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Although only a few frontotemporal lobar degeneration (FTLD) patients develop frank amyotrophic lateral sclerosis (ALS), motor neuron dysfunctions (MNDys) occur in larger proportion of patients. The aim this study is to evaluate MNDys and ALS sample consecutively enrolled sporadic FTLD patients.Clinical neurophysiological evaluations (i.e. needle electromyography) assessed lower (LMN) upper (UMN) function at the baseline 70 probable (i.e., 26 behavioural variant-bvFTD, 20 primary progressive aphasias-PPAs 24 corticobasal syndrome-CBS). To obtain more accurate estimation, quantitative scales were also applied ALSFRS-r UMN scale). Patients screened for MAPT, GRN C9orf72 mutations. A mean clinical follow-up 27.8 ± 22.4 months progression presentation ALS.Five genetic cases identified. Within patients, relative low rate was diagnosed as (5%), while higher (17%) showed MNDys. Thirteen (20%) presented with isolated signs LMN and/or dysfunction, 8 (12%) neurogenic changes electromyography. No differences phenotype disease duration found between positive negative Clinical highly associated electromyographic findings. At follow-up, no patient developed ALS.Neurophysiological examinations revealed mild not fulfilling criteria ALS. This condition did evolve two years. These results, indicating subclinical corticospinal tracts neurons, suggest that may be risk than general population.