We present a case series of children with relapsing inflammatory demyelination who live on the island of Borneo, a relatively rural region of South East Asia with a warm, tropical climate. The four cases are a 9 year old girl with a polyfocal clinically isolated syndrome who had a single recurrence of symptoms soon after completing steroid taper, a 6 year old boy with relapsing-remitting multiple sclerosis (MS), an 8 year old boy with relapsing neuromyelitis optica and an 8 year old boy with chronic recurrent optic neuritis (CRION). The clinical presentation and neuroimaging features in our children are similar those seen in children living in temperate world regions. The new recommendations by the International Pediatric Multiple Sclerosis Study Group (IPMSSG) in classifying childhood inflammatory demyelinating disorders are useful in our children. This report hopes to raise awareness of relapsing inflammatory demyelinating syndromes in children living in a region with a low risk for MS.

Background & Objective: Continuous electroencephalography (cEEG) is valuable in the diagnosis and management of refractory status epilepticus (RSE) but requires intensive skilled interpretation. Density spectral array (DSA) is a quantitative analytic tool used to screen cEEG recordings for seizures. This study aims to determine the optimal amplitude setting and to compare the use of single-averagetrendgraph display and eight-trendgraph display in seizure detection with DSA. Methods: Five excerpts from pediatric cEEG recordings with RSE were identified. In Phase 1 of the study, each of 4 readers determined the DSA amplitude setting one most preferred for each excerpt, and marked all seizures in one excerpt using one’s preferred setting. Inter-rater agreement in seizure detection was measured. In Phase 2, readers marked all seizures in all excerpts, first using single-average-trendgraph display, and then using eight-trendgraph display after a wash-out period. Intra-rater agreement in seizure detection between the two display methods was calculated. Results: In Phase 1, DSA readers’ choice of preferred amplitude settings varied widely but inter-rater agreement in seizure detection was high. In Phase 2, seizure detection using single- and eight-trendgraph displays showed high agreement with each other and, where they disagreed, single-average-trendgraph was more sensitive. Additionally, low seizure-to-background amplitude ratio in EEG recordings was associated with worse detection sensitivity/specificity. Conclusions: DSA amplitude settings do not affect seizure detection. Single-trendgraph display is comparable to eight-trendgraph display in screening cEEG for seizures. Seizure detection with DSA performs better in cEEG recordings with high seizure-to-background amplitude ratio.

AIM:To report unusual presentation and good outcome of neuromyelitis optica(NMO) in a child.METHODS:Case Report.RESULTS: An 8-year-old girl presented with 5-day history of sudden bilateral visual deterioration followed by left lower limb weakness. Visual acuity was perception to light in both eyes. Funduscopy revealed bilateral hyperaemic swollen optic discs. MRI of brain and spine revealed enhancing white matter lesions in the right frontal lobe and spinal cord at C5 level. She was diagnosed NMO and treated with intravenous methylprednisolone and tapering doses of oral prednisolone as maintenance therapy.Symptoms gradually improved 1 month after treatment. However, she presented with similar presentation 1 week after stopping oral prednisolone. MRI of brain and spine were reviewed and showed enhancing lesion in the right frontal lobe and longitudinal segment of spinal cord from C3 to C5 level. She was promptly given intravenous methylprednisolone for 5 days followed by prolonged tapering of oral prednisolone over 6 months period. After 2 months, she was able to walk and attend activities of school. Visual acuity was improved to 6/10 in both eyes and neurological examination was normal. There was no recurrence during the next year. Final visual acuity was 6/7.5 in the the right eye and 6/10 in the left eye.CONCLUSION: The diagnosis of NMO should be kept in mind although it is unusual presentation in child presented with bilateral visual loss and unilateral lower limb weakness. Early diagnosis and treatments would yield good outcome to the patient.

INTRODUCTIONMortality risk prediction scores are important for benchmarking quality of care in paediatric intensive care units (PICUs). We aimed to benchmark PICU outcomes at our hospital against the Pediatric Index of Mortality 2 (PIM2) mortality risk prediction score, and evaluate differences in diagnosis on admission and outcomes between Malaysian and immigrant children.

METHODSWe prospectively collected demographic and clinical data on paediatric medical patients admitted to the PICU of Sabah Women's and Children's Hospital in Kota Kinabalu, Sabah, Malaysia. The PIM2 risk score for mortality was tabulated.

RESULTSOf the 131 patients who met the inclusion criteria, data was available for 115 patients. The mean age of the patients was 2.6 ± 3.8 years, with 79% of the cohort aged less than five years. Patients were mainly of Kadazan (38%) and Bajau (30%) descent, and 26% of patients were non-citizens. Leading diagnoses on admission were respiratory (37%), neurological (18%) and infectious (17%) disorders. Out of the 29 patients who died, 23 (79%) were Malaysians and the main mortality diagnostic categories were respiratory disorder (22%), septicaemia (22%), haemato-oncological disease (17%) and neurological disorder (13%). Calculated standardised mortality ratios (SMRs) were not significantly > 1 for any patient category for variables such as age and admission diagnosis. However, infants less than two years old with comorbidities were significantly worse (SMR 2.61, 95% confidence interval 1.02-6.66).

CONCLUSIONThe patient profile at our centre was similar to that reported from other PICUs in Asia. The PIM2 score is a useful mortality risk prediction model for our population.

Adolescent ; Borneo ; Child ; Child, Preschool ; Comorbidity ; Critical Care ; methods ; Critical Illness ; mortality ; Female ; Humans ; Infant ; Intensive Care Units, Neonatal ; Intensive Care Units, Pediatric ; Malaysia ; Male ; Mortality ; Prospective Studies ; Quality of Health Care ; Risk Assessment ; Severity of Illness Index

No abstract available.
Hong Kong* ; Rehabilitation* ; Stroke*