/M2), but because of the elevated heart rate, the cardiac index did not decrease. All hemodynamics normalized within days. CONCLUSION: Fulminant EV71 infection may lead to severe neurologic complications and acute PE. The acute PE and cardiopulmonary decompensation in EV71 infection are not directly caused by viral myocarditis. The mechanism of PE may be related to increased pulmonary vascular permeability caused by brainstem lesions and/or systemic inflammatory response instead of increased pulmonary capillary hydrostatic pressure.

Brain Diseases↗

Referral diagnosis of Prader-Willi syndrome and Angelman syndrome based on methylation-specific polymerase chain reaction.

BACKGROUND AND PURPOSE: Prader-Willi syndrome (PWS) and Angelman syndrome (AS) are associated with distinct phenotypes that include mental retardation. Both PWS and AS are caused by loss of function of genes located in chromosome 15q11-q13, an area subject to genomic imprinting. Methylation-specific polymerase chain reaction (M-PCR), based on parent of origin specific DNA methylation at the promoter region of the small nuclear ribonucleoprotein polypeptide N gene (SNRPN), can provide accurate and rapid diagnosis for nearly all PWS patients while it is less accurate for AS patients. We report the development of a referral system for molecular diagnosis of PWS and AS based on M-PCR. METHODS: Pediatric geneticists, psychiatrists, or neurologists were asked to evaluate phenotypes of patients with PWS or AS and complete a questionnaire designed according to the consensus criteria to diagnose these conditions. Molecular analysis based on M-PCR was performed for patients with a score of at least two. RESULTS: A total of 108 patients with suspected PWS and 20 patients with suspected AS were referred for diagnostic testing. PWS was diagnosed in 26 of these patients and AS in two. Among the major diagnostic criteria for PWS, excessive weight gain, developmental delay, and hyperphagia were more prevalent in older patients (> or = 1 yr) than in younger patients. Cerebral hypotonia and developmental delay were significantly more prevalent in older PWS patients than in non-PWS patients. CONCLUSION: M-PCR is a cost-effective method for the diagnosis of PWS and AS. The limitations of current scoring systems and the low cost of M-PCR suggest that routine molecular screening is justified for patients suspected of having PWS.

Adolescent↗

Neurophysiological studies in a case of Sjögren-Larsson syndrome.

We describe a 4-year-old boy with Sjögren-Larsson syndrome (SLS). Clinical neurophysiological studies, including electroencephalography (EEG) and brainstem auditory evoked potential (BAEP), visual evoked potentials (VEP), and short-latency somatosensory evoked potential (SSEP) tests, were performed. The results of multimodality evoked potential tests suggested that SLS may involve dysfunction of various sensory pathways.

Child, Preschool↗