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Herein, we report the first three Austrian and two German patients with , presenting a detailed description of their clinical phenotype and disease course. Our report adds to the knowledge of this extremely rare disorder, and highlights that must also be considered in the differential diagnosis of complicated HSP outside the Amish community. Herein, we report the first three Austrian and two German patients with SPG21, presenting a detailed description of their clinical phenotype and disease course. Our report adds to the knowledge of