Monomelic neurogenic syndromes: a prospective study

Monomelic neurogenic syndromes are rare. Their classification and prognostic features have not been addressed in the European population. A prospective study of 17 patients with monomelic neurogenic amyotrophy, of upper or lower limb onset, with progression limited to one limb for three or more years. Clinical and neurophysiological studies were performed in the subsequent 3 or more years. Fifteen patients were of European origin and two were Asian. Those presenting with proximal monomelic weakness or with involvement of the posterior compartment of the lower leg showed no further progression after the initial period of development of the syndrome. Brisk reflexes in wasted muscles did not predict progression. Electromyographic signs of denervation in the opposite limb at presentation did not predict later progression. Transcranial magnetic stimulation (TMS) features of corticospinal dysfunction were a useful predictor of subsequent progression (p=0.01). One patient with lower limb onset developed conduction block with weakness in an upper limb nine years after presentation, and this upper limb weakness responded to IVIg therapy. This adult-onset European group of patients is different as compared with juvenile-onset Asian cases. The clinical syndromes appear heterogeneous, but neurophysiological investigations, in particular TMS, can be helpful in determining prognosis. Multifocal motor neuropathy should be considered when there is progression, even years after onset.