7 results on '"Chong, W."'
Search Results
2. Magnetic resonance imaging pattern recognition in childhood bilateral basal ganglia disorders
- Author
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Mohammad, Shekeeb S., Angiti, Rajeshwar Reddy, Biggin, Andrew, Morales-Briceño, Hugo, Goetti, Robert, Pérez-Dueñas, Belén, Gregory, Allison, Hogarth, Penelope, Ng, Joanne, Papandreou, Apostolos, Bhattacharya, Kaustuv, Rahman, Shamima, Prelog, Kristina, Webster, Richard I, Wassmer, Evangeline, Hayflick, Susan, Livingston, John, Kurian, Manju, Chong, W. Kling, Dale, Russell C., and Universitat Autònoma de Barcelona
- Subjects
Pattern recognition ,Basal ganglia ,Striatal necrosis ,Striatum ,MRI - Abstract
Bilateral basal ganglia abnormalities on MRI are observed in a wide variety of childhood disorders. MRI pattern recognition can enable rationalization of investigations and also complement clinical and molecular findings, particularly confirming genomic findings and also enabling new gene discovery. A pattern recognition approach in children with bilateral basal ganglia abnormalities on brain MRI was undertaken in this international multicentre cohort study. Three hundred and five MRI scans belonging to 201 children with 34 different disorders were rated using a standard radiological scoring proforma. In addition, literature review on MRI patterns was undertaken in these 34 disorders and 59 additional disorders reported with bilateral basal ganglia MRI abnormalities. Cluster analysis on first MRI findings from the study cohort grouped them into four clusters: Cluster 1-T-weighted hyperintensities in the putamen; Cluster 2-T-weighted hyperintensities or increased MRI susceptibility in the globus pallidus; Cluster 3-T-weighted hyperintensities in the globus pallidus, brainstem and cerebellum with diffusion restriction; Cluster 4-T-weighted hyperintensities in the basal ganglia. The 34 diagnostic categories included in this study showed dominant clustering in one of the above four clusters. Inflammatory disorders grouped together in Cluster 1. Mitochondrial and other neurometabolic disorders were distributed across clusters 1, 2 and 3, according to lesions dominantly affecting the striatum (Cluster 1: glutaric aciduria type 1, propionic acidaemia, 3-methylglutaconic aciduria with deafness, encephalopathy and Leigh-like syndrome and thiamine responsive basal ganglia disease associated with SLC19A3), pallidum (Cluster 2: methylmalonic acidaemia, Kearns Sayre syndrome, pyruvate dehydrogenase complex deficiency and succinic semialdehyde dehydrogenase deficiency) or pallidum, brainstem and cerebellum (Cluster 3: vigabatrin toxicity, Krabbe disease). The Cluster 4 pattern was exemplified by distinct T-weighted hyperintensities in the basal ganglia and other brain regions in genetically determined hypermanganesemia due to SLC39A14 and SLC30A10. Within the clusters, distinctive basal ganglia MRI patterns were noted in acquired disorders such as cerebral palsy due to hypoxic ischaemic encephalopathy in full-term babies, kernicterus and vigabatrin toxicity and in rare genetic disorders such as 3-methylglutaconic aciduria with deafness, encephalopathy and Leigh-like syndrome, thiamine responsive basal ganglia disease, pantothenate kinase-associated neurodegeneration, TUBB4A and hypermanganesemia. Integrated findings from the study cohort and literature review were used to propose a diagnostic algorithm to approach bilateral basal ganglia abnormalities on MRI. After integrating clinical summaries and MRI findings from the literature review, we developed a prototypic decision-making electronic tool to be tested using further cohorts and clinical practice. Analysing a cohort of 201 children with bilateral basal ganglia MRI abnormalities in conjunction with a literature review, we propose a stepwise approach for 93 different disorders. Disorders cluster based on MRI changes in the putamen, globus pallidus alone or with brainstem and cerebellar changes and with T2W versus T1W hyperintensities in the basal ganglia
- Published
- 2020
3. Pantothenate kinase 2 mutation with classic pantothenate-kinase-associated neurodegeneration without ‘eye-of-the-tiger’ sign on MRI in a pair of siblings
- Author
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Zolkipli, Zarazuela, Dahmoush, Hisham, Saunders, Dawn E., Chong, W. K. Kling, and Surtees, Robert
- Published
- 2006
- Full Text
- View/download PDF
4. Paediatric MOG antibody–associated ADEM with complex movement disorder: A case report.
- Author
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Sa, Mario, Thornton, Rachel, Chong, W. K. Kling, Kaliakatsos, Marios, and Hacohen, Yael
- Subjects
MULTIPLE sclerosis ,DEMYELINATION ,NEUROMYELITIS optica ,ENCEPHALOMYELITIS ,IMMUNOGLOBULINS - Abstract
Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are a well-recognized cause of acquired demyelinating syndromes in both adult and children. Despite basal ganglia involvement on imaging, movement disorder is not a cardinal feature. We describe a 2-year-9-month-old girl who presented with severe encephalopathy with aphasia, seizures and a complex movement disorder with dystonic posturing and tonic eye deviation. Neuroimaging revealed subtle asymmetrical predominantly white matter signal changes. MOG-Abs were positive in the serum. Other known pathogenic autoantibodies including N-methyl-D-aspartate receptor antibodies (NMDAR-Abs) were negative. The patient made a complete recovery following 2-week corticosteroid treatment. This case highlights the need for MOG-Ab testing in children with suspected autoimmune encephalopathies. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
5. Cerebral Magnetic Resonance Imaging in Children With Prenatal Drug Exposure.
- Author
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Sirnes, Eivind, Elgen, Irene B., Chong, W. K., Griffiths, Silja T., and Aukland, Stein Magnus
- Subjects
COMPARATIVE studies ,CONFIDENCE intervals ,LONGITUDINAL method ,MAGNETIC resonance imaging ,RESEARCH funding ,T-test (Statistics) ,DATA analysis ,CONTROL groups ,DATA analysis software ,DESCRIPTIVE statistics ,PRENATAL exposure delayed effects ,ODDS ratio - Abstract
This study aimed to evaluate cerebral magnetic resonance imaging (MRI) scans of children with prenatal drug exposure in a clinical context. A hospital-based cohort of 10- to 14-year-old children, prenatally exposed to alcohol, opioids, and polysubstances, and a group of sex- and age-matched controls were examined with cerebral MRI. Scans obtained from 34 exposed children and 40 controls were scored based on the presence and degree of pathology by an experienced pediatric neuroradiologist blinded to the participants’ background. Overall visual detectable MRI pathology was found in 35% of the exposed children and 33% of the controls (odds ratio = 1.08; 95% confidence interval = 0.36-3.25). No specific imaging pattern following prenatal drug exposure was seen by the means of simple visual analysis of cerebral MRI scans. Although cerebral MRI is feasible, it is probably of limited value in the clinical assessment of children with prenatal drug exposure. [ABSTRACT FROM AUTHOR]
- Published
- 2017
- Full Text
- View/download PDF
6. Hereditary folate malabsorption: effect of systemic folate supplements on myelination.
- Author
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Paul, Siba Prosad, Candy, David C. A., Clayton, Peter, Chong, W. K., and Wallace, Ann
- Abstract
Folate deficiency is a rare but important cause of macrocytic anaemia in infancy. This article describes folate deficiency in a four-month-old male infant who presented with non-specific symptoms, but was found to have megaloblastic anaemia with very low serum folate due to congenital folate malabsorption. The critical role of folic acid in brain development is highlighted. [ABSTRACT FROM AUTHOR]
- Published
- 2011
7. Is streamlined evaluation of children for epilepsy surgery possible?
- Author
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Patil, Shekhar G., Cross, J. Helen, Chong, W. Kling, Boyd, Stewart G., Harkness, William J., Neville, Brian G. R., and Scott, Rod C.
- Subjects
CHILDHOOD epilepsy ,JUVENILE diseases ,MAGNETIC resonance imaging ,ELECTROENCEPHALOGRAPHY ,TREATMENT of epilepsy - Abstract
The presurgical evaluation of children with intractable epilepsy includes evaluation by an experienced clinician, MRI, video EEG, and functional imaging techniques to localize seizure onset. However, the contributions of each investigation to surgical decision making has not been systematically assessed. Data used for decision on eligibility for surgery on 353 children was discussed at a presurgical multidisciplinary meeting and systematically recorded. The relationships between MRI, EEG, SPECT findings, and the probability of being offered epilepsy surgery were investigated retrospectively using a quick unbiased statistical tree (QUEST). Sixteen children were offered nonresective surgery. Of the remaining, 236 (70%) were offered resective surgery. The proportion of children with a localized lesion on MRI offered resective surgery was 92%[95% CI: 88 to 95%], and EEG telemetry did not modify decision making in this group (p < 0.001). In children with bilateral MRI changes or normal scan the probability of being offered resective surgery was 78% in those with localized ictal onset on EEG compared to 9% with nonlocalized EEG (p < 0.001). SPECT did not appear to systematically influence decision making in any group. Children with medically intractable epilepsy and localized lesions on MRI may not necessarily need ictal EEG recordings or SPECT prior to offering resective surgery. More targeted use of EEG telemetry could allow more children with less obvious surgical targets to be investigated without increasing resources. [ABSTRACT FROM AUTHOR]
- Published
- 2008
- Full Text
- View/download PDF
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