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Persistent and transient "conduction block" in motor neuron diseases

D J Lange1, W Trojaborg, T D McDonald

  • 1Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY 10032-2603.

Muscle & Nerve
|September 1, 1993
PubMed
Summary

Persistent conduction block in motor neuron disease may be identifiable and indicates prolonged survival. This finding aids in diagnosing motor neuron disease variants, distinguishing them from amyotrophic lateral sclerosis (ALS).

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Area of Science:

  • Neurology
  • Neurophysiology

Background:

  • Conduction block, a peripheral nerve dysfunction, can occur in motor neuron disease (MND).
  • Diagnosing conduction block in MND is challenging due to a lack of universally accepted criteria.
  • Persistent conduction block in peripheral neuropathies suggests a potential diagnostic marker.

Purpose of the Study:

  • To investigate the persistence and diagnostic utility of conduction block in patients with motor neuron disease.
  • To determine if persistent conduction block is associated with prolonged survival and distinct clinical features.

Main Methods:

  • Repeated electrophysiological studies (nerve conduction studies) in 9 patients with various forms of MND.
  • Assessment of motor evoked response amplitude loss between distal and proximal stimulation sites.

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  • Evaluation of clinical features, upper motor neuron signs, and immunological markers.

    • Main Results:

    • Five patients exhibited persistent focal amplitude and area loss, indicative of conduction block, over 12-36 months.
    • These patients had prolonged symptom duration (4-13 years), were male, and rarely presented with definite upper motor neuron signs.
    • Four patients showed transient amplitude loss, had shorter symptom duration, and typically presented with definite upper motor neuron signs.

    Conclusions:

    • Persistent conduction block in MND may represent a distinct subtype with prolonged survival.
    • This finding offers a reproducible method for identifying conduction block in MND.
    • Patients with persistent conduction block may present with clinical syndromes indistinguishable from ALS, except for the rarity of definite upper motor neuron signs.