Publications by authors named "Claire Spooner"

Aim: Hypovitaminosis A is a leading cause of preventable childhood blindness, especially in developing nations. Vitamin A is a fat-soluble essential micronutrient that serves vital functions in the visual system and in regulating bone resorption. We report on a series of four children with mixed nutritional and compressive optic neuropathy and provide a review of the literature.

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Myelin oligodendrocyte glycoprotein (MOG) antibodies have a strong association with acute disseminated encephalomyelitis (ADEM) in children, and bilateral and recurrent optic neuritis in children and adults. Recent reports suggest that seizures and encephalopathy may occur in children and adults with MOG antibody-associated disease. We describe the clinical, laboratory, and radiological course of four MOG antibody-positive children who first presented with isolated seizures without fulfilling clinical or radiological criteria for ADEM or other central nervous system demyelination syndromes, who months to years later developed more typical demyelination.

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Article Synopsis
  • The study investigates how childhood-onset temporal lobe epilepsy (TLE) affects developmental milestones and identifies predictors for long-term outcomes in individuals.
  • A cohort of 54 individuals with TLE was evaluated, revealing three distinct groups based on their achievement of developmental tasks: Normal (52%), Altered (37%), and Delayed (11%), with significant cognitive performance differences among the groups.
  • Medical factors like seizure chronicity, cognitive ability, the potential for surgical intervention, and gender were found to predict group membership, highlighting the importance of monitoring developmental tasks to provide better support for affected individuals and their families.
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Objectives: To determine whether there is a relationship between electroencephalography patterns and hypoglycemia, by using simultaneous cot-side amplitude integrated electroencephalography (aEEG) and continuous interstitial glucose monitoring, and whether non-glucose cerebral fuels modified these patterns.

Study Design: Eligible babies were ≥ 32 weeks gestation, at risk for hypoglycemia, and admitted to the neonatal intensive care unit. Electrodes were placed in C3-P3, C4-P4 O1-O2 montages.

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Article Synopsis
  • This study investigates how childhood-onset temporal lobe epilepsy (TLE) affects long-term psychological health, analyzing outcomes related to the condition and its treatment.
  • Approximately 13 years post-seizure onset, researchers assessed 53 patients through interviews and self-report questionnaires on quality of life, depression, self-esteem, and anxiety.
  • Four distinct psychological outcome groups emerged: those with spontaneous remission had minimal issues, surgery patients had varied adjustments, non-seizure-free surgical patients experienced significant depression, and those with ongoing epilepsy faced severe chronic illness effects.
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Background: Zellweger syndrome spectrum disorders are caused by mutations in any of at least 12 different PEX genes. This includes PEX16, which encodes an integral peroxisomal membrane protein involved in peroxisomal membrane assembly. PEX16-defective patients have been reported to have a severe clinical presentation.

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