Motor neuron dysfunctions in the frontotemporal lobar degeneration spectrum: a clinical and neurophysiological study

Journal of the Neurological Sciences
Chiara CeramiStefano F Cappa

Abstract

Although only a few frontotemporal lobar degeneration (FTLD) patients develop frank amyotrophic lateral sclerosis (ALS), motor neuron dysfunctions (MNDys) occur in a larger proportion of patients. The aim of this study is to evaluate MNDys and ALS in a sample of consecutively enrolled sporadic FTLD patients. Clinical and neurophysiological evaluations (i.e. needle electromyography) assessed lower (LMN) and upper (UMN) motor neuron function at the baseline in 70 probable FTLD patients (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). Patients were screened for MAPT, GRN and C9orf72 mutations. A mean clinical follow-up of 27.8±22.4 months assessed MNDys progression and the clinical presentation of ALS. Five genetic cases were identified. Within the sample of sporadic patients, a relative low rate of FTLD patients was diagnosed as probable ALS (5%), while a higher proportion of patients (17%) showed clinical and neurophysiological MNDys. Thirteen patients (20%) presented with isolated clinical signs of LMN and/or UMN dysfunction, and 8 patients (12%) showed neurogenic...Continue Reading

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Citations

Mar 27, 2016·Journal of Neurochemistry·Tiffany W Todd, Leonard Petrucelli
Apr 8, 2017·Journal of Alzheimer's Disease : JAD·Tim Van LangenhoveJohn R Hodges
Sep 17, 2018·Database : the Journal of Biological Databases and Curation·Subodh Kumar MishraAmit Kumar
Oct 12, 2018·BioMed Research International·Maria ArioliNicola Canessa
Jul 8, 2021·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Marta GromichoMamede De Carvalho

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