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Research paper
Diagnostic accuracy of electrically elicited multiplet discharges in patients with motor neuron disease
  1. Boudewijn T H M Sleutjes1,
  2. Inger Montfoort1,
  3. Pieter A van Doorn2,
  4. Gerhard H Visser1,3,
  5. Joleen H Blok1,4
  1. 1Department of Clinical Neurophysiology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands
  2. 2Department of Neurology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands
  3. 3Department of Clinical Neurophysiology, SEIN Heemstede, Heemstede, The Netherlands
  4. 4Department of Clinical Physics, Máxima Medical Centre, Veldhoven, The Netherlands
  1. Correspondence to Boudewijn T H M Sleutjes, Department of Clinical Neurophysiology, Erasmus MC, University Medical Centre Rotterdam, P.O. Box 2040, Rotterdam 3000 CA, The Netherlands; b.sleutjes{at}erasmusmc.nl

Abstract

Objective To determine and compare the diagnostic accuracy of electrically elicited multiplet discharges (MDs) and fasciculation potentials (FPs) in motor neuron disease (MND).

Methods Patients were eligible when they had MND in their differential diagnosis and were referred for electromyogram (EMG). Stimulated high-density surface EMG of the thenar muscles was performed on the same day as standard EMG examination. High-density recordings were analysed for presence of MDs and needle EMG of any muscle investigated in the cervical region for presence of FPs.

Results Of the 61 patients enrolled in this diagnostic study, 24 patients were clinically diagnosed with amyotrophic lateral sclerosis (ALS) and 11 patients with progressive muscular atrophy (PMA). Another diagnosis was made in 26 patients. Sixteen patients in whom MDs were detected were diagnosed with either ALS (n=11) or PMA (n=5; sensitivity=47.1%, PPV=94.1%). MDs were detected in only one patient initially diagnosed with PMA, but in whom later on, multifocal motor neuropathy could not be excluded (specificity=96.2%). Electrically elicited MDs had a higher specificity than FPs (96.2% vs 53.9%, p<0.001, n=26) and lower sensitivity (47.1% vs 85.3%, p=0.002, n=34). When considering presence of MDs in MND as neurogenic EMG abnormality, lower motor neuron involvement of ≥1 EMG region increased from 50% to 73.5% (p=0.008, n=34).

Conclusions Electrically evoked MDs are highly specific for ALS and PMA and are an early sign of lower motor neuron dysfunction.

  • EMG
  • ALS
  • NEUROPHYSIOL, CLINICAL
  • MOTOR NEURON DISEASE

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