Fig. 12.1
Representative examples of single motor unit potentials (SMUPs) stimulating the right ulnar nerve and recording abductor digiti minimi using the multipoint approach. One set of SMUPs had amplitudes ranging from 23–30 μV (a), while the other had amplitudes ranging from 112–128 μV (b). Note the similar morphology of the waveforms in each set of tracings. The full examination identified 8 distinct sets of SMUPs that averaged 116.68 μV. With a CMAP amplitude recorded to be 1.9 mV at the same site, the MUNE was calculated to be 16.285
Combined Multipoint Incremental Method
The combined multipoint incremental method involves the use of a single recording site for a nerve/muscle pair, and a defined set of stimulation points, typically three [13]. At each stimulation site, the nerve is stimulated at low currents that are increased slowly, as in the multipoint approach. However, once the first motor unit action potential is obtained and recorded, the current should continue to be increased slowly until a second incremental (“all or none”) jump in the action potential amplitude occurs. Once the second is recorded, then the stimulation is again increased slowly until a third incremental jump occurs. This protocol is repeated at each of the three stimulation sites, yielding a total of 9 measurements. At each site, the first motor unit amplitude is equivalent to the first action potential obtained, while the second is calculated by subtracting the first from the second, while the third is calculated by subtracting the second from the third. These nine motor unit amplitudes are then averaged and used for the standard MUNE calculation (Fig. 12.2). Redundant motor unit action potentials are tolerated in this protocol, as opposed to the multipoint stimulation technique.
Fig. 12.2
Images of SMUPs stimulating the right ulnar nerve and recording abductor digiti minimi using the incremental approach. Three increments are recorded at each of three different sites: 2 cm proximal to the wrist crease (a), 6 cm proximal to the wrist crease (b), and 1 cm proximal to the ulnar groove at the elbow (c). The average increment, representing the SMUP, for the nine stimulations is 38.9 μV. With a CMAP recorded at 9.5 mV, the MUNE is then calculated to be 244.2
MUNE is increasingly used as an outcome measure for clinical trials in spinal muscular atrophy [7, 14, 15], and has yielded interesting information about the disease, including natural history data [16, 17] and evidence suggesting that new motor development may be occurring as a compensatory mechanism in spinal muscular atrophy [8]. Thus far, it does not appear to have obvious diagnostic applications in routine clinical settings, as it is not clear that it would detect signs of motor unit dysfunction that would not be seen by other means, such as CMAP amplitudes and signs of denervation/reinnervation on needle examination. However, as more data accumulate regarding the correlation of MUNE values with disease progression, such applications may be proposed in the future.
References
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McComas AJ, Sica RE, Brandstater ME. Further motor unit studies in Duchenne muscular dystrophy. J Neurol Neurosurg Psychiatry. 1977;40(12):1147–51.CrossrefPubMedPubMedCentral