To investigate whether BBB impairment was associated with neuroinflammation and, specifically to astrogliosis and microgliosis, we carried out immunohistochemistry against CoIV (blood vessels marker), GFAP (astrocyte marker) and Iba1 (microglia marker) in striata tissue from MJD patients and control individuals. This evidence concerns the gene AIF1 and Spinocerebellar ataxia type 3.