The frontotemporal syndrome of ALS is associated with poor survival

Journal of Neurology

Volumn 263, Issue 12, 2016, Pages 2476-2483

  Govaarts, Rosanne ^a   Beeldman, Emma ^a   Kampelmacher, Mike J ^b   van Tol, Marie Jose ^c   van den Berg, Leonard H ^d   van der Kooi, Anneke J ^a   Wijkstra, Peter J ^c   Zijnen Suyker, Marianne ^e   Cobben, Nicolle A M ^f   Schmand, Ben A ^a   de Haan, Rob J ^a   de Visser, Marianne ^a   Raaphorst, Joost ^a,g  

^a UNIVERSITY OF AMSTERDAM   (Netherlands)

^b UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^c UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

^d UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^e ERASMUS MEDICAL CENTER   (Netherlands)

^f MAASTRICHT UNIVERSITY MEDICAL CENTER   (Netherlands)

^g RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Amyotrophic lateral sclerosis; Behavioral changes; Cognitive impairment; Frontotemporal syndrome; Non invasive ventilation; Survival

Indexed keywords

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; BEHAVIOR CHANGE; CLINICAL ARTICLE; COGNITION; COGNITIVE DEFECT; DISEASE DURATION; EXECUTIVE FUNCTION; FEMALE; FRONTOTEMPORAL SYNDROME; HOSPITAL ANXIETY AND DEPRESSION SCALE; HUMAN; LANGUAGE; MALE; MEDIAN SURVIVAL TIME; MEMORY; MINI MENTAL STATE EXAMINATION; NEUROPSYCHOLOGICAL TEST; NONINVASIVE VENTILATION; ONSET AGE; PATIENT CODING; PHYSICAL DISABILITY; PRIORITY JOURNAL; RISK FACTOR; SURVIVAL; VERY ELDERLY; VITAL CAPACITY; COGNITION DISORDERS; COHORT ANALYSIS; COMPLICATION; FRONTOTEMPORAL DEMENTIA; GENETICS; MENTAL DISORDERS; MIDDLE AGED; MORTALITY; PROCEDURES; PSYCHOLOGICAL RATING SCALE; QUESTIONNAIRE; SURVIVAL ANALYSIS; TRINUCLEOTIDE REPEAT;

C9ORF72 PROTEIN, HUMAN; PROTEIN;

ADULT; AGED; AGED, 80 AND OVER; AMYOTROPHIC LATERAL SCLEROSIS; COGNITION DISORDERS; COHORT STUDIES; FEMALE; FRONTOTEMPORAL DEMENTIA; HUMANS; MALE; MENTAL DISORDERS; MIDDLE AGED; NEUROPSYCHOLOGICAL TESTS; NONINVASIVE VENTILATION; PROTEINS; PSYCHIATRIC STATUS RATING SCALES; SURVEYS AND QUESTIONNAIRES; SURVIVAL ANALYSIS; TRINUCLEOTIDE REPEATS;

EID: 84991387576     PISSN: 03405354     EISSN: 14321459     Source Type: Journal    
DOI: 10.1007/s00415-016-8290-1     Document Type: Article

References (39)

1. Abdulla S, Machts J, Kaufmann J, Patrick K, Kollewe K, Dengler R, Heinze HJ, Petri S, Vielhaber S, Nestor PJ (2014) Hippocampal degeneration in patients with amyotrophic lateral sclerosis. Neurobiol Aging 35:2639–2645
2. Beeldman E, Jaeger B, Raaphorst J, Seelen M, Veldink J, van den Berg L, de Visser M, Schmand B (2014) The verbal fluency index: Dutch normative data for cognitive testing in ALS. Amyotroph Later Scler Frontotemp Degener 15:388–391
3. Beeldman E, Raaphorst J, Klein Twennaar M, de Visser M, Schmand BA, de Haan RJ (2015) The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry 87:611–619
4. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ (2006) Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 5:140–147
5. Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Later Scler 1:293–299
6. Byrne S, Elamin M, Bede P, Shatunov A, Walsh C, Corr B, Heverin M, Jordan N, Kenna K, Lynch C, McLaughlin RL, Iyer PM, O’Brien C, Phukan J, Wynne B, Bokde AL, Bradley DG, Pender N, Al-Chalabi A, Hardiman O (2012) Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol 11:232–240
7. Caga J, Turner MR, Hsieh S, Ahmed RM, Devenney E, Ramsey E, Zoing MC, Mioshi E, Kiernan MC (2016) Apathy is associated with poor prognosis in amyotrophic lateral sclerosis. Eur J Neurol Off J Eur Fed Neurol Soc 23:891–897
8. Cedarbaum JM, Stambler N (1997) Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials. J Neurol Sci 152(Suppl 1):S1–S9
9. Chio A, Calvo A, Ghiglione P, Mazzini L, Mutani R, Mora G (2010) Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry 81:1141–1143
10. Chio A, Ilardi A, Cammarosano S, Moglia C, Montuschi A, Calvo A (2012) Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV. Neurology 78:1085–1089
11. Chio A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, Traynor BG (2009) Prognostic factors in ALS: a critical review. Amyotroph Later Scler 10:310–323
12. Creemers H, Grupstra H, Nollet F, van den Berg LH, Beelen A (2015) Prognostic factors for the course of functional status of patients with ALS: a systematic review. J Neurol 262:1407–1423
13. Cummings JL, Mega M, Gray K, Rosenberg-Thompson S, Carusi DA, Gornbein J (1994) The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology 44:2308–2314
14. Database municipal personal records (2015) http://www.government.nl/issues/identification-documents/the-municipal-personal-records-database. Accessed 24 Feb 2015
15. Elamin M, Phukan J, Bede P, Jordan N, Byrne S, Pender N, Hardiman O (2011) Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology 76:1263–1269
16. Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198
17. Goldstein LH, Abrahams S (2013) Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol 12:368–380
18. Hu WT, Shelnutt M, Wilson A, Yarab N, Kelly C, Grossman M, Libon DJ, Khan J, Lah JJ, Levey AI, Glass J (2013) Behavior matters—cognitive predictors of survival in amyotrophic lateral sclerosis. PLoS One 8:e57584
19. Huisman MH, de Jong SW, van Doormaal PT, Weinreich SS, Schelhaas HJ, van der Kooi AJ, de Visser M, Veldink JH, van den Berg LH (2011) Population based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology. J Neurol Neurosurg Psychiatry 82:1165–1170
20. Lillo P, Mioshi E, Zoing MC, Kiernan MC, Hodges JR (2011) How common are behavioural changes in amyotrophic lateral sclerosis? Amyotroph Later Scler 12:45–51
21. Martin NH, Lawrence V, Murray J, Janssen A, Higginson I, Lyall R, Burman R, Leigh PN, Al-Chalabi A, Goldstein LH (2016) Decision making about gastrostomy and noninvasive ventilation in amyotrophic lateral sclerosis. Qual Health Res 26:1366–1381
22. Merrilees J, Klapper J, Murphy J, Lomen-Hoerth C, Miller BL (2010) Cognitive and behavioral challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis. Amyotroph Later Scler 11:298–302
23. Mioshi E, Caga J, Lillo P, Hsieh S, Ramsey E, Devenney E, Hornberger M, Hodges JR, Kiernan MC (2014) Neuropsychiatric changes precede classic motor symptoms in ALS and do not affect survival. Neurology 82:149–155
24. Montuschi A, Iazzolino B, Calvo A, Moglia C, Lopiano L, Restagno G, Brunetti M, Ossola I, Lo Presti A, Cammarosano S, Canosa A, Chio A (2015) Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy. J Neurol Neurosurg Psychiatry 86:168–173
25. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554
26. Olney RK, Murphy J, Forshew D, Garwood E, Miller BL, Langmore S, Kohn MA, Lomen-Hoerth C (2005) The effects of executive and behavioral dysfunction on the course of ALS. Neurology 65:1774–1777
27. Phukan J, Pender NP, Hardiman O (2007) Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol 6:994–1003
28. Raaphorst J, Beeldman E, de Visser M, de Haan RJ, Schmand B (2012) A systematic review of behavioural changes in motor neuron disease. Amyotroph Later Scler 13:493–501
29. Raaphorst J, Beeldman E, Schmand B, Berkhout J, Linssen WH, Van den Berg LH, Pijnenburg YA, Grupstra HF, Weikamp JG, Schelhaas HJ, Papma JM, van Swieten JC, de VM, de Haan RJ (2012) The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS. Neurology 79:1377–1383
30. Raaphorst J, de Visser M, van Tol MJ, Linssen WH, van der Kooi AJ, de Haan RJ, Van den Berg LH, Schmand B (2011) Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy. J Neurol Neurosurg Psychiatry 82:170–175
31. Raaphorst J, Tuijp J, Verweij L, Westermann EJ, van der Kooi AJ, Gaytant MA, van den Berg LH, de Visser M, Kampelmacher MJ (2013) Treatment of respiratory impairment in patients with motor neuron disease in the Netherlands: patient preference and timing of referral. Eur J Neurol 20:1524–1530
32. Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, van Swieten JC, Seelaar H, Dopper EG, Onyike CU, Hillis AE, Josephs KA, Boeve BF, Kertesz A, Seeley WW, Rankin KP, Johnson JK, Gorno-Tempini ML, Rosen H, Prioleau-Latham CE, Lee A, Kipps CM, Lillo P, Piguet O, Rohrer JD, Rossor MN, Warren JD, Fox NC, Galasko D, Salmon DP, Black SE, Mesulam M, Weintraub S, Dickerson BC, Diehl-Schmid J, Pasquier F, Deramecourt V, Lebert F, Pijnenburg Y, Chow TW, Manes F, Grafman J, Cappa SF, Freedman M, Grossman M, Miller BL (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134:2456–2477
33. Schmand B, Groenink SC, van den Dungen M (2008) Letter fluency: psychometric properties and Dutch normative data. Tijdschr Gerontol Geriatr 39:64–76
34. Shoesmith CL, Findlater K, Rowe A, Strong MJ (2007) Prognosis of amyotrophic lateral sclerosis with respiratory onset. J Neurol Neurosurg Psychiatry 78:629–631
35. Strong MJ, Grace GM, Freedman M, Lomen-Hoerth C, Woolley S, Goldstein LH, Murphy J, Shoesmith C, Rosenfeld J, Leigh PN, Bruijn L, Ince P, Figlewicz D (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Later Scler 10:131–146
36. Taylor LJ, Brown RG, Tsermentseli S, Al-Chalabi A, Shaw CE, Ellis CM, Leigh PN, Goldstein LH (2013) Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis? J Neurol Neurosurg Psychiatry 84:494–498
37. van Rheenen W, van Blitterswijk M, Huisman MH, Vlam L, van Doormaal PT, Seelen M, Medic J, Dooijes D, de Visser M, van der Kooi AJ, Raaphorst J, Schelhaas HJ, van der Pol WL, Veldink JH, van den Berg LH (2012) Hexanucleotide repeat expansions in C9ORF72 in the spectrum of motor neuron diseases. Neurology 79:878–882
38. Woolley SC, Katz JS (2008) Cognitive and behavioral impairment in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am 19:607–617
39. Zigmond AS, Snaith RP (1983) The hospital anxiety and depression scale. Acta Psychiatr Scand 67:361–370