FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4366448152> ?p ?o ?g. }

• W4366448152 endingPage "4116" @default.
• W4366448152 startingPage "4105" @default.
• W4366448152 abstract "Abstract Increasing evidence shows that disease spreading in amyotrophic lateral sclerosis (ALS) follows a preferential pattern with more frequent involvement of contiguous regions from the site of symptom onset. The aim of our study was to assess if: (i) the burden of upper (UMN) and lower motor neuron (LMN) involvement influences directionality of disease spreading; (ii) specific patterns of disease progression are associated with motor and neuropsychological features of different ALS subtypes (classic, bulbar, primary lateral sclerosis, UMN-predominant, progressive muscular atrophy, flail arm, flail leg); and (iii) specific clinical features may help identify ALS subtypes, which remain localized to the site of onset for a prolonged time (regionally entrenching ALS). A single-centre, retrospective cohort of 913 Italian ALS patients was evaluated to assess correlations between directionality of the disease process after symptom onset and motor/neuropsychological phenotype. All patients underwent an extensive evaluation including the following clinical scales: Penn Upper Motor Neuron Score (PUMNS), MRC Scale for Muscle Strength and the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). The most frequent initial spreading pattern was that towards adjacent horizontal regions (77.3%), which occurred preferentially in patients with lower MRC scores (P = 0.038), while vertical diffusion (21.1%) was associated with higher PUMNS (P &lt; 0.001) and with reduced survival (P &lt; 0.001). Non-contiguous disease spreading was associated with more severe UMN impairment (P = 0.003), while contiguous disease pattern with lower MRC scores. Furthermore, non-contiguous disease spreading was associated with more severe cognitive impairment in both executive and visuospatial ECAS domains. Individuals with regionally entrenching ALS were more frequently female (45.6% versus 36.9%; P = 0.028) and had higher frequencies of symmetric disease onset (40.3% versus 19.7%; P &lt; 0.001) and bulbar phenotype (38.5% versus 16.4%; P &lt; 0.001). Our study suggests that motor phenotypes characterized by a predominant UMN involvement are associated with a vertical pattern of disease progression reflecting ipsilateral spreading within the motor cortex, while those with predominant LMN involvement display more frequently a horizontal spreading from one side of the spinal cord to the other. These observations raise the hypothesis that one of the mechanisms underlying disease spreading in ALS pathology is represented by diffusion of toxic factors in the neuron microenvironment. Finally, it is possible that in our cohort, regionally entrenching ALS forms are mainly observed in patients with atypical bulbar phenotypes, characterized by a slowly progressive course and relatively benign prognosis." @default.
• W4366448152 created "2023-04-22" @default.
• W4366448152 creator A5001016290 @default.
• W4366448152 creator A5014425322 @default.
• W4366448152 creator A5025840282 @default.
• W4366448152 creator A5028975603 @default.
• W4366448152 creator A5032993233 @default.
• W4366448152 creator A5035957460 @default.
• W4366448152 creator A5041312078 @default.
• W4366448152 creator A5047027591 @default.
• W4366448152 creator A5062637279 @default.
• W4366448152 creator A5066201036 @default.
• W4366448152 creator A5072540006 @default.
• W4366448152 creator A5074423844 @default.
• W4366448152 creator A5083304018 @default.
• W4366448152 creator A5085059200 @default.
• W4366448152 date "2023-04-19" @default.
• W4366448152 modified "2023-10-14" @default.
• W4366448152 title "Regional spreading pattern is associated with clinical phenotype in amyotrophic lateral sclerosis" @default.
• W4366448152 cites W1503916499 @default.
• W4366448152 cites W1560180811 @default.
• W4366448152 cites W1972539656 @default.
• W4366448152 cites W1981295136 @default.
• W4366448152 cites W1992271952 @default.
• W4366448152 cites W1993184479 @default.
• W4366448152 cites W1993984727 @default.
• W4366448152 cites W1997983266 @default.
• W4366448152 cites W2040891140 @default.
• W4366448152 cites W2070046685 @default.
• W4366448152 cites W2073987119 @default.
• W4366448152 cites W2079683596 @default.
• W4366448152 cites W2101748834 @default.
• W4366448152 cites W2107315373 @default.
• W4366448152 cites W2108381339 @default.
• W4366448152 cites W2119432914 @default.
• W4366448152 cites W2121554950 @default.
• W4366448152 cites W2129432034 @default.
• W4366448152 cites W2137641915 @default.
• W4366448152 cites W2146214940 @default.
• W4366448152 cites W2154446698 @default.
• W4366448152 cites W2157557701 @default.
• W4366448152 cites W2161938104 @default.
• W4366448152 cites W2220628142 @default.
• W4366448152 cites W2297401979 @default.
• W4366448152 cites W2338131205 @default.
• W4366448152 cites W2562172900 @default.
• W4366448152 cites W2564558365 @default.
• W4366448152 cites W2735438407 @default.
• W4366448152 cites W2740040314 @default.
• W4366448152 cites W2765761153 @default.
• W4366448152 cites W2794654744 @default.
• W4366448152 cites W2888767757 @default.
• W4366448152 cites W2989995720 @default.
• W4366448152 cites W3036124676 @default.
• W4366448152 cites W3047494286 @default.
• W4366448152 cites W4205539471 @default.
• W4366448152 cites W4210998205 @default.
• W4366448152 cites W4280493798 @default.
• W4366448152 cites W4293318792 @default.
• W4366448152 doi "https://doi.org/10.1093/brain/awad129" @default.
• W4366448152 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37075222" @default.
• W4366448152 hasPublicationYear "2023" @default.
• W4366448152 type Work @default.
• W4366448152 citedByCount "2" @default.
• W4366448152 countsByYear W43664481522023 @default.
• W4366448152 crossrefType "journal-article" @default.
• W4366448152 hasAuthorship W4366448152A5001016290 @default.
• W4366448152 hasAuthorship W4366448152A5014425322 @default.
• W4366448152 hasAuthorship W4366448152A5025840282 @default.
• W4366448152 hasAuthorship W4366448152A5028975603 @default.
• W4366448152 hasAuthorship W4366448152A5032993233 @default.
• W4366448152 hasAuthorship W4366448152A5035957460 @default.
• W4366448152 hasAuthorship W4366448152A5041312078 @default.
• W4366448152 hasAuthorship W4366448152A5047027591 @default.
• W4366448152 hasAuthorship W4366448152A5062637279 @default.
• W4366448152 hasAuthorship W4366448152A5066201036 @default.
• W4366448152 hasAuthorship W4366448152A5072540006 @default.
• W4366448152 hasAuthorship W4366448152A5074423844 @default.
• W4366448152 hasAuthorship W4366448152A5083304018 @default.
• W4366448152 hasAuthorship W4366448152A5085059200 @default.
• W4366448152 hasBestOaLocation W43664481521 @default.
• W4366448152 hasConcept C118552586 @default.
• W4366448152 hasConcept C126322002 @default.
• W4366448152 hasConcept C14216870 @default.
• W4366448152 hasConcept C169900460 @default.
• W4366448152 hasConcept C2776728740 @default.
• W4366448152 hasConcept C2776752467 @default.
• W4366448152 hasConcept C2777373429 @default.
• W4366448152 hasConcept C2779134260 @default.
• W4366448152 hasConcept C2780596555 @default.
• W4366448152 hasConcept C2781172350 @default.
• W4366448152 hasConcept C71924100 @default.
• W4366448152 hasConcept C72563966 @default.
• W4366448152 hasConcept C99508421 @default.
• W4366448152 hasConceptScore W4366448152C118552586 @default.
• W4366448152 hasConceptScore W4366448152C126322002 @default.
• W4366448152 hasConceptScore W4366448152C14216870 @default.
• W4366448152 hasConceptScore W4366448152C169900460 @default.

• next