Background Identifying genetic syndromes that lead to significant atopic disease can open
new pathways for investigation and intervention in allergy. Objective We sought to define a
genetic syndrome of severe atopy, increased serum IgE levels, immune deficiency,
autoimmunity, and motor and neurocognitive impairment. Methods Eight patients from 2
families with similar syndromic features were studied. Thorough clinical evaluations,
including brain magnetic resonance imaging and sensory evoked potentials, were …