Journal of Neurology

, Volume 250, Issue 11, pp 1279–1292

The spectrum of lower motor neuron syndromes

Authors

    • Dept. of Neurology, Rudolf Magnus Institute for NeurosciencesUniversity Medical Centre Utrecht
  • L. H. Van den Berg
    • Dept. of Neurology, Rudolf Magnus Institute for NeurosciencesUniversity Medical Centre Utrecht
  • J. Visser
    • Dept. of Neurology, Academic Medical CenterUniversity of Amsterdam
  • M. de Visser
    • Dept. of Neurology, Academic Medical CenterUniversity of Amsterdam
  • H. Franssen
    • Dept. of Clinical Neurophysiology, Rudolf Magnus Institute for NeurosciencesUniversity Medical Centre Utrecht
  • J. H. J. Wokke
    • Dept. of Neurology, Rudolf Magnus Institute for NeurosciencesUniversity Medical Centre Utrecht
REVIEW

DOI: 10.1007/s00415-003-0235-9

Cite this article as:
Van den Berg-Vos, R.M., Van den Berg, L.H., Visser, J. et al. J Neurol (2003) 250: 1279. doi:10.1007/s00415-003-0235-9

Abstract.

This review discusses the most important lower motor neuron syndromes. This relatively rare group of syndromes has not been well described clinically. Two subgroups can be distinguished: patients in whom motor neurons (lower motor neuron disease (LMND)) are primarily affected or motor axons and their surrounding myelin (multifocal motor neuropathy (MMN)), both leading to muscle atrophy and weakness.

Both hereditary and sporadic forms of LMND have been described. The discussion of recent advances in the genetic knowledge of several hereditary forms of LMND may lead to a better understanding of the pathophysiology and the development of therapeutic strategies. By contrast, the pathogenesis of sporadic LMND is largely unknown. It is, therefore, difficult to consider the various sporadic forms of LMND, discussed in this review, as separate diseases. Because the diagnostic and therapeutic options may differ, it would seem rational to consider sporadic LMND as a spectrum of syndromes which can be distinguished from each other on the basis of clinical presentation.

MMN is a lower motor neuron syndrome with presumed immunemediated pathogenesis. Evidence of motor conduction block on nerve conduction studies and a positive response to treatment with intravenous immunoglobulins (IVIg) are considered the most relevant criteria for the diagnosis of MMN. As it is treatable, it is important to distinguish MMN from LMND. Careful electrophysiological analysis in the search for conduction block is, therefore, required in all adult patients with pure lower motor neuron syndromes. For the individual patient, dist inction between the various lower motor neuron syndromes is important as it enables the physician to provide adequate information over the disease course in LMND and to facilitate early treatment in MMN.

Key words

motor neuron diseaselower motor neuron syndromeslower motor neuron diseaseamyotrophic lateral sclerosismultifocal motor neuropathy
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Copyright information

© Steinkopff Verlag 2003