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Basal Ganglia Disease Mimicking Acute Encephalitis Syndrome Among Infants of Bodo Tribe, Assam
Jemin J Webster1, Shafini Beryl2, Koshy George3
1Baptist Christian Hospital, Tezpur, Assam. jeminwebster@hotmail.com.
Insights
Thiamine supplementation significantly reduced mortality in infants with acute encephalitis syndrome and basal ganglia infarcts. This finding suggests potential benefits for thiamine deficiency in affected infants.
Area of Science:
- Neurology
- Pediatrics
- Nutritional Neuroscience
Background:
- Acute encephalitis syndrome (AES) in infants can lead to severe neurological complications, including basal ganglia infarcts.
- Bilateral symmetrical basal ganglia infarcts in infants are associated with high mortality and poor neurodevelopmental outcomes.
- The role of specific nutritional deficiencies, such as thiamine, in AES with basal ganglia involvement is not well-established.
Purpose of the Study:
- To investigate the impact of thiamine administration on mortality and neurodevelopmental outcomes in infants diagnosed with AES and bilateral symmetrical basal ganglia infarcts.
- To explore potential etiological links, including subclinical thiamine deficiency, mitochondrial disorders, or genetic mutations, in this patient cohort.
Main Methods:
- Retrospective review of hospital records for infants diagnosed with AES and bilateral symmetrical basal ganglia infarcts between 2011-2015 at a single center in Assam.
- Comparison of mortality rates and neurodevelopmental outcomes between infants who received thiamine (as part of multivitamin injection) and those who did not.
Main Results:
- A significantly lower mortality rate was observed in infants treated with thiamine (3.7%) compared to those who did not receive thiamine (86.9%).
- The relative risk of death was substantially reduced with thiamine treatment (RR 0.04; 95% CI 0.006-0.29; P<0.001).
- Normal neurodevelopmental outcomes were observed in two infants who received thiamine during follow-up.
Conclusions:
- Thiamine administration appears to be associated with a dramatic reduction in mortality for infants suffering from AES with bilateral symmetrical basal ganglia infarcts.
- The findings suggest a potential role for subclinical thiamine deficiency in the pathogenesis of this condition.
- Further investigation into mitochondrial diseases and SLC19A3 gene mutations is warranted in this population.
Abstract:
We conducted a review of hospital records of infants with acute encephalitis syndrome with bilateral symmetrical basal ganglia infarcts, between 2011-2015, at a single center in Assam. Thiamine (as part of multivitamin injection) was used in the treatment of 23 infants and not used in 27; Only 1 (3.7%) infant died in the former group and 20 infants (86.9%) died in the latter [RR (95% CI) 0.04 (0.006,0.29); P<0.001). Two infants on follow-up had normal development, both in the thiamine group. The study suggests the possibility of subclinical thiamine deficiency, mitochondrial diseases, or SLC19A3 gene mutation in this population.
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