The second patient, already described in Olivieri et al., had a notable immunological involvement which included thyroiditis, cANCA positivity, antiphospholipid antibodies and cerebral ischemia [25]: In addition to the “classic” leukoencephalopathy picture, brain MRI showed at 5 years of age a first ischemic event at the level of the left caudate nucleus and the follow up showed additional lacunar infarct outcomes and slight enlargement of the other homolateral malacic area. This evidence concerns the gene PRTN3 and brain ischemia.