Mastaglia et al. (1999) reported a 4-generation Macedonian family with autosomal dominant HMSN characterized by variable severity and motor nerve conduction velocities in the intermediate range. Affected members displayed a symmetric pattern of distal muscle atrophy, weakness, and ... Mastaglia et al. (1999) reported a 4-generation Macedonian family with autosomal dominant HMSN characterized by variable severity and motor nerve conduction velocities in the intermediate range. Affected members displayed a symmetric pattern of distal muscle atrophy, weakness, and sensory impairment in the lower limbs and to a lesser extent in the upper limbs. Motor NCVs ranged from 24-41 m/s for the median nerve and from 33-48 m/s for the ulnar nerve. Nerve biopsy of 2 patients showed primarily axonal degeneration, but also areas of segmental demyelination and remyelination without onion bulb formation. Mastaglia et al. (1999) called the disorder in this family an 'intermediate' form of HSMN between CMT types 1 (see 118200) and 2 (see 118210).