Motor Unit Integrity in Multifocal Motor Neuropathy: A Systematic Evaluation with CMAP Scans

Abstract

Introduction/Aims: Progressive axonal loss in multifocal motor neuropathy (MMN) is often assessed with nerve conduction studies (NCS), by recording maximum compound muscle action potentials (CMAPs). However, reinnervation maintains the CMAP amplitude until a significant portion of the motor unit (MU) pool is lost. Therefore, we performed more informative CMAP scans to study MU characteristics in a large cohort of patients with MMN. Methods: We derived the maximum CMAP amplitude (CMAP max), an MU number estimate (MUNE), and the largest MU amplitude stimulus current required to elicit 5%, 50%, and 95% of CMAP max (S5, S50, S95) and relative ranges ([S95 − S5] × 100 / S50) from the scans. These metrics were compared with clinical, laboratory, and NCS results. Results: Forty MMN patients and 24 healthy controls were included in the study. CMAP max and MUNE were reduced in MMN patients (both P <.001). Largest MU amplitude as a percentage of CMAP max was increased in MMN patients (P <.001). Disease duration and treatment duration were not associated with MUNE. Relative range was larger in patients with anti-GM1 antibodies than in those without anti-GM1 antibodies (P =.016) and controls (P <.001). The largest MU amplitudes were larger in patients without anti-GM1 antibodies than in patients with anti-GM1 antibodies (P =.037) and controls (P =.044). Discussion: We found that MU loss is common in MMN and accompanied by enlarged MUs. Presence of anti-GM1 antibodies was associated with increased relative range of MU thresholds and reduction in largest MU amplitude. Our findings indicate that CMAP scans complement routine NCS, and may have potential for practical monitoring of treatment efficacy and disease progression.