Huei-Shyong Wang

Chang Gung Memorial Hospital, T’ai-pei, Taipei, Taiwan

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Publications (96)131.9 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: In this retrospective study, we collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of seizure after ischemic stroke for the period of January 1996 to July 2006. Thirty-nine out of 94 children with ischemic stroke had poststroke seizures. Thirty-three out of 39 children with poststroke seizures had new onset seizures but only data of 28 were available. Infection was the most common etiology in the early poststroke seizure group (52.4%) but not in the late poststroke seizure group (0%). Infarction involving arterial ischemic stroke of anterior circulation were the most common in both the early poststroke seizure (61.9%) and the late poststroke seizure group (57.1%). Epilepsy was the most common sequelae in both the early poststroke seizure (38.1%) and late poststroke seizure group (100%). Children who had initial focal neurological sign (100% vs. 38.1%; P=0.007) or the focal cortical dysfunction on EEG (85.7% vs. 33.3%; P=0.029) were prone to develop late poststroke seizures. Late poststroke seizures had a high risk of developing poststroke epilepsy (100% vs. 38.1%; P=0.007). We conclude that seizures commonly occur in childhood ischemic stroke. Most poststroke seizures developed at an early stage. Infection was the most common etiology that caused early poststroke seizures in childhood ischemic stroke. Initial focal neurological signs and focal cortical dysfunction on EEG are risk factors for developing epilepsy. Poststroke seizures did not affect mortality, but there was a significant difference in normal outcome and epilepsy between those with or without poststroke seizures.
    Brain & development 04/2009; 31(4):294-9. · 1.74 Impact Factor
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    ABSTRACT: Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.
    Pediatric Neurology 03/2009; 40(2):84-7. · 1.50 Impact Factor
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    ABSTRACT: The authors discuss the current management for cerebellopontine angle (CPA) tumors in children. CPA tumors accounted for 1% to 3% of intracranial tumors in children. There had been much controversy with the management of these tumors. A total of 29 eligible patients were enrolled to the study and 5 patients had multiple lesions at diagnosis. Eight patients with tumors exclusively confined in the CPA. Sixteen patients with tumors occurred predominantly within CPA and 5 arising from the vicinity and growing mainly into the CPA. Twelve tumors were located in the right CPA (41%) and 5 (17%) on the left. Thirteen of the 29 patients developed hydrocephalus and 3 required placement of a shunt. Lesions of the CPAs were divided into those native to the angle and those extending to the angle from adjacent structures. Gross total removal was achieved in 9 cases, subtotal in 14, and 2 had biopsies only. Four patients were diagnosed with pontine glioma solely by magnetic resonance imaging without histologic confirmation. Two died soon after the operation. Ten patients died with a mortality rate of 34.5%. The median follow-up in this study was 38 months (range: 4 to 225 mo). The CPA is a rare location for lesions in children, with clear predominance on the right side. Although low-grade lesions are more frequent, the histology varies widely and is limited by the lack of radiologic-pathologic correlation.
    Journal of Pediatric Hematology/Oncology 01/2009; 31(11):832-834. · 0.96 Impact Factor
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    ABSTRACT: Tuberous sclerosis complex is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of signs and clinical features. Occlusive vascular disease and aneurysms of the cerebral and visceral arteries were described in these patients. We report on an 8-month-old boy who manifested tuberous sclerosis complex with multiple intracranial aneurysms involving the internal carotid artery and middle cerebral artery. The unusual features of our case favor the concepts of the presence of an arterial dysplasia in tuberous sclerosis complex, and of developmental defects as the cause of intracranial aneurysms.
    Pediatric Neurology 12/2008; 39(5):365-7. · 1.50 Impact Factor
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    ABSTRACT: Acute encephalitis with refractory repetitive partial seizure (AERRPS) is a peculiar type of post-encephalitic/encephalopathic epilepsy. Here we report an analysis of AERRPS in a series of children and propose an effective treatment option for seizure control in these children. We retrospectively reviewed cases of AERRPS treated in a pediatric intensive care unit, between February 2002 and June 2006. Clinical characteristics were systemically assessed. Burst suppression coma was induced by high-dose suppressive therapy; 24-h electroencephalogram (EEG) monitoring was performed on each patient. The goal of treatment was to achieve complete clinical seizure control or burst-suppression pattern on EEG, aiming for an interburst interval of >5s. Brain imaging was done for each patient. There were nine patients (seven boys), aged 5-15 years. Clinical symptoms included fever (100%), upper respiratory symptoms (66.7%) and altered consciousness (66.7%). All patients received multiple high-dose suppressive drugs and were intubated with/without inotropic agents. Seizures in three patients were stopped after high-dose lidocaine infusion (6-8 mg/kg/h) in the acute stage and three patients were stopped after high dose phenobarbital (serum level 60-80 ug/mL) combined with high-dose oral topiramate (15-20 mg/kg/day). Follow-up for this study was 16-61 months. Two subjects died while seven developed epilepsy and/or neurologic deficits; none returned to baseline. All survivors were discharged and continued multiple antiepileptic medications. Our data indicates that children with AERRPS have high mortality and morbidity rates. High-dose topiramate combined with high-dose lidocaine infusion or high-dose phenobarbital in the acute stage might be an effective treatment option for children with AERRPS.
    Brain & development 11/2008; 31(8):605-11. · 1.74 Impact Factor
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    ABSTRACT: To delineate the relationship between neurological severity and neuroimage of lesion load including specific topography of supratentorial cortical tubers and white matter lesions in tuberous sclerosis complex (TSC). Twenty-five TSC patients more than 2 years of age who underwent conventional and fluid-attenuated inversion recovery sequence (FLAIR) magnetic resonance imaging (MRI) were retrospectively studied. Neurological severity score was designated for three items: seizure, developmental delay and/or mental retardation, and autism. A neuroimaging scoring system was designed to evaluate the load of the cerebrum lesions with respect to location and size of cortical tubers and white matter lesions based on FLAIR MRI. A linear trend was observed between MRI lesion score and neurological severity score (r=0.511; p=0.009). The lesion score in the left temporal lobe has positive correlation to neurological severity score (r=0.609; p=0.001). The brain lesion load was positively correlated with neurological prognosis in TSC patients. Patients with larger lesion load in the left temporal lobe may be correlated with increased neurological severity in right-handed patients with TSC.
    European Journal of Paediatric Neurology 04/2008; 12(2):108-12. · 1.93 Impact Factor
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    ABSTRACT: In this retrospective study, we reviewed the charts and collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of ischemic stroke for the period of January 1996 to July 2006. Ninety-four children were enrolled. Eighty-eight had arterial ischemic stroke and six had sinovenous thrombosis. Twenty-nine percent of the children had seizures. Twenty-six percent had diffuse neurological signs and 76% had focal neurological signs. Risk factors included vascular disease (33%), infection (27%), metabolic disorders (18%), trauma (11%), prothrombotic states (13%), cardiac disease (10%), and mitochondrial disease (6%). Ten percent (n=9) had no identifiable cause. Twenty-two percent of the children had more than one risk factor. Anterior territory (70%) was more involved than posterior territory (18%) in arterial ischemic stroke. Unilateral infarctions were more common on the left side (51%) than on the right (24.5%). Neurological deficits were present in 45% (n=34/75) of the children; the most frequent deficit was motor impairment (24%). Seven children (9%) died in the acute stage. There were 12 children (16%) who had recurrent stroke and 8 children (8/12) who had underlying vascular disease. The vascular disease included moyamoya disease (5), CNS lupus (1) and ill-defined vasculopathy (2). The etiology pattern in Taiwan was different from that in Western countries. Vascular disease was a significant risk factor for recurrence in childhood ischemic stroke.
    Brain and Development 02/2008; 30(1):14-9. · 1.54 Impact Factor
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    ABSTRACT: Encephalitis is an acute infection of brain parenchyma characterized clinically by fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. Here we report an analysis of status epilepticus (SE) related presumed encephalitis in a series of children. We retrospectively reviewed cases of SE related presumed encephalitis treated in the pediatric intensive care unit, between February 2002 and June 2006. Factors evaluated included age, sex, clinical symptoms, seizure type, presence of SE or refractory status epilepticus (RSE), initial electroencephalogram (EEG) finding, neuroimaging study, cerebrospinal fluid (CSF) and outcome. There were 46 patients (19 girls and 27 boys), aged 8 months to 16 years. Twenty (43.4%) of 46 children developed RSE. The major clinical symptoms included fever (100%), upper respiratory symptoms (56.5%) and altered level of consciousness (45.6%). The initial seizure type was categorized as focal (23.9%), generalized (34.8%), primary focal and secondary generalized (41.3%). Initial EEG revealed a focal (30.8%), or multifocal (19.2%) epileptiform discharge in the SE group and a focal (5%), or multifocal (70%) or generalized (25%) epileptiform discharge in the RSE group. The time of follow-up for this study was 6 months to 51 months. In the SE group, 4 died, 16 developed epilepsy and/or neurologic deficits, and 6 returned to baseline. In the RSE group, 6 died, 13 developed epilepsy and/or neurologic deficits, and none returned to baseline. All survivors were discharged on antiepileptic medications. Our data indicated that children of SE related presumed encephalitis had a high mortality and morbidity. Outcome was related to multifocal or generalized abnormalities of the initial EEG and presence of RSE.
    European Journal of Paediatric Neurology 02/2008; 12(1):32-7. · 1.93 Impact Factor
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    ABSTRACT: Diabetes ketoacidosis (DKA) is one of the common complications of type I insulin-dependent diabetes mellitus. Neurological deterioration during an episode of DKA is usually assumed to be caused by cerebral edema and cerebral vascular accidents. However, hemorrhagic stroke is a very rare complication of juvenile DKA. We describe a girl who had newly diagnosed insulin-dependent diabetes mellitus with juvenile DKA developed intracerebral hemorrhage.
    Brain and Development 02/2008; 30(1):91-3. · 1.54 Impact Factor
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    ABSTRACT: Posterior circulation infarction is uncommon in children. Vertebral artery dissection is an unusual cause of posterior circulation infarction in children. We report on a 12-year-old boy with spontaneous left-extracranial vertebral artery dissection associated with isolated ipsilateral superior cerebellar artery territory infarction, diagnosed clinically and by brain computed tomography, magnetic resonance imaging, and magnetic resonance angiography. Cerebral angiography demonstrated a flame-like occlusion of the left vertebral artery at level C(2)-C(3), and indicated that artery-to-artery embolus may be a mechanism of superior cerebellar artery territory infarction. We emphasize that vertebral artery dissection should be considered in a child with acute signs of posterior circulation ischemia.
    Pediatric Neurology 01/2008; 37(6):435-7. · 1.50 Impact Factor
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    ABSTRACT: Congenital dermal sinuses are relatively uncommon, and result from abnormal neurulation. A lack of awareness about this problem causes these patients to manifest significant mortality and morbidity. Spinal intramedullary abscesses are rare and potentially devastating lesions. Intraspinal epidermoids are also unusual lesions. We report on a boy, aged 1 year and 5 months, who had a lower-back hairy hemangioma at birth without further evaluation, and dermal sinus tract without surgical intervention at age 7 months. He also manifested rapidly progressive paraplegia with urine and stool retention, and was found to have a spinal intramedullary abscess with an epidermoid as the result of a dermal sinus. This patient illustrates the importance of the recognition and evaluation of skin markers, and of an awareness and complete neurological assessment of all patients with a congenital dermal sinus, because of the potential for intradural extension and a frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.
    Pediatric Neurology 09/2007; 37(2):144-7. · 1.50 Impact Factor
  • Po-Cheng Hung, Huei-Shyong Wang
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    ABSTRACT: Several mitochondrial diseases are known to occasionally involve the cerebral white matter, but in these cases the major findings are involved in the basal ganglia and brainstem. We report a case of diffuse white matter involvement in an infant with mitochondrial disease that was diagnosed via mitochondrial DNA analysis. Mitochondrial disease should be considered in the diagnosis of diffuse leukoencephalopathy in infancy.
    Journal of Clinical Ultrasound 07/2007; 35(5):277-80. · 0.80 Impact Factor
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    ABSTRACT: Vertebral defects, anal atresia, cardiovascular anomalies, tracheoesophageal fistulas (TEFs), renal anomalies, and limb defects (most often of the radius) are commonly associated and known collectively by the acronym VACTERL. The authors studied these nonrandomly associated birth defects to determine if a further relationship exists between VACTERL association and the presence of a tethered spinal cord (TSC). From 2001 to 2004, 12 patients with VACTERL association who were treated operatively by a single pediatric surgeon underwent magnetic resonance (MR) imaging to evaluate the intraspinal abnormalities that may cause tethering. Three patients were excluded from the study due to complications after surgery for TEF. Coincidentally, these three patients did not have imperforate ani. In the remaining nine patients, seven had associated urogenital anomalies, and six of these also had high-type imperforate ani. Five of the six patients and the one patient with low-type imperforate anus and a urogenital anomaly were found to have TSCs. In the remaining two patients without urogenital anomalies there was a high-type imperforate anus without a TSC in one patient and a low-type imperforate anus with a TSC in the other. All seven patients with TSCs underwent successful untethering. The lesions contributing to TSC included terminal filum lipomas (TFLs) in five patients, an intramedullary ependymal cyst in one patient, and a lipo-meningomyelocele in another patient. The authors found that in patients with VACTERL association there was a high incidence of TSC (seven of nine patients) if an imperforate anus was present as one of the anomalies. In patients with VACTERL association and urogenital anomalies, the incidence of TSC was even higher (86%). Five of the seven cases of TSC in the present study were caused by a TFL, a lesion that can be easily and safely managed surgically. The authors conclude that MR imaging is essential for ruling out the possibility of a TSC in patients with VACTERL association combined with urogenital anomalies or an imperforate anus.
    Journal of Neurosurgery 04/2007; 106(3 Suppl):201-4. · 3.23 Impact Factor
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    ABSTRACT: Internal carotid artery dissection is an important cause of ischemic stroke in children and young patients. Children presenting with gross neurological abnormalities after blunt trauma to the head or neck should be considered to have sustained injury to the carotid arteries until proven otherwise. Treatment options include observation, anticoagulation and endovascular stenting, and aggressive surgical repair of the carotid artery injury. We present the case of a 7-year-old boy who had a dissection of his right internal carotid artery after a dangerous position of head upside down from a water slide.
    Pediatric emergency care 04/2007; 23(3):166-8. · 0.92 Impact Factor
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    ABSTRACT: Tourette's syndrome is manifested in a broad spectrum of motor, vocal, and behavioral disturbances. Movement disorders, such as tics, may contribute to the development of cervical myelopathy owing to the effects of involuntary movements on the neck. However, the association of cervical myelopathy with motor tics of the head and neck is rare. We report here a case of a violent, repetitive neck extension due to Tourette's syndrome that developed cervical myelopathy caused by cervical disc herniation.
    Brain and Development 04/2007; 29(2):61-3. · 1.54 Impact Factor
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    ABSTRACT: Transcranial color Doppler sonography (TCCD) is a useful tool for intracranial investigation. Using TCCD to calculate total cerebral blood flow volume (TCBFV) can be a useful indicator for intracranial hemodynamic status. We performed a series study of TCCD on 60 healthy kindergarten students. Peak-systolic, end-diastolic, and mean blood velocities of major cerebral arteries, and depth of flow waves were measured. We also collected Gosling pulsatile index (PI) and Pourcelot resistance index (RI) of the arteries. TCBFV was calculated from the mean blood flood velocity and vessel chamber size of the internal carotid artery (ICA) and basilar artery (BA). Fifty children completed the examinations. The TCBFV was 1538+/-416 ml/min with mean cerebral blood flow volume of 571+/-241 ml/min for the ICA system and 983+/-343 ml/min for the BA system. PI, RI, and the velocities of A1, A2, M1, M2, BA, ICA, and TCBFV were not significantly different between girls and boys in this age group. In this study, we used TCCD to determine the normal data of main cerebral arteries and TCBFV of pre-school children in Taiwan. The reference data of velocities and other parameters of main cerebral arteries from our study may serve as a guide for additional pediatric cerebral hemodynamic studies.
    Brain and Development 04/2007; 29(2):64-8. · 1.54 Impact Factor
  • American Journal of Emergency Medicine 02/2007; 25(1):118-20. · 1.15 Impact Factor
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    Po-Cheng Hung, Huei-Shyong Wang
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    ABSTRACT: We report two male Taiwanese siblings in whom the T-->C point mutation at nucleotide 9176 of the mitochondrial ATPase 6 gene (m.9176T>C mutation) was associated with early onset hypotonia, lactic acidosis, and death due to respiratory arrest at 7 and 10 months old. Brain MRI showed lesions over diffuse white matter and the bilateral posterior limbs of the internal capsule. The m.9176T>C mutation is suggested as the cause of the bilateral striatal necrosis and Leigh syndrome. However, leukodystrophy in Leigh syndrome associated with m.9176T>C mutation has never been reported before. We suggest that m.9176T>C mutation could be a new aetiology for leukodystrophy in children with Leigh syndrome.
    Developmental Medicine & Child Neurology 02/2007; 49(1):65-7. · 3.29 Impact Factor
  • I-Jun Chou, Kuang-Lin Lin, Huei-Shyong Wang, Chao-Jan Wang
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    ABSTRACT: Antiepileptic drugs have been shown to be associated with a lowering of bone mineral density in childhood and adolescence, which are critical periods of skeletal mineralization. A lower peak bone mass attained at the end of adolescence is associated with greater involutional osteoporosis and risk for fracture in the elderly. Our purpose was to evaluate the effects of carbamazepine and valproate monotherapy on bone mineral density in children in Taiwan. From November 1995 to April 2005, forty-two children with uncomplicated epilepsy, who were treated with either carbamazepine (n=21) or valproate (n=21) monotherapy for more than 6 months, were enrolled in this study. All subjects were 5 to 18 years of age, seizure-free for 5 months or more, with normal daily activity, and normal diet. Lumbar bone mineral density of L1 to L4 was measured by dual-energy X-ray absorptiometry. The mean serum levels of carbamazepine and valproate were 5.12 +/- 2.15 mcg/ml and 49.61 +/- 20.84 mcg/ml, respectively. Treatment durations were 37.05 +/- 31.11 months and 22.86 +/- 18.84 months, respectively. The serum levels of calcium and phosphate in both groups were within therapeutic range. The serum level of alkaline phosphatase was significantly higher in the carbamazepine group (264.71 +/- 66.91, U/L) than in the valproate group (179.48 +/- 79.37, U/L). Three patients (140%) had bone mineral density Z-score of -2.0 or lower in the carbamazepine-treated group, but none in the valproate-treated group (p=0.232). Comparing the Z-score in carbamazapine- and valproate-monotherapy children, 7 (33%) had Z-score of -1.5 or lower in the carbamazepine-treated group, and none in the valporate-treated group had Z-score of -1.5 or lower (p=0.009). Four (57%) patients in the 7 carbamazepine-treated children with Z-score of -1.5 or lower had serum drug level lower than therapeutic range. Children receiving carbarmazepine monotherapy had increased frequency of lower bone density than children receiving valproate monotherapy.
    Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi 01/2007; 48(6):317-22.
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    ABSTRACT: Wilson disease is an autosomal recessive inborn error of copper metabolism that leads to neurologic symptoms and variable degrees of hepatic damage. The most common characteristic signs clinically are liver disease, psychiatric disease, neurologic disease, or a combination of these. Early recognition by means of clinical signs and an early initiation of therapy using chelators or zinc-salts are essential for a good outcome and prognosis. This report describes a male suffering from Wilson disease who exhibited an unusual presentation that included psychological manifestations without hepatic involvement. He was initially treated for attention-deficit hyperactivity disorder and a seizure disorder until brain imaging established the diagnosis of Wilson disease.
    Pediatric Neurology 11/2006; 35(4):284-6. · 1.50 Impact Factor

Publication Stats

509 Citations
131.90 Total Impact Points


  • 2002–2014
    • Chang Gung Memorial Hospital
      • • Division of Pediatric Neurology
      • • Division of Pediatric Critical Care
      • • Division of Hematology and Oncology
      T’ai-pei, Taipei, Taiwan
  • 2004–2010
    • National Taiwan University Hospital
      • Department of Surgery
      Taipei, Taipei, Taiwan
  • 2002–2010
    • Chang Gung University
      • • College of Medicine
      • • Division of Pediatric Neurology
      Taoyuan, Taiwan, Taiwan
  • 2003
    • Saint Paul's Hospital
      T’ai-pei, Taipei, Taiwan