Brain atrophy in LGI1-ADAM22 complex deficiency may result from different mechanisms, including impaired functional maturation of postnatal synapses and seizure-related brain damage, as frequently described in other early-onset epileptic encephalopathies.11,34 This study widens the imaging spectrum associated with ADAM22 variants, including cerebellar atrophy with prevalent vermian involvement, corpus callosum and/or anterior commissure hypoplasia and cerebral white matter signal changes. This evidence concerns the gene LGI1 and Epileptic encephalopathy.