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Brain, Vol. 126, No. 5, 1036-1047, May 2003
© 2003 Guarantors of Brain
doi: 10.1093/brain/awg117

Sporadic lower motor neuron disease with adult onset: classification of subtypes

R. M. Van den Berg-Vos1, J. Visser4, H. Franssen2, M. de Visser4, J. M. B. V. de Jong4, S. Kalmijn3, J. H. J. Wokke1 and L. H. Van den Berg1

Departments of 1 Neurology and 2 Clinical Neurophysiology of the Rudolf Magnus Institute for Neurosciences, 3 Julius Centre for General Practice and Patient Oriented Research, University Medical Centre Utrecht and the 4 Department of Neurology, Academic Medical Centre, Amsterdam, The Netherlands

Correspondence to: L. H. Van den Berg, MD, PhD, Department of Neurology, University Medical Centre Utrecht, PO Box 85500, 3508 GA Utrecht, The Netherlands E-mail: l.h.vandenberg{at}neuro.azu.nl

The discovery of the genetic basis of hereditary lower motor neuron disease (LMND) and the recognition of multifocal motor neuropathy as a distinct clinical entity necessitate a new classification of LMND. To this end, we studied the clinical and electrophysiological features of 49 patients with sporadic adult-onset LMND in a cross-sectional study. Disease duration was more than 4 years to exclude the majority of patients with amyotrophic lateral sclerosis. Based on the pattern of weakness, we identified three groups: 13 patients with generalized weakness (group 1); eight patients with symmetrical, distal muscle weakness (group 2); and 28 patients with non-generalized asymmetrical weakness of the arms in most patients (group 3). Group 3 could be subdivided into patients with weakness in predominantly the distal (group 3a) or the proximal (group 3b) muscle groups, both with disease progression to adjacent spinal cord segments. Distinctive features of group 1 were an older age at onset, more severe weakness and muscle atrophy, lower reflexes, greater functional impairment, more widespread abnormalities on concentric needle EMG, respiratory insufficiency and serum M-protein. In groups 2 and 3, concentric needle EMG findings also suggested a more widespread disease process. Retrospectively, the prognosis of sporadic adult-onset LMND appears to be favourable, because clinical abnormalities were still confined to one limb in most patients after a median disease duration of 12 years. We propose to classify the patients in the different subgroups as slowly progressive spinal muscular atrophy (group 1), distal spinal muscular atrophy (group 2), segmental distal spinal muscular atrophy (group 3a) and segmental proximal spinal muscular atrophy (group 3b). The described clinical phenotypes may help to distinguish between different LMND forms.


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