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Table E-1. Key Words Used in the Literature Search FTD

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Table E-1. Key Words Used in the Literature Search FTD Table e-1. Key words used in the literature search FTD, frontotemporal dementia, FTLD, OR amyotrophic lateral sclerosis, ALS, motor pick’s disease, frontal variant FTD, neuron(e) disease, MND, Gehrig* frontal variant dementia, bv-FTD, f-FTD, frontotemporal (lobar) degeneration, presenile dementia AND cognition, mild cognitive impairment, cognition disorders, language disorders, (neuro)psychological tests, language (tests), memory, MMSE, Mini Mental State Examination, theory of mind, Frontal Assessment Battery, Boston Naming, Wisconsin Card Sorting Test, Stroop test, trail making test, aptitude tests Table e-2. Overview of tests used in the cognitive domains. Cognitive domain Cognitive skills tested within the cognitive domain Frequently used neuropsychological tests within the cognitive domain Social cognition Social perception, recognition of expressed emotions, Ekman 60 faces test, faux pas test, reading the mind in the eyes test, understanding social causality and theory of mind theory of mind, The Awareness of Social Inference Test Verbal memory Recall of unassociated words and short stories Auditory verbal learning test, logical memory test, recognition memory test – words, free and cued selective reminding test Fluency Mental lexicon and executive control Letter fluency, category fluency, design fluency Executive functions Mental flexibility, task-switching, strategic planning and Trail making test part B, stroop test part B, Iowa gambling test, suppression ability Wisconsin card sorting test, hayling sentence completion test, brixton test Visual memory Visuospatial memory and visual recognition of objects and faces Rey complex figure test part B, face recognition task, doors test, recognition memory test – faces Visuoconstruction Ability to copy complex figures Rey complex figure test part A Language Naming and picture and word comprehension Boston naming test, peabody picture vocabulary test, piramids and palm trees test, token test, western aphasia battery, Sydney language battery, British picture vocabulary scale Attention Verbal and visual working memory and associability Digit span forward, digit span backward, visual span, letter number sequencing test Psychomotor speed Processing speed and mental flexibility Trail making test part A, stroop test part A, symbol digit substitution test Visuoperception Object and space perception Judgement of line orientation test, visual object and space perception battery Legend. Only frequently used tests are included in this table, i.e. tests used in three or more studies Table e-3. Characteristics of the included articles Author, year Newcastle Ottawa scale Participants Age of Disease Years of Disease (p/c) patients duration education severity Selection Comparability Exposure Irish 2013(1) 4 2 1 10/10 62.8 4.8 11.8 81.1# Cruz de Souza 2010(2) 3 2 1 17/17 71.1 - 5.2 - Chiong 2013(3) 2 2 1 10/16 61.2 - 16.6 1.1* 6.1§ Kumfor 2013(4) 3 2 1 11/15 66.7 6.1 11.2 77.7# Diehl-Schmid 2007(5) 3 2 1 25/33 63.2 - 12.8 - Fine 2009(6) 2 2 1 25/9 58.6 - 16.5 - O’Callaghan 2013(7) 3 2 1 11/15 63.1 1.0 12.2 - Carey 2008(8) 2 2 1 44/ 27 61.5 - 15.3 - Banks 2008(9) 2 2 1 11/15 63.8 5.6 15.9 - Irish 2013(10) 1 2 1 15/15 63.9 3.0 12.3 78.5# Ash 2009(11) 2 2 1 12/10 64.8 4.1 16.0 - Couto 2013(12) 3 2 1 12/18 69.8 - 16.0 - Cosentino 2006(13) 2 2 1 12/15 67.4 6.0 15.8 - Bertoux 2013(14) 3 2 1 20/30 69.2 3.3 9.9 - Bertoux 2012(15) 2 2 1 37/30 65.0 - 10.6 126.5$ Collette 2007(16) 2 2 1 13/28 65.7 - - 127.3$ Collette 2010(17) 4 2 1 12/20 67.5 - 11.6 118.7$ Fernandez-Duque 2 2 1 6/10 63.7 - 16.5 125.7$ 2005(18) Fernandez-Duque 2 2 1 10/14 61,0 - 16.3 - 2007(19) Fernandez-Duque 2 2 1 11/12 60.6 3.4 16.1 126.4$ 2009(20) Fernandez-Duque 2 2 1 9/10 62.3 - 16.2 126.0$ 2010(21) Filippi 2013(22) 4 2 1 12/30 59.0 2.7 11.0 4.8§ Downey 2013(23) 3 2 1 20/20 64,0 5.5 13.0 - Funkiewiez 2012(24) 3 2 1 22/30 65.5 - 5.3 131.4$ Gleichgerrcht 2 2 1 25/26 70.0 - 2011(25) 15.9 0.8* 80.1# Gleichgerrcht 4 2 1 35/14 68.5 - 2012(26) 13.6 81.9# Gregory 2002(27) 4 2 1 19/16 58.6 - 11.6 81.4# Hodges 1999(28) 3 2 1 9/9 57.0 - 10.0 - Hornberger 2011(29) 3 2 1 14/18 59.3 3.7 11.8 75.6# Hsieh 2012(30) 3 2 1 8/15 62.9 - 12.3 1.4* 79.6# Irish 2011(31) 3 2 1 15/19 61.6 3.2 12.3 78.1# Isella 2008(32) 3 2 1 18/40 65.1 - 7.5 1.1* Johns 2009(33) 4 2 1 17/20 66.6 - 11.5 - Johnson 2011(34) 3 2 1 11/17 59.8 - 16.2 6.1§ Kloeters 2013(35) 3 2 1 18/28 60.8 - 11.6 80.1# Kugo 2007(36) 3 2 1 23/25 64.7 5.0 11.2 1.3* Kumfor 2011(37) 3 2 1 16/37 61.5 3.5 12.1 82.8# Laisney 2009(38) 2 2 1 18/18 67.2 2.7 11.2 - Le Bouc 2012(39) 2 2 1 11/20 58.7 - 11.5 - Lough 2006(40) 2 2 1 31/13 61.1 - - 86.5# Manes 2011(41) 2 2 1 43/14 68.8 - 13.6 0.7* 78.9# Matuszewski 2006(42) 2 2 1 20/21 67.9 - - - McKinnon 2008(43) 2 2 1 8/16 59.0 3.0 15.5 - Mendez 1998(44) 3 2 1 31/31 65.6 2.7 14.1 1.4* Miller 2012(45) 3 2 1 17/36 61.0 3.7 12.0 86.0# 0.7* Omar 2011(46) 2 2 1 16/21 64.7 6.9 14.1 - Omar 2013(47) 3 2 1 12/17 66.1 - - - Pachana 1996(48) 2 2 1 15/16 63.9 - 15.2 - Peelle 2007(49) 2 2 1 7/20 60.0 - 14.6 - Pennington 2011(50) 2 2 1 14/15 59.7 3.4 10.5 - Possin 2012(51) 3 2 1 32/37 59.4 - 16.6 - Possin 2011(52) 4 2 1 48/94 61.8 - 16.6 - Rahman 1999(53) 3 2 1 8/8 57.9 3.4 - - Rankin 2009(54) 3 2 1 20/13 60.0 - 16.7 1.1* 6.6§ Ricci 2012(55) 3 2 1 15/28 65.7 - 10.3 - Ricci 2012(55) 3 2 1 11/15 59.8 - 12.6 - Savage 2014(56) 2 2 1 25/30 62.5 - 11.8 77.5# Silveri 2003(57) 2 2 1 17/34 72.1 - 8.9 - Souchay 2003(58) 3 2 1 6/16 57.7 - 9.8 - Stopford 2012(59) 4 2 1 26/26 64.0 6.0 - 1.0* Torralva 2007(60) 3 2 1 20/10 67.2 - 12.8 85.6# Torralva 2009(61) 3 2 1 35/14 67.2 - 13.6 81.9# Viskontas 2011(62) 2 2 1 12/21 61.2 - 15.3 0.9* 5.5§ Wicklund 2004(63) 3 2 1 16/20 65.1 4.4 15.7 - Baez 2014(64) 2 2 1 37/30 66.0 - 13.7 - Bertoux 2014(65) 3 2 1 44/22 67.0 - 11.0 - Hsieh 2013(66) 3 2 1 9/15 62.5 - 10.9 4.9§ Irish 2014(67) 2 2 1 19/19 63.6 3.9 12.6 77.5# Irish 2014(68) 3 2 1 10/14 63.6 5.0 10.9 77.2# Kamminga 2014(69) 2 2 1 8/11 64.1 5.1 11.6 83.5# Kumfor 2014(70) 2 2 1 20/24 66.6 5.2 12.2 79.7# Lagarde 2013(71) 2 2 1 16/18 69.3 5.3 11.8 124.7$ Lemos 2014(72) 4 2 1 32/32 68.6 - 7.0 1.0* Russo 2014(73) 3 2 1 27/40 66.5 3.3 13.6 6.2§ Ash 2016(74) 3 2 1 54/27 63.7 4.2 15.7 - Baez 2016(75) 2 2 1 26/23 66.1 - 15.2 - Baez 2017(76) 2 2 1 16/22 65.8 - 14.8 - Balconi 2015(77) 2 2 1 16/20 65.6 - 7.0 - Bertoux 2016(78) 2 2 1 71/60 65.9 3.3 11.4 - Bertoux 2015(79) 2 2 1 60/30 66.1 3.2 10.3 - Brioschi 2015(80) 2 2 1 28/18 59.2 - 15.9 - Cavedo 2014(81) 3 2 1 16/22 69.0 - 12.0 - Cerciello 2017(82) 2 2 1 9/20 72.3 - 9.5 - Chiong 2016(83) 2 2 1 25/58 64.0 - 16.9 7.0§ Clark 2015(84) 3 2 1 22/21 67.0 9 13.9 - Clark 2015(85) 2 2 1 15/21 65.0 6.3 17.0 - Cohen 2016(86) 2 2 1 11/39 68.0 8.5 10.2 - Consonni 2017(87) 2 2 1 15/48 64.4 3.5 - - Cook 2014(88) 2 2 1 33/15 62.0 5 16.0 - 2 1 0.8* 34/25 67.1 - Custodio 2016(89) 3 11.7 76.6# Dermody 2016(90) 3 2 1 24/22 63.0 3.5 12.4 74.2# Dermody 2015(91) 4 2 1 12/12 63.2 5.1 11.6 78.8# Fernandez 2017(92) 3 2 1 26/24 71.3 3.9 8.0 70.4# Flanagan 2016(93) 3 2 1 39/61 60.6 3.5 12.3 - Fletcher 2015(94) 2 2 1 16/26 66.0 8.3 14.6 - Hafkemeijer 2017(95) 2 2 1 12/22 64.7 5.0 - 1.3* Hardy 2016(96) 2 2 1 24/24 64.4 7.8 14.8 - Hutchings 2015(97) 2 2 1 16/17 64.1 5.1 11.8 - Irish 2016(98) 3 2 1 15/20 63.5 3.8 11.6 79.4# Johnen 2016(99) 4 2 1 24/35 64.9 2.7 11.2 - Kamminga 2015(100) 3 2 1 19/20 60.5 4.3 11.3 79.0# Keri 2014(101) 4 2 1 16/20 58.9 - 11.7 - # Kumfor 2015(102) 4 2 1 13/11 62.5 5.5 12.0 77.0 Kumfor 2016(103) 3 2 1 13/16 60.2 4.3 11.8 77.5# Lagarde 2015(104) 3 2 1 18/18 69.7 5.4 12.0 126.3$ Leslie 2016(105) 4 2 1 14/33 62.7 4.8 12.2 68.2# Lin 2016(106) 2 2 1 15/15 62.1 5.1 11.4 - Mahoney 2015(107) 3 2 1 23/18 63.8 - 15.5 - Mandelli 2016(108) 3 2 1 23/34 62.9 - 16.1 - Metzger 2016(109) 3 2 1 8/8 67.6 - - - O'Callaghan 2 1 22/22 64.8 2.3 2016(110) 3 12.0 - Primativo 2017(111) 2 2 1 12/38 67.7 9.2 14.0 - Ramanan 2015(112) 4 2 1 17/35 67.0 3.0 15.8 1.37* 62.3# Santamaria-Garcia 2 2 1 20/20 58.9 3.1 14.8 - 2017(113) Scherling 2017(114) 3 2 1 17/35 62.1 - 16.5 6.4§ Sedeno 2016(115) 2 2 1 14/12 66.4 - 14.7 - Tan 2015(116) 3 2 1 23/15 62.0 4.0 12.0 74.0# Torralva 2015(117) 4 2 1 40/18 67.2 - 15.0 70.4# Tu 2015(118) 3 2 1 24/23 64.7 6.6 11.8 82.1# Wong 2014(119) 3 2 1 22/35 61.2 3.8 11.3 7.1§ 76.0# Wong 2016(120) 3 2 1 5.6§ 22/38 61.0 3.6 11.8 76.3# Legend.
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