Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniques.

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  • Author(s): Theys PA;Theys PA; Peeters E; Robberecht W
  • Source:
    Journal of neurology [J Neurol] 1999 Jun; Vol. 246 (6), pp. 438-42.
  • Publication Type:
    Comparative Study; Journal Article
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 0423161 Publication Model: Print Cited Medium: Print ISSN: 0340-5354 (Print) Linking ISSN: 03405354 NLM ISO Abbreviation: J Neurol Subsets: MEDLINE
    • Publication Information:
      Original Publication: Berlin ; New York, Springer-Verlag
    • Subject Terms:
      Evoked Potentials, Somatosensory* ; Motor Neurons* ; Neural Conduction*; Amyotrophic Lateral Sclerosis/*physiopathology; Adult ; Aged ; Amyotrophic Lateral Sclerosis/diagnosis ; Disease Progression ; Electrophysiology ; Female ; Humans ; Male ; Middle Aged ; Peroneal Nerve/physiopathology ; Prospective Studies ; Sensory Thresholds ; Sural Nerve/physiopathology ; Temperature
    • Abstract:
      Although amyotrophic lateral sclerosis is a degenerative disease of the upper and lower motor neurons, there is evidence that the disease can affect other systems, including the sensory system. On the other hand, within the motor neuron pool there is possibly a predilection of the degenerative process for the motor neurons fibers with the fastest conduction velocity (MNFCV). We studied these two aspects of the disease in a group of 50 patients by prospectively assessing several sensory indices and by studying the selectivity of the spinal motor neuron loss. At baseline, nerve conduction studies and somatosensory evoked potentials showed abnormal slowing in the peripheral and central sensory pathways. Thermal thresholds for heating were elevated but were normal for cooling. In more than 60% of the patients at least one of the sensory tests studied was abnormal. However, except for a significant decrease in the amplitude of the sensory nerve action potentials of the sural nerves, these afferent dysfunctions were not progressive over the follow-up period of 6 months, in contrast to the marked deterioration in motor functions. Three different statistical models were applied to evaluate the presence of demyelination, selective loss of MNFCV, or the purely random degeneration of fast- and slow-conducting motor neurons. These data indicate a selective loss of the MNFCV and suggest that subclinical abnormalities of the sensory system in ALS are often present but almost nonprogressive. Furthermore, the amyotrophic lateral sclerosis disease process seems preferentially to affect MNFCV.
    • Publication Date:
      Date Created: 19990804 Date Completed: 19990914 Latest Revision: 20190831
    • Publication Date:
      20231215
    • Accession Number:
      10.1007/s004150050379
    • Accession Number:
      10431767