Beside autosomal dominant SMA lower extremity-predominant 1 (SMALED1, OMIM #58600) caused by mutations in dynein cytoplasmic 1 heavy chain 1 protein (DYNC1H1; OMIM *600112), we and others have recently identified heterozygous variants in BICD2 (OMIM *609797) as causative for autosomal dominant SMA, lower extremity-predominant, 2, (SMALED2; OMIM #615520) and hereditary spastic paraplegia (HSP) (Neveling et al., 2013; Oates et al., 2013; Peeters et al., 2013). Here, BICD2 is linked to hereditary spastic paraplegia.