Infantile Tremor Syndrome Followed by West Syndrome: Effect or Continuation of Spectrum?

J Trop Pediatr. 2020 Oct 1;66(5):556-560. doi: 10.1093/tropej/fmaa014.

Abstract

Background: Infantile tremor syndrome (ITS) is a nutritional deficiency syndrome, frequently reported from the Indian subcontinent caused by vitamin B12 deficiency. The West syndrome (WS), on the other hand, is a type of epileptic encephalopathy with variable etiology.

Case series: We present a series of five children who presented with symptoms consistent with ITS and received standard intramuscular vitamin B12 therapy to which good response was observed. All these children were readmitted with WS with a time lag varying from 2 to 12 months. Magnetic resonance imaging brain and metabolic screen were within normal limits. Three out of five patients responded well to adrenocorticotropic hormone (ACTH), remaining two required additional drugs. We hypothesize that WS could have developed after ITS due to developmental desynchronization.

Conclusion: In the Indian scenario, it is particularly important to be aware of appearance of WS after ITS, because of high prevalence of ITS and devastating nature of WS.

Keywords: West syndrome; infantile tremor syndrome; vitamin B12 deficiency.

Publication types

  • Case Reports

MeSH terms

  • Adrenocorticotropic Hormone / therapeutic use
  • Brain / diagnostic imaging
  • Child
  • Humans
  • Infant
  • Injections, Intramuscular
  • Magnetic Resonance Imaging
  • Spasms, Infantile / diagnosis*
  • Spasms, Infantile / drug therapy
  • Spasms, Infantile / etiology
  • Syndrome
  • Tremor / drug therapy*
  • Tremor / etiology
  • Vitamin B 12 / administration & dosage*
  • Vitamin B 12 / therapeutic use
  • Vitamin B 12 Deficiency / drug therapy*

Substances

  • Adrenocorticotropic Hormone
  • Vitamin B 12