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Abstract
Our objective was to assess the association between amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases such as Alzheimer's disease (AD), frontotemporal dementia (FTD) and Parkinson's disease (PD). From May 2007 through August 2012 we investigated 146 patients with newly diagnosed ALS and 146 age- and gender-matched controls. Each individual was screened for cardinal extrapyramidal signs (neurological examination) and cognitive dysfunction (Mini Mental State Examination, MMSE and Frontal Assessment Battery, FAB). Results demonstrated that rigidity was present in 8.2% of cases and 2.1% of controls (adjusted odds ratio, adjOR 5.7; 95% CI 1.5–22.0). The corresponding percentages for bradykinesia and postural instability were, respectively, 8.2 vs. 2.7% (adjOR 4.8; 95% CI 1.4–16.5) and 2.7 vs. 9.6% (adjOR 0.3; 95% CI 0.1–0.9). FAB ≤ 13.4 was recorded in 24.8 vs. 9.6%; adjOR 2.9; 95% CI 1.5–5.7). Tremor and abnormal FAB score were predicted by an older age at onset while an abnormal FAB score was associated with cramps and family history of neurodegenerative diseases. In conclusion, our data support the notion that newly diagnosed ALS carries a higher than expected risk of extrapyramidal signs and FTD.
Acknowledgements
The study has been funded by the Italian Ministry of Health (RFFS-2006-7-335969).
Declaration of interest: E. Pupillo has received funding from the American ALS Association and Italian Ministry of Health for data management and data monitoring of an observational study protocol. She receives funding from Italian Drug Agency (AIFA) for data monitoring and study management of randomized clinical trials. P. Messina has received funding from Sanofi-Aventis, EISAI, Lombardy Region, and the American ALS Association for the data analysis and data management of RCT and observational study protocol. C. Lunetta has received compensation for board membership by ITALFARMACO. M. Corbo has received compensation for board membership by ITALFARMACO. M. Filosto has received funding from Genzyme, CSL Behring and Baxter.
J. Mandrioli has received research grants from Regione EmiIia Romagna (Programma di ricerca Regione-Università 2010–2012, area 2, Ricerca per il Governo Clinico), and she has received compensation for board membership by ITALFARMACO. Emilia Romagna Registry for ALS is supported by a grant from the Emilia Romagna Regional Health Authority. E. Beghi has received payment for board membership by VIROPHARMA and EISAI; has received funding for travel and speaker honoraria from UCB-Pharma, GSK and also for educational presentations from GSK; has received grants for research activities from the Italian Drug Agency, Italian Ministry of Health, Sanofi-Aventis and the American ALS Association.
Bianchi, Chiveri, Lorusso, Marin, Riva, Sasanelli and Tremolizzo report no conflicts of interest.