Are electrophysiological features related to disability in patients with anti-MAG neuropathy?

Neurophysiol Clin

Referral centre for ALS and Neuromuscular diseases, La Timone Hospital, Aix-Marseille University, 264, rue Saint-Pierre, 13005 Marseille, France; Inserm UMR S 910, Medical Genetics and Functional Genomics, Aix-Marseille université, 13005 Marseille, France.

Published: February 2017

Objectives: To explore clinical-neurophysiological correlations in anti-myelin-associated glycoprotein (anti-MAG) neuropathy.

Methods: Clinical and electrophysiological data of 42 patients with anti-MAG neuropathy were retrospectively analysed. Disability was evaluated using the Overall Neuropathy Limitation Scale (ONLS), motor impairment through MRC sum score and sensory deficiency through INCAT sensory score. Compound motor action potential (CMAP) sum score was calculated adding the distal CMAP amplitude of the median, ulnar, tibial and fibular nerves of both sides.

Results: In multivariate analysis, motor impairment was associated with CMAP sum score (r=0.35, P=0.047) and distal motor latency in the median nerves (r=-0.45 P=0.012), sensory deficiency was related to motor conduction velocity in the median nerve (r=-0.65. P=0.02). Disability was correlated with CMAP sum score (r=-0.37, P=0.022).

Conclusion: Electrophysiological features are associated with clinical involvement in anti-MAG neuropathy. Reduction of CMAP amplitudes reflects distal motor latency delay and is mainly due to axonal loss. Since it is related to muscle weakness and disability, CMAP sum score may be a good marker in the evaluation of patients with anti-MAG neuropathy.

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http://dx.doi.org/10.1016/j.neucli.2017.01.002DOI Listing

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