Grave Long-term Outcomes for Children With Thiamine Deficiency

Seven Israeli infants who were fed a thiamine-deficient soy-based formula remain moderately to severely neurologically impaired at 5 years. Long-term impairment includes various degrees of mental retardation and motor, brainstem, or basal-ganglia dysfunction. The grave outcomes for these children, who displayed severe epileptic seizures during infancy, are presented in the latest print issue of Neurology.

A total of 20 Israeli infants were "seriously" affected after being fed the kosher baby formula, Remedia Super Soya 1—which was produced in 2003 by Humana, a German company, and imported by Remedia, an Israeli company. Infants quickly developed the known symptoms of cardiomyopathy or neurologic dysfunction that are associated with wet and dry beriberi, respectively. At least 3 infants died. Tests of the formula revealed negligible amounts of thiamine.

In an accompanying editorial, child neurologist Marc Patterson reviews the effects of deficient thiamine, or vitamin B1, which cannot be synthesized or stored to any significant degree by humans. In addition to the long-described forms of beriberi, thiamine deficiency is also manifest in Wernicke encephalopathy and Korsakoff syndrome—amnestic conditions associated with inveterate alcoholism and diencephalic injury. Leigh disease, a metabolic pediatric encephalopathy associated with thiamine deficiency, is neuropathologically similar to Wernicke disease, minus the mammillary-body lesions. Infantile thiamine deficiency, itself, is very rare in developed nations and is seen primarily in infants who are breastfed by thiamine-deficient mothers. It has a better prognosis, however, than Leigh disease.

According to the Jerusalem Post, parents of the affected infants received substantial financial compensation from the formula manufacturer and the Israeli importer. The Israeli State Attorney's office is prosecuting 3 Remedia officials and 5 Health Ministry workers—including pediatrician Dorit Nitzan-Kaluski, the former head of the Food Service Division and coauthor of the 2005 report of the incident in Pediatrics.

Depicted chemical structure of thiamine from Wikipedia.