The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis

References

• Hardiman O, Al-Chalabi A, Chio A, et al. Amyotrophic lateral sclerosis. Nat Rev Dis Primers. 2017 Oct 20;3:17085. PubMed PMID: 29052611.
   PubMed Web of Science ®Google Scholar
• Kiernan M, Vucic S, Cheah B, et al. Amyotrophic lateral sclerosis. Lancet. 2011;277:942–955.
   Google Scholar
• Dharmadasa T, Matamala JM, Huynh W et al. Motor neurone disease. Handbook of clinical neurology. editors, Vinken PJ, Bruyn GW.2018. Vol. 159 p.345–357. DOI: 10.1016/b978-0-444-63916-5.00022-7. PubMed PMID: 30482326; eng.
   Google Scholar
• Strong MJ. Revisiting the concept of amyotrophic lateral sclerosis as a multisystems disorder of limited phenotypic expression. Curr Opin Neurol. 2017 Dec;30(6):599–607. PubMed PMID: 28914734.
   PubMed Web of Science ®Google Scholar
• Phukan J, Elamin M, Bede P, et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012 Jan;83(1):102–108. PubMed PMID: 21836033.
   PubMed Web of Science ®Google Scholar
• Elamin M, Bede P, Byrne S, et al. Cognitive changes predict functional decline in ALS: a population-based longitudinal study. Neurology. 2013 Apr 23;80(17):1590–1597. PubMed PMID: 23553481.
   PubMed Web of Science ®Google Scholar
• Massman PJ, Sims J, Cooke N, et al. Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1996 Nov;61(5):450–455. PubMed PMID: 8937336; PubMed Central PMCID: PMCPMC1074039.
   PubMed Web of Science ®Google Scholar
• Lomen-Hoerth C, Murphy J, Langmore S, et al. Are amyotrophic lateral sclerosis patients cognitively normal? [Research Support, Non-U.S. Gov’t]. Neurology. 2003 Apr 8;60(7):1094–1097. PubMed PMID: 12682312.
   PubMed Web of Science ®Google Scholar
• Irwin D, Lippa CF, Swearer JM. Cognition and amyotrophic lateral sclerosis (ALS). Am J Alzheimers Dis Other Demen. 2007 Aug-Sep;22(4):300–312. PubMed PMID: 17712161.
   PubMedGoogle Scholar
• Ringholz GM, Appel SH, Bradshaw M, et al. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. 2005 Aug 23;65(4):586–590. PubMed PMID: 16116120.
   PubMed Web of Science ®Google Scholar
• Xu Z, Alruwaili ARS, Henderson RD, et al. Screening for cognitive and behavioural impairment in amyotrophic lateral sclerosis: frequency of abnormality and effect on survival. J Neurol Sci. 2017 May 15;376:16–23. PubMed PMID: 28431606.
   PubMed Web of Science ®Google Scholar
• Consonni M, Iannaccone S, Cerami C, et al. The cognitive and behavioural profile of amyotrophic lateral sclerosis: application of the consensus criteria. Behav Neurol. 2013;27(2):143–153. PubMed PMID: 23001631; PubMed Central PMCID: PMCPMC5215720.
   PubMed Web of Science ®Google Scholar
• Montuschi A, Iazzolino B, Calvo A, et al. Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy. J Neurol Neurosurg Psychiatry. 2015 Feb;86(2):168–173. PubMed PMID: 24769471.
   PubMed Web of Science ®Google Scholar
• Renton AE, Majounie E, Waite A, et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011 Oct 20;72(2):257–268. PubMed PMID: 21944779; PubMed Central PMCID: PMCPMC3200438.
   PubMed Web of Science ®Google Scholar
• DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011 Oct 20;72(2):245–256. PubMed PMID: 21944778; PubMed Central PMCID: PMCPMC3202986.
   PubMed Web of Science ®Google Scholar
• Mioshi E, Lillo P, Yew B, et al. Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes. Neurology. 2013 Mar 19;80(12):1117–1123. PubMed PMID: 23427327.
   PubMed Web of Science ®Google Scholar
• Phukan J, Pender NP, Hardiman O. Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol. 2007 Nov;6(11):994–1003. PubMed PMID: 17945153.
   PubMed Web of Science ®Google Scholar
• Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017 May;18(3–4):153–174. PubMed PMID: 28054827.
   PubMed Web of Science ®Google Scholar
• Strong MJ, Grace GM, Freedman M, et al. Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009 Jun;10(3):131–146. PubMed PMID: 19462523.
   PubMed Web of Science ®Google Scholar
• Strong MJ. The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008 Dec;9(6):323–338. PubMed PMID: 18752088.
   PubMed Web of Science ®Google Scholar
• Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011 Sep;134(Pt 9):2456–2477. PubMed PMID: 21810890; PubMed Central PMCID: PMCPMC3170532.
   PubMed Web of Science ®Google Scholar
• Neary D, Snowden JS, Mann DM. Cognitive change in motor neurone disease/amyotrophic lateral sclerosis (MND/ALS). J Neurol Sci. 2000 Nov 1;180(1–2):15–20. PubMed PMID: 11090859.
   PubMed Web of Science ®Google Scholar
• Peavy GM, Herzog AG, Rubin NP, et al. Neuropsychological aspects of dementia of motor neuron disease: a report of two cases. Neurology. 1992 May;42(5):1004–1008. PubMed PMID: 1579222.
   PubMed Web of Science ®Google Scholar
• Woolley SC, Jonathan SK. Cognitive and behavioral impairment in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am. 2008 Aug;19(3):607–17, xi. PubMed PMID: 18625419.
   PubMed Web of Science ®Google Scholar
• Brooks BR, Miller RG, Swash M, et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2000 Dec;15:293–299. official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases PubMed PMID: 11464847.
   PubMed Web of Science ®Google Scholar
• Lomen-Hoerth C, Anderson T, Miller B. The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology. 2002 Oct 8;59(7):1077–1079. PubMed PMID: 12370467.
   PubMed Web of Science ®Google Scholar
• Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998 Dec;51(6):1546–1554. PubMed PMID: 9855500.
   PubMed Web of Science ®Google Scholar
• Guedj E, Le Ber I, Lacomblez L, et al. Brain spect perfusion of frontotemporal dementia associated with motor neuron disease. Neurology. 2007 Jul 31;69(5):488–490. PubMed PMID: 17664410.
   PubMed Web of Science ®Google Scholar
• Murphy JM, Henry RG, Langmore S, et al. Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol. 2007 Apr;64(4):530–534. PubMed PMID: 17420314.
   PubMedGoogle Scholar
• Neumann M, Sampathu D, Kwong L, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. 2006;314:130–133.
   PubMed Web of Science ®Google Scholar
• Ludolph AC, Langen KJ, Regard M, et al. Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography study. Acta Neurol Scand. 1992 Feb;85(2):81–89. PubMed PMID: 1574993.
   PubMed Web of Science ®Google Scholar
• Abrahams S, Leigh PN, Harvey A, et al. Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS). Neuropsychologia. 2000;38(6):734–747. PubMed PMID: 10689049.
   PubMed Web of Science ®Google Scholar
• Portet F, Cadilhac C, Touchon J, et al. Cognitive impairment in motor neuron disease with bulbar onset. Amyotroph Lateral Scler. 2001 Mar;21:23–29. official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases. PubMed PMID: 11465929.
   PubMed Web of Science ®Google Scholar
• Strong MJ, Grace GM, Orange JB, et al. A prospective study of cognitive impairment in ALS. Neurology. 1999 Nov 10;53(8):1665–1670. PubMed PMID: 10563610.
   PubMed Web of Science ®Google Scholar
• Rakowicz WP, Hodges JR. Dementia and aphasia in motor neuron disease: an underrecognised association? J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):881–889. PubMed PMID: 9854965; PubMed Central PMCID: PMCPMC2170423.
   PubMed Web of Science ®Google Scholar
• Frank B, Haas J, Heinze HJ, et al. Relation of neuropsychological and magnetic resonance findings in amyotrophic lateral sclerosis: evidence for subgroups. Clin Neurol Neurosurg. 1997 May;99(2):79–86. PubMed PMID: 9213049.
   PubMed Web of Science ®Google Scholar
• Mantovan MC, Baggio L, Dalla Barba G, et al. Memory deficits and retrieval processes in ALS. Eur J Neurol. 2003 May;10(3):221–227. PubMed PMID: 12752394.
   PubMed Web of Science ®Google Scholar
• Stojkovic T, Stefanova E, Pekmezovic T, et al. Executive dysfunction and survival in patients with amyotrophic lateral sclerosis: preliminary report from a Serbian centre for motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener. 2016 Oct-Nov;17(7–8):543–547. PubMed PMID: 27494074.
   PubMed Web of Science ®Google Scholar
• Jelsone-Swain L, Persad C, Votruba KL, et al. The relationship between depressive symptoms, disease state, and cognition in amyotrophic lateral sclerosis. Front Psychol. 2012;3:542. PubMed PMID: 23411492; PubMed Central PMCID: PMCPMC3571885.
   PubMed Web of Science ®Google Scholar
• Rippon GA, Scarmeas N, Gordon PH, et al. An observational study of cognitive impairment in amyotrophic lateral sclerosis. Arch Neurol. 2006 Mar;63(3):345–352. PubMed PMID: 16533961.
   PubMedGoogle Scholar
• Raaphorst J, de Visser M, van Tol MJ, et al. Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy. J Neurol Neurosurg Psychiatry. 2011 Feb;82(2):170–175. PubMed PMID: 20562407.
   PubMed Web of Science ®Google Scholar
• Kourtesis P, Christidi F, Margioti E, et al. The Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen (ECAS): sensitivity in differentiating between ALS and Alzheimer’s disease in a Greek population. Amyotroph Lateral Scler Frontotemporal Degener. 2019 Aug 30:1–8. DOI: 10.1080/21678421.2019.1655059. PubMed PMID: 31469297.
   PubMed Web of Science ®Google Scholar
• Machts J, Bittner V, Kasper E, et al. Memory deficits in amyotrophic lateral sclerosis are not exclusively caused by executive dysfunction: a comparative neuropsychological study of amnestic mild cognitive impairment. BMC Neurosci. 2014 Jun 30;15:83. PubMed PMID: 24981872; PubMed Central PMCID: PMCPMC4086690.
   PubMed Web of Science ®Google Scholar
• Agarwal S, Highton-Williamson E, Caga J, et al. Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum. J Neurol. 2018 Aug;265(8):1819–1828. PubMed PMID: 29868980.
   PubMed Web of Science ®Google Scholar
• Abrahams S, Goldstein LH, Al-Chalabi A, et al. Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1997 May;62(5):464–472. PubMed PMID: 9153602; PubMed Central PMCID: PMCPMC486852.
   PubMed Web of Science ®Google Scholar
• Rusina R, Ridzon P, Kulist’ak P, et al. Relationship between ALS and the degree of cognitive impairment, markers of neurodegeneration and predictors for poor outcome. A prospective study. Eur J Neurol. 2010 Jan;17(1):23–30. PubMed PMID: 19572947.
   PubMed Web of Science ®Google Scholar
• Xu W, Chi L, Row BW, et al. Increased oxidative stress is associated with chronic intermittent hypoxia-mediated brain cortical neuronal cell apoptosis in a mouse model of sleep apnea. Neuroscience. 2004;126(2):313–323. PubMed PMID: 15207349.
   PubMed Web of Science ®Google Scholar
• Huynh W, Sharplin LE, Caga J, et al. Respiratory function and cognitive profile in amyotrophic lateral sclerosis. Eur J Neurol. 2019. DOI:10.1111/ene.14130.
   PubMedGoogle Scholar
• Palmieri A, Mento G, Calvo V, et al. Female gender doubles executive dysfunction risk in ALS: a case-control study in 165 patients. J Neurol Neurosurg Psychiatry. 2015 May;86(5):574–579. PubMed PMID: 25063584.
   PubMed Web of Science ®Google Scholar
• Raaphorst J, Beeldman E, De Visser M, et al. A systematic review of behavioural changes in motor neuron disease [Review]. Amyotroph Lateral Scler. 2012 Oct;13(6):493–501. PubMed PMID: 22424127.
   PubMed Web of Science ®Google Scholar
• Burke T, Pinto-Grau M, Lonergan K, et al. A Cross-sectional population-based investigation into behavioral change in amyotrophic lateral sclerosis: subphenotypes, staging, cognitive predictors, and survival. Ann Clin Transl Neurol. 2017 May;4(5):305–317. PubMed PMID: 28491898; PubMed Central PMCID: PMCPMC5420811.
   PubMed Web of Science ®Google Scholar
• Grossman AB, Woolley-Levine S, Bradley WG, et al. Detecting neurobehavioral changes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2007 Feb;8(1):56–61. PubMed PMID: 17364437.
   PubMed Web of Science ®Google Scholar
• Flaherty-Craig C, Eslinger P, Stephens B, et al. A rapid screening battery to identify frontal dysfunction in patients with ALS. Neurology. 2006 Dec 12;67(11):2070–2072. PubMed PMID: 17159125.
   PubMed Web of Science ®Google Scholar
• Lillo P, Mioshi E, Zoing MC, et al. How common are behavioural changes in amyotrophic lateral sclerosis? Amyotroph Lateral Scler. 2011 Jan;12(1):45–51. PubMed PMID: 20849323.
   PubMed Web of Science ®Google Scholar
• Terada T, Obi T, Yoshizumi M, et al. Frontal lobe-mediated behavioral changes in amyotrophic lateral sclerosis: are they independent of physical disabilities? J Neurol Sci. 2011 Oct 15;309(1–2):136–140. PubMed PMID: 21782199.
   PubMed Web of Science ®Google Scholar
• Witgert M, Salamone AR, Strutt AM, et al. Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis. Eur J Neurol. 2010 Jan;17(1):103–110. PubMed PMID: 19874396.
   PubMed Web of Science ®Google Scholar
• Gibbons ZC, Richardson A, Neary D, et al. Behaviour in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2008 Apr;9(2):67–74. PubMed PMID: 18427998.
   PubMed Web of Science ®Google Scholar
• Ahmed RM, Caga J, Devenney E, et al. Cognition and eating behavior in amyotrophic lateral sclerosis: effect on survival. J Neurol. 2016 Aug;263(8):1593–1603. PubMed PMID: 27260291.
   PubMed Web of Science ®Google Scholar
• Beeldman E, Raaphorst J, Klein Twennaar M, et al. The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry. 2016 Jun;87(6):611–619. PubMed PMID: 26283685.
   PubMed Web of Science ®Google Scholar
• Santangelo G, Siciliano M, Trojano L, et al. Apathy in amyotrophic lateral sclerosis: insights from dimensional apathy scale. Amyotroph Lateral Scler Frontotemporal Degener. 2017 Aug;18(5–6):434–442. PubMed PMID: 28431489.
   PubMed Web of Science ®Google Scholar
• Radakovic R, Stephenson L, Colville S, et al. Multidimensional apathy in ALS: validation of the dimensional apathy scale. J Neurol Neurosurg Psychiatry. 2016 Jun;87(6):663–669. PubMed PMID: 26203157.
   PubMed Web of Science ®Google Scholar
• Roche JC, Rojas-Garcia R, Scott KM, et al. A proposed staging system for amyotrophic lateral sclerosis. Brain. 2012 Mar;135(Pt(3)):847–852. PubMed PMID: 22271664; PubMed Central PMCID: PMCPMC3286327.
   PubMedGoogle Scholar
• Radakovic R, Stephenson L, Newton J, et al. Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis [Research Support, Non-U.S. Gov’t]. Cortex. 2017 Sep;94:142–151. PubMed PMID: 28759804.
   PubMed Web of Science ®Google Scholar
• Kim HJ, Oh SI, de Leon M, et al. Structural explanation of poor prognosis of amyotrophic lateral sclerosis in the non-demented state. Eur J Neurol. 2017 Jan;24(1):122–129. PubMed PMID: 27753163; PubMed Central PMCID: PMCPMC5436047.
   PubMed Web of Science ®Google Scholar
• Bede P, Hardiman O. Lessons of ALS imaging: pitfalls and future directions - a critical review. Neuroimage Clin. 2014;4:436–443. PubMed PMID: 24624329; PubMed Central PMCID: PMCPMC3950559.
   PubMed Web of Science ®Google Scholar
• Bede P, Iyer PM, Schuster C, et al. The selective anatomical vulnerability of ALS: ‘disease-defining’ and ‘disease-defying’ brain regions. Amyotroph Lateral Scler Frontotemporal Degener. 2016 Oct-Nov;17(7–8):561–570. PubMed PMID: 27087114.
   PubMed Web of Science ®Google Scholar
• Schuster C, Elamin M, Hardiman O, et al. Presymptomatic and longitudinal neuroimaging in neurodegeneration–from snapshots to motion picture: a systematic review. J Neurol Neurosurg Psychiatry. 2015 Oct;86(10):1089–1096. PubMed PMID: 25632156.
   PubMed Web of Science ®Google Scholar
• Abe K. Cognitive function in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2000 Dec;1(5):343–347. official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases. PubMed PMID: 11464853.
   Web of Science ®Google Scholar
• Kato S, Hayashi H, Yagishita A. Involvement of the frontotemporal lobe and limbic system in amyotrophic lateral sclerosis: as assessed by serial computed tomography and magnetic resonance imaging. J Neurol Sci. 1993 May;116(1):52–58. PubMed PMID: 8509805.
   PubMed Web of Science ®Google Scholar
• Woolley SC, Zhang Y, Schuff N, et al. Neuroanatomical correlates of apathy in ALS using 4 Tesla diffusion tensor MRI. Amyotroph Lateral Scler. 2011 Jan;12(1):52–58. PubMed PMID: 21271791.
   PubMed Web of Science ®Google Scholar
• Brettschneider J, Del Tredici K, Toledo JB, et al. Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Ann Neurol. 2013 Jul;74(1):20–38. PubMed PMID: 23686809; PubMed Central PMCID: PMCPMC3785076.
   PubMed Web of Science ®Google Scholar
• Bede P, Bokde A, Elamin M, et al. Grey matter correlates of clinical variables in amyotrophic lateral sclerosis (ALS): a neuroimaging study of ALS motor phenotype heterogeneity and cortical focality. J Neurol Neurosurg Psychiatry. 2013 Jul;84(7):766–773. PubMed PMID: 23085933.
   PubMed Web of Science ®Google Scholar
• Terada T, Miyata J, Obi T, et al. Frontal assessment battery and frontal atrophy in amyotrophic lateral sclerosis. Brain Behav. 2017 Jun;7(6):e00707. PubMed PMID: 28638712; PubMed Central PMCID: PMCPMC5474710.
   PubMed Web of Science ®Google Scholar
• Agosta F, Ferraro PM, Riva N, et al. Structural brain correlates of cognitive and behavioral impairment in MND. Hum Brain Mapp. 2016 Apr;37(4):1614–1626. PubMed PMID: 26833930.
   PubMed Web of Science ®Google Scholar
• Schuster C, Kasper E, Dyrba M, et al. Cortical thinning and its relation to cognition in amyotrophic lateral sclerosis. Neurobiol Aging. 2014 Jan;35(1):240–246. PubMed PMID: 23992619.
   PubMed Web of Science ®Google Scholar
• Tsujimoto M, Senda J, Ishihara T, et al. Behavioral changes in early ALS correlate with voxel-based morphometry and diffusion tensor imaging. J Neurol Sci. 2011 Aug 15;307(1–2):34–40. PubMed PMID: 21641004.
   PubMed Web of Science ®Google Scholar
• Mezzapesa DM, Ceccarelli A, Dicuonzo F, et al. Whole-brain and regional brain atrophy in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol. 2007 Feb;28(2):255–259. PubMed PMID: 17296989.
   PubMed Web of Science ®Google Scholar
• Lillo P, Mioshi E, Burrell JR, et al. Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum. PloS One. 2012;7(8):e43993. PubMed PMID: 22952843; PubMed Central PMCID: PMCPMC3430626.
   PubMed Web of Science ®Google Scholar
• Hornberger M, Geng J, Hodges JR. Convergent grey and white matter evidence of orbitofrontal cortex changes related to disinhibition in behavioural variant frontotemporal dementia. Brain. 2011 Sep;134(Pt 9):2502–2512. PubMed PMID: 21785117.
   PubMed Web of Science ®Google Scholar
• Tan RH, Devenney E, Dobson-Stone C, et al. Cerebellar integrity in the amyotrophic lateral sclerosis-frontotemporal dementia continuum. PloS One. 2014;9(8):e105632. PubMed PMID: 25144223; PubMed Central PMCID: PMCPMC4140802.
   PubMed Web of Science ®Google Scholar
• Machts J, Loewe K, Kaufmann J, et al. Basal ganglia pathology in ALS is associated with neuropsychological deficits. Neurology. 2015 Oct 13;85(15):1301–1309. PubMed PMID: 26385880.
   PubMed Web of Science ®Google Scholar
• Turken A, Whitfield-Gabrieli S, Bammer R, et al. Cognitive processing speed and the structure of white matter pathways: convergent evidence from normal variation and lesion studies. Neuroimage. 2008 Aug 15;42(2):1032–1044. PubMed PMID: 18602840; PubMed Central PMCID: PMCPMC2630965.
   PubMed Web of Science ®Google Scholar
• Mesulam M. Brain, mind, and the evolution of connectivity. Brain Cogn. 2000 Feb;42(1):4–6. PubMed PMID: 10739582.
   PubMed Web of Science ®Google Scholar
• Bede P, Bokde AL, Byrne S, et al. Multiparametric MRI study of ALS stratified for the C9orf72 genotype. Neurology. 2013 Jul 23;81(4):361–369. PubMed PMID: 23771489; PubMed Central PMCID: PMCPMC3772833.
   PubMed Web of Science ®Google Scholar
• Keller J, Bohm S, Aho-Ozhan HEA, et al. Functional reorganization during cognitive function tasks in patients with amyotrophic lateral sclerosis. Brain Imaging Behav. 2018 Jun;12(3):771–784. PubMed PMID: 28600740.
   PubMed Web of Science ®Google Scholar
• Chio A, Pagani M, Agosta F, et al. Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 2014 Dec;13(12):1228–1240. PubMed PMID: 25453462.
   PubMed Web of Science ®Google Scholar
• Garraux G, Salmon E, Degueldre C, et al. Medial temporal lobe metabolic impairment in dementia associated with motor neuron disease. J Neurol Sci. 1999 Oct 15;168(2):145–150. PubMed PMID: 10526199.
   PubMed Web of Science ®Google Scholar
• Canosa A, Pagani M, Cistaro A, et al. 18F-FDG-PET correlates of cognitive impairment in ALS. Neurology. 2016 Jan 5;86(1):44–49. PubMed PMID: 26590270.
   PubMed Web of Science ®Google Scholar
• Huynh W, Simon NG, Grosskreutz J, et al. Assessment of the upper motor neuron in amyotrophic lateral sclerosis. Clin Neurophysiol. 2016 Jul;127(7):2643–2660. PubMed PMID: 27291884; eng.
   PubMed Web of Science ®Google Scholar
• Dharmadasa T, Huynh W, Tsugawa J, et al. Implications of structural and functional brain changes in amyotrophic lateral sclerosis. Expert Rev Neurother. 2018;18:407–419.
   PubMed Web of Science ®Google Scholar
• Talbot PR, Goulding PJ, Lloyd JJ, et al. Inter-relation between “classic” motor neuron disease and frontotemporal dementia: neuropsychological and single photon emission computed tomography study. J Neurol Neurosurg Psychiatry. 1995 May;58(5):541–547. PubMed PMID: 7745399; PubMed Central PMCID: PMCPMC1073482.
   PubMed Web of Science ®Google Scholar
• Vercelletto M, Ronin M, Huvet M, et al. Frontal type dementia preceding amyotrophic lateral sclerosis: a neuropsychological and SPECT study of five clinical cases. Eur J Neurol. 1999 May;6(3):295–299. PubMed PMID: 10210909.
   PubMed Web of Science ®Google Scholar
• Abe K, Fujimura H, Toyooka K, et al. Single-photon emission computed tomographic investigation of patients with motor neuron disease. Neurology. 1993 Aug;43(8):1569–1573. PubMed PMID: 8351013.
   PubMed Web of Science ®Google Scholar
• Abe K, Fujimura H, Toyooka K, et al. Cognitive function in amyotrophic lateral sclerosis. J Neurol Sci. 1997 May 1;148(1):95–100. PubMed PMID: 9125395.
   PubMed Web of Science ®Google Scholar
• Birn RM, Kenworthy L, Case L, et al. Neural systems supporting lexical search guided by letter and semantic category cues: a self-paced overt response fMRI study of verbal fluency. Neuroimage. 2010 Jan 1;49(1):1099–1107. PubMed PMID: 19632335; PubMed Central PMCID: PMCPMC2832834.
   PubMed Web of Science ®Google Scholar
• Mitsuyama Y. Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiatry. 1984 Sep;47(9):953–959. PubMed PMID: 6481390; PubMed Central PMCID: PMCPMC1027997.
   PubMed Web of Science ®Google Scholar
• Okamoto K, Hirai S, Yamazaki T, et al. New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett. 1991 Aug 19;129(2):233–236. PubMed PMID: 1660578.
   PubMed Web of Science ®Google Scholar
• Wightman G, Anderson VE, Martin J, et al. Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia. Neurosci Lett. 1992 May 25;139(2):269–274. PubMed PMID: 1376881.
   PubMed Web of Science ®Google Scholar
• Anderson VE, Cairns NJ, Leigh PN. Involvement of the amygdala, dentate and hippocampus in motor neuron disease. J Neurol Sci. 1995 May;129(Suppl):75–8. PubMed PMID: 7595627.
   PubMed Web of Science ®Google Scholar
• Yoshida M. Amyotrophic lateral sclerosis with dementia: the clinicopathological spectrum. Neuropathology. 2004 Mar;24(1):87–102. PubMed PMID: 15068178.
   PubMed Web of Science ®Google Scholar
• Arai T, Hasegawa M, Akiyama H, et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun. 2006 Dec 22;351(3):602–611. PubMed PMID: 17084815.
   PubMed Web of Science ®Google Scholar
• Wilson CM, Grace GM, Munoz DG, et al. Cognitive impairment in sporadic ALS: a pathologic continuum underlying a multisystem disorder. Neurology. 2001 Aug 28;57(4):651–657. PubMed PMID: 11524474.
   PubMed Web of Science ®Google Scholar
• Prudlo J, Konig J, Schuster C, et al. TDP-43 pathology and cognition in ALS: A prospective clinicopathologic correlation study. Neurology. 2016 Sep 6;87(10):1019–1023. PubMed PMID: 27488596.
   PubMed Web of Science ®Google Scholar
• Gregory JM, McDade K, Bak TH, et al. Executive, language and fluency dysfunction are markers of localised TDP-43 cerebral pathology in non-demented ALS. J Neurol Neurosurg Psychiatry. 2020 Feb;91(2):149–157. PubMed PMID: 31515300.
   PubMed Web of Science ®Google Scholar
• Parvizi J, Coburn KL, Shillcutt SD, et al. Neuroanatomy of pathological laughing and crying: a report of the American Neuropsychiatric Association committee on research. J Neuropsychiatry Clin Neurosci. 2009 Winter;21(1):75–87. PubMed PMID: 19359455.
   PubMed Web of Science ®Google Scholar
• Kringelbach ML. The human orbitofrontal cortex: linking reward to hedonic experience. Nat Rev Neurosci. 2005 Sep;6(9):691–702. PubMed PMID: 16136173.
   PubMed Web of Science ®Google Scholar
• Moretti R, Torre P, Antonello RM, et al. Complex cognitive disruption in motor neuron disease. Dement Geriatr Cogn Disord. 2002;14(3):141–150. PubMed PMID: 12218257.
   PubMed Web of Science ®Google Scholar
• Kim SM, Lee KM, Hong YH, et al. Relation between cognitive dysfunction and reduced vital capacity in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2007 Dec;78(12):1387–1389. PubMed PMID: 17557798; PubMed Central PMCID: PMCPMC2095584.
   PubMed Web of Science ®Google Scholar
• Newsom-Davis IC, Lyall RA, Leigh PN, et al. The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study. J Neurol Neurosurg Psychiatry. 2001 Oct;71(4):482–487. PubMed PMID: 11561031; PubMed Central PMCID: PMCPMC1763518.
   PubMed Web of Science ®Google Scholar
• Byrne S, Elamin M, Bede P, et al. Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol. 2012 Mar;11(3):232–240. PubMed PMID: 22305801; PubMed Central PMCID: PMCPMC3315021.
   PubMed Web of Science ®Google Scholar
• Chio A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler. 2009 Oct-Dec;10(5–6):310–323. PubMed PMID: 19922118; PubMed Central PMCID: PMCPMC3515205.
   PubMed Web of Science ®Google Scholar
• Creemers H, Grupstra H, Nollet F, et al. Prognostic factors for the course of functional status of patients with ALS: a systematic review. J Neurol. 2015 Jun;262(6):1407–1423. PubMed PMID: 25385051.
   PubMed Web of Science ®Google Scholar
• Elamin M, Bede P, Montuschi A, et al. Predicting prognosis in amyotrophic lateral sclerosis: a simple algorithm. J Neurol. 2015 Jun;262(6):1447–1454. PubMed PMID: 25860344; PubMed Central PMCID: PMCPMC4469087.
   PubMed Web of Science ®Google Scholar
• Shoesmith CL, Findlater K, Rowe A, et al. Prognosis of amyotrophic lateral sclerosis with respiratory onset. J Neurol Neurosurg Psychiatry. 2007 Jun;78(6):629–631. PubMed PMID: 17088331; PubMed Central PMCID: PMCPMC2077959.
   PubMed Web of Science ®Google Scholar
• Lillo P, Garcin B, Hornberger M, et al. Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis. Arch Neurol. 2010 Jul;67(7):826–830. PubMed PMID: 20625088.
   PubMedGoogle Scholar
• Hothorn T, Jung HH. RandomForest4Life: a random forest for predicting ALS disease progression. Amyotroph Lateral Scler Frontotemporal Degener. 2014 Sep;15(5–6):444–452. PubMed PMID: 25141076.
   PubMed Web of Science ®Google Scholar
• Westeneng HJ, Debray TPA, Visser AE, et al. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol. 2018 May;17(5):423–433. PubMed PMID: 29598923.
   PubMed Web of Science ®Google Scholar
• Elamin M, Phukan J, Bede P, et al. Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology. 2011 Apr 5;76(14):1263–1269. PubMed PMID: 21464431.
   PubMed Web of Science ®Google Scholar
• Gordon PH, Goetz RR, Rabkin JG, et al. A prospective cohort study of neuropsychological test performance in ALS. Amyotroph Lateral Scler. 2010 May 3;11(3):312–320. PubMed PMID: 20225929.
   PubMed Web of Science ®Google Scholar
• Hu WT, Shelnutt M, Wilson A, et al. Behavior matters–cognitive predictors of survival in amyotrophic lateral sclerosis. PloS One. 2013;8(2):e57584. PubMed PMID: 23460879; PubMed Central PMCID: PMC3583832.
   PubMed Web of Science ®Google Scholar
• Govaarts R, Beeldman E, Kampelmacher MJ, et al. The frontotemporal syndrome of ALS is associated with poor survival. J Neurol. 2016 Dec;263(12):2476–2483. PubMed PMID: 27671483; PubMed Central PMCID: PMCPMC5110703.
   PubMed Web of Science ®Google Scholar
• Olney RK, Murphy J, Forshew D, et al. The effects of executive and behavioral dysfunction on the course of ALS. Neurology. 2005 Dec 13;65(11):1774–1777. PubMed PMID: 16344521.
   PubMed Web of Science ®Google Scholar
• Caga J, Turner MR, Hsieh S, et al. Apathy is associated with poor prognosis in amyotrophic lateral sclerosis [Research Support, Non-U.S. Gov’t]. Eur J Neurol. 2016 May;23(5):891–897. PubMed PMID: 26822417.
   PubMed Web of Science ®Google Scholar
• Burke T, Elamin M, Galvin M, et al. Caregiver burden in amyotrophic lateral sclerosis: a cross-sectional investigation of predictors [Research Support, Non-U.S. Gov’t]. J Neurol. 2015 Jun;262(6):1526–1532. PubMed PMID: 25904206.
   PubMed Web of Science ®Google Scholar
• Caga J, Hsieh S, Highton-Williamson E, et al. Apathy and its impact on patient outcome in amyotrophic lateral sclerosis. J Neurol. 2018 Jan;265(1):187–193. PubMed PMID: 29189922.
   PubMed Web of Science ®Google Scholar
• Hodges JR, Davies R, Xuereb J, et al. Survival in frontotemporal dementia. Neurology. 2003 Aug 12;61(3):349–354. PubMed PMID: 12913196.
   PubMed Web of Science ®Google Scholar
• Hu WT, Seelaar H, Josephs KA, et al. Survival profiles of patients with frontotemporal dementia and motor neuron disease. Arch Neurol. 2009 Nov;66(11):1359–1364. PubMed PMID: 19901167; PubMed Central PMCID: PMCPMC2881327.
   PubMedGoogle Scholar
• Abrahams S, Leigh PN, Goldstein LH. Cognitive change in ALS: a prospective study. Neurology. 2005 Apr 12;64(7):1222–1226. PubMed PMID: 15824350.
   PubMed Web of Science ®Google Scholar
• Kasper E, Zydatiss K, Schuster C, et al. No Change in executive performance in ALS patients: a longitudinal neuropsychological study. Neurodegener Dis. 2016;16(3–4):184–191. PubMed PMID: 26613522.
   PubMed Web of Science ®Google Scholar
• Burkhardt C, Neuwirth C, Weber M. Longitudinal assessment of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): lack of practice effect in ALS patients? Amyotroph Lateral Scler Frontotemporal Degener. 2017 May;18(3–4):202–209. PubMed PMID: 28165766.
   PubMed Web of Science ®Google Scholar
• Robinson KM, Lacey SC, Grugan P, et al. Cognitive functioning in sporadic amyotrophic lateral sclerosis: a six month longitudinal study. J Neurol Neurosurg Psychiatry. 2006 May;77(5):668–670. PubMed PMID: 16614030; PubMed Central PMCID: PMCPMC2117453.
   PubMed Web of Science ®Google Scholar
• Geser F, Prvulovic D, O’Dwyer L, et al. On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia. Prog Neurobiol. 2011 Dec;95(4):649–662. PubMed PMID: 21911035; PubMed Central PMCID: PMCPMC3230745.
   PubMed Web of Science ®Google Scholar
• Ravits JM, La Spada AR. ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration. Neurology. 2009 Sep 8;73(10):805–811. PubMed PMID: 19738176; PubMed Central PMCID: PMC2739608.
   PubMed Web of Science ®Google Scholar
• Fralick M, Sacks CA, Kesselheim AS. Assessment of use of combined dextromethorphan and quinidine in patients with dementia or Parkinson disease after US food and drug administration approval for pseudobulbar affect. JAMA Intern Med. 2019 Feb 1;179(2):224–230. PubMed PMID: 30615021; PubMed Central PMCID: PMCPMC6439654.
   PubMed Web of Science ®Google Scholar
• Brooks BR, Thisted RA, Appel SH, et al. Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology. 2004 Oct 26;63(8):1364–1370. PubMed PMID: 15505150.
   PubMed Web of Science ®Google Scholar
• Merrilees J, Klapper J, Murphy J, et al. Cognitive and behavioral challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010 May 3;11(3):298–302. PubMed PMID: 20222805; PubMed Central PMCID: PMC2908374.
   PubMed Web of Science ®Google Scholar
• Trojsi F, Santangelo G, Caiazzo G, et al. Neuropsychological assessment in different King’s clinical stages of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17(3–4):228–235. PubMed PMID: 26905940.
   PubMed Web of Science ®Google Scholar
• Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006 Feb;5(2):140–147. PubMed PMID: 16426990.
   PubMed Web of Science ®Google Scholar
• Ahmed RM, Highton-Williamson E, Caga J, et al. Lipid metabolism and survival across the frontotemporal dementia-amyotrophic lateral sclerosis spectrum: relationships to eating behavior and cognition. J Alzheimers Dis. 2018;61(2):773–783. PubMed PMID: 29254092.
   PubMed Web of Science ®Google Scholar
• Strutt AM, Palcic J, Wager JG, et al. Cognition, behavior, and respiratory function in amyotrophic lateral sclerosis. ISRN Neurol. 2012;2012:912123. PubMed PMID: 22852095; PubMed Central PMCID: PMCPMC3407622.
   PubMedGoogle Scholar
• Mioshi E, Dawson K, Mitchell J, et al. The Addenbrooke’s Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening. Int J Geriatr Psychiatry. 2006 Nov;21(11):1078–1085. PubMed PMID: 16977673.
   PubMed Web of Science ®Google Scholar
• Hsieh S, S S, Hoon C, et al. Validation of the Addenbrooke’s Cognitive Examination III in frontotemporal dementia and Alzheimer’s disease. Dement Geriatr Cogn Disord. 2013;36(3–4):242–250. PubMed PMID: 23949210.
   PubMed Web of Science ®Google Scholar
• Ahn SW, Kim SH, Kim JE, et al. Frontal assessment battery to evaluate frontal lobe dysfunction in ALS patients. Can J Neurol Sci. 2011 Mar;38(2):242–246. PubMed PMID: 21320827.
   PubMed Web of Science ®Google Scholar
• Dubois B, Slachevsky A, Litvan I, et al. The FAB: a Frontal assessment battery at bedside. Neurology. 2000 Dec 12;55(11):1621–1626. PubMed PMID: 11113214.
   PubMed Web of Science ®Google Scholar
• Abrahams S, Newton J, Niven E, et al. Screening for cognition and behaviour changes in ALS [Research Support, Non-U.S. Gov’t]. Amyotroph Lateral Scler Frontotemporal Degener. 2014 Mar;15(1–2):9–14. PubMed PMID: 23781974.
   PubMed Web of Science ®Google Scholar
• Niven E, Newton J, Foley J, et al. Validation of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): A cognitive tool for motor disorders. Amyotroph Lateral Scler Frontotemporal Degener. 2015 Jun;16(3–4):172–179. PubMed PMID: 25967542.
   PubMed Web of Science ®Google Scholar
• Woolley SC, York MK, Moore DH, et al. Detecting frontotemporal dysfunction in ALS: utility of the ALS Cognitive Behavioral Screen (ALS-CBS) [Multicenter Study]. Amyotroph Lateral Scler. 2010 May 03;11(3):303–311. PubMed PMID: 20433413.
   PubMed Web of Science ®Google Scholar
• Mioshi E, Hsieh S, Caga J, et al. A novel tool to detect behavioural symptoms in ALS [Case ReportsResearch Support, Non-U.S. Gov’t]. Amyotroph Lateral Scler Frontotemporal Degener. 2014 Jun;15(3–4):298–304. PubMed PMID: 24863641.
   PubMed Web of Science ®Google Scholar
• Raaphorst J, Beeldman E, Schmand B, et al. The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS [Comparative Study]. Neurology. 2012 Sep 25;79(13):1377–1383. PubMed PMID: 22972650.
   PubMed Web of Science ®Google Scholar
• Beeldman E, Jaeger B, Raaphorst J, et al. The verbal fluency index: dutch normative data for cognitive testing in ALS. Amyotroph Lateral Scler Frontotemporal Degener. 2014 Sep;15(5–6):388–391. PubMed PMID: 24862654.
   PubMed Web of Science ®Google Scholar
• Lillo P, Savage S, Mioshi E, et al. Amyotrophic lateral sclerosis and frontotemporal dementia: A behavioural and cognitive continuum. Amyotroph Lateral Scler. 2012 Jan;13(1):102–109. PubMed PMID: 22214356.
   PubMed Web of Science ®Google Scholar
• Raaphorst J, Beeldman E, Jaeger B, et al. Is the Frontal assessment battery reliable in ALS patients? Amyotroph Lateral Scler Frontotemporal Degener. 2013 Jan;14(1):73–74. PubMed PMID: 22889176.
   PubMed Web of Science ®Google Scholar
• Proudfoot M, Menke RA, Sharma R, et al. Eye-tracking in amyotrophic lateral sclerosis: A longitudinal study of saccadic and cognitive tasks. Amyotroph Lateral Scler Frontotemporal Degener. 2015;17(1–2):101–111. PubMed PMID: 26312652; PubMed Central PMCID: PMCPMC5127416.
   PubMed Web of Science ®Google Scholar