EVOKED-POTENTIAL STUDY IN FACIO-SCAPULO-HUMERAL MUSCULAR-DYSTROPHY

Nerve conduction velocities (NCVs), somatosensory (SEPs) and auditory evoked potentials (BAEPs) were recorded in 9 patients with facio-scapu lo-humeral dystrophy (FSHD) and in 20 age-matched controls. In FSHD pa tients a significant increase of the nerve distal sensory latencies an d of the absolute SEP latencies revealed a subclinical involvement of the afferent sensory pathways, as well as the abnormal slowing of the later components of the BAEPs, pointed to a central auditory dysfuncti on. Moreover all patients underwent brain MRI that showed the presence of white matter hyperintense lesions in 4 of them (44%). No correlati ons were found between individual or total number of SEP and BAEP abno rmal electrophysiological parameters and severity of WMHL, age, age at onset, duration of the disease or muscular impairment. These findings make the interpretation and pathophysiology of the nervous damage in FSHD rather uncertain. More studies are required to better define the aspects of neurogenic involvement in this type of muscular dystrophy.