Variable pyramidal tract signs of hyperreflexia and spasticity with distal motor neuropathy can be found in association with pathogenic variants in several genes.10-12 Monoallelic pathogenic variants in BSCL2 (HGNC:15832) encoding an ER membrane protein lead to a wide spectrum of highly variable neurological disorders ranging from pure dHMN type V without spasticity and CMT2 to Silver syndrome (SPG17-MIM#270685) and ‘juvenile amyotrophic lateral sclerosis-mimics’.14 Monoallelic pathogenic SETX (HGNC:445; MIM#608465) variants can also manifest as dHMN accompanied with pyramidal signs.15 The gene discussed is SETX; the disease is juvenile amyotrophic lateral sclerosis.