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Pure lower motoneurone syndrome in a series of six patients

P. Larrodé-Pellicer, C. Iñíguez-Martínez, S. Santos-Lasaosa, S. Adelantado, P. González, F. Morales-Asín [REV NEUROL 2002;34:105-108] PMID: 11988902 DOI: https://doi.org/10.33588/rn.3402.2001324

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Volumen 34 | Number 02 | Nº of views of the article 8.842 | Nº of PDF downloads 694 | Article publication date 16/01/2002

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ABSTRACT

INTRODUCTION Lower motoneurone syndromes (SMNI) have been defined by their clinical pattern, anomalies in nerve conduction and the presence of antiglycolipid antibodies. The possible response to immunotherapy is another characteristic which makes these syndromes interesting.

PATIENTS AND METHODS We studied the clinical and electrophysiological data, clinical progress and response to treatment of a series of six patients, with a pure lower motoneurone syndrome, with a follow-up period of between 5 and 13 years.

CONCLUSIONS The clinical phenotype of our patients was: male, middle-aged, the clinical topography showed only lower motoneurone involvement, predominantly or exclusively limited to the upper limbs, asymmetrical, distal rather than proximal, with a chronic course progressing initially but later becoming stable. From the electrophysiological point of view some of our cases were of SMNI. In these the conduction block was not permanent although there were signs of axon loss and multifocal demyelination. Response to treatment with intravenous immunoglobulins was seen in patients with anti-GMI antibodies of IgM type and recent signs of clinical deterioration, whether there were conduction blocks present or not. In the cases which responded to this treatment there was tolerance and dependence following repeated transfusions of IV IgG. KeywordsAntiganglioside antibodies Conduction blocks Immunotherapy Intravenous immunoglobulin Lower motoneurone syndrome Multi-focal motor neuropathy CategoriesNervios periféricos, unión neuromuscular y músculo

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Original

Pure lower motoneurone syndrome in a series of six patients