Child's Nervous System

, 25:1477 | Cite as

Infantile encephalopathy due to vitamin deficiency in industrial countries

  • Ibrahim Abu-Kishk
  • Marianna Rachmiel
  • Chen Hoffmann
  • Eli Lahat
  • Gideon Eshel
Original Paper



Severe avitaminosis causing life-threatening conditions in the infantile age group is extremely uncommon and has been reported in babies with malabsorption receiving prolonged inadequate vitamin supplements.

Case reports

We report two infants who presented with neurological deterioration. Immediate work-up and treatment for infectious and inborn metabolic disorders were initiated and the diagnosis, made with a few days delay, was prolonged avitaminosis of thiamine (B1) and cobalamin (B12). B1 deficiency was suspected when further neurological deterioration was observed during administration of intravenous fluids containing glucose in an infant with high lactate levels in the cerebrospinal fluid. High transketolase activity that normalized after thiamine treatment and the findings in the MRI and MRS of the brain confirmed the suspected diagnosis. B12 deficiency was suspected in an infant of a strict vegetarian mother who presented with neurological deterioration and severe megaloblastic anemia. The diagnosis was confirmed when low serum levels of B12 and methylmalonic aciduria were detected and treatment with B12 resulted in normalization of urinary methymalonic acid.


Avitaminosis, even in industrialized countries, should be considered in an atypical age group with no known risk factors. Early diagnosis and prompt treatment may accomplish a quick recovery with fewer sequelae.


Wernicke's encephalopathy Thiamine deficiency Cobalamin deficiency Avitaminosis 


  1. 1.
    Decker MJ, Isaacman DJ (2000) A common cause of altered mental status occurring at an uncommon age. Pediatr Emerg Care 16:94–96PubMedCrossRefGoogle Scholar
  2. 2.
    Harper CG, Giles M, Finlay-Jones R (1986) Clinical signs in the Wernicke–Korsakoff complex: a retrospective analysis of 131 cases diagnosed at necropsy. J Neurol Neurosurg Psychiatry 49:341–345PubMedCrossRefGoogle Scholar
  3. 3.
    Vasconcelos MM, Silva KP, Vidal G, Silva AF, Domingues RC, Berditchevsky CR (1999) Early diagnosis of pediatric Wernicke’s encephalopathy. Ped Neuro 20:289–294CrossRefGoogle Scholar
  4. 4.
    Fattal-Valevski A, Kesler A, Sela BA, Nitzan-Kaluski D, Rotstein M, Mesterman R, Toledano-Alhadef H, Stolovitch C, Joffmann C, Globus O, Eshel G (2005) Outbreak of life-threatening thiamine deficiency in infants in Israel caused by a defective soy-based formula. Pediatrics 115:e233–e238PubMedCrossRefGoogle Scholar
  5. 5.
    Allen LH (1994) Vitamin B12 metabolism and status during pregnancy, lactation and infancy. Adv Exp Med Biol 352:173–186PubMedGoogle Scholar
  6. 6.
    Almadan MS, Awamy BH, Mulhim IA (1993) Nutritional vitamin B12 deficiency in infancy. Indian J Pediatr 60:683–685PubMedCrossRefGoogle Scholar
  7. 7.
    Garewal G, Narang A, Das KC (1988) Infantile tremor syndrome: a vitamin B12 deficiency syndrome in infants. J Trop Pediatr 34:174–178PubMedGoogle Scholar
  8. 8.
    Taneja S, Bhandari N, Strand TA, Sommerfelt H, Refsum H, Ueland PM, Schneede J, Bahl R, Bhan MK (2007) Cobalamin and folate status in infants and young children in a low–to–middle income community in India. Am J Clin Nutr 86:1302–1309PubMedGoogle Scholar
  9. 9.
    Koguchi K, Nakatsuji Y, Abe K, Sakoda S (2004) Wernicke's encephalopathy after glucose infusion. Neurology 62:512Google Scholar
  10. 10.
    Navarro D, Zwingmann C, Chatauret N, Butterworth RF (2008) Glucose loading precipitates focal lactic acidosis in the vulnerable medial thalamus of thiamine-deficient rats. Metab Brain Dis 23:115–122PubMedCrossRefGoogle Scholar
  11. 11.
    Kornreich L, Bron-Harlev E, Hoffmann C, Schwarz M, Konen O, Schoenfeld T, Straussberg R, Nahum E, Ibrahim AK, Eshel G, Horev G (2005) Thiamine deficiency in infants: MR findings in the brain. AJNR Am J Neuroradiol 26:1668–1674PubMedGoogle Scholar
  12. 12.
    Sparacia G, Banco A, Lagalla R (1999) Reversible MRI abnormalities in an unusual paediatric presentation of Wernicke’s encephalopathy. Ped Radiol 29:581–584CrossRefGoogle Scholar
  13. 13.
    Wighton MC, Manson JI, Speed I, Robertson E, Chapman E (1979) Brain damage in infancy and dietary vitamin B12 deficiency. Med J Aust 2:1–3PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Ibrahim Abu-Kishk
    • 1
    • 2
  • Marianna Rachmiel
    • 1
    • 2
  • Chen Hoffmann
    • 2
    • 4
  • Eli Lahat
    • 2
    • 3
  • Gideon Eshel
    • 1
    • 2
  1. 1.Pediatric Intensive Care UnitAssaf Harofeh Medical CenterTel-AvivIsrael
  2. 2.The Sackler Faculty of MedicineTel-Aviv UniversityTel-AvivIsrael
  3. 3.Pediatric Neurology UnitAssaf Harofeh Medical CenterTel-AvivIsrael
  4. 4.Department of ImagingChaim Sheba Medical CenterTel-AvivIsrael

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