The Frontotemporal Syndromes of ALS. Clinicopathological Correlates
Abstract
Amyotrophic lateral sclerosis (ALS) is increasingly recognized to be a syndromic disorder in which the degeneration of motor neurons is frequently accompanied by a range of syndromes reflective of frontotemporal dysfunction, including a behavioural or cognitive syndrome, a dysexecutive syndrome or a frontotemporal dementia. Both sporadic and familial variants of ALS can be affected. The anatomic substrate of each is a frontotemporal lobar degeneration (FTLD) characterized by superficial linear spongiosus, atrophy and neuronal loss, and both astrocytic and neuronal deposition of TDP-43 as pathological inclusions. Largely unrecognized however is the extent of alterations in tau protein metabolism, particularly in cognitively impaired patients (ALSci). This includes hyper-phosphorylation (pThr(175)) and tau phosphatase resistance, increased fibril formation ex vivo of tau isolated from ALSci and tau immunoreactive aggregates in neurons, dystrophic neurites and astrocytes. In this article, we will review the contemporary clinical, genetic and neuropathological characteristics of the frontotemporal syndromes of ALS and propose that as opposed to being a FTLD in which TDP-43 is the primary disease protein (FTLD-TDP) and that the frontotemporal syndromes of ALS represent a hybrid of both TDP-43 and tau pathology.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Alteration in tau protein metabolism including hyperphosphorylation, tau phosphatase resistance, and deposition of PHF-tau intracellular aggregates may be found in diseases characterized by abnormal TDP-43 metabolism, such as ALS.
“There is also evidence that alteration in tau protein metabolism including hyperphosphorylation, tau phosphatase resistance, and deposition of PHF-tau intracellular aggregates may be found in diseases characterized by abnormal TDP-43 metabolism, such as ALS [177].”
From The neuropathology of sport (2014) · cites this paper as [177]
Cites
Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576 · see citing works
Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., Mann, D., Tsuchiya, K., Yoshida, M., Hashizume, Y., et al. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.10.093 · Cited by 2,732 · see citing works
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Vance, C. A., Rogelj, B., Hortobágyi, T., Vos, K. J. D., Nishimura, A. L., Sreedharan, J., Hu, X., Smith, B. N., Ruddy, D., Wright, P. D., et al. (2009) · Science · doi:10.1126/science.1165942 · Cited by 2,531 · see citing works
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
Kwiatkowski, T. J., Bosco, D. A., LeClerc, A. L., Tamrazian, E., Vanderburg, C. R., Russ, C., Davis, A., Gilchrist, J. M., Kasarskis, E. J., Munsat, T. L., et al. (2009) · Science · doi:10.1126/science.1166066 · Cited by 2,526 · see citing works
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
Lagier-Tourenne, C., Polymenidou, M., & Cleveland, D. W. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq137 · Cited by 1,021 · see citing works
Mackenzie, I. R. A., Neumann, M., Bigio, E. H., Cairns, N. J., Alafuzoff, I., Kril, J., Kovacs, G. G., Ghetti, B., Halliday, G., Holm, I. E., et al. (2010) · Acta Neuropathologica · doi:10.1007/s00401-009-0612-2 · Cited by 1,012 · see citing works
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
Mackenzie, I. R., Rademakers, R., & Neumann, M. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70195-2 · Cited by 977 · see citing works
TDP‐43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease
Amador‐Ortiz, C., Lin, W., Ahmed, Z., Personett, D., Davies, P., Duara, R., Graff‐Radford, N. R., Hutton, M. L., & Dickson, D. W. (2007) · Annals of Neurology · doi:10.1002/ana.21154 · Cited by 916 · see citing works
Prevalence and patterns of cognitive impairment in sporadic ALS
Ringholz, G. M., Appel, S. H., Bradshaw, M. R., Cooke, N., Mosnik, D. M., & Schulz, P. E. (2005) · Neurology · doi:10.1212/01.wnl.0000172911.39167.b6 · Cited by 843 · see citing works
Turner, M. R., Cagnin, A., Turkheimer, F. E., Miller, C. C. J., Shaw, C. E., Brooks, D. J., Leigh, P. N., & Banati, R. B. (2004) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.12.012 · Cited by 737 · see citing works
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
Wegorzewska, I., Bell, S., Cairns, N. J., Miller, T. M., & Baloh, R. H. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0908767106 · Cited by 706 · see citing works
The overlap of amyotrophic lateral sclerosis and frontotemporal dementia
Lomen, C. E., Anderson, T. T., & Miller, B. L. (2002) · Neurology · doi:10.1212/wnl.59.7.1077 · Cited by 699 · see citing works
Are amyotrophic lateral sclerosis patients cognitively normal?
Lomen-Hoerth, C., Murphy, J. M., Langmore, S. E., Kramer, J. H., Olney, R. K., & Miller, B. L. (2003) · Neurology · doi:10.1212/01.wnl.0000055861.95202.8d · Cited by 616 · see citing works
Wils, H., Kleinberger, G., Janssens, J., Pereson, S., Joris, G., Cuijt, I., Smits, V., Groote, C. C., Broeckhoven, C. V., & Kumar‐Singh, S. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0912417107 · Cited by 592 · see citing works
Strong, M. J., Grace, G. M., Freedman, M., Lomen-Hoerth, C., Woolley, S. C., Goldstein, L. H., Murphy, J. M., Shoesmith, C. L., Rosenfeld, J., Leigh, P. N., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802654364 · Cited by 543 · see citing works
Self-citationVance, C. A., Al‐Chalabi, A., Ruddy, D., Smith, B. N., Hu, X., Sreedharan, J., Siddique, T., Schelhaas, H. J., Küsters, B., Troost, D., et al. (2006) · Brain · doi:10.1093/brain/awl030 · Cited by 392 · see citing works
A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia
Morita, M., Al‐Chalabi, A., Andersen, P. M., Hosler, B. A., Sapp, P. C., Englund, E. M., Mitchell, J. E., Habgood, J., de Belleroche, J., Xi, J., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000200048.53766.b4 · Cited by 356 · see citing works
Millecamps, S., Salachas, F., Cazeneuve, C., Gordon, P., Bricka, B., Camuzat, A., Guillot‐Noël, L., Russaouen, O., Bruneteau, G., Pradat, P., et al. (2010) · Journal of Medical Genetics · doi:10.1136/jmg.2010.077180 · Cited by 308 · see citing works
Geser, F., Brandmeir, N. J., Kwong, L. K., Martinez‐Lage, M., Elman, L. B., McCluskey, L. F., Xie, S. X., Lee, V. M. ‐., & Trojanowski, J. Q. (2008) · Archives of Neurology · doi:10.1001/archneur.65.5.636 · Cited by 307 · see citing works
Andersen, P. M. (1997) · Brain · doi:10.1093/brain/120.10.1723 · Cited by 301 · see citing works
Hirano, A., Dembitzer, H. M., Kurland, L. T., & Zimmerman, H. M. (1968) · PubMed · Cited by 300 · see citing works
Lynch, T., Sano, M. C., Marder, K. S., Bell, K. L., Foster, N. L., Defending, R. F., Sima, A. A. F., Keohane, C., Nygaard, T. G., Fahn, S., et al. (1994) · Neurology · doi:10.1212/wnl.44.10.1878 · Cited by 287 · see citing works
Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis.
Massman, P. J., Sims, J., Cooke, N., Haverkamp, L. J., Appel, V., & Appel, S. H. (1996) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.61.5.450 · Cited by 251 · see citing works
Blair, I. P., Williams, K. L., Warraich, S. T., Durnall, J. C., Thoeng, A., Manavis, J., Blumbergs, P. C., Vucic, S., Kiernan, M. C., & Nicholson, G. A. (2009) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2009.194399 · Cited by 250 · see citing works
A prospective study of cognitive impairment in ALS
Strong, M. J., Grace, G. M., Orange, J. B., Leeper, H. A., Menon, R. S., & Aere, C. (1999) · Neurology · doi:10.1212/wnl.53.8.1665 · Cited by 247 · see citing works
Self-citationTHE FINE STRUCTURE OF SOME INTRAGANGLIONIC ALTERATIONS
HIRANO, A., Dembitzer, H. M., Kurland, L. T., & Zimmerman, H. M. (1968) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-196804000-00001 · Cited by 246 · see citing works
Wightman, G., Anderson, V., Martin, J., Swash, M., Anderton, B. H., Neary, D., Mann, D. M. A., Luthert, P. J., & Leigh, P. N. (1992) · Neuroscience Letters · doi:10.1016/0304-3940(92)90569-s · Cited by 233 · see citing works
Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex on Guam
Hirano, A. (1966) · Archives of Neurology · doi:10.1001/archneur.1966.00470130039004 · Cited by 226 · see citing works
Tsai, K., Yang, C.-H., Fang, Y.-H., Cho, K.-H., Chien, W., Wang, W., Wu, T., Lin, C., Fu, W., & Shen, C.-K. J. (2010) · The Journal of Experimental Medicine · doi:10.1084/jem.20092164 · Cited by 226 · see citing works
Moisse, K., Volkening, K., Leystra-Lantz, C., Welch, I., Hill, T., & Strong, M. J. (2009) · Brain Research · doi:10.1016/j.brainres.2008.10.021 · Cited by 210 · see citing works
Self-citationFrameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia
Yan, J., Deng, H., Siddique, N. A., Fecto, F., Chen, W., Yang, Y., Liu, E., Donkervoort, S. C., Zheng, J. G., Shi, Y., et al. (2010) · Neurology · doi:10.1212/wnl.0b013e3181f07e0c · Cited by 208 · see citing works
Transgenic Rat Model of Neurodegeneration Caused by Mutation in the TDP Gene
Zhou, H., Huang, C., Chen, H., Wang, D., Landel, C. P., Xia, P. Y., Bowser, R. P., Liu, Y.-J., & Xia, X. (2010) · PLoS Genetics · doi:10.1371/journal.pgen.1000887 · Cited by 200 · see citing works
Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family
Boxer, A. L., Mackenzie, I. R., Boeve, B. F., Baker, M., Seeley, W. W., Crook, R., Feldman, H., Hsiung, G. R., Rutherford, N. J., Laluz, V., et al. (2010) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2009.204081 · Cited by 194 · see citing works
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)
Strong, M. J. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2009.09.029 · Cited by 185 · see citing works
Self-citationTDP-43 in aging and Alzheimer's disease - a review.
Wilson, A. C., Dugger, B. N., Dickson, D. W., & Wang, D.-S. (2011) · PubMed Central · Cited by 165 · see citing works
Gitcho, M. A., Bigio, E. H., Mishra, M., Johnson, N., Weıntraub, S., Mesulam, M. M., Rademakers, R., Chakraverty, S., Cruchaga, C., Morris, J. C., et al. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0571-7 · Cited by 161 · see citing works
Distinct cerebral lesions in sporadic and ‘D90A’ SOD1 ALS: studies with [11C]flumazenil PET
Turner, M. R., Hammers, A., Al‐Chalabi, A., Shaw, C. E., Andersen, P. M., Brooks, D. J., & Leigh, P. N. (2005) · Brain · doi:10.1093/brain/awh509 · Cited by 160 · see citing works
Deficits in emotional and social cognition in amyotrophic lateral sclerosis.
Girardi, A., MacPherson, S. E., & Abrahams, S. (2010) · Neuropsychology · doi:10.1037/a0020357 · Cited by 154 · see citing works
The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis
Strong, M. J. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802372371 · Cited by 148 · see citing works
Self-citationThe cognitive profile of amyotrophic lateral sclerosis: A meta-analysis
Raaphorst, J., de Visser, M., Linssen, W. H. J. P., de Haan, R. J., & Schmand, B. A. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482960802645008 · Cited by 148 · see citing works
Beneficial vascular risk profile is associated with amyotrophic lateral sclerosis
Sutedja, N. A., van der Schouw, Y. T., Fischer, K., Sizoo, E. M., Huisman, M. H. B., Veldink, J. H., & van den Berg, L. H. (2011) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2010.236752 · Cited by 144 · see citing works
Neurobehavioral Features in Frontotemporal Dementia With Amyotrophic Lateral Sclerosis
Lillo, P., Garcin, B., Hornberger, M., Bak, T. H., & Hodges, J. R. (2010) · Archives of Neurology · doi:10.1001/archneurol.2010.146 · Cited by 143 · see citing works
Evidence for astrocytosis in ALS demonstrated by [11C](l)-deprenyl-D2 PET
Johansson, A., Engler, H., Blomquist, G., Scott, B., Wall, A. E., Aquilonius, S., Långström, B., & Askmark, H. (2007) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2007.01.057 · Cited by 136 · see citing works
Cognitive impairment in sporadic ALS
Wilson, C., Grace, G. M., Muñoz, D. G., et al. (2001) · Neurology · doi:10.1212/wnl.57.4.651 · Cited by 131 · see citing works
Association of apolipoprotein E ∈4 allele with bulbar-onset motor neuron disease
Al‐Chalabi, A., Bakker, M. C., Shaw, C. E., Lloyd, C., Enayat, Z. E., Leigh, P. N., Sham, P. C., Ball, D. M., & Powell, J. F. (1996) · The Lancet · doi:10.1016/s0140-6736(96)90343-8 · Cited by 114 · see citing works
Gellera, C., Colombrita, C., Ticozzi, N., Castellotti, B., Bragato, C., Ratti, A., Taroni, F., & Silani, V. (2007) · Neurogenetics · doi:10.1007/s10048-007-0111-3 · Cited by 113 · see citing works
Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review
Giordana, M. T., Ferrero, P., Grifoni, S., Pellerino, A., Naldi, A., & Montuschi, A. (2010) · Neurological Sciences · doi:10.1007/s10072-010-0439-6 · Cited by 108 · see citing works
SOD1 mutations in amyotrophic lateral sclerosis
Battistini, S., Giannini, F., Greco, G., Bibbò, G., Ferrera, L., Marini, V., Causarano, R., Casula, M., Lando, G., Patrosso, M. C., et al. (2005) · Journal of Neurology · doi:10.1007/s00415-005-0742-y · Cited by 107 · see citing works
[11C]-WAY100635 PET demonstrates marked 5-HT1A receptor changes in sporadic ALS
Turner, M. R. (2005) · Brain · doi:10.1093/brain/awh428 · Cited by 105 · see citing works
FUS Mutations in Frontotemporal Lobar Degeneration with Amyotrophic Lateral Sclerosis
Broustal, O., Camuzat, A., Guillot‐Noël, L., Guy, N., Millecamps, S., Deffond, D., Lacomblez, L., Golfier, V., Hannequin, D., Salachas, F., et al. (2010) · Journal of Alzheimer s Disease · doi:10.3233/jad-2010-100837 · Cited by 100 · see citing works
Association of APOE ε4 allele with survival in amyotrophic lateral sclerosis
Drory, V. E., Birnbaum, M., Korczyn, A. D., & Chapman, J. (2001) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(01)00569-x · Cited by 100 · see citing works
Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases
Donaghy, C. G., Thurtell, M. J., Pioro, E. P., Gibson, J. M., & Leigh, R. J. (2010) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2010.212407 · Cited by 95 · see citing works
The basic aspects of therapeutics in amyotrophic lateral sclerosis
Strong, M. J. (2003) · Pharmacology & Therapeutics · doi:10.1016/s0163-7258(03)00040-8 · Cited by 94 · see citing works
Self-citationSOD1 and cognitive dysfunction in familial amyotrophic lateral sclerosis
Wicks, P. J., Abrahams, S., Papps, B., Al‐Chalabi, A., Shaw, C. E., Leigh, P. N., & Goldstein, L. H. (2009) · Journal of Neurology · doi:10.1007/s00415-009-0078-0 · Cited by 91 · see citing works
Hirano, A. (1967) · Archives of Neurology · doi:10.1001/archneur.1967.00470220021003 · Cited by 90 · see citing works
Microtubule-associated tau protein positive neuronal and glial inclusions in ALS
Yang, W., Sopper, M. M., Leystra‐Lantz, C., et al. (2003) · Neurology · doi:10.1212/01.wnl.0000099372.75786.f8 · Cited by 83 · see citing works
Self-citationPathoanatomic Changes in Amyotrophic Lateral Sclerosis on Guam
Malamud, N. (1961) · Archives of Neurology · doi:10.1001/archneur.1961.00450160051003 · Cited by 83 · see citing works
Emotional Perception Deficits in Amyotrophic Lateral Sclerosis
Zimmerman, E. K., Eslinger, P. J., Simmons, Z., & Barrett, A. M. (2007) · Cognitive and Behavioral Neurology · doi:10.1097/wnn.0b013e31804c700b · Cited by 82 · see citing works
Yang, W., Leystra‐Lantz, C., & Strong, M. J. (2008) · Brain Research · doi:10.1016/j.brainres.2007.12.031 · Cited by 78 · see citing works
Self-citationA CASE OF ALS-FTD IN A LARGE FALS PEDIGREE WITH A K17I ANG MUTATION
van Es, M. A., Diekstra, F. P., Veldink, J. H., et al. (2009) · Neurology · doi:10.1212/01.wnl.0000339487.84908.00 · Cited by 76 · see citing works
Strong, M. J. (2001) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100001505 · Cited by 73 · see citing works
Self-citationStrong, M. J., & Gordon, P. H. (2005) · Amyotrophic Lateral Sclerosis · doi:10.1080/14660820410021267 · Cited by 71 · see citing works
Self-citationClinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation
Ito, H., Nakamura, M., Komure, O., Ayaki, T., Wate, R., Maruyama, H., Nakamura, Y., Fujita, K., Kaneko, S., Okamoto, Y., et al. (2011) · Acta Neuropathologica · doi:10.1007/s00401-011-0842-y · Cited by 61 · see citing works
Alzheimer disease pathology in amyotrophic lateral sclerosis
Bowser, R. P., & Hamilton, R. L. (2004) · Acta Neuropathologica · doi:10.1007/s00401-004-0843-1 · Cited by 61 · see citing works
Tau protein hyperphosphorylation in sporadic ALS with cognitive impairment
Strong, M. J., Yang, W., Strong, W., Leystra‐Lantz, C., Jaffe, H., & Pant, H. C. (2006) · Neurology · doi:10.1212/01.wnl.0000218161.15834.db · Cited by 58 · see citing works
Self-citationChanges in memory for emotional material in amyotrophic lateral sclerosis (ALS)
Papps, B., Abrahams, S., Wicks, P. J., Leigh, P. N., & Goldstein, L. H. (2005) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2004.11.027 · Cited by 58 · see citing works
Inferring thought and action in motor neurone disease
Gibbons, Z. C., Snowden, J. S., Thompson, J. C., Happe, F. G. E., Richardson, A. M. T., & Neary, D. (2006) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2006.10.008 · Cited by 54 · see citing works
Hulette, C. M., Pericak‐Vance, M. A., Roses, A. D., Schmechel, D. E., Yamaoka, L. H., Gaskell, P. C., Welsh-Bohmer, K. A., Crowther, R. A., & Spillantini, M. G. (1999) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199908000-00008 · Cited by 52 · see citing works
Tau protein aggregation in the frontal and entorhinal cortices as a function of aging
Yang, W., Ang, L. C., & Strong, M. J. (2005) · Developmental Brain Research · doi:10.1016/j.devbrainres.2005.02.004 · Cited by 48 · see citing works
Self-citationMasè, G., Ros, S., Gemma, A., et al. (2001) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(01)00625-6 · Cited by 45 · see citing works
Gohar, M., Yang, W., Strong, W., Volkening, K., Leystra‐Lantz, C., & Strong, M. J. (2008) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2008.05791.x · Cited by 38 · see citing works
Self-citationApolipoprotein E ɛ4 in bulbar-onset motor neuron disease
SMITH, R. G., Haverkamp, L. J., Case, S., Appel, V., & Appel, S. H. (1996) · The Lancet · doi:10.1016/s0140-6736(05)64502-3 · Cited by 38 · see citing works
Colombrita, C., Onesto, E., Tiloca, C., Ticozzi, N., Silani, V., & Ratti, A. (2011) · PubMed · doi:10.4449/aib.v149i1.1261 · Cited by 32 · see citing works
Neuropsychological Functioning in PLS: A Comparison with ALS
Grace, G. M., Orange, J. B., Rowe, A., et al. (2011) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100120803 · Cited by 29 · see citing works
Forno, L. S., Langston, J. W., Herrick, M. K., Wilson, J. D., & Murayama, S. (2002) · Acta Neuropathologica · doi:10.1007/s00401-001-0509-1 · Cited by 25 · see citing works
Martinaud, O., Laquerrière, A., Guyant‐Maréchal, L., et al. (2005) · Acta Neuropathologica · doi:10.1007/s00401-005-1028-2 · Cited by 23 · see citing works
Jellinger, K. A. (2005) · European Journal of Neurology · doi:10.1111/j.1468-1331.2004.01089.x · Cited by 23 · see citing works
Amyotrophic lateral sclerosis: contemporary concepts in etiopathogenesis and pharmacotherapy
Strong, M. J. (2004) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.13.12.1593 · Cited by 20 · see citing works
Self-citationGoetz, C. G., & Pappert, E. J. (1997) · Annals of Neurology · doi:10.1002/ana.410410325 · Cited by 20 · see citing works
Lack of association of apolipoprotein E ϵ4 allele with bulbar‐onset motor neuron disease
Bachus, R., Bader, S., Gessner, R. V., et al. (1997) · Annals of Neurology · doi:10.1002/ana.410410326 · Cited by 19 · see citing works
[Amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam].
Okumura, H., & Tashiro, K. (2004) · PubMed · Cited by 18 · see citing works
Cognition in amyotrophic lateral sclerosis
Murphy, J. M., Ahmed, F., & Lomen‐Hoerth, C. (2012) · Oxford University Press eBooks · doi:10.1093/med/9780199590674.003.0010 · Cited by 3 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
The Neuropathology of Chronic Traumatic Encephalopathy
McKee, A. C., Stein, T. D., Kiernan, P. T., & Alvarez, V. E. (2015) · Brain Pathology · doi:10.1111/bpa.12248 · Cited by 585 · see citing works
McKee, A. C., Daneshvar, D. H., Alvarez, V. E., & Stein, T. D. (2014) · Acta Neuropathologica · doi:10.1007/s00401-013-1230-6 · Cited by 422 · see citing works
Differential autophagy power in the spinal cord and muscle of transgenic ALS mice
Crippa, V., Boncoraglio, A., Galbiati, M., Aggarwal, T., Rusmini, P., Giorgetti, E., Cristofani, R., Carra, S., Pennuto, M., & Poletti, A. (2013) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2013.00234 · Cited by 63 · see citing works
- Authors
- Strong, M. J., & Yang, W.
- Affiliations
- Western University (CA)
- Corresponding author
- Strong, Michael Joseph
- Venue
- Journal of Molecular Neuroscience
- Published
- 2011-08-02
- Volume
- 45
- Issue
- 3
- Pages
- 648-655
- ISSN
- 0895-8696 (ISSN-L)
- 1559-1166
- OpenAlex
- W2055152437
- Cited by
- 41
- References
- 82
- Citations by year
- 2024: 2 · 2022: 1 · 2020: 1 · 2019: 5 · 2017: 3 · 2016: 6 · 2015: 8 · 2014: 4 · 2013: 5 · 2012: 6
- FWCI
- 3.01
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Journal of Molecular Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Keywords
- amyotrophic lateral sclerosis, frontotemporal lobar degeneration, TDP-43, tau hyperphosphorylation, frontotemporal dementia, dysexecutive syndrome, behavioral variant frontotemporal dementia, clinicopathological correlation
- MeSH terms
- Amyotrophic Lateral Sclerosis, DNA-Binding Proteins, Humans, Syndrome, tau Proteins, Frontotemporal Lobar Degeneration
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
