Motor nerve conduction studies on children with spinal muscular atrophy.
Median and posterior tibial motor nerve conduction studies were performed on 10 children with spinal muscular atrophy (SMA). Three patients with SMA type I, in whom rapid deterioration occurred, showed reduced motor nerve conduction velocity and a remarkably low M-wave amplitude in both nerves. In type II and III patients, the motor nerve conduction velocity was normal in the median nerve, although the M-wave amplitude was small in the tibial nerve. In four patients, a reduction of the M-wave amplitude was observed as clinical symptoms advanced. These findings may suggest that motor conduction studies in spinal muscular atrophy provide complementary information for understanding the pathogenesis and are also useful to clarify the heterogeneity of this disease.
Miyanomae
Y
Y
Department of Pediatrics, Kyoto City Child Welfare Center, Japan.
Takeuchi
Y
Y
Nishimura
A
A
Kawase
S
S
Hirai
K
K
Ochi
M
M
Sawada
T
T
D002648:Child; D004594:Electrophysiology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008475:Median Nerve / Q000502:physiology; D009046:Motor Neurons / Q000502:physiology*; D009134:Muscular Atrophy, Spinal / Q000503:physiopathology*; D009431:Neural Conduction / Q000502:physiology*; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology; D013979:Tibial Nerve / Q000502:physiology