A rare axonal hereditary motor and sensory neuropathy characterized by early onset (<10 years) progressive distal muscle weakness and wasting of the lower limbs and later, to a lesser extent the upper limbs resulting in foot and wrist drop, areflexia, skeletal deformities (kyphoscoliosis, pes cavus with flattening, joint contractures), mild sensory impairment with vibration sense reduced to a greater extent than pain, optic atrophy and hearing loss. Wheelchair dependence by adolescence is usual and respiratory impairment with diaphragmatic paralysis may develop. The disease is Monarch Disease Ontology term MONDO_0019549 (severe early-onset axonal neuropathy due to MFN2 deficiency). Also known as: AR-CMT2, Ouvrier type, SEOAN due to MFN2 deficiency, autosomal recessive Charcot-Marie-Tooth disease, Ouvrier type.