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We assessed the cutaneous silent period (CSP) in 24 patients with Fabry disease with small-fiber sensory neuropathy and 12 normal subjects to test the hypothesis that small-diameter afferents are responsible for producing the CSP. Sensory nerve conduction studies and quantitative sensory testing for cold and vibration detection thresholds were also measured. Overall, Fabry patients had impaired thermal, but not vibration, detection thresholds, with greatest impairment in the feet. In the upper extremity, CSP latencies, duration, and suppression of electromyographic activity (EMG) did not differ. In the lower extremity, patients had reduced suppression of EMG during the CSP compared to normal controls. CSP durations exhibited a bimodal distribution in patients, including a subset of seven patients with durations shorter than all controls. This subset had profound loss of thermal sensation in the feet, but this was also true of some patients who had normal CSPs. Patients with shortened CSPs had modestly elevated vibration thresholds and reduced sensory potentials in comparison to patients with normal CSPs. Reduced CSPs in Fabry patients are associated with, but not entirely explained by, the severity of small-fiber neuropathy as measured by quantitative sensory testing. The possibility that large-diameter fibers provide a minor contribution to producing the CSP should be considered.

The transient suppression of muscle contraction during the cutaneous silent period (CSP) could be produced either through postsynaptic inhibition of motoneurons or through presynaptic inhibition of the excitatory inputs to motoneurons that sustain voluntary contraction. We sought to delineate the mechanisms underlying the CSP in hand muscles by measuring changes in H-reflexes and motor-evoked potentials (MEPs) produced by transcranial magnetic stimulation (TMS) during the CSP in 10 healthy volunteers. H-reflexes and MEPs both measure the excitability of the motoneuron pool and activate similar subpopulations of motoneurons through different pathways. Inhibition of H-reflexes and MEPs of similar size was maximal at the midpoint of the CSP and gradually returned to baseline. The similar time course of recovery suggests that the H-reflex and MEP are affected by inhibition at a common site, most likely postsynaptic inhibition of the motoneurons.