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Unlabelled: Despite advancement in medical care, prejudice and misunderstanding of epilepsy still exist. In this study, we investigate the problems faced by epileptic children, at home and in school, and make suggestions for improvement. Questionnaires were randomly distributed to parents of epileptic children attending normal and special classes (groups A and B, respectively). Return of questionnaires was anonymous. Ninety-one percent responded. Of the responders, 56 children were in Group A and 30 in Group B. Chronic and intractable epilepsy was more frequently observed in Group B than in Group A patients (47% vs 14%, p < 0.05). Main family concerns were seizures, school performance and side effects of medication. Half of the parents complained that their children were more restless and short-tempered. Only 43% of parents were aware that seizures were caused by abnormal brain discharges. Twenty percent thought swimming should be prohibited even if seizures could be controlled. Schools were informed of the disease by 84% of the families. Only 29% of parents knew the name and dose of the current medication. Information was considered adequate in 27% of patients. Drug compliance was better in epileptic children with associated handicaps than in those without handicaps. Half of the parents requested more information about epilepsy and closer communication between teachers and physicians.Conclusions: To establish comprehensive care that satisfies the needs of epileptic children and their families, further training of medical specialists in epilepsy and enhancement of networks among relevant organizations are needed. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barré syndrome (GBS) is a rare acquired immune-mediated polyneuropathy. Updated population-based data concerning paediatric GBS is needed., Methods: Paediatric patients aged below 18 years diagnosed with GBS between 2009 and 2018 in all 11 paediatric departments in Hong Kong were identified from the Hong Kong Hospital Authority Clinical Data Analysis and Reporting System. The collected data from medical health records were reviewed by paediatric neurologist from each department. Estimated incidence of paediatric GBS was calculated. We also compared our findings with other paediatric GBS studies in Asia., Results: 63 subjects of paediatric GBS were identified, giving an estimated annual incidence of 0.62 per 100,000 population. Half of the subjects had acute inflammatory demyelinating polyneuropathy (AIDP) (n = 31; 49.2%), one quarter had Miller Fisher Syndrome (MFS) (n = 16; 25.4%), one-fifth had axonal types of GBS (n = 12; 19.0%), and four were unclassified. Paediatric subjects with axonal subtypes of GBS compared to the other 2 subtypes, had significantly higher intensive care unit (ICU) admission rates (p = 0.001) and longest length of stay (p = 0.009). With immunomodulating therapy, complete recovery was highest in those with MFS (100%), followed by AIDP (87.1%) and axonal GBS (75%). Our study also confirms a higher MFS rate for paediatric GBS in East Asia region and our study has the highest MFS rate (25.4%)., Conclusion: Our population-based 10-year paediatric GBS study provides updated evidence on estimated incidence, healthcare burden and motor outcome of each subtype of paediatric GBS and confirmed a higher occurrence of paediatric MFS in East Asia., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Objective: Early onset drug-resistant epilepsy is a neurologic disorder in which 2 antiepileptic drugs fail to maintain the seizure-free status of the patient. Heterogeneous clinical presentations make the diagnosis challenging. We aim to identify the underlying genetic causes of a pediatric cohort with drug-resistant epilepsy and evaluate whether the findings can provide information on patient management., Methods: We include patients with drug-resistant epilepsy onset before 18 years of age. Singleton clinical chromosomal microarray (CMA) followed by whole exome sequencing (WES) was performed using genomic DNA. In the first-tier analysis of the exome data, we aimed to identify disease-causing mutations in 546 genes known to cause, or to be associated with, epilepsy. For negative cases, we proceeded to exome-wide analysis. Rare coding variants were interrogated for pathogenicity based on the American College of Medical Genetics and Genomics (ACMG) guidelines., Results: We recruited 50 patients. We identified 6 pathogenic or likely pathogenic mutations, giving a diagnostic yield of 12%. Mutations were found in 6 different genes: SCN8A , SCN1A , MECP2 , CDKL5 , DEPDC5 , and CHD2 . The CDKL5 variant was found to be mosaic. One variant of unknown significance (VUS) in KCNT1 was found in a patient with compatible clinical features. Of note, a reported pathogenic SCN5A mutation known to contribute to Brugada syndrome, was also found in the patient with an SCN1A mutation., Significance: Our study suggests that singleton WES is an effective diagnostic tool for drug-resistant epilepsy. Genetic diagnosis can help to consolidate the clinical diagnosis, to facilitate phenotypic expansion, and to influence treatment and management options for seizure control in our patients. In our study, a significant portion of the genetic findings are known to be associated with an increased risk of sudden unexpected death in epilepsy (SUDEP). These findings could assist with more appropriate management in patients with epilepsy.

Background: Chromosomal microarray offers superior sensitivity for identification of submicroscopic copy number variants (CNV) and it is advocated to be the first tier genetic testing for patients with autism spectrum disorder (ASD). In this regard, diagnostic yield of array comparative genomic hybridization (CGH) for ASD patients is determined in a cohort of Chinese patients in Hong Kong., Methods: A combined adult and paediatric cohort of 68 Chinese ASD patients (41 patients in adult group and 27 patients in paediatric group). The genomic DNA extracted from blood samples were analysed by array CGH using NimbleGen CGX-135K oligonucleotide array., Results: We identified 15 CNV and eight of them were clinically significant. The overall diagnostic yield was 11.8 %. Five clinically significant CNV were detected in the adult group and three were in the paediatric group, providing diagnostic yields of 12.2 and 11.1 % respectively. The most frequently detected CNV was 16p13.11 duplications which were present in 4 patients (5.9 % of the cohort)., Conclusions: In this study, a satisfactory diagnostic yield of array CGH was demonstrated in a Chinese ASD patient cohort which supported the clinical usefulness of array CGH as the first line testing of ASD in Hong Kong.

Purpose: This study evaluated self-esteem in adolescents with epilepsy and its association with psychosocial and disease-related variables., Methods: This was a cross-sectional study with patients enrolled between January and June 2010. Culture-Free Self-Esteem Inventory for Children (CFSEI-2) was administered to 140 children with epilepsy and 50 children with asthma, aged 10-18years attending mainstream schools., Results: Adolescents with epilepsy had a significantly lower overall self-esteem score when compared with those with asthma, 17±5.21 versus 19.4±3.83, respectively (P=0.005). Thirty-one (22.1%) children with epilepsy compared with 4 (8.3%) with asthma had overall self-esteem score below the cutoff (P=0.034). There was a significant correlation between overall self-esteem score and duration of epilepsy, Hospital Anxiety and Depression Scale (HADS) anxiety score, HADS depression score, and Strengths and Weaknesses of ADHD symptoms and Normal-Behaviors (SWAN) rating combined score. The impact of various correlates on individual domains was not identical. Independent factors associated with low overall self-esteem were HADS depression score (OR: 1.62; 95% CI: 1.2, 2.2; P=0.002), duration of epilepsy (OR: 1.4; 95% CI: 1.04, 1.88; P=0.024), and father employment status economically inactive (OR: 11.9; 95% CI: 1.07, 125; P=0.044). Seizure-free ≥12months was a favorable factor that was less likely to be associated with low self-esteem (OR: 0.14; 95% CI: 0.02, 0.81; P=0.028)., Conclusion: Self-esteem was compromised in adolescents with epilepsy. A significant correlation between self-esteem and psychological comorbidities was demonstrated. Enhancing social support and education programs may improve the self-esteem and, ultimately, the lives of adolescents living with epilepsy., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: We examined attention-deficit hyperactivity disorder in adolescents with epilepsy and the association with seizure-related and sociodemographic variables., Methods: Strengths and Weakness of Attention-Deficit Hyperactivity Disorder Symptoms and Normal Behaviors rating scale was administered to 122 children with epilepsy and 50 children with asthma, aged 10 to 18 years attending mainstream schools., Results: Twenty-nine (23.7%) adolescents with epilepsy compared with five (10%) with asthma had attention deficit hyperactivity disorder (P = 0.037). Adolescents with epilepsy had a significantly higher score in the inattention subscale when compared with those with asthma (-0.25 ± 1.2 vs -0.64 ± 1.07, P = 0.049). Combined subtype was most frequent in the epilepsy group. Oppositional defiant disorders were more prevalent in those having attention deficit hyperactivity disorder. Psychiatric assistance had only been provided to one third of our patients with epilepsy and attention deficit hyperactivity disorder at the time of study. There was a negative correlation between attention deficit hyperactivity disorder scores and age of seizure onset. A positive correlation was observed between the number of antiepileptic drugs and the inattentive subscale score. The impact of various correlates on individual subtypes was not identical. Independent risk factors associated with attention deficit hyperactivity disorder were medical comorbidities (odds ratio = 12.82, 95% confidence interval 4.44, 37.03, P < 0.0001) and age at seizure onset (odds ratio = 0.73, 95% confidence interval 0.56, 0.94, P = 0.016)., Conclusions: Attention deficit hyperactivity disorder is overrepresented in adolescents with epilepsy; screening for its symptoms should be an integral part of management in adolescents with epilepsy., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The present study examined anxiety and depression in adolescents with epilepsy and the association of these disorders with seizure-related and sociodemographic variables. The Hospital Anxiety and Depression Scale was administered to 140 children with epilepsy and 50 children with asthma aged 10 to 18 years attending mainstream schools. Adolescents with epilepsy had significantly higher scores on the depression subscale than those with asthma (5.2 ± 3.3 vs 4.2 ± 3.2, P = .032). Anxiety subscale scores and the frequency of anxiety and depression in both the epilepsy and asthma groups were not statistically significant. In the epilepsy group, 32.8% had anxiety and 22.1% had depression. Factors associated with anxiety were older age at the time of the study and polytherapy (2 or more antiepileptic drugs). Adolescents who had been seizure-free for 12 months or more at time of the study were less likely to experience anxiety. Factors associated with depression were medical comorbidities, female gender, frequent seizures, and younger age of seizure onset. A common risk factor for both anxiety and depression was the duration of epilepsy. Anxiety and depression were also highly associated with each other. Affective disorders are common in epilepsy and screening for psychiatric symptoms is required., (© The Author(s) 2015.)

Purpose: To determine whether a brief student survey can differentiate among third-year clerkship student's professionalism experiences and whether sharing specific feedback with surgery faculty and residents can lead to improvements., Methods: Medical students completed a survey on professionalism at the conclusion of each third-year clerkship specialty rotation during academic years 2007-2010., Results: Comparisons of survey items in 2007-2008 revealed significantly lower ratings for the surgery clerkship on both Excellence (F = 10.75, p < 0.001) and Altruism/Respect (F = 15.59, p < 0.001) subscales. These data were shared with clerkship directors, prompting the surgery department to discuss student perceptions of professionalism with faculty and residents. Postmeeting ratings of surgery professionalism significantly improved on both Excellence and Altruism/Respect dimensions (p < 0.005 for each)., Conclusions: A brief survey can be used to measure student perceptions of professionalism and an intervention as simple as a surgery department openly sharing results and communicating expectations appears to drive positive change in student experiences., (Copyright © 2012 Association of Program Directors in Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To investigate whether requests for standard paediatric electroencephalograms accord with guideline recommendations, subsequent changes in clinical management according to reported results, and extent to which the service meets waiting time targets., Design: Case series., Setting: Regional hospital, Hong Kong., Patients: All patients aged less than 18 years who underwent electroencephalography between December 2009 and February 2010., Main Outcome Measures: Appropriateness of the electroencephalogram request and the impact of its findings on clinical management., Results: A total of 109 patients were recruited, but requests for standard electroencephalograms were considered 'inappropriate' with respect to guidelines in 44% of the patients, of which 50% were made to diagnose 'funny turns'. The standard electroencephalogram contributed to the diagnosis or management in only 28% of patients. In all of the latter, the request for an electroencephalogram had been appropriate. Nonspecialists made referrals for 86% of the patients. Inadequate information was provided in 66% of the requests. Standard electroencephalograms were performed within guideline targets, the wait being less than 4 weeks in 95% of requests., Conclusion: An effective electroencephalogram service was being provided, though abuses were common. These were mainly because of misconceptions regarding the role and limitations of standard electroencephalograms. Through an educative, non-confrontational approach, and with time to explain guideline recommendations to clinicians, sustainable change in practice could be achieved so as to benefit patients, clinicians, and service provision.

This study sought to evaluate seizure-related injuries in children under age 18 years with newly diagnosed epilepsy presenting at the Department of Pediatrics, Tuen Mun Hospital, in 2002-2003. All children were interviewed before January 2005. Children with seizure-related injuries were compared with those without injuries for identification of risk factors. One hundred and twenty-two children were surveyed. No patient died during a seizure. Eleven (9%) children manifested seizure-related injuries. Injury occurred at a mean age of 11.6 years, and epilepsy was diagnosed at a mean age of 13.8 years, in those with seizure-induced injuries. Injury occurred at first seizure presentation or upon diagnosis of epilepsy in 72.7% patients. Ten (90.9%) children were not receiving antiepileptic drugs at their time of injury. The mean age of seizure onset was 10.7 years in patients with injuries, and 6.7 years in control subjects (P = 0.007). Seizures resulting in injuries were generalized tonic-clonic in 72.7% (P = 0.045; odds ratio, 3.77; 95% confidence interval, 0.95-14.98). Idiopathic etiology was evident in 54.5%, and normal neurodevelopmental status in 72.7%, of patients with injuries. Age of seizure onset was the only independent variable retained after multivariate analysis. Soft-tissue and dental injuries comprised 91.7% and 75%, respectively, of injuries occurring at home. The risk of seizure-related injuries was substantial, especially before epilepsy was diagnosed, but most of these injuries were minor. These unique data could help in parental counseling., (Copyright 2010 Elsevier Inc. All rights reserved.)

Aim: To describe the disease burden, clinical pattern and outcome of influenza-related hospitalisations in children., Methods: This is a retrospective study carried out in a regional hospital in Hong Kong. Children hospitalised with established diagnosis of influenza infection from January to June of 2005 were studied. Length of hospitalisation, demographic characteristics, symptoms, clinical diagnosis and complications of influenza infection were analysed., Results: Influenza A infection accounted for 93.5% of these hospitalisations. Children less than 5 years of age comprised 70% of admission. Highest rate of admission occurred in May and April. One fourth of emergency admission during the study period and over 70% in the peak season was a result of influenza-related illness. Underlying medical disease was observed in 14.6% of children. Mean duration of hospitalisation was 3.0 days. Fever was the commonest presenting symptoms. Fever lasting for 7 days or more was observed in one-fifth of patients. Respiratory tract diseases (upper and lower) were the most frequent non-neurological diagnosis. Febrile convulsion was the complication observed in 27.6% of admission. One patient died as a result of acute necrotising encephalopathy., Conclusion: Influenza contributed to heavy health-care burden. Mortality was rare but did occur. Hospitalisations occurred in both healthy children and those with underlying chronic illness. Young children played an important role in such hospitalisations. Means to prevent influenza-associated morbidity and mortality especially among young children are needed.

An infant presented with failure to thrive and developmental regression. Physical examination revealed an irritable child with swollen, erythematous extremities, and elevated blood pressure. Extensive investigations, including a metabolic work-up and neuroimaging, were unrevealing. Exposure to self-purchased medication was initially denied. The physical signs were suggestive of acrodynia. Mercury poisoning was ultimately established by measuring paired blood and urine mercury levels. On further enquiry, it was revealed that the child had been given a Chinese medicinal product for 4 months. He responded well to a chelating agent. Acrodynia is a childhood disease considered to be of historical interest only, but making a diagnosis of mercury poisoning is rewarding because the response to treatment is good. This case highlights the common misconception that alternative medicines are safe and benign.

Objective: The primary aim of our study was to compare the health-related quality of life (HRQL) of children with epilepsy in Hong Kong with that of children with epilepsy in Canada, and to explore possible factors affecting these findings. A second interest was to determine agreement between proxy reports and self-ratings among children with epilepsy in Hong Kong, compare these with findings in Canada, and identify factors that influence the concordance., Methods: Child self-report and parent-proxy questionnaires on an epilepsy-specific HRQL measure, appropriately translated and validated in Chinese, were administered to 266 Hong Kong children and their parents. An unpaired t test was used to compare the scores with published results from 381 Canadian children and their parents, who used the original English version of the measure. Demographic characteristics of the two groups were compared using t tests, chi2 tests, and Fisher's exact tests. Agreement between parents' and children's scores was evaluated with intraclass correlation coefficients (ICCs) and standardized response means (SRMs). The total HRQL score differences between parents and children in Hong Kong were compared with those in Canada using an unpaired t test. Factors that might affect the parent-child score difference were studied using Pearson correlation analysis, chi2 test, and analysis of variance. Factors studied included: sex, current age, age at diagnosis, duration of epilepsy, number of antiepileptic drugs used, type of seizure, seizure severity, cognition of the child, the type of school attended, presence of neurological problems, presence of behavioral problems, recent health care usage, education and employment status of both parents, housing status of the family, and relationship of the proxy respondent to the child., Results: (1) In contrast to the Canadian sample, Hong Kong children with epilepsy were older (P<0.01), had a longer duration of epilepsy (P<0.01) and less severe seizures (P<0.01), and were more likely to attend normal schools (P<0.01). Children in Hong Kong reported more interpersonal/social difficulties (P<0.01), more worries (P<0.01), and more secrecy about their epilepsy (P<0.01). Parents in Hong Kong believed that their children perceived more worries (P<0.01) and were more secretive about their epilepsy (P<0.01). (2) Moderate to good agreement between parent-proxy response scores and child self-report scores was demonstrated (ICC=0.50-0.69, SRM=0.19-0.33). The total HRQL score differences between parent and child in Hong Kong were not different from those in Canada. None of the factors studied were related to the parent-child score difference., Conclusions: Youth with epilepsy in Hong Kong and their parents reported poorer quality of life than children with epilepsy in Canada. Further studies are necessary to identify the determinants of HRQL in children with epilepsy in different cultures. Acceptable agreement between the two ratings suggests that proxy reports can be used when child self-reports cannot be obtained.

Hospitalizations due to breakthrough seizures were studied in children with newly diagnosed epilepsy to evaluate (1) risk factors associated with such admissions, in particular the withholding of medication and subtherapeutic dose of anticonvulsants, and (2) the impact of the unscheduled hospitalization on subsequent seizure outcome. We recruited patients aged less than 18 years with newly diagnosed epilepsy who presented to the pediatric department in Tuen Mun Hospital between January 2002 and December 2003. Patients with acute seizure-related hospitalization within 1 year after diagnosis were included as cases (n = 36); patients with no such hospitalization were included as controls (n = 86). Treatment effect was analyzed at the last follow-up visit before January 2005. Significant univariate association was observed between seizure-related hospitalization and age at seizure onset (OR = 0.91, 95% CI 0.84, 1), idiopathic epilepsy (OR = 0.35, 95% CI 0.15, 0.81), no antiepileptic drug (OR = 3.67, 95% CI 1.51, 8.18), and subtherapeutic doses of antiepileptic drug (OR = 9.9, 95% CI 2.2, 43.8). Independent risk factors of such hospitalizations were etiology of epilepsy, no antiepileptic drug, subtherapeutic dose of antiepileptic drug, and history of febrile convulsion. Sixty-four percent of the cohort was seizure-free. Acute seizure-related hospitalization was associated with less favorable outcome (OR = 3.79, 95% CI 1.48, 9.26).

The aims of the present study are to identify predisposing factors of febrile seizures in influenza A infection and to clarify the special characteristics of febrile seizures in children with influenza A infection. Between January and July 2005, children hospitalized because of febrile seizures and subsequently confirmed influenza A infection were enrolled as subjects. Age-matched control subjects were those admitted as a result of influenza A infection but no febrile seizures (control 1) and children who developed febrile seizures with negative viral studies (control 2). Significant factors for the development of febrile seizures include: history of febrile seizures, family history of seizure disorders, and coexisting gastroenteritis. Independent risk factor for febrile seizures was history of febrile seizures (odds ratio 7.58, 95% confidence interval CI 1.48 to 38.84, P = 0.015). When compared with children who developed febrile seizures with negative virus studies, children who developed febrile seizures in influenza A infection had a significantly higher maximum body temperature, shorter duration of fever before seizure onset, and more frequent occurrence of partial seizures. Current episode represented first seizure in 26.5% of children infected with influenza A as compared with 50% of children whose virus studies were negative (P = 0.04). The findings suggest that effective vaccination may prevent development of febrile seizures, especially in those patients with past history of febrile seizures. Rapid diagnostic testing for influenza infection in the management of complex febrile seizures, especially during influenza season, is cost-effective.

We describe a fulminant picture of anticonvulsant hypersensitivity syndrome (AHS) and the possible role of nitrazepam. A 5-month-old boy developed fever and rash after the use of phenobarbitone. Allergy to phenobarbitone was suspected. Nitrazepam was substituted for seizure control. Over the next few days he progressively collapsed with fever, facial oedema and multi-organ involvement. The diagnosis of AHS was delayed because nitrazepam has not been implicated in the development of cross-sensitivity. AHS is a severe multi-organ reaction to aromatic anti-epileptic drugs. It has been thought to occur as a consequence of pre-existing pharmacogenetic and immunologic abnormalities. Careful selection of anti-epileptic drugs is essential as cross-sensitivity is common. Intermittent benzodiazepines have been recommended in managing breakthrough seizures in AHS. However, the structure of benzodiazepines contains aromatic rings and potential cross-reactivity cannot be totally ignored. Although we do not have direct proof, we believe that nitrazepam prolonged the clinical course.

Background: Although studies have ascertained that ten percent of soldiers killed in battle bleed to death from extremity wounds, little data exists on exsanguination and mortality from extremity injuries in civilian trauma. This study examined the treatment course and outcomes of civilian patients who appear to have exsanguinated from isolated penetrating extremity injuries., Methods: Five and 1/2 years' data (Aug 1994 to Dec 1999) were reviewed from two Level I trauma centers that receive 95% of trauma patients in metropolitan Houston, TX. Records (hospital trauma registries, emergency medical system (EMS) and medical examiner data) were reviewed on all patients with isolated extremity injuries who arrived dead at the trauma center or underwent cardiopulmonary resuscitation (CPR) or emergency center thoracotomy (ECT)., Results: Fourteen patients meeting inclusion criteria were identified from over 75,000 trauma emergency center (EC) visits. Average age was 31 years and 93% were males. Gunshot wounds accounted for 50% of the injuries. The exsanguinating wound was in the lower extremity in 10/14 (71%) patients and proximal to the elbow or knee in 12/14 (86%). Ten (71%) had both a major artery and vein injured; one had only a venous injury. Prehospital hemorrhage control was primarily by gauze dressings. Twelve (86%) had "signs of life" in the field, but none had a discernable blood pressure or pulse upon arrival at the EC. Prehospital intravenous access was not obtained in 10 patients (71%). Nine patients underwent ECT, and nine were initially resuscitated (eight with ECT and one with CPR). Those undergoing operative repair received an average of 26 +/- 14 units of packed red blood cells. All patients died, 93% succumbing within 12 hours., Conclusion: Although rare, death from isolated extremity injuries does occur in the civilian population. The majority of injuries that lead to immediate death are proximal injuries of the lower extremities. The cause of death in this series appears to have been exsanguination, although definitive etiology cannot be discerned. Intravenous access was not obtainable in the majority of patients. Eight patients (57%) had bleeding from a site that anatomically might have been amenable to tourniquet control. Patients presenting to the EC without any detectable blood pressure and who received either CPR or EC thoracotomy all died.

The interrelationship between magnetic resonance imaging findings, types of cerebral palsy, and gestation was studied. We analyzed the magnetic resonance imaging of brain in 122 children with spastic cerebral palsy. Forty-three patients had spastic hemiplegia, 61 had spastic diplegia, and 18 had spastic tetraplegia. Magnetic resonance imaging abnormalities were observed in 75% of patients. Periventricular leukomalacia accounted for 66% of abnormalities observed in patients with spastic diplegia; other types of brain lesions were uncommon. In patients with spastic tetraplegia, two types of magnetic resonance imaging abnormalities predominated: congenital brain anomalies and term-type brain injuries, 42% and 33% respectively. Types of magnetic resonance imaging abnormalities were more heterogeneous in patients with spastic hemiplegia. Preterm brain injuries (periventricular leukomalacia and posthemorrhagic porencephaly) were observed often in patients born at preterm but were also observed in patients born at term. Term-type brain injuries (term-type border-zone infarct, basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia) were observed only in patients born at or near term. We conclude that magnetic resonance imaging findings for patients with spastic cerebral palsy were closely related to types of cerebral palsy and gestation at birth. Magnetic resonance imaging in patients with perinatal brain injury may reflect pathologic changes and is useful in understanding and evaluating cerebral palsy.

Objective: To examine variables that might predict abnormal outcome of status epilepticus among children., Design: Retrospective study., Setting: Regional hospital, Hong Kong., Patients: All children younger than 15 years who were admitted to the paediatric intensive care unit with status epilepticus between 1997 and 2002., Main Outcome Measures: Neurodevelopmental outcomes., Results: Two of the 25 patients died, resulting in a mortality rate of 8%. No deaths were due to status epilepticus itself. No patient with febrile or idiopathic status epilepticus developed epilepsy. Neurological deterioration was observed in a quarter (six of 23) of the survivors. Symptomatic aetiology (acute or remote) and refractory status epilepticus were associated with adverse outcomes (P<0.05). Young age at status epilepticus (<12 months) and duration of status epilepticus (>60 minutes) tended to be more frequent among those who developed adverse outcome. Rectal diazepam was given before hospital arrival in only four patients., Conclusions: Paediatric patients with status epilepticus who had normal neurodevelopmental status before the onset of an attack and who did not sustain an acute insult to the central nervous system or have progressive encephalopathy, had favourable outcomes. Prompt use of rectal diazepam or buccal midazolam administered by caretakers or paramedics should be encouraged.

Objective: To determine whether practice parameters are applied to the management of children with febrile convulsion., Design: Retrospective study., Setting: Paediatric department of a public hospital, Hong Kong., Methods: Practice parameters of the American Academy of Pediatrics and audit measures recommended by the Joint Working Group of the Research Unit of the Royal College of Physicians and the British Paediatric Association were employed as standards. Records between January and April 2000 with the diagnostic coding of febrile convulsion, convulsion, status epilepticus, or meningitis/encephalitis/encephalopathy were reviewed. Areas assessed were appropriate documentation of hospital records and unit statistics (adverse outcomes, inappropriate investigations and treatment)., Results: Ninety-four consecutive records were evaluated. In the documentation of hospital notes, accurate description of seizure was observed in 92%, incorrect diagnosis or coding in 12%, and presence/absence of signs of meningitis and parental counselling documented in 64% and 85%, respectively. Regarding unit statistics, investigations performed included a complete blood count, blood glucose, serum calcium, serum electrolytes, renal function tests, liver function tests, chest X-ray, and urinalysis. The mean number of routine investigations was seven. The average length of stay was 2 days. There were no cases of delay in the diagnosis of central nervous system infection. Inappropriate investigations and treatment were as follows: electroencephalography 11%, computed tomography brain scan 2%, and maintenance anticonvulsants 2%. All patients were discharged home with panadol regardless of clinical state., Conclusions: The present study showed that the use of unnecessary investigations was common. Investigations, though resulting in significant expense, proved to be of little diagnostic value. Diagnostic procedures should be performed only when specifically called for by the patient's condition or medical history.

The aim of the study was to evaluate and compare the image quality of the 3D TOF MRA acquired with a small FOV and low phase encodes with those MR angiographic images acquired with standard pulse sequence parameters. Twenty patients who were referred to our institution for MR imaging of the brain and strictly satisfied the selection criteria were included in this study. Apart from the routine protocol for MR imaging of the brain, 3D TOF MRA of the circle of Willis with a small FOV and a standard FOV were performed. The image quality of all MRA was evaluated by two independent observers who were blind to the pulse sequence parameters. From the standard FOV MRA, 22.5, 12.5, and 5% of the patients were graded as mild, moderate, and severe stenosis of the internal carotid artery, respectively. On the contrary, no apparent stenosis was observed from the small FOV MRA with low phase encodes. Regarding the reduction in MR artifacts and acquisition time achieved with the small FOV 3D TOF MRA with low phase encodes, this might be a useful MR angiographic technique to be used in routine clinical practice.

The goal of this study is identify early predictors of intractability in childhood epilepsy. A cohort of epileptic children living in the northwest sector of Hong Kong was prospectively identified and monitored. Treatment effect was analyzed at the last follow-up before July 1, 2000. Cases were patients who had an average of at least one unprovoked seizure per month during an observational period of at least 2 years. Controls were children having achieved at least 2 seizure-free years. Strong univariate association was observed between intractability and the following factors: high initial seizure frequency, remote symptomatic etiology, infantile spasms and mixed seizure types, abnormal neurologic status, history of status epilepticus, neonatal seizures, and early breakthrough attacks after treatment initiation. Independent predictors of intractability with multiple regression were abnormal neurodevelopmental status, symptomatic etiology, and more than three seizures in the second 6 months after treatment. Our study suggested that risk of developing intractable epilepsy might be predicted, to some extent, at the early course of illness in children with abnormal neurologic status and lack of early response to treatment.

Objective: To assess the value of clinical and/or radiographic prognostic indices in predicting the clinical course and outcome of patients with acute pancreatitis, in the intensive care unit., Design: Retrospective, single institution review., Setting: An adult medical and surgical intensive care unit in a public, urban teaching hospital., Patients: Patients with acute pancreatitis requiring intensive care unit admission between January 1, 1997 and June 30, 2000., Interventions: Standard care., Measurements and Main Results: A total of 477 patients were hospitalized with the diagnosis of acute pancreatitis. Of these, 28 patients (6%) were admitted to the intensive care unit. Ranson's, Imrie scores, Acute Physiologic and Chronic Health Evaluation (APACHE) II and III scores, simplified acute physiology scores, and multiple organ dysfunction scores were tabulated at 1, 2, 3, 7, and 14 days after intensive care unit admission. Abdominal computed tomography was available for review for 24 of the 28 patients (86%), where the mean Balthazar's computed tomography index was 4.5 +/- 0.4 (range = 2 to 10). Hospital mortality rate for the intensive care unit patients was 14% (4 of 28). The intensive care unit length of stay ranged from 1 to 79 days (mean 15 days, median 5 days). Fifty-seven percent of the patients developed organ dysfunction, and 36% of the patients required mechanical ventilatory support, ranging in duration from 1 to 70 days. Infectious morbidity occurred in 43% of patients. Thirty-six percent of the patients required operative intervention for intraabdominal complications. APACHE II scores at 7 days after intensive care unit admission correlated closely with ventilator days (r2 =.90; p =.003) and correlated with the occurrence of infectious complications (r2 =.71; p =.02). Patient age, APACHE III, simplified acute physiology scores, multiple organ dysfunction scores, Ranson, Imrie, computed tomography, and APACHE II scores before day 7 did not closely correlate with the occurrence of adverse clinical outcome., Conclusions: The clinical course and outcomes of intensive care unit patients with acute pancreatitis can be highly variable. An APACHE II score <10 during the initial 48 hrs correlated with mild pancreatitis and uncomplicated intensive care unit course; however, multifactorial prognosticators were not useful for the early identification of patients who developed complications or required extended intensive care unit care.

Moyamoya syndrome has been reported in association with Down syndrome. In paediatric patients, the usual presentation is that of ischaemic stroke. We report a 9-year-old boy with Down syndrome and moyamoya syndrome who presented with acute-onset left hemiparesis. This is the first such reported case in Hong Kong. There is growing evidence that the chromosomal abnormalities in patients with Down syndrome may contribute to a vulnerability for the development of moyamoya syndrome. A high index of suspicion is necessary to make the correct diagnosis. Medical and surgical management strategies for this disease are discussed. Surgical intervention should proceed without delay, if indicated, to prevent further neurological deterioration. A multidisciplinary approach is recommended for the rehabilitation of these patients.

Background: Surgical wound infection and intra-abdominal abscess remain common infectious complications after appendectomy, especially in the setting of a perforated or gangrenous appendix. We therefore developed a clinical protocol for the management of appendicitis to decrease postoperative infectious complications., Methods: Between January 1, 1999, and December 31, 1999, 206 patients with appendicitis were treated on protocol. Retrospectively, the charts were reviewed for all protocol patients as well as for 232 patients with appendicitis treated in the year prior to protocol initiation. Data were collected on surgical wound infections and intra-abdominal abscesses., Results: There were significantly fewer infectious complications in the protocol group than in the nonprotocol group (20 [9%] versus 8 [4%]; P <0.05). In patients with a perforated or gangrenous appendix, the infectious complication rate was reduced from 33% to 13% (P <0.05)., Conclusions: The incidence of infectious complications after appendectomy can be significantly reduced with a standardized approach to antibiotic therapy and wound management.

Unlabelled: D-2-Hydroxyglutaric aciduria (D-2-HG) is a rare metabolic disorder. First reported in 1980, it does not have any well-recognized presentation or specific treatment regimen. Neuroimaging findings are heterogeneous. Subdural haemorrhage has not been a feature of any of the reported cases. This report presents a boy with bilateral subdural haematoma in whom non-accidental injury was initially suspected and subsequent metabolic investigation led to a diagnosis of D-2-HG., Conclusion: In the management of childhood subdural haemorrhages, it is very important that potentially treatable metabolic disorders are detected and that parents are not wrongly accused of injuring their children.

Objective: To assess the utility of triage guidelines for patients with cholelithiasis and suspected choledocholithiasis, incorporating selective use of magnetic resonance cholangiography (MRC) and endoscopic retrograde cholangiopancreatography (ERCP) before laparoscopic cholecystectomy (LC)., Summary Background Data: ERCP is the most frequently used modality for the diagnosis and resolution of choledocholithiasis before LC. MRC has recently emerged as an accurate, noninvasive modality for the detection of choledocholithiasis. However, useful strategies for implementing this diagnostic modality for patient evaluation before LC have not been investigated., Methods: During a 16-month period, the authors prospectively evaluated all patients before LC using triage guidelines incorporating patient information obtained from clinical evaluation, serum chemistry analysis, and abdominal ultrasonography. Patients were then assigned to one of four groups based on the level of suspicion for choledocholithiasis (group I, extremely high; group 2, high; group 3, moderate; group 4, low). Group 1 patients underwent ERCP and clearance of common bile duct stones; group 2 patients underwent MRC; group 3 patients underwent LC with intraoperative cholangiography; and group 4 patients underwent LC without intraoperative cholangiography., Results: Choledocholithiasis was detected in 43 of 440 patients (9.8%). The occurrence of choledocholithiasis among patients in the four groups were 92.6% (25/27), 32.4% (12/37), 3.8% (2/52), and 0.9% (3/324) for groups 1, 2, 3, and 4, respectively (P <.001). MRC was used for 8.4% (37/440) of patients. Patient triage resulted in the identification of common bile duct stones during preoperative ERCP in 92.3% (36/39) of the patients. Unsuspected common bile duct stones occurred in six patients (1.4%)., Conclusions: The probability of choledocholithiasis can be accurately assessed based on information obtained during the initial noninvasive evaluation. Stratification of risks for choledocholithiasis facilitates patient management with the most appropriate diagnostic studies and interventions, thereby improving patient care and resource utilization.

The present study describes the characteristics of epilepsy in a cohort of Chinese epileptic children. All children with active epilepsy who were under 15 years of age and residing in the catchment area of Tuen Mun Hospital, Hong Kong were monitored. Etiology, seizure types, and epilepsy syndromes were classified according to the recent guidelines of the International League Against Epilepsy. A total of 309 children were recruited into the study. The etiology of epilepsy was idiopathic in 42% of the children, cryptogenic in 16.8%, and remote symptomatic in 40.8%. Perinatal factors were the most frequently found cause of epilepsy. Seizure types were partial in 48.5% of the children and generalized in 46.9%. Epilepsy syndromes could be classified in all but seven patients, with 48.2% localization related and 49.5% generalized. Generalized seizures were more prevalent in children less than 5 years of age. Additional neuroimpairments affected 36% of our epileptic children. Sixty-nine percent of patients were seizure free for more than 1 year. The authors conclude that the International League Against Epilepsy can be applied successfully to a population-based cohort of Chinese epileptic children. A larger, longitudinal epidemiologic study is needed to answer questions concerning the true prevalence, incidence, types, and etiologies in the Chinese population.

Parkinsonism complicating systemic lupus erythematosus (SLE) is extremely rare. We report two girls with SLE who developed extrapyramidal parkinsonian features after an initial stormy course. One patient presented with generalized tonic clonic seizure and was then noted to have akinetic mutism and masked face. MRI brain revealed abnormal signals in bilateral basal ganglia and single photon emission computed tomography (SPECT) showed hypoperfusion in the same area. EEG background was slow and disorganized. Symptoms persisted despite high dose intravenous methylprednisolone and cyclophosphamide. Intravenous immunoglobulin (IVIG) was prescribed empirically and was followed by complete recovery. Both EEG and MRI brain were normal on follow-up. The second patient was found unconscious and then developed bradykinesia, mutism and shuffling gait. MRI and SPECT both detected abnormalities in basal ganglia. EEG was slow. Intravenous immunoglobulin was given after methylprednisolone and cyclophosphamide. This was followed by clinical improvement. The pathogenesis of basal ganglia injury in SLE, along with the management of cerebral lupus and the mechanisms of action of IVIG, are discussed.

Epilepsy occurs in 15-60% of children with cerebral palsy; however, its clinical course is not well defined. This retrospective study reviewed the prevalence, nature, and prognosis of epilepsy in cerebral palsy. Thirty-two of 85 children with cerebral palsy seen in the Neurodevelopmental Clinic in Tuen Mun Hospital between 1990 and 1995 had epilepsy. A control group of 59 epileptic children with normal neurodevelopment status was seen during the same period. Epilepsy most commonly affected patients with spastic tetraplegia and those with mental subnormality. When compared with controls, children with cerebral palsy had a higher incidence of epilepsy with onset within the first year of age (47% vs 10%), history of neonatal seizures (19% vs 3%), status epilepticus (16% vs 1.7%), polytherapy (25% vs 3%), and treatment with second-line antiepileptic drugs (31% vs 6.7%). They had a lower incidence of generalized seizures (28% vs 59%) and remaining seizure free (37% vs 90%). Factors associated with a seizure-free period of 1 year or more in epileptic children with cerebral palsy were normal intelligence, single seizure type, monotherapy, and spastic diplegia. Epilepsy was common in children with cerebral palsy. Further larger studies are required to delineate other prognostic factors.