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Biomedical subjects

W J Shian

Publications and source records attributed to W J Shian.

At least 19 recordsLinked to original sources
PubMed1996

Epstein-Barr virus encephalitis and encephalomyelitis: MR findings.

The purpose of this project is to investigate the clinical and brain MR characteristics of Epstein-Barr virus (EBV) encephalitis and encephalomyelitis. Clinical and 30 MR findings of 29 patients with EBV encephalitis or encephalomyelitis were retrospectively reviewed. Patients included 24 with encephalitis, 3 with encephalomyelitis, and 2 with brain-stem encephalitis. Altered consciousness, seizures, visual hallucination, and acute psychotic reaction were the common presentations. Eight patients had positive MR findings. These included T2 prolongation over gray and white matter, periventricular leukomalacia, and brain atrophy. Transient T2 prolongation over gray and white matter was found in one patient. Our results indicate that EBV encephalitis and encephalomyelitis have a wide range of both clinical and MR findings. The MR lesions may disappear in a short period, so the timing for the MR scan may be critical.

Adolescent↗
PubMed1995

Lumboperitoneal shunt complicated with chronic tonsillar herniation: a case report.

Lumbopritoneal shunt (L-P shunt) has been widely accepted as a treatment for communicating hydrocephalus. The technique offers many advantages such as easy insertion, a low infection rate, a reduced incidence of brain damage, et centera. Despite these advantages, there are also some associated complications, such as tonsillar herniation. This complication rarely occurs, but, when it does, it develops gradually. This report concerns an eight-year-old girl, who had received L-P shunt at the age of 20 days; she then developed tonsillar herniation, requiring operative decompression, eight years later. This case illustrates the necessity for carefully follow up patients who have undergone the L-P shunt procedure.

Cerebellar Diseases↗
PubMed1995

Unilateral porencephalic cyst presenting as infantile spasms: a case report.

Multiple etiological factors have been associated with infantile spasms (IS), and are usually linked to diffuse or multifocal brain damage. It is generally believed that infants with symptomatic IS have a significantly higher incidence of mental retardation and epilepsy than those with cryptogenic IS. However, IS secondary to focal brain lesions in which surgical removal of the lesions has resulted in cessation of generalized spasms and subsequent normal development are rarely reported. A seven-month-old male infant who manifested head nodding and generalized flexor spasms one month prior to the admission is reported. Waking interictal electroencephalography revealed a pattern of burst-suppression variant of hypsarrhythmia. Brain magnetic resonance imaging disclosed a huge porencephalic cyst over the left temporo-parietal region. The infant underwent craniotomy with corpus callosotomy, causing marked improvement in seizures. He attained subsequent stable psychomotor development.

Brain Diseases↗
PubMed1994

Fatal brainstem encephalitis caused by Epstein-Barr virus.

An 8-month-old male infant, previously well, developed acute changes of consciousness associated with high fever, vomiting, and respiratory failure. Brain CT showed hypodensity of the brainstem, which had shown hyperechogenicity on brain ultrasonography. Brainstem encephalitis caused by Epstein-Barr virus (EBV) was diagnosed, based on the clinical presentation, neuroimaging and paired serological examinations. The patient expired eventually due to central failure. We suggest that EBV infection should be a differential diagnosis in cases of brainstem encephalitis.

Brain Stem↗
PubMed1994

MRI of Joubert's syndrome.

OBJECTIVE: To report five cases of the rare Joubert's syndrome. SUBJECTS: All five cases were studied by 1.0-Tesla MRI. All the patients showed typical clinical manifestations of Joubert's syndrome including neonatal respiratory abnormalities, developmental delay, ataxia, retinal atrophy and nystagmus. RESULTS: The T1WI of MRI showed characteristic MRI features of Joubert's syndrome including dilatation of the fourth ventricle with some appearing bat-wing shaped, elongation and stretching of the superior cerebellar peduncles, dysphasia of the vermis, widening of the foramen of Magendie and the posterior cistern. One case was associated with encephalomeningocele. CONCLUSIONS: MRI can provide characteristic findings of Joubert's syndrome and confirm the clinical diagnosis.

Brain↗
PubMed1994

Leigh syndrome with progressive ventriculomegaly.

A 6-month-old female infant was diagnosed with Leigh syndrome after an abnormal muscle specimen was obtained and after magnetic resonance imaging demonstrated characteristic cranial lesions. She presented with episodic hyperventilation, myoclonus, ophthalmoplegia, hypotonia, and elevation of lactate in the cerebrospinal fluid and blood. A series of cranial ultrasounds revealed progressive ventricular enlargement before the typical lesions were detected by magnetic resonance imaging. Myelin destruction is believed to play an important role in the early stage of Leigh syndrome. Ultrasonography may provide a convenient way to document changes in brain that provide early suspicion of Leigh syndrome.

Brain↗
PubMed1994

Tc-99m HMPAO brain SPECT findings in pediatric viral encephalitis.

Two children were diagnosed with viral encephalitis, due to Epstein-Barr virus infection in one case and to herpes simplex virus infection in the other. Tc-99m HMPAO brain SPECT was arranged to detect changes in regional cerebral blood flow (rCBF) secondary to viral encephalitis. During the acute episode, Tc-99m HMPAO brain SPECT showed that the two cases had increased rCBF. After the acute episode, follow-up brain SPECT was arranged 6 months later. The rCBF in one case was restored to normal on the second brain SPECT, and that in the other case was decreased. The child with normal rCBF in the follow-up brain SPECT had better learning ability and intelligence than the other child with decreased rCBF.

Brain↗
PubMed1994

Varicella-zoster virus infection in children with malignancy.

BACKGROUND: Immunocompromised children are potentially threatened by infections, among which, the highly contagious chickenpox infection is the most common. In the past six months, there has been a spate of five chickenpox infections in children with malignancy, all of whom were receiving chemotherapy at that time. METHODS: The cases of 17 children with malignancies, who suffered from varicella-zoster infection during a period of chemotherapy at Taichung Veterans General Hospital were reviewed. RESULTS: The diagnoses of their neoplasms were 12 acute lymphoblastic leukemia (ALL), 2 lymphoma, 3 solid tumors. The mean age was 6.8 +/- 4.0 year-old (range 3 to 15 year-old). The average duration from chickenpox skin eruption to admission was 3.3 +/- 1.8 days. Four patients suffered from abdominal pain and three of them died soon; three of them suffered from back pain and one died later. Seven of these 11 patients had impaired liver function (GOT > 45 U/L), of whom 4 died later. There were seven patients with pneumonitis, of whom five died later. Among 12 patients with ALL, 3 had absolute lymphocyte counts (ALC) < 500/mm3, but only 1 died later; 9 had ALC > 500/mm3, of whom 4 had pneumonitis, and all died later. Four patients developed disseminated intravascular coagulopathy, and three of them died later. Seven patients were prescribed acyclovir within three days after first skin eruption, none of these died. Ten patients were prescribed acyclovir three days or more after first skin eruption and five of them died later. Five patients were prescribed intravenous immunoglobulin (IVIG) within three days after first skin eruption, and none of them died; of the seven patients prescribed IVIG three days or more after first skin eruption, three died later. CONCLUSIONS: Abdominal pain and disseminated intravascular coagulopathy (DIC) were signs of visceral dissemination. Severe liver function impairment, pneumonitis and DIC were the principal causes of death. Early administration of acyclovir and intravenous immunoglobulin (IVIG) can probably effectively prevent the dissemination of varicella-zoster virus (VZV). While varicella-zoster immunoglobulin (VZIG) was unavailable, IVIG was still valuable in treating VZV infection.

Acyclovir↗
PubMed1994

Guillain-Barre syndrome in infants and children.

BACKGROUND: Guillain-Barre syndrome (GBS) occurs at all ages, and population-based studies give it a crude average annual incidence rate ranging from 0.4 to 1.7 per 100,000 population. This retrospective study is performed for fifteen Chinese infants and children in light of the fact that GBS infrequently occurs during childhood years. METHODS: Hospital records of fifteen Chinese infants and children, i.e. eleven males and four females, with GBS were included. Their ages ranged from one to sixteen years old, with a mean average of 8.5 years. Their performances were graded on a scale from 0 to 6 according to the GBS Study Group. Their performances were graded under categories of Grades 2 (2/15), 3 (2/15), 4 (8/15) and 5 (3/15). RESULTS: Seven patients (46.7%) had preceding viral illness, occurring from seven to fourteen days prior to admission, with a mean average of twelve days. In addition to weakness and areflexia, musculoskeletal pain and subjective paresthesia were the most common presentations upon admission. The later the cerebrospinal fluid (CSF) was examined, the more cases had CSF features of increased protein level with no or mild pleocytosis. Eleven patients underwent serological examinations, and seven of their tests yielded positive for Epstein-Barr virus in five and Mycoplasma pneumoniae in two. Seven patients underwent electromyography and nerve conduction velocity measurement, all exhibiting the characteristic features of demyelination. Twelve patients had supportive treatment, and three of them required mechanical ventilation. Intravenous gamma globulin was administered in two patients, and oral prednisolone was administered in one patient. It took one to six months with a mean average of 3.4 months for patients' performance returning to Grade 0. All of the patients survived without residual disability. CONCLUSIONS: Some infectious pathogens could play a role in the pathogenesis of GBS. Furthermore, complete recovery may be the rule for most infants and children with GBS.

Adolescent↗
PubMed1994

Clinical study of epileptic children with history of febrile convulsion.

BACKGROUND: Clinically, some epileptic syndromes have been linked to febrile convulsions (FC), but the exact relationship between FC and later epilepsy remains a mystery. METHODS: Detailed histories of 81 Chinese children among 1950 pediatric epileptics with a history of FC were obtained retrospectively. The clinical courses and risk factors were analyzed. RESULTS: According to their epileptic patterns, the children were divided into a generalized group (Group G, 45/81), a partial group (Group P, 30/81) and those with severe myoclonic epilepsy in infancy (6/81). Patients of Group P had an earlier age of onset of FC with a larger number of risk factors than those of Group G (p < 0.01), and patients of Group G had a shorter interval from the last FC to later epilepsy than those of Group P (p < 0.01). More risk factors of FC were present in children of Group P; these included especially focal seizures, prolonged duration and retarded psychomotor development (p < 0.01). CONCLUSIONS: For group of epileptics in this study, genetic factors might predispose for the expression of FC and later epilepsy, and anticonvulsant prophylaxis for FC seemed to be unnecessary.

Child, Preschool↗
PubMed1994

Dural arteriovenous malformation with symmetrical calcification of the basal ganglia: a case report.

Dural arteriovenous malformations associated with symmetrical calcification of the basal ganglia are rare in children. This report concerns a 22-month-old female infant who was admitted with the problem of acute onset of status epilepticus. Physical examination revealed a grade II/VI heart systolic murmur over the left sternal border, and engorged scalp veins. Neurologically, left side hemiparesis, brisk deep tendon reflexes, bilateral presence of Babinski sign and ankle clonus were present. Brain computed tomography without contrast medium showed cortical atrophy and symmetrical calcification of the basal ganglia. Brain magnetic resonance imaging showed a signal-voided tortuous structure over the right parietal region. Cerebral angiography disclosed a dural arteriovenous malformation, located over the right parietal region, which had two major feeding arteries from the branch of the anterior cerebral artery and the parietal branch of the middle cerebral artery, and had direct venous drainage into the superior sagittal sinus. The patient received craniotomy with ligation of feeding arteries. She became seizure-free, and her neurological deficits improved gradually.

Basal Ganglia↗
PubMed1994

Viral encephalitis in children: detection with technetium-99m HMPAO brain single-photon emission CT and its value in prediction of outcome.

PURPOSE: To assess the role of regional cerebral blood flow measured by technetium-99m hexamethyl propyleneamine oxime (HMPAO) brain single-photon emission CT (SPECT) in viral encephalitis of children. METHODS: Eighteen children diagnosed as having viral encephalitis (12 Epstein-Barr virus, 4 herpes simplex virus, and 2 Japanese B virus) underwent Tc-99m HMPAO brain SPECT and brain MR and/or CT. RESULTS: During the acute episode, 4 (22%) of the 18 patients had localized abnormality on brain MR and/or CT. Tc-99m HMPAO brain SPECT in the acute phase showed that 17 (94%) of the 18 patients had increased regional cerebral blood flow and 1 (6%) of the 18 children had a normal brain SPECT. Follow-up brain SPECT was performed at least 15 days after the acute episode. In 17 patients with abnormal first brain SPECT, 12 (71%) had normal second brain SPECT and 5 (29%) had decreased regional cerebral blood flow. The group of patients with normal regional cerebral blood flow on the follow-up brain SPECT had a better outcome than the group of patients with decreased regional cerebral blood flow. CONCLUSIONS: (a) The Tc-99m HMPAO brain SPECT was abnormal more often than CT or MR in children with acute viral encephalitis and provided better location. (b) In acute episodes of encephalitis, most patients showed locally increased regional cerebral blood flow. (c) After acute episodes, the regional cerebral blood flow returned to normal in most cases. (d) A normal Tc-99m HMPAO brain SPECT in the subacute phase usually indicates a good clinical outcome (no neurologic defect) 1 year after the acute illness in children with viral encephalitis.

Adolescent↗
PubMed1994

Clinical observation of neonatal gastrointestinal perforation.

BACKGROUND: Gastrointestinal perforation is an infrequent occurrence in neonates. Experience in its management is presented, while also attempting to analyze the factors affecting outcome. METHODS: From 1983 to 1992, 31 neonates with gastrointestinal perforation were treated at Taichung Veterans General Hospital. The medical records of these patients were reviewed thoroughly. In the meantime, Mann-Whitney U and Yates' correction chi square tests were used to analyze the factors predicting the outcomes. RESULTS: There were 21 males and 10 females, among whom 16 were premature births. The median age at diagnosis was 5 day; half (16/31) had occurred during the first 5 days of life. Abdominal distension was the most common manifestation (87%). Hemograms at admission showed leukopenia in 32% (10/31) and thrombocytopenia in 40% (8/20) of the patients. Metabolic acidosis was present in 46% (13/28) of the patients. Peritoneal fluid and preoperative blood cultures were positive in 71% (17/24) and 50% (13/26) of the patients, and both of them had Gram-negative enterobacteriacea as the most common pathogen. The overall mortality rate was 58%. The highest mortality rate was associated with gastric perforation (100%), followed by small bowel (50%) and colon (50%) perforations. The predominant cause of perforation was necrotizing enterocolitis (14/31), with the most common site the terminal ileum (16/29). Four patients with necrotizing enterocolitis had multiple perforations. Others who underwent surgery showed single perforation. The major cause of death in those patients who received operation was sepsis (8/13). An initial arterial pH value higher than 7.25, and surgical procedure performed within two days after disease onset may predict a favorable outcome (p < 0.01). CONCLUSIONS: Gastrointestinal perforation is a life-threatening complication in neonates. A better survival rate can be obtained by cooperation among neonatologists, pediatric surgeons and the nursing staff taking care of these high risk babies.

Chi-Square Distribution↗
PubMed1994

Childhood absence epilepsy.

BACKGROUND: Childhood absence epilepsy (CAE) is a relatively rare form of idiopathic generalized epilepsy, occurring in previously normal children with a strong genetic predisposition. The annual incidence of CAE has been estimated at 6.3/100,000 to 8/100,000 children aged 0-15 years. METHODS: Medical and electroencephalographic records of 50 Chinese children with CAE were reviewed. RESULTS: There were 22 males and 28 females. The age of onset ranged from 3 to 12 years with a mean of 6.9 +/- 2.4 years. Six children (12%) had positive familial epileptic history and 11 patients (22%) had a past history of febrile convulsion. Electroencephalographically, there were posterior delta rhythm (6/50), frontocentral (1/50), and centrotemporal (1/50) spikes in addition to the characteristic 3 Hz spike and wave complex. All patients received regular or irregular treatment with valproate. Sixteen patients (32%) evolved to generalized tonic-clonic seizure (GTCS) with a mean of 2.1 +/- 1.6 years of lag from stopping valproate. The patients with GTCS had significant late onset of age, a longer active period, and longer treatment duration than those without GTCS (p < 0.01). There were significantly more patients with irregular treatment evolving to GTCS than those with regular treatment (p < 0.01). None of the patients with posterior delta rhythm evolved to GTCS. CONCLUSIONS: Regular treatment with valproate is the modality of choice in the management of CAE. Posterior delta rhythm seems to be a good prognostic factor electroencephalographically.

Child↗
PubMed1994

Leukemoid reaction in a child with appendiceal abscess: a case report.

A case of appendiceal abscess presenting leukemoid reaction is reported. A seven-month-old male infant was admitted because of fever and diarrhea. Anemic conjunctiva and mild abdominal distension were the only physical findings on arrival. Progressive leukocytosis with left shift maturation was noted during hospitalization. The maximal white blood cell count was 178,000/cumm, with a high leukocyte alkaline phosphatase score. On the fourth day of admission, a palpable right lower quadrant abdominal mass with local tenderness was found. Appendiceal abscess was diagnosed by both ultrasonography and computed tomography. The infant received triple antibiotic therapy consisting of ampicillin, gentamicin, and clindamycin for 10 days; the mass disappeared two weeks later. As appendiceal abscess is a rare occurrence in infants and its production of a leukemoid reaction is even rarer, the case described here may cause a diagnostic challenge, and deserves attention from clinicians.

Abscess↗
PubMed1994

Juvenile myoclonic epilepsy.

BACKGROUND: Recognition of the significance of juvenile myoclonic epilepsy (JME) in the English-language neurological literature is relatively new. There are many factors responsible for delay in diagnosis of JME, including lack of familiarity with the syndrome, failure to elicit a history of myoclonic jerking and absence or generalized tonic-clonic seizures predating myoclonic jerks in some patients. METHODS: The medical and electroencephalographic (EEG) records of seven Chinese children with JME, four males and three females, were reviewed. RESULTS: The age of onset ranged from 10 to 14 years with mean 11.8 +/- 1.6 years. The precipitating factors were sleep deprivation (5/7), photostimulation (3/7), hyperventilation (2/7) and menstruation (1/3). Childhood and juvenile absence epilepsies predated JME in three and one patients, respectively. All patients had concomitant grand mal on awakening. Generalized 3-4 Hz polyspikes-wave complexes occurred in all patients, and two patients had additional 3-4 Hz spike-wave complexes. These activities were provoked with photic stimulation (3/7) and hyperventilation (2/7). All patients were treated with valproate for more than four years, and relapse occurred six months to one year after discontinuation of valproate. CONCLUSIONS: JME is a benign generalized epilepsy with a strong genetic basis. Valproate is the drug of choice up to now, but long-term (life-long) medication may be needed.

Adolescent↗
PubMed1994

Acute transverse myelitis in children: clinical analysis of seven cases.

BACKGROUND: The reported annual incidence of acute transverse myelitis (ATM) is 1 per 3.4 million. Because of its rarity, we performed this retrospective clinical investigation. METHODS: Hospital records of seven Chinese children with ATM were reviewed thoroughly. The diagnosis of ATM were made clinically with or without neuroimages. RESULTS: Five males and two females were included, of age from three to sixteen years with a mean of 8.6 years. The common presentations included weakness of lower extremities, acute flaccid paralysis, urinary distention, constipation, hyporeflexia, sensory impairment and paresthesia. Slightly elevated protein level in cerebrospinal fluid was noted. Mycoplasma pneumoniae, varicella/zoster virus and Epstein-Barr virus infections were confirmed serologically in one, one, and two patients, respectively. All patients received supportive treatment. The duration required to reach peak severity from onset of illness ranged from six to forty-eight hours. All but one patient recovered, and one to six months were required to achieve maximum improvement. CONCLUSIONS: Some infectious pathogens may play a role in the pathogenesis of ATM. In general, the prognosis of childhood ATM is good.

Acute Disease↗