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http://hdl.handle.net/1942/34217
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DC Field | Value | Language |
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dc.contributor.author | Rusina, Robert | - |
dc.contributor.author | Vandenberghe, Rik | - |
dc.contributor.author | BRUFFAERTS, Rose | - |
dc.date.accessioned | 2021-06-04T14:22:31Z | - |
dc.date.available | 2021-06-04T14:22:31Z | - |
dc.date.issued | 2021 | - |
dc.date.submitted | 2021-06-01T13:19:20Z | - |
dc.identifier.citation | Diagnostics, 11 (4) , (Art N° 624) | - |
dc.identifier.uri | http://hdl.handle.net/1942/34217 | - |
dc.description.abstract | Amyotrophic lateral sclerosis (ALS) has long been considered to be a purely motor disorder. However, it has become apparent that many ALS patients develop cognitive and behavioral manifestations similar to frontotemporal dementia and the term amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD) is now used in these circumstances. This review is intended to be an overview of the cognitive and behavioral manifestations commonly encountered in ALS patients with the goal of improving case-oriented management in clinical practice. We introduce the principal ALS-FTSD subtypes and comment on their principal clinical manifestations, neuroimaging findings, neuropathological and genetic background, and summarize available therapeutic options. Diagnostic criteria for ALS-FTSD create distinct categories based on the type of neuropsychological manifestations, i.e., changes in behavior, impaired social cognition, executive dysfunction, and language or memory impairment. Cognitive impairment is found in up to 65%, while frank dementia affects about 15% of ALS patients. ALS motor and cognitive manifestations can worsen in parallel, becoming more pronounced when bulbar functions (affecting speech, swallowing, and salivation) are involved. Dementia can precede or develop after the appearance of motor symptoms. ALS-FTSD patients have a worse prognosis and shorter survival rates than patients with ALS or frontotemporal dementia alone. Important negative prognostic factors are behavioral and personality changes. From the clinician's perspective, there are five major distinguishable ALS-FTSD subtypes: ALS with cognitive impairment, ALS with behavioral impairment, ALS with combined cognitive and behavioral impairment, fully developed frontotemporal dementia in combination with ALS, and comorbid ALS and Alzheimer's disease. Although the most consistent ALS and ALS-FTSD pathology is a disturbance in transactive response DNA binding protein 43 kDa (TDP-43) metabolism, alterations in microtubule-associated tau protein metabolism have also been observed in ALS-FTSD. Early detection and careful monitoring of cognitive deficits in ALS are crucial for patient and caregiver support and enable personalized management of individual patient needs. | - |
dc.description.sponsorship | Supported by the Ministry of Health, Czech Republic (TN64190), by the Grant Agency of the Ministry of Health (NV18-01-00399, NV19-04-00090), and by Charles University (Project Progress Q35/LF3). | - |
dc.language.iso | en | - |
dc.publisher | MDPI | - |
dc.rights | 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/). | - |
dc.subject.other | neurodegeneration | - |
dc.subject.other | amyotrophic lateral sclerosis | - |
dc.subject.other | behavioral impairment | - |
dc.subject.other | frontotemporal lobar degeneration | - |
dc.subject.other | dementia | - |
dc.title | Cognitive and Behavioral Manifestations in ALS: Beyond Motor System Involvement | - |
dc.type | Journal Contribution | - |
dc.identifier.issue | 4 | - |
dc.identifier.volume | 11 | - |
local.format.pages | 11 | - |
local.bibliographicCitation.jcat | A1 | - |
dc.description.notes | Rusina, R (corresponding author), Charles Univ Prague, Fac Med 3, Dept Neurol, Prague 14059, Czech Republic.; Rusina, R (corresponding author), Thomayer Univ Hosp, Prague 14059, Czech Republic. | - |
dc.description.notes | robert.rusina@lf3.cuni.cz; rik.vandenberghe@uzleuven.be; | - |
dc.description.notes | rose.bruffaerts@kuleuven.be | - |
dc.description.other | Rusina, R (corresponding author), Charles Univ Prague, Fac Med 3, Dept Neurol, Prague 14059, Czech Republic ; Thomayer Univ Hosp, Prague 14059, Czech Republic. robert.rusina@lf3.cuni.cz; rik.vandenberghe@uzleuven.be; rose.bruffaerts@kuleuven.be | - |
local.publisher.place | ST ALBAN-ANLAGE 66, CH-4052 BASEL, SWITZERLAND | - |
local.type.refereed | Refereed | - |
local.type.specified | Review | - |
local.bibliographicCitation.artnr | 624 | - |
dc.identifier.doi | 10.3390/diagnostics11040624 | - |
dc.identifier.isi | WOS:000642944900001 | - |
dc.identifier.eissn | 2075-4418 | - |
local.provider.type | wosris | - |
local.uhasselt.uhpub | yes | - |
local.description.affiliation | [Rusina, Robert] Charles Univ Prague, Fac Med 3, Dept Neurol, Prague 14059, Czech Republic. | - |
local.description.affiliation | [Rusina, Robert] Thomayer Univ Hosp, Prague 14059, Czech Republic. | - |
local.description.affiliation | [Vandenberghe, Rik; Bruffaerts, Rose] KU, Leuven Brain Inst LBI, Lab Cognit Neurol, Dept Neurosci, B-3000 Leuven, Belgium. | - |
local.description.affiliation | [Vandenberghe, Rik; Bruffaerts, Rose] Univ Hosp, Dept Neurol, B-3000 Leuven, Belgium. | - |
local.description.affiliation | [Bruffaerts, Rose] Hasselt Univ, Biomed Res Inst, B-3590 Diepenbeek, Belgium. | - |
local.uhasselt.international | yes | - |
item.fullcitation | Rusina, Robert; Vandenberghe, Rik & BRUFFAERTS, Rose (2021) Cognitive and Behavioral Manifestations in ALS: Beyond Motor System Involvement. In: Diagnostics, 11 (4) , (Art N° 624). | - |
item.validation | ecoom 2022 | - |
item.accessRights | Open Access | - |
item.fulltext | With Fulltext | - |
item.contributor | Rusina, Robert | - |
item.contributor | Vandenberghe, Rik | - |
item.contributor | BRUFFAERTS, Rose | - |
crisitem.journal.issn | 2075-4418 | - |
crisitem.journal.eissn | 2075-4418 | - |
Appears in Collections: | Research publications |
Files in This Item:
File | Description | Size | Format | |
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diagnostics-11-00624-v2.pdf | Published version | 275.51 kB | Adobe PDF | View/Open |
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