The GFAP variant, when expressed in older ages, causes neuronal loss in the brain especially in the brain stem [5]. In some reports, neuronal loss in basal ganglia are found in adult-onset GFAP variant [6–8], that presence of striatonigral neuronal loss on FP-CIT PET with GFAP variant was first described in this case. An unusual pattern of dopamine depletion with a rostrocaudal gradient in the putamen and caudate nucleus were seen, unlike in patients with PD which shows preferential depletion in posterior putamen with relative sparing of caudate nucleus [9]. The atypical dopamine depletion pattern has not been described or investigated in other Alexander disease patients in the literature and that it could also relate to the interaction of co-pathology.