title: Thiamine deficiency in infancy: long-term follow-up.
abstract: BACKGROUND
  In 2003, several hundred Israeli infants risked thiamine deficiency after being fed a soy-based formula deficient in thiamine. Approximately 20 patients were seriously affected, and three of them died. We report the clinical presentation of acute encephalopathy in 11 children and the long-term sequelae of eight children who initially survived.
  
  
  METHODS
  In the acute phase, six had bulbar signs, five had ophthalmologic signs and two had phrenic neuropathy. Three of the five patients with cardiac involvement had cardiomyopathy and died in the acute phase. One patient presented with a complete atrioventricular block.
  
  
  RESULTS
  In the long-term, one patient, who was in a chronic vegetative state, died after 6 years. Seven children exhibited mental retardation and motor abnormalities, six developed severe epilepsy, two early kyphoscoliosis, and one patient remained with a complete atrioventricular block.
  
  
  CONCLUSIONS
  Infants who survive severe infantile thiamine deficiency have serious residual motor and cognitive sequelae as well as epilepsy.
authors.affiliation: The Pediatric Neurology and Developmental Unit, Loewenstein Rehabilitation Hospital, Raanana, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: aviva100@bezeqint.net.
authors.forename: Aviva
authors.initials: A
authors.lastname: Mimouni-Bloch
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Epilepsy Service, Schneider Children's Medical Center, Petach Tikva, Israel.
authors.forename: Hadassa
authors.initials: H
authors.lastname: Goldberg-Stern
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Neurology Unit, Schneider Children's Medical Center, Petach Tikva, Israel.
authors.forename: Rachel
authors.initials: R
authors.lastname: Strausberg
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Rehabilitation, Sheba Medical Center, Ramat Gan, Israel.
authors.forename: Amichai
authors.initials: A
authors.lastname: Brezner
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Child Neurology and Rehabilitation, Assaf Harofeh Medical Center, Zerifin, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Heyman
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Child Development Center, Schneider Children's Medical Center, Petach Tikva, Israel.
authors.forename: Dov
authors.initials: D
authors.lastname: Inbar
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Epilepsy Service, Schneider Children's Medical Center, Petach Tikva, Israel.
authors.forename: Sara
authors.initials: S
authors.lastname: Kivity
authors.affiliation: Soroka Medical Center, Beer-Sheva, Israel; Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
authors.forename: Alex
authors.initials: A
authors.lastname: Zvulunov
authors.affiliation: Soroka Medical Center, Beer-Sheva, Israel; Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Sztarkier
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Cardiology Unit, Schneider Children's Medical Center, Petach Tikva, Israel.
authors.forename: Rami
authors.initials: R
authors.lastname: Fogelman
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Neurology Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Aviva
authors.initials: A
authors.lastname: Fattal-Valevski
mesh_terms: D002648:Child; D004827:Epilepsy / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D041943:Infant Formula; D008607:Intellectual Disability / Q000209:etiology; D007557:Israel; D007738:Kyphosis / Q000209:etiology; D008297:Male; D009069:Movement Disorders / Q000209:etiology; D018458:Persistent Vegetative State / Q000209:etiology; D012600:Scoliosis / Q000209:etiology; D013832:Thiamine Deficiency / Q000150:complications*; D013997:Time Factors
keywords: Wernike encephalopathy; atrioventricular block; cardiomyopathy; thiamine; vitamin deficiency