PMC:6610326 / 5246-7082
Annnotations
{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/6610326","sourcedb":"PMC","sourceid":"6610326","source_url":"https://www.ncbi.nlm.nih.gov/pmc/6610326","text":"ALS shares clinical and pathological features with frontotemporal dementia (FTD), a type of dementia that involves impaired judgment and executive skills. In FTD, the loss of cortical MNs is accompanied by loss of neurons in the frontal and temporal cortices, which correlates clinically with the symptoms of FTD (Neumann et al., 2006; Burrell et al., 2016). The relationship between ALS and FTD has been confirmed through genetic studies, and these two conditions are now considered to be at opposite ends of the same disease continuum (Taylor et al., 2016; Shahheydari et al., 2017). Hence, while ALS was historically judged as a disorder affecting the motor system only, it is now recognized that non-motor features are present (Fang et al., 2017). A wealth of evidence also demonstrates that ALS is a heterogeneous disorder. The clinical symptoms, including the proportion of UMN and LMN signs, age of onset, disease duration, and association with other conditions, are major features contributing to its highly variable phenotypes. As well as the development of FTD (Strong and Yang, 2011), ALS can also involve cognitive impairment in up to 50% of patients (Tsermentseli et al., 2012), the autonomic nervous system (Piccione et al., 2015), supranuclear gaze systems (van der Graaff et al., 2009; Donaghy et al., 2011), and extrapyramidal motor signs (Pradat et al., 2002). Sensory, olfactory and visual dysfunction have also been described in some patients (Bede et al., 2016). In addition, there are also other conditions affecting MNs that share similarities, but also striking differences, to ALS. In particular, primary lateral sclerosis (PLS) affects UMNs but it progresses much slowly than ALS. It also has a significantly lower mortality rate (Tartaglia et al., 2007), consistent with the relative resistant of LMNs in ALS.","tracks":[{"project":"2_test","denotations":[{"id":"31316328-17023659-38515282","span":{"begin":330,"end":334},"obj":"17023659"},{"id":"31316328-26987909-38515283","span":{"begin":352,"end":356},"obj":"26987909"},{"id":"31316328-27830784-38515284","span":{"begin":553,"end":557},"obj":"27830784"},{"id":"31316328-28539871-38515285","span":{"begin":579,"end":583},"obj":"28539871"},{"id":"31316328-28805578-38515286","span":{"begin":745,"end":749},"obj":"28805578"},{"id":"31316328-21809041-38515287","span":{"begin":1089,"end":1093},"obj":"21809041"},{"id":"31316328-21396632-38515288","span":{"begin":1185,"end":1189},"obj":"21396632"},{"id":"31316328-25211238-38515289","span":{"begin":1239,"end":1243},"obj":"25211238"},{"id":"31316328-19070491-38515290","span":{"begin":1296,"end":1300},"obj":"19070491"},{"id":"31316328-21097546-38515291","span":{"begin":1318,"end":1322},"obj":"21097546"},{"id":"31316328-11921138-38515292","span":{"begin":1372,"end":1376},"obj":"11921138"},{"id":"31316328-27087114-38515293","span":{"begin":1477,"end":1481},"obj":"27087114"},{"id":"31316328-17296839-38515294","span":{"begin":1775,"end":1779},"obj":"17296839"}],"attributes":[{"subj":"31316328-17023659-38515282","pred":"source","obj":"2_test"},{"subj":"31316328-26987909-38515283","pred":"source","obj":"2_test"},{"subj":"31316328-27830784-38515284","pred":"source","obj":"2_test"},{"subj":"31316328-28539871-38515285","pred":"source","obj":"2_test"},{"subj":"31316328-28805578-38515286","pred":"source","obj":"2_test"},{"subj":"31316328-21809041-38515287","pred":"source","obj":"2_test"},{"subj":"31316328-21396632-38515288","pred":"source","obj":"2_test"},{"subj":"31316328-25211238-38515289","pred":"source","obj":"2_test"},{"subj":"31316328-19070491-38515290","pred":"source","obj":"2_test"},{"subj":"31316328-21097546-38515291","pred":"source","obj":"2_test"},{"subj":"31316328-11921138-38515292","pred":"source","obj":"2_test"},{"subj":"31316328-27087114-38515293","pred":"source","obj":"2_test"},{"subj":"31316328-17296839-38515294","pred":"source","obj":"2_test"}]},{"project":"0_colil","denotations":[{"id":"31316328-17023659-630739","span":{"begin":330,"end":334},"obj":"17023659"},{"id":"31316328-26987909-630740","span":{"begin":352,"end":356},"obj":"26987909"},{"id":"31316328-27830784-630741","span":{"begin":553,"end":557},"obj":"27830784"},{"id":"31316328-28539871-630742","span":{"begin":579,"end":583},"obj":"28539871"},{"id":"31316328-28805578-630743","span":{"begin":745,"end":749},"obj":"28805578"},{"id":"31316328-21809041-630744","span":{"begin":1089,"end":1093},"obj":"21809041"},{"id":"31316328-21396632-630745","span":{"begin":1185,"end":1189},"obj":"21396632"},{"id":"31316328-25211238-630746","span":{"begin":1239,"end":1243},"obj":"25211238"},{"id":"31316328-19070491-630747","span":{"begin":1296,"end":1300},"obj":"19070491"},{"id":"31316328-21097546-630748","span":{"begin":1318,"end":1322},"obj":"21097546"},{"id":"31316328-11921138-630749","span":{"begin":1372,"end":1376},"obj":"11921138"},{"id":"31316328-27087114-630750","span":{"begin":1477,"end":1481},"obj":"27087114"},{"id":"31316328-17296839-630751","span":{"begin":1775,"end":1779},"obj":"17296839"}],"attributes":[{"subj":"31316328-17023659-630739","pred":"source","obj":"0_colil"},{"subj":"31316328-26987909-630740","pred":"source","obj":"0_colil"},{"subj":"31316328-27830784-630741","pred":"source","obj":"0_colil"},{"subj":"31316328-28539871-630742","pred":"source","obj":"0_colil"},{"subj":"31316328-28805578-630743","pred":"source","obj":"0_colil"},{"subj":"31316328-21809041-630744","pred":"source","obj":"0_colil"},{"subj":"31316328-21396632-630745","pred":"source","obj":"0_colil"},{"subj":"31316328-25211238-630746","pred":"source","obj":"0_colil"},{"subj":"31316328-19070491-630747","pred":"source","obj":"0_colil"},{"subj":"31316328-21097546-630748","pred":"source","obj":"0_colil"},{"subj":"31316328-11921138-630749","pred":"source","obj":"0_colil"},{"subj":"31316328-27087114-630750","pred":"source","obj":"0_colil"},{"subj":"31316328-17296839-630751","pred":"source","obj":"0_colil"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"2_test","color":"#93a0ec","default":true},{"id":"0_colil","color":"#baec93"}]}]}}