Motor XXXX in the @entity747 spectrum: A clinical and neurophysiological study.

@entity1 @entity409 @entity747 @entity21864 @entity3301 @entity746 @entity528 @entity3668
BACKGROUND: Although only a few @entity747 ( @entity747 ) @entity1 develop frank @entity746 ( @entity746 ), motor @entity528 (MNDys) occur in a larger proportion of @entity1 . The aim of this study is to evaluate MNDys and @entity746 in a sample of consecutively enrolled sporadic @entity747 @entity1 . METHODS: Clinical and neurophysiological evaluations (i.e. needle electromyography) assessed lower (LMN) and upper (UMN) motor neuron function at the baseline in 70 probable @entity747 @entity1 (i.e., 26 behavioural variant-bvFTD, 20 primary progressive aphasias-PPAs and 24 corticobasal syndrome-CBS). To obtain a more accurate estimation, quantitative scales were also applied (i.e. ALSFRS-r and UMN scale). @entity1 were screened for @entity3668 , @entity21864 and @entity3301 mutations. A mean clinical follow-up of 27.8  22.4 months assessed MNDys progression and the clinical presentation of @entity746 . RESULTS: Five genetic cases were identified. Within the sample of sporadic @entity1 , a relative low rate of @entity747 @entity1 was diagnosed as probable @entity746 (5%), while a higher proportion of @entity1 (17%) showed clinical and neurophysiological MNDys. Thirteen @entity1 (20%) presented with isolated clinical signs of @entity409 , and 8 @entity1 (12%) showed neurogenic changes at the electromyography. No differences in @entity747 phenotype and disease duration were found between MNDys positive and negative @entity1 . Clinical MNDys were highly associated with positive electromyographic findings. At follow-up, no MNDys positive @entity1 developed @entity746 . CONCLUSION: Neurophysiological and clinical examinations revealed mild MNDys in @entity747 @entity1 not fulfilling criteria for @entity746 . This condition did not evolve at a mean follow-up of two years. These results, indicating a @entity528 and lower motor neurons, suggest that @entity747 @entity1 may be more at risk of MNDys than the general population.
