Distinctive cognitive impairments and a specific neurobehavioral syndrome are reported in 11 of 13 children who had failed to thrive on chloride-deficient formula during infancy. They were examined four to nine years later at the Division of Pediatric Neurology, UCLA School of Medicine, Los Angeles, CA and the Department of Neurology, Boston University School of Medicine, Boston, MA. All patients during infancy had developed symptoms of hypokalemic, hypochloremic metabolic alkalosis and/or growth deceleration, concurrently with their exposure to chloride-deficient Neo-Mull-Soy formula. Head circumference at follow-up was within normal limits, three children had hypotonia, two diffuse hyperreflexia, one had choreiform movements and one bilateral intention tremor. The EEG was abnormal in six, with paroxysmal discharges seen in five children. CT and MRI studies were normal. Intelligence was within the average range for 11 of the 13 children tested. All had a neurobehavioral syndrome consisting of a language disorder primarily involving articulation, word finding and naming; visual-motor and fine motor difficulties; and attention deficit disorder often featuring repetitive behaviors, withdrawal and “over-focus”. [1]

COMMENT. A syndrome characterized by loss of appetite, failure to gain weight, muscular weakness, lethargy, vomiting and severe hypochloremic, hypokalemic, metabolic alkalosis has been described in infants fed chloride-deficient soy-based infant formulas. Clinical features resemble Bartter Syndrome, but the renal biopsy is normal and infants recover when dietary chloride supplements are provided. Previous follow-up reports have drawn attention to developmental delay [2] and the present study demonstrates specific cognitive, language and attentional deficits in later childhood.