Maroofian et al. delineate a new form of autosomal recessive distal hereditary motor neuropathy (dHMN) with pyramidal features associated with RTN2 deficiency, and conclude that the disorder shares similarities with SIGMAR1-related dHMN and Silver-like syndromes. The gene discussed is SIGMAR1; the disease is neuronopathy, distal hereditary motor, autosomal recessive.