ICNC Abstracts, ICNC 2018

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A unique variant of West Syndrome:Vitamin B12 responsive epileptic encephalopathy
Harshkumar Arvindbhai Patel, Prarthana Kharod, Prarthana Kharod

Last modified: 2018-09-09

Abstract


Aim

In developing countries vitaminB12 deficiency is common in infants which is mainly nutritional. Neurological symptoms of vitaminB12 deficiency are heterogeneous and include apathy,hyoptonia,feeding difficulty, developmentaldelay or regression, involuntary movements, seizures and coma. Anecdotal case reports of west syndromes due to vitamin b12 deficiency have been described. The objective of this study to describe clinical profile of infants presenting with west syndrome due to vitaminB12 deficiency.

Methods

During study period from april 2016 to march 2018 total 11 infants were included at two centres. Their clinical, biochemical, radiological and electrophysiological details were recorded and short term neurological follow up was obtained.

Results

Out of 11 infants 7 (64%) were boys and 4 (36%) were girls. Mean age was 10.2+ 2.8 months. Median age of onset of west syndrome was 9 months. Vitamin B12 deficiency was diagnosed based on increased homocystine levels. Six children out of 11 were treated with vitaminB12 and folic acid and 5 were treated with high dose oral steroids, Valproate along with vitaminB12. All patients were responded and spasms free. Median age of response was 8 weeks after the onset of treatment. Gain of developmental milestones was noted in all patients however 6 children had delayed milestones till last visit.

Conclusion

West syndrome is infantile onset epileptic encephalopathy warranting aggressive treatment. This study highlights potentially treatable entity as a cause of West syndrome. Vitamin B12 deficiency should be considered while managing infants with West syndrome as prompt recognition and early treatment has substantial­­implications throughout life.



Keywords


Infantile spasms,west syndrome,vitamin B12 deficiency

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