Garg, N;
Howells, J;
Yiannikas, C;
Vucic, S;
Krishnan, AV;
Spies, J;
Bostock, H;
... Kiernan, MC; + view all
(2017)
Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss.
Clinical Neurophysiology
, 128
(10)
pp. 2022-2028.
10.1016/j.clinph.2017.07.414.
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Bostock_2017 Garg Motor unit remodelling in MMN accepted Clin Neurophysiol.pdf - Accepted Version Download (605kB) | Preview |
Abstract
OBJECTIVE: To estimate the degree of axonal loss in patients diagnosed with multifocal motor neuropathy (MMN) using a novel assessment of motor unit numbers and size. METHODS: Automated motor unit number estimation using a compound muscle action potential (CMAP) scan was undertaken in median nerves with conduction block. Results were compared with 30 age-matched healthy controls. RESULTS: Compared with healthy controls, MMN patients had fewer motor units (MMN: 33±11vs HC: 93±36 [mean±SD]; p<0.0001) and larger 'size of the largest unit' (MMN: 1.2±0.5mVvs HC: 0.4±0.1mV; p<0.0001), despite having normal distal CMAP amplitudes (MMN: 7.6±1.8mVvs HC: 8.7±2.5mV; p=0.24). CONCLUSIONS: MMN is associated with marked axonal loss which may be masked by striking re-innervation resulting in preservation of distal CMAP amplitudes. SIGNIFICANCE: Assessment of motor unit properties should be incorporated into assessment of disease progression in MMN, given that nerve conduction studies are insensitive to motor unit remodelling.
| Type: | Article |
|---|---|
| Title: | Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss |
| Location: | Netherlands |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.clinph.2017.07.414 |
| Publisher version: | http://dx.doi.org/10.1016/j.clinph.2017.07.414 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Autoimmune diseases, Axonal degeneration, Multifocal motor neuropathy, Neuromuscular disease, Neurophysiology, Action Potentials, Adult, Aged, Axons, Cohort Studies, Female, Humans, Male, Middle Aged, Motor Neuron Disease, Nerve Degeneration, Neural Conduction, Polyneuropathies, Prospective Studies, Recruitment, Neurophysiological |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1571586 |
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