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Steven Shinn-Forng Peng

Publications and source records attributed to Steven Shinn-Forng Peng.

10 recordsLinked to original sources
PubMed2026

Long-term seizure outcomes and factors associated with response to adjunctive everolimus in TSC-associated epilepsy.

BACKGROUND: Everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, is increasingly used in tuberous sclerosis complex (TSC)-associated epilepsy; however, long-term real-world outcomes and factors associated with favorable response remain unclear. This study aimed to evaluate the long-term seizure outcomes of adjunctive everolimus and explore clinical factors associated with treatment response. METHODS: We retrospectively recruited 21 patients with active TSC-associated epilepsy receiving adjunctive everolimus and assessed seizure outcomes during follow-up. Clinical characteristics were compared between responders and non-responders at 1 year after treatment initiation. RESULTS: Over a median treatment duration of 72 months, responder rates ranged from 53.8% to 64.7%, and seizure-free rates ranged from 33.3% to 41.2%. Responders had fewer involved organ systems at baseline (median 3 vs. 4, p = 0.020) and lower anti-seizure medication burden (median 2 vs. 4, p = 0.045). Younger age at treatment initiation showed a trend toward improved response. CONCLUSION: Adjunctive everolimus was associated with sustained long-term seizure reduction in this real-world cohort. In exploratory analyses, fewer involved organ systems and fewer baseline ASMs were associated with favorable treatment response. These findings require validation in larger prospective cohorts.

Epilepsy↗
PubMed2005

Persistent pulmonary hypertension in a neonate with vein of Galen arteriovenous malformation.

Vein of Galen aneurysmal malformation (VGAM) often leads to death in the neonatal period, mainly due to congestive heart failure. Chronic and excessive pulmonary flow in utero and postnatally is attributed to large VGAM and right ventricular overload. We report a male neonate with VGAM complicated by severe heart failure and persistent pulmonary hypertension. Endovascular coil embolization of VGAM was performed, resulting in improvement of congestive heart failure; however, severe persistent pulmonary hypertension led to death 2 days after the embolization. Postmortem examination showed marked right and left ventricular hypertrophy and impressive muscular thickening of intra-alveolar arterioles. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension. Therefore, early delivery and repair of VGAM should be considered before the onset of persistent pulmonary hypertension.

Humans↗
PubMed2004

Cerebral diffusion tensor images in children with tuberous sclerosis: a preliminary report.

BACKGROUND: In tuberous sclerosis (TS), tubers usually involve the white matter. Diffusion tensor (DT) images are used to demonstrate white-matter tracts. OBJECTIVE: To determine the changes in DT indices in supratentorial tubers and associated changes in the white-matter tracts adjacent to tubers in patients with TS. MATERIALS AND METHODS: The DT imaging indices, including first, second and third eigenvalues (EVs), apparent diffusion coefficients (ADCs), and fractional anisotropy (FA) in the white-matter lesions of tubers, were assessed in seven patients with TS exhibiting developmental delay and compared with controls. RESULTS: EV1, EV2, EV3, ADC and FA of the white-matter lesions of tubers were significantly different from contralateral unremarkable regions of the brain and from controls (P<0.05). The number of frontal and parietal tubers was significantly negatively correlated with EV1 of the superior longitudinal fasciculi of TS patients (r=-0.60, P =0.04). In addition, TS patients had significantly larger ADCs in the corona radiata and sagittal stratum than the control subjects. EV3s of the inferior longitudinal fasciculus and sagittal stratum were significantly more increased in the TS patients than in the control subjects. CONCLUSIONS: EV1, EV2, EV3, ADC and FA maps are potential tools for demonstrating cerebral white-matter changes owing to TS.

Adolescent↗
PubMed2004

Diffusion tensor images in children with early-treated, chronic, malignant phenylketonuric: correlation with intelligence assessment.

BACKGROUND AND PURPOSE: Diffusion tensor (DT) images can provide information about the nature of white matter changes, including axonal loss and demyelination. We applied DT imaging to verify white matter changes in patients with malignant phenylketonuria (PKU) and to correlate the findings with clinical intelligence quotients (IQs). METHODS: We compared DT images with T2-weighted images in 12 patients with early-treated, chronic, stable malignant PKU and 12 age-matched control subjects. DT parameters included first, second, and third eigenvalues (EV1-3), apparent diffusion coefficients (ADCs), and fractional anisotropy (FA). Regions of interest were placed the frontoparietal, parieto-occipital, frontal and central white matter and in the anterior and posterior corpus callosum. Eight patients older than 3 years underwent IQ assessment including verbal, performance, and full-scale IQ tests. RESULTS: In the eight patients older than 3 years, no definite abnormal signal intensity changes were found on T2-weighted images. EV2, EV3, and FA of the parieto-occipital white matter were significantly different in patients and control subjects older than 3 years. EV3 and ADC of the parieto-occipital white matter were significantly and negatively correlated with verbal IQ (r = -0.79, P = .04) and performance IQ (r = -0.93, P = .03). FA of the parieto-occipital central white matter was positively correlated with verbal IQ (r = 0.75, P = .05). CONCLUSION: Though treated early, patients with chronic, stable malignant PKU had abnormal DT findings in the parieto-occipital central white matter. EV2, EV3, and FA maps are potential tools for demonstrating brain changes due to malignant PKU.

5-Hydroxytryptophan↗
PubMed2002

Mesenteric lipoblastoma: case report.

We present a rare case of mesenteric lipoblastoma in a 16-month-old girl. The US, CT and MRI features of this unusual tumour are described and correlated with the pathology findings. MRI more clearly suggested the presence of fat components in the tumour. In addition, multiplanar MR images demonstrated the anatomical extent better, which was essential for successful complete tumour excision.

Female↗
PubMedDate not supplied

Diffusion-weighted images in children with meningoencephalitis.

PURPOSE: The objective of the study was to evaluate the maps of apparent diffusion coefficients (ADCs) and diffusion-weighted (DW) images in demonstrating meningoencephalitic lesions in children. MATERIALS AND METHODS: Between May 1998 and May 2000, 18 infants and children (4.5-190 months old) suffering from meningoencephalitis were included in the study. The diagnoses were bacterial meningoencephalitis in 8 and aseptic or viral in 10 patients. All 18 patients had brain MRI examinations. In the axial plane, three pulse sequences were performed on all patients: (1) FSE T2W images; (2) fast FLAIR images; (3) single-shot echoplanar DW images were acquired. Another 18 patients from the control group also received DW image examination. ADCs were computed for all regions on each DW image. RESULTS: The absolute values of CNRs of lesions on T2W (7.27+/-5.51), FLAIR (5.56+/-5.03) and DW (13.36+/-16.64) images were significantly greater than those on ADC maps (0.42+/-0.30) in the study group of patients (P<.01). In addition, absolute CNRs on DW images were significantly greater than on T2W and FLAIR images (P<.01). However, lesions on ADC maps in the study group have significantly greater CNRs than in the control group (0.13+/-0.12) (P<.01). CNRs on initial DW images from patients with atrophy or swelling of meningoencephalitic lesions were significantly different from the CNRs of those patients without significant changes in meningoencephalitic lesions (P=.02<.05). CONCLUSION: The DW image is a sensitive tool for detecting meningoencephalitic lesions and is better than FSE T2W and fast FLAIR images in CNRs. Diffusion MR techniques provides new ways to possibly predict the outcome of intracranial infectious diseases in children.

Brain Mapping↗
PubMedDate not supplied

Schizencephaly: correlation between clinical and neuroimaging features.

Schizencephaly is an uncommon congenital brain malformation. We report our experience of 13 patients with schizencephaly and evaluate the clinical, neuroradiologic, electroencephalographic (EEG), and nosological features. Of these 13 patients, 8 were unilateral forms, 5 were bilateral forrms and 11 were open-lip type schizencephaly. One patient was proven to have cytomegalovirus (CMV) infection. The clinicalfeatures and neurodevelopmental outcomes are variable. Although seizure developed in 9 patients (5 patients from unilateral and 4 from bilateral forms), the severity of epilepsy was not totally related to the degree of malformations. The neurodevelopmental outcome depended on the extent of schizencephaly as well as the seizure control. Those with bilateral forms and intractable seizures had the worst outcome. Other central nervous system (CNS) anomalies were observed in 11 patients. Six out of 11 patients had focal cortical dysplasia. We conclude that children with schizencephaly usually have variable neurological impairment. Earlier diagnosis of schizencephaly and related CNS malformation with neuroimaging is helpful in predicting the neurodevelopmental outcomes in these patients.

Adolescent↗
PubMedDate not supplied

Adrenoleukodystrophy: clinical analysis of 9 Taiwanese children.

X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder and identified in many races without apparent predilection for any race. This study was designed to investigate the clinical and therapeutic aspects of X-ALD in Taiwanese children with this disorder. We retrospectively reviewed all children admitted to NTUH from Nov. 1993 to Aug. 2002 with the diagnosis of ALD, defined by increased very long chain fatty acid (VLCFA). The mean age at diagnosis of the patients was 7.4 years (range, 2.8 to 13 years). Seven out of 9 patients had abnormal brain magnetic resonance image (MRI) studies. Three patients received bone marrow transplantation. Of these, two died of severe graft-versus-host disease and the other remained stable. Of the remaining 6 patients, two patients were in vegetative status and the other two patients were neurologically normal. X-ALD in Taiwanese children had similar clinical manifestations as reviewed in western countries. Symmetrical demyelination in parieto-occipital region and the accumulation of contrast material at the edge of the lesion are the typical MRI findings. Proton MR spectroscopy (MRS) can be used to evaluate either the asymptomatic patient or patient with normal brain image. Performance of T-cell depletion bone marrow transplantation or cord blood transplantation is suggested for X-ALD with early cerebral involvement.

Adolescent↗
PubMedDate not supplied

Cerebral infarction associated with possible enteroviral infection in an infant.

Enterovirus infection has been rarely reported to cause cerebral infarction in infants. We describe a 2-month-old boy with right focal seizure and right hemiparesis associated with enterovirus infection during an epidemic of enterovirus 71 infection in Taiwan in 1998. Magnetic resonance imaging and angiography showed vasculitis in the left anterior cerebral artery with cerebral infarction. In the unclarified pathogenesis of cerebral disease in enterovirus infection, this case suggests focal vasculitis with subsequent cerebral infarction. Enterovirus-related vasculitis of the central nervous system is thus another consideration when facing a child with focal seizure, acute hemiplegia and cerebral infarction.

Anticonvulsants↗