Background: An upper motor neuron (UMN) lesion in amyotrophic lateral sclerosis (ALS) is often difficult to identify because clinical signs may be discrete or masked by severe simultaneous LMN lesions. We compared the diagnostic sensitivity of transcranial magnetic stimulation (TMS) to cranial muscles and limb muscles in the detection of UMN lesions.
Design: We investigated corticobulbar and corticospinal tract function to the tongue/orofacial muscles and abductor digiti minimi/tibial anterior muscles with TMS in 51 patients with ALS to compare the diagnostic yield in the detection of UMN dysfunction. An UMN lesion was assumed when the following were found: the peripheral conduction time and amplitude of the M-wave were within the normal range, the response to cortical stimulation was absent, the TMS evoked/M-wave amplitude ratio was reduced, and the central motor conduction time or the interside difference was delayed (> mean+2.5 SD).
Results: On the basis of these criteria a UMN lesion to the orofacial muscles was identified in 24 patients (47%), to the tongue in 27 (53%), and to the upper and lower limbs in 13 (25%) and 22 patients (43%), respectively. Combined abnormalities from all sites increased the diagnostic yield to 39 patients (76%). TMS of the limb muscles confirmed a UMN lesion in only 15 (54%) of the 28 patients with clinically confirmed UMN involvement. This number increased to 23 patients (82%) if tongue and orofacial muscles were taken into acount.
Conclusion: Our results indicate the early and in most cases subclinical corticobulbar tract involvement of the central motor pathways to the orofacial muscles and tongue in ALS. TMS of the tongue and orofacial muscles had a higher sensitivity in identifying UMN lesions than that of the upper and lower limbs.