Epilepsy in children with cerebral palsy.

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.

Epidemiology of childhood epilepsy in a cohort of 309 Chinese children.

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.

3D time of flight MR angiography: acquisition with small field of view and low phase encodes.

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.

Features predicting adverse outcomes of status epilepticus in childhood.

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.

Management of febrile convulsion: scene in a regional hospital.

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.

Moyamoya syndrome in a child with Down syndrome.

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.

Mortality from isolated civilian penetrating extremity injury.

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.

Neurodevelopmental profile of Down syndrome in Chinese children.

OBJECTIVE: To give an overall appraisal of the clinical features of Down syndrome (DS) in Chinese children with emphasis on the neurodevelopmental outcome, and to compare the related complications with that of other races. METHODOLOGY: The records of 124 Chinese children with DS assessed at the Child Assessment Centre of the University Department of Paediatrics in the Duchess of Kent Children's Hospital from 1985 to 1993 were reviewed. RESULTS: Thirty-one per cent of patients had microcephaly. Eighty-five percent (33/39) when assessed in the first year of life had a developmental quotient (DQ) above 50 but only 29% (2/7) had DQ above 50 when assessed after the age of 5. Only two patients (1.6%) had epilepsy: infantile spasms (1) and Lennox-Gastaut syndrome (1). Hearing impairment was found in 45% of children with mild conductive hearing impairment being the most common. CONCLUSIONS: Chinese children with DS, when compared with other races, were similarly intellectually disabled, but were less likely to develop epilepsy.

Parkinsonism as unusual neurological complication in childhood systemic lupus erythematosus.

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.

Parental perception, worries and needs in children with epilepsy.

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.

Fatal meningoencephalitis due to Bacillus anthracis.

We report the first case of fatal anthrax meningoencephalitis in Hong Kong over the past 60 years. A 13 year-old boy presented with right lower quadrant pain, diarrhoea and progressive headache. Lumbar puncture yielded gram positive bacilli initially thought to be Bacillus cereus, a contaminant. He was treated with ampicillin and cefotaxime, but died 3 days after hospitalization. The organism isolated from blood and cerebrospinal fluid was later identified as Bacillus anthracis.

Paediatric Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is characterized by a dull, persistent pain around the affected eye, ophthalmoplegia and, sometimes, involvement of other cranial nerves passing through the cavernous sinus. Corticosteroid administration is valuable in the treatment and frequently has a dramatic effect. We report a boy with Tolosa-Hunt syndrome who fails to respond to the initial steroid treatment. The role of the MRI in the management of this condition is discussed.

Status epilepticus in 37 Chinese children: aetiology and outcome.

OBJECTIVE: The aetiology and clinical features of status epilepticus (SE) are described, with the aim of defining any relationship between risk factors and clinical outcome. METHODOLOGY: A retrospective review was performed of 37 Chinese children admitted to Queen Mary Hospital, Hong Kong, from 1989 to 1993 with the diagnosis of SE. RESULTS: Eighty-six per cent had onset before 5 years of age; 60% were due to an acute central nervous system (CNS) insult, 11% were idiopathic, 13% had a pre-existing CNS insult, 5% were febrile and 11% were due to progressive encephalopathy. An abnormal neurological status was present in 24% before the episode of SE, and a history of seizures before the onset of SE was present in 35% of patients. Fifty-four per cent of the episodes of SE were generalized. The mortality rate was 11% during the period of follow up but no deaths were attributed to SE. Neurological sequelae were observed in 27% of patients and recurrent SE occurred in 12%. CONCLUSIONS: In those patients with normal neurological status before an episode of SE and without acute CNS insult or progressive encephalopathy, the outcome was favourable.

D-2-Hydroxyglutaric aciduria and subdural haemorrhage.

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.

Patient evaluation and management with selective use of magnetic resonance cholangiography and endoscopic retrograde cholangiopancreatography before laparoscopic cholecystectomy.

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.