Abstract

IntroductionNodding syndrome (NS) is associated with high anion gap, biotinidase and acetyl carnitine deficiency, vitamin B6 and D deficiency and internal displacement. The objective of this study was to conduct a metabolic analysis on NS children and review literature on its similarities with ASD.MethodsWe conducted biochemical analysis on blood and urine of NS children at Hope for HumaNs (HfH) centre in 2014 and reviewed literature on its similarities with ASD. Ethical approval was obtained from an IRB. Data analysis was conducted using STATA version 12 and a p-value less than 0.05 was considered significant.ResultsWe found biotinidase deficiency in NS with a mean 1.98 95% CI(1.61, 2.34; p < 0.001); Acetyl carnitine deficiency 16.92 95% CI(16.10,17.75; p<0.001); Low BMI-for-age 16.92 95% CI(16.10,17.75; p = 0.42); Age 14.08 95% CI(0.78,4.660; p = 0.007); IDP duration 4.82 95% CI(4.48, 5.21; p = 0.92); Age at NS onset 8.02 95% CI(7.03, 9.01; p = 0.001); NS associated with multiple nodding episodes (χ2)=22.15, p=0.005; NS siblings with NS (χ2) = 9.68, p = 0.004; NS were in IDPs (χ2) = 22.15, p = 0.005.ConclusionThese findings are indicative that NS is associated with biotinidase and acetyl carnitine deficiency, IDPs, and environmental exposures. There are no new cases of NS reported by Ugandan MOH and WHO since 2012 when the IDP camps were disbanded and communities resettled in their own communities and feed on their own grown foods. Perhaps NS may be akin to Autism Spectrum Disorder (ASD). This finding will help support all efforts towards the treatment and rehabilitation of NS children.

Highlights

  • Nodding syndrome (NS) is associated with high anion gap, biotinidase and acetyl carnitine deficiency, vitamin B6 and D deficiency and internal displacement

  • All NS children were in IDPs (Figure 2) and the majority (77%) had dropped-out of school

  • NS children were in IDPs, fed on IDP diets which were mainly foreign to their GI microbiome and other environmental exposures

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Summary

Introduction

Nodding syndrome (NS) is associated with high anion gap, biotinidase and acetyl carnitine deficiency, vitamin B6 and D deficiency and internal displacement. The objective of this study was to conduct a metabolic analysis on NS children and review literature on its similarities with ASD. Case series and case reports conducted in Uganda identified high anion gap metabolic acidosis among NS children compared to their sex-and-age matched controls [9,10,11]. This researcher avers that nodding episodes are precipitated by sights of local food, starvation, exposure to cold weather/temperatures or cold water, physical exercises and there is an association with high anion gap [9,12].

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