Cutaneous silent period in patients with spinal muscular atrophy type 2 and type 3

The aim of this study was to examine cutaneous silent periods (CSPs) in spinal muscular atrophy (SMA) type 2 and type 3 patients. Fourteen patients with SMA and 14 healthy subjects were included. CSPs were recorded from thenar muscles after painful stimulation of the index finger during slight thumb abduction. CSP parameters (including onset latency, duration, index of suppression, long-loop reflex, and post-inhibitory rebound activity) were measured. All parameters were compared between SMA patients and healthy subjects using the Mann-Whitney U test. We then correlated CSP parameters to Hammersmith Functional Motor Scale Expanded (HFMSE) scores in SMA patients by Spearman-rho testing. No CSP parameter except E3% (i.e., the post-inhibitory rebound activity relative to pre-stimulus baseline) differed significantly between SMA patients and healthy subjects. E3% was significantly smaller in patients than healthy individuals. HFMSE scores of SMA patients correlated negatively with CSP duration and positively with E3%. Smaller E3%, correlating with longer CSP duration, in SMA patients with more severe clinical findings and therefore more marked motoneuron loss suggests that the E3 component following the CSP is directly affected by motoneuron loss.