الفهرس 
Amyotrophic Lateral Sclerosis OverviewOnly 14 pages are availabe for public view
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Abstract
Background: Recently, cognitive and behavioral changes in ALS are featured as an integral part of the disease. A noticeable proportion of ALS patients present with a full-blown picture of frontotemporal dementia (typically behavioral variant), which is considered as the most common form of cognitive impairment in ALS with an average percentage of 25-33% of the patients being affected.
Aim: Comparing cognitive functions in ALS patients and healthy controls and comparing cognitive functions in patients with early and late onset amyotrophic lateral sclerosis.
Method: A case control study, conducted from September 2020 to April 2021, included 30 ALS patients (15 early onset cases, 15 late onset cases); who attended the neuromuscular unit of Ain Shams University Hospitals and fulfilled the criteria of diagnosis of ALS and 30 healthy matched controls, assessed by Edinburg cognitive and behavioral ALS screen (ECAS) and ALS patients were assessed by King’s clinical staging scale and Amyotrophic lateral sclerosis Functional Rating Scale-Revised (ALSFRS-R).
Results: A total of 30 ALS cases and 30 matched healthy controls were enrolled, their sociodemographic data were statistically insignificant. Regarding cognitive and behavioral assessment using the ECAS, both ECAS mean total score and sub-domains mean scores were significantly lower in ALS patients compared to controls (p-value <0.001), with statistically significant relation between ALS clinical staging and ECAS total scores (p-value <0.001). Furthermore, it was found that mean scores of nearly all ECAS domains are lower in early onset group than late onset but with a non-statistically significant difference.
Conclusion: ALS cause significant cognitive impairment, with significant relation between functional status and clinical staging of the disease with the severity of cognitive and behavioural dysfunctions and although early onset cases had had lower score on ECAS compared to those with late onset, but it was non-significant.