Motor neuron dysfunctions in the frontotemporal lobar degeneration spectrum: A clinical and neurophysiological study

Journal of the Neurological Sciences

Volumn 351, Issue 1-2, 2015, Pages 72-77

  Cerami, C ^a,b   Marcone, A ^b   Crespi, C ^a,b   Iannaccone, S ^b   Marangoni, C ^b   Dodich, A ^a,b   Giusti, M C ^b   Zamboni, M ^b   Golzi, V ^b   Cappa, S F ^b,c  

^a VITA SALUTE SAN RAFFAELE UNIVERSITY   (Italy)

^b SAN RAFFAELE HOSPITAL   (Italy)

^c SCUOLA UNIVERSITARIA SUPERIORE IUSS PAVIA   (Italy)

Author keywords

Amyotrophic lateral sclerosis; Electromyography; Frontotemporal dementia; Frontotemporal lobar degeneration; Motor neuron dysfunction; Pyramidal signs

Indexed keywords

ADULT; AGED; ALS FUNCTIONAL RATING SCALE REVISED; ARTICLE; C9ORF72 GENE; CLINICAL EXAMINATION; CORTICOBASAL DEGENERATION; DISEASE SEVERITY; ELECTROMYOGRAPHY; FEMALE; FRONTOTEMPORAL DEMENTIA; GENE MUTATION; GENETIC SCREENING; GRN GENE; HIGH RISK PATIENT; HUMAN; MAJOR CLINICAL STUDY; MALE; MAPT GENE; MIDDLE AGED; MOTONEURON; MOTOR NEURON DISEASE; NEUROPHYSIOLOGY; PHENOTYPE; PRIMARY PROGRESSIVE APHASIA; PRIORITY JOURNAL; PYRAMIDAL TRACT; RATING SCALE; UMN SCALE; AMYOTROPHIC LATERAL SCLEROSIS; COMORBIDITY; FOLLOW UP; FRONTOTEMPORAL LOBAR DEGENERATION; PATHOPHYSIOLOGY;

AGED; AMYOTROPHIC LATERAL SCLEROSIS; COMORBIDITY; FEMALE; FOLLOW-UP STUDIES; FRONTOTEMPORAL LOBAR DEGENERATION; HUMANS; MALE; MIDDLE AGED; MOTOR NEURON DISEASE;

EID: 84926407860     PISSN: 0022510X     EISSN: 18785883     Source Type: Journal    
DOI: 10.1016/j.jns.2015.02.039     Document Type: Article

References (41)

1. H.R. Morris, A.J. Waite, N.M. Williams, J.W. Neal, and D.J. Blake Recent advances in the genetics of the ALS-FTLD complex Curr Neurol Neurosci Rep 12 3 Jun 2012 243 250
2. T. Van Langenhove, J. van der Zee, and C. Van Broeckhoven The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum Ann Med 44 8 Dec 2012 817 828
3. C. Lomen-Hoerth Clinical phenomenology and neuroimaging correlates in ALS-FTD J Mol Neurosci 45 3 Nov 2011 656 662
4. R. Ferrari, D. Kapogiannis, E.D. Huey, and P. Momeni FTD and ALS: a tale of two diseases Curr Alzheimer Res 8 3 May 2011 273 294
5. M. Neumann, D.M. Sampathu, and L.K. Kwong Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Science 314 2006 130 133
6. L.K. Kwong, M. Neumann, D.M. Sampathu, V.M. Lee, and J.Q. Trojanowski TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease Acta Neuropathol 114 2007 63 70
7. K.A. Josephs, R.C. Petersen, and D.S. Knopman Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP Neurology 66 1 Jan 10 2006 41 48
8. A.E. Brenton, E. Majounie, and A. Waite A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD Neuron 72 2 Oct 20 2011 257 268
9. M. DeJesus-Hernandez, I.R. Mackenzie, and B.F. Boeve Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS Neuron 72 2 Oct 20 2011 245 256
10. P. Lillo, and J.R. Hodges Frontotemporal dementia and motor neuron disease: overlapping clinic-pathological disorders J Clin Neurosci 16 9 Sep 2009 1131 1135
11. P. Lillo, S. Savage, E. Mioshi, M.C. Kiernan, and J.R. Hodges Amyotrophic lateral sclerosis and frontotemporal dementia: a behavioural and cognitive continuum Amyotroph Lateral Scler 13 1 2012 Jan 102 109
12. J.M. Murphy, R.G. Henry, S. Langmore, J.H. Kramer, B.L. Miller, and C. Lomen-Hoerth Continuum of frontal lobe impairment in amyotrophic lateral sclerosis Arch Neurol 64 4 Apr 2007 530 534
13. S. Abrahams, P.N. Leigh, A. Harvey, G.N. Vythelingum, D. Grisé, and L.H. Goldstein Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS) Neuropsychologia 38 6 2000 734 747
14. M. Consonni, S. Iannaccone, and C. Cerami The cognitive and behavioural profile of amyotrophic lateral sclerosis: application of the consensus criteria Behav Neurol 27 2 2013 143 153
15. J. Phukan, M. Elamin, and P. Bede The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study J Neurol Neurosurg Psychiatry 83 1 Jan 2012 102 108
16. C. Cerami, A. Dodich, and N. Canessa Emotional empathy in amyotrophic lateral sclerosis: a behavioural and voxel-based morphometry study Amyotroph Lateral Scler Frontotemporal Degener 15 1-2 Mar 2014 21 29
17. C. Lomen-Hoerth, J. Murphy, S. Langmore, J.H. Kramer, R.K. Olney, and B. Miller Are amyotrophic lateral sclerosis patients cognitively normal? Neurology 60 2003 1094 1097
18. C. Lomen-Hoerth, T. Anderson, and B. Miller The overlap of amyotrophic lateral sclerosis and frontotemporal dementia Neurology 59 2002 1077 1079
19. J.R. Hodges, R. Davies, J. Xuereb, J. Kril, and G. Halliday Survival in frontotemporal dementia Neurology 61 2003 349 354
20. E.D. Roberson, J.H. Hesse, and K.D. Rose Frontotemporal dementia progresses to death faster than Alzheimer disease Neurology 65 5 Sep 13 2005 719 725
21. Y. Mitsuyama, and S. Takamiya Presenile dementia with motor neuron disease in Japan. A new entity? Arch Neurol 36 1979 592 593
22. J.R. Burrell, M.C. Kiernan, S. Vucic, and J.R. Hodges Motor neuron dysfunction in frontotemporal dementia Brain 134 Pt 9 Sep 2011 2582 2594
23. T.H. Bak, and J.R. Hodges Motor neuron disease, dementia and aphasia: coincidence, co-occurrence or continuum? J Neurol 248 2001 260 270
24. T.H. Bak, and J.R. Hodges The effects of motor neuron disease on language: further evidence Brain Lang 89 2004 354 361
25. M. de Carvalho, R. Dengler, and A. Eisen Electrodiagnostic criteria for diagnosis of ALS Clin Neurophysiol 119 3 Mar 2008 497 503
26. P. Ekman, and W. Friesen Pictures of facial affect 1976 Consulting Psychologists Press Palo Alto
27. A. Dodich, C. Cerami, and N. Canessa Emotion recognition from facial expressions: a normative study of the Ekman 60-Faces Test in the Italian population Neurol Sci 35 7 Jul 2014 1015 1021
28. C. Cerami, A. Dodich, and N. Canessa Neural correlates of empathic impairment in the behavioral variant of frontotemporal dementia Alzheimers Dement 10 6 Nov 2014 827 834
29. M.H. Davis Measuring individual differences in empathy: evidence for a multidimensional approach J Pers Soc Psychol 44 1983 113 126
30. C. Cerami, A. Marcone, D. Galimberti, C. Villa, E. Scarpini, and S.F. Cappa From genotype to phenotype: two cases of genetic frontotemporal lobar degeneration with premorbid bipolar disorder J Alzheimers Dis 27 4 2011 791 797
31. C. Cerami, A. Marcone, and D. Galimberti Novel evidence of phenotypical variability in the hexanucleotide repeat expansion in chromosome 9 J Alzheimers Dis 35 3 Jan 1 2013 455 462
32. K. Rascovsky, J.R. Hodges, and D. Knopman Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Brain 134 Pt 9 2011 2456 2477
33. M.L. Gorno-Tempini, A.E. Hillis, and S. Weintraub Classification of primary progressive aphasia and its variants Neurology 76 2011 1006 1014
34. M.J. Armstrong, I. Litvan, and A.E. Lang Criteria for the diagnosis of corticobasal degeneration Neurology 80 5 2013 496 503
35. C.M. Ellis, A. Simmons, and D.K. Jones Diffusion tensor MRI assesses corticospinal tract damage in ALS Neurology 53 5 Sep 22 1999 1051 1058
36. J.M. Cedarbaum, N. Stambler, and E. Malta The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III) J Neurol Sci 169 1-2 Oct 31 1999 13 21
37. B.R. Brooks, R.G. Miller, M. Swash, and T.L. Munsat World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis Amyotroph Lateral Scler Other Motor Neuron Disord 1 5 Dec 2000 293 299
38. C. Cerami, A. Marcone, and D. Galimberti Novel missense progranulin gene mutation associated with the semantic variant of primary progressive aphasia J Alzheimers Dis 36 3 Jan 1 2013 415 420
39. R. Ferrari, D.G. Hernandez, and M.A. Nalls Frontotemporal dementia and its subtypes: a genome-wide association study Lancet Neurol 13 7 Jul 2014 686 699
40. K. Tsuchiya, S. Murayama, and K. Mitani Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases Acta Neuropathol 109 4 Apr 2005 353 366
41. K.A. Josephs, O. Katsuse, and D.A. Beccano-Kelly Atypical progressive supranuclear palsy with corticospinal tract degeneration J Neuropathol Exp Neurol 65 4 Apr 2006 396 405