Objective: To determine whether there is a relation between electrically evoked multiplet discharges (MDs) and motor axonal excitability properties. We hypothesized that electrically evoked MDs share their underlying pathophysiological mechanism with fasciculations. Methods: High-density surface EMG and motor nerve excitability recordings of the thenar muscles were performed in 22 patients with motor neuron disease (MND) in their differential diagnosis and who were referred for EMG examination. Results: Supernormality (hyperexcitable phase following the refractory period) was significantly increased in patients with MDs (n= 10) compared to patients without MDs (n= 12) (25.5% vs 17.0%; p= 0.02). Depolarizing threshold electrotonus differed significantly between both groups as well (TEdpeak, 76.6% vs 66.6%, p<. 0.01; TEd90-100. ms, 51.7% vs 44.3%, p<. 0.01). Conclusions: Our findings imply that the same pathophysiological excitability changes are involved in generating MDs and fasciculations. Yet, MDs may be quantified more easily, and may be more specific for abnormal distal excitability than fasciculations, because fasciculations may originate along the motor axon as well as in the neuron cell body. Significance: MDs are potentially useful as objective measure of increased distal axonal excitability at individual motor unit level and might complement clinical studies in MND.

Axonal excitability, Motor neuron disease, Multiplet discharges, Supernormality
dx.doi.org/10.1016/j.clinph.2014.10.145, hdl.handle.net/1765/85415
Clinical Neurophysiology
Department of Neurology

Sleutjes, B.T.H.M, Montfoort, I, van Doorn, P.A, Visser, G.H, & Blok, J.H. (2015). Increased supernormality in patients with multiplet discharges: Evidence for a common pathophysiological mechanism behind multiplets and fasciculations. Clinical Neurophysiology, 126(7), 1440–1445. doi:10.1016/j.clinph.2014.10.145