Language in corticobasal syndrome: a systematic review

A search was performed in the Medline/PubMed database, according to the PRISMA criteria, using the keywords “corticobasal syndrome” OR “corticobasal degeneration” AND “language”. Articles on CBS covering language assessment that were written in English were included, with no constraints on the publication date.

Results:

A total of 259 articles were found and 35 were analyzed, consisting of 531 participants. Twenty-eight studies showed heterogeneous language deficits and seven mentioned nonfluent primary progressive aphasia. The most used tests were the Western Aphasia Battery (8 studies) and the Boston Naming Test (8 studies).

Conclusion:

It was not possible to identify a unique linguistic profile in CBS.

Keywords:
corticobasal syndrome; language; neurocognitive disorders; language tests

Authors, year of publication	Sample size	Gender (male/female)	Age (years)	Disease duration (years)
Kertesz et al., 2000²2. Kertesz A, Martinez-Lage P, Davidson W, Munoz D. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology. 2000;55(9):1368-75. https://doi.org/10.1212/wnl.55.9.1368 https://doi.org/10.1212/wnl.55.9.1368...	35	movement disorder=5/10 cognitive disorder=14/6	movement disorder=61.9 cognitive disorder=63.6	movement disorder=5.4 cognitive disorder=7.1
Frattali et al., 2000⁴²42. Frattali CM, Grafman J, Patronas N, Makhlouf F, Litvan I. Language disturbances in corticobasal degeneration. Neurology. 2000;54(4):990-2. https://doi.org/https://doi.org/10.1212/WNL.54.4.990 https://doi.org/https://doi.org/10.1212/...	15	8/7	67.7	4.5
Graham et al., 2003⁵⁰50. Graham NL, Bak T, Patterson K, Hodges JR. Language function and dysfunction in corticobasal degeneration. Neurology. 2003;61(4):493-9. https://doi.org/10.1212/01.wnl.0000081230.09863.ed https://doi.org/10.1212/01.wnl.000008123...	10	7/3	67.6	3.35
Frattali et al., 2003²⁶26. Frattali CM, Duffy JR, Litvan I, Patsalides AD, Grafman J. Yes/no reversals as neurobehavioral sequela: A disorder of language, praxis, or inhibitory control? Eur J Neurol. 2003;10(1):103-6. https://doi.org/10.1046/j.1468-1331.2003.00545.x https://doi.org/10.1046/j.1468-1331.2003...	prospective study=34 retrospective study=9	prospective study=18/16 retrospective study=4/5	prospective study=67.91 retrospective study=71.3	prospective study=3.8 retrospective study=2.78
Gorno-Tempini et al., 2004²⁰20. Gorno-Tempini M-L, Murray RC, Rankin KP, Weiner MW, Miller BL. Clinical, cognitive and anatomical evolution from nonfluent progressive aphasia to corticobasal syndrome: a Case Report. Neurocase. 2004;10(6):426-36. https://doi.org/10.1080/13554790490894011 https://doi.org/10.1080/1355479049089401...	1	0/1	not applicable	not applicable
McMonagle et al., 2006³3. McMonagle P, Blair M, Kertesz A. Corticobasal degeneration and progressive aphasia. Neurology. 2006;67(8):1444-51. https://doi.org/10.1212/01.wnl.0000240215.43492.01 https://doi.org/10.1212/01.wnl.000024021...	55	motor onset=10/9 cognitive onset=16/20	n/a	motor onset=2.7 cognitive onset=3.6
McMillan et al., 2006³²32. McMillan CT, Clark R, Moore P, Grossman M. Quantifier comprehension in corticobasal degeneration. Brain Cogn. 2006;62(3):250-60. https://doi.org/10.1016/j.bandc.2006.06.005 https://doi.org/10.1016/j.bandc.2006.06....	16	n/a	66.3	n/a
Cotelli et al., 2006²⁹29. Cotelli M, Borroni B, Manenti R, Alberici A, Calabria M, Agosti C, et al. Action and object naming in frontotemporal dementia, progressive supranuclear palsy, and corticobasal degeneration. Neuropsychology. 2006;20(5):558-65. https://doi.org/10.1037/0894-4105.20.5.558 https://doi.org/10.1037/0894-4105.20.5.5...	10	n/a	63.8	n/a
Cotelli et al., 2007³⁰30. Cotelli M, Borroni B, Manenti R, Ginex V, Calabria M, Moro A, et al. Universal grammar in the frontotemporal dementia spectrum: evidence of a selective disorder in the corticobasal degeneration syndrome. Neuropsychologia. 2007;45(13):3015-23. https://doi.org/10.1016/j.neuropsychologia.2007.05.012 https://doi.org/10.1016/j.neuropsycholog...	11	n/a	64.6	n/a
Donovan et al., 2007²³23. Donovan NJ, Kendall DL, Moore AB, Rosenbek JC, Rothi LJG. Why consider impaired social language usage in a case of corticobasal degeneration? Clin Neuropsychol. 2007;21(1):190-203. https://doi.org/10.1080/13854040600634584 https://doi.org/10.1080/1385404060063458...	1	0/1	60	4
Koenig et al., 2007²⁸28. Koenig P, Smith EE, Moore P, Glosser G, Grossman M. Categorization of novel animals by patients with Alzheimer's disease and corticobasal degeneration. Neuropsychology. 2007;21(2):193-206. https://doi.org/10.1037/0894-4105.21.2.193 https://doi.org/10.1037/0894-4105.21.2.1...	experiment 1=8 experiment 2=9	experiment 1=3/5 experiment 2=5/4	experiment 1=64.5 experiment 2=70.1	n/a
Silveri and Ciccarelli, 2007³¹31. Silveri MC, Ciccarelli N. The deficit for the word-class “verb” in corticobasal degeneration: Linguistic expression of the movement disorder? Neuropsychologia. 2007;45(11):2570-9. https://doi.org/10.1016/j.neuropsychologia.2007.03.014 https://doi.org/10.1016/j.neuropsycholog...	5	2/3	63.8	1.6
Halpern et al., 2007²⁷27. Halpern C, Clark R, Moore P, Cross K, Grossman M. Too much to count on: Impaired very small numbers in corticobasal degeneration. Brain Cogn. 2007;64(2):144-9. https://doi.org/10.1016/j.bandc.2007.01.006 https://doi.org/10.1016/j.bandc.2007.01....	16	9/7	67.07	3.9
Kim et al., 2008⁴⁵45. Kim SK, Park KW, Kang D-Y, Cha JK, Kim S-H, Kim JW. Severe Episodic Memory Impairment in a Patient with Clinical Features Compatible with Corticobasal Degeneration. J Clin Neurol. 2008;4(2):94. https://doi.org/10.3988/jcn.2008.4.2.94 https://doi.org/10.3988/jcn.2008.4.2.94...	1	1/0	55	n/a
Shelley et al., 2009¹²12. Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH. Is the pathology of corticobasal syndrome predictable in life? Mov Disord. 2009;24(11):1593-9. https://doi.org/10.1002/mds.22558 https://doi.org/10.1002/mds.22558...	12	6/6	75.5	8.08
Gross et al., 2010²⁴24. Gross RG, Ash S, McMillan CT, Gunawardena D, Powers C, Libon DJ, et al. Impaired Information Integration Contributes to Communication Difficulty in Corticobasal Syndrome. Gon Bahv Neurol. 2010;23(1):1-7. https://doi.org/10.1097/WNN.0b013e3181c5e2f8 https://doi.org/10.1097/WNN.0b013e3181c5...	20	9/11	67.4	3.9
Valverde et al., 2011³⁵35. Valverde AH, Costa S, Timoteo Â, Ginestal R, Pimentel J. Rapidly progressive corticobasal degeneration syndrome. Case Rep Neurol. 2011;3(2):185-90. https://doi.org/10.1159/000329820 https://doi.org/10.1159/000329820...	1	0/1	74	0,25
Borroni et al., 2011⁴⁷47. Borroni B, Premi E, Agosti C, Alberici A, Cerini C, Archetti S, et al. CSF Alzheimer's disease-like pattern in corticobasal syndrome: Evidence for a distinct disorder. J Neurol Neurosurg Psychiatry. 2011;82(8):834-8. https://doi.org/10.1136/jnnp.2010.221853 https://doi.org/10.1136/jnnp.2010.221853...	30	21/9	63.5	2.5
Troiani et al., 2011³³33. Troiani V, Clark R, Grossman M. Impaired verbal comprehension of quantifiers in corticobasal syndrome. Neuropsychology. 2011;25(2):159-65. https://doi.org/10.1037/a0021448 https://doi.org/10.1037/a0021448...	11	n/a	65.5	n/a
Passov et al., 2011³⁶36. Passov V, Gavrilova RH, Strand E, Cerhan JH, Josephs KA. Sporadic corticobasal syndrome with progranulin mutation presenting as progressive apraxic agraphia. Arch Neurol. 2011;68(3):376-80. https://doi.org/10.1001/archneurol.2011.26 https://doi.org/10.1001/archneurol.2011....	1	0/1	49	2
Dopper et al., 2011⁴¹41. Dopper EGP, Seelaar H, Chiu WZ, De Koning I, Van Minkelen R, Baker MC, et al. Symmetrical corticobasal syndrome caused by a novel c.314dup progranulin mutation. J Mol Neurosci. 2011;45(3):354-8. https://doi.org/10.1007/s12031-011-9626-z https://doi.org/10.1007/s12031-011-9626-...	1	1/0	61	2
Caso et al., 2012²¹21. Caso F, Onofrio F, Falautano M, Todeschini P, Migliaccio R, Comi G, et al. From primary progressive aphasia to corticobasal syndrome: two clinical and rCBF functional reports. Neurocase. 2012;19(2):1-7. https://doi.org/https://doi.org/10.1080/13554794.2012.667117 https://doi.org/https://doi.org/10.1080/...	2	0/2	case 1=64 case 2=70	case 1=2 case 2=4
Assal et al., 2012³⁷37. Assal F, Laganaro M, Remund CD, Paquier CR. Progressive crossed-apraxia of speech as a first manifestation of a probable corticobasal degeneration. Behav Neurol. 2012;25(4):285-9. https://doi.org/10.3233/BEN-2012-110219 https://doi.org/10.3233/BEN-2012-110219...	1	0/1	64	n/a
Mathew et al., 2012⁴4. Mathew R, Bak TH, Hodges JR. Diagnostic criteria for corticobasal syndrome: a comparative study. J Neurol Neurosurg Psychiatry. 2012;83(4):405-10. https://doi.org/10.1136/jnnp-2011-300875 https://doi.org/10.1136/jnnp-2011-300875...	40	22/18	70	initial assessment=3 follow-up=4.9
Sakurai et al., 2013³⁸38. Sakurai Y, Ishii K, Sonoo M, Saito Y, Murayama S, Iwata A, et al. Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake. J Neurol. 2013;260(8):1982-91. https://doi.org/10.1007/s00415-013-6908-0 https://doi.org/10.1007/s00415-013-6908-...	1	0/1	65	n/a
Burrell et al., 2013¹⁵15. Burrell JR, Hornberger M, Villemagne VL, Rowe CC, Hodges JR. Clinical profile of PiB-positive corticobasal syndrome. PLoS ONE. 2013;8(4):e61025. https://doi.org/10.1371/journal.pone.0061025 https://doi.org/10.1371/journal.pone.006...	14	7/7	66.1	2.9
Turaga et al., 2013⁵¹51. Turaga S, Mridula R, Borgohain R. Cerebral glucose metabolism, clinical, neuropsychological, and radiological profile in patients with corticobasal syndrome. Neurol India. 2013;61(1):7. https://doi.org/10.4103/0028-3886.107916 https://doi.org/10.4103/0028-3886.107916...	17	11/6	66.35	4.06
Marshall et al., 2015⁴⁰40. Marshall CR, Guerreiro R, Thust S, Fletcher P, Rohrer JD, Fox NC. A novel MAPT mutation causing corticobasal syndrome led by progressive apraxia of speech. J Alzheimer's Dis. 2015;48(4):923-6. https://doi.org/10.3233/JAD-150477 https://doi.org/10.3233/JAD-150477...	1	0/1	47	1
Abe et al., 2016¹³13. Abe Y, Kimura N, Goto M, Aso Y, Matsubara E. Brain perfusion in corticobasal syndrome with progressive aphasia. Dement Geriatr Cogn Dis Extra. 2016;6(1):133-41. https://doi.org/10.1159/000443329 https://doi.org/10.1159/000443329...	26	9/17	76	2.3
Di Stefano et al., 2016¹⁶16. Di Stefano F, Kas A, Habert MO, Decazes P, Lamari F, Lista S, et al. The phenotypical core of Alzheimer-related and nonrelated variants of the corticobasal syndrome: a systematic clinical, neuropsychological, imaging, and biomarker study. Alzheimer's Dement. 2016;12(7):786-95. https://doi.org/10.1016/j.jalz.2016.02.005 https://doi.org/10.1016/j.jalz.2016.02.0...	45	23/22	69.2	3.2
Ash et al., 2016³⁴34. Ash S, Ternes K, Bisbing T, Min NE, Moran E, York C, et al. Dissociation of quantifiers and object nouns in speech in focal neurodegenerative disease. Neuropsychologia. 2016;89:141-52. https://doi.org/10.1016/j.neuropsychologia.2016.06.013 https://doi.org/10.1016/j.neuropsycholog...	33	15/18	65.3	4.2
Kim et al., 2016²²22. Kim JS, Lee GB, Hong YJ, Park KW. Corticobasal degeneration presenting as non-fluent/agrammatic primary progressive aphasia: a case report. Dement Neurocognitive Disord. 2016;15(2):55. https://doi.org/10.12779/dnd.2016.15.2.55 https://doi.org/10.12779/dnd.2016.15.2.5...	1	0/1	58	4
Magdalinou et al., 2018²⁵25. Magdalinou NK, Golden HL, Nicholas JM, Witoonpanich P, Mummery CJ, Morris HR, et al. Verbal adynamia in parkinsonian syndromes: behavioral correlates and neuroanatomical substrate. Neurocase. 2018;24(4):204-12. https://doi.org/10.1080/13554794.2018.1527368 https://doi.org/10.1080/13554794.2018.15...	4	n/a	n/a	n/a
Mazzon et al., 2018³⁹39. Mazzon G, Menichelli A, Fabretto A, Cattaruzza T, Manganotti P. A new MAPT deletion in a case of speech apraxia leading to corticobasal syndrome. Neurocase. 2018;24(3):140-4. https://doi.org/https://doi.org/10.1080/13554794.2018.1492729 https://doi.org/https://doi.org/10.1080/...	1	1/0	74	1
Dodich et al., 2019¹⁴14. Dodich A, Cerami C, Inguscio E, Iannaccone S, Magnani G, Marcone A, et al. The clinico-metabolic correlates of language impairment in corticobasal syndrome and progressive supranuclear palsy. Neuroimage Clin. 2019;24:102009. https://doi.org/10.1016/j.nicl.2019.102009 https://doi.org/10.1016/j.nicl.2019.1020...	33	15/18	70.4	3.06

Authors, year of publication	Main speech/language results	Speech and language tests or abilities tested	Classification of the language evaluation	Quality of studies
Kertesz et al., 2000²2. Kertesz A, Martinez-Lage P, Davidson W, Munoz D. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology. 2000;55(9):1368-75. https://doi.org/10.1212/wnl.55.9.1368 https://doi.org/10.1212/wnl.55.9.1368...	Initially, only word finding difficulties; verbal apraxia in 3/35 patients	WAB	Comprehensive assessment	High
Frattali et al., 2000⁴²42. Frattali CM, Grafman J, Patronas N, Makhlouf F, Litvan I. Language disturbances in corticobasal degeneration. Neurology. 2000;54(4):990-2. https://doi.org/https://doi.org/10.1212/WNL.54.4.990 https://doi.org/https://doi.org/10.1212/...	Anomic, Broca's and transcortical motor aphasia	WAB (1st section)	Restricted assessment	Moderate
Graham et al., 2003⁵⁰50. Graham NL, Bak T, Patterson K, Hodges JR. Language function and dysfunction in corticobasal degeneration. Neurology. 2003;61(4):493-9. https://doi.org/10.1212/01.wnl.0000081230.09863.ed https://doi.org/10.1212/01.wnl.000008123...	Specific linguistic deficit involving phonologic processing	Letter fluency (FAS), semantic fluency, picture naming, word-picture matching, PPT, Single-word reading (The surface list), nonword reading, oral spelling, phoneme blending and phoneme segmentation	Restricted assessment	Moderate
Frattali et al., 2003²⁶26. Frattali CM, Duffy JR, Litvan I, Patsalides AD, Grafman J. Yes/no reversals as neurobehavioral sequela: A disorder of language, praxis, or inhibitory control? Eur J Neurol. 2003;10(1):103-6. https://doi.org/10.1046/j.1468-1331.2003.00545.x https://doi.org/10.1046/j.1468-1331.2003...	Aphasia, without details	WAB (1st section)	Restricted assessment	Moderate
Gorno-Tempini et al., 2004²⁰20. Gorno-Tempini M-L, Murray RC, Rankin KP, Weiner MW, Miller BL. Clinical, cognitive and anatomical evolution from nonfluent progressive aphasia to corticobasal syndrome: a Case Report. Neurocase. 2004;10(6):426-36. https://doi.org/10.1080/13554790490894011 https://doi.org/10.1080/1355479049089401...	nf-PPA	Motor speech evaluation, BDAE (verbal agility component, repetition), WAB (spontaneous speech section, written picture description, repetition, auditory word recognition, sequential command), BNT, PPT, CYCLE-R, PALPA (Regularity and Reading, Lexical Morphology and Grammatical Class, Homophone Decision), Gathercole and Baddeley's Non-Word Repetition task	Comprehensive assessment	High
McMonagle; Blair; Kertesz, 2006³3. McMonagle P, Blair M, Kertesz A. Corticobasal degeneration and progressive aphasia. Neurology. 2006;67(8):1444-51. https://doi.org/10.1212/01.wnl.0000240215.43492.01 https://doi.org/10.1212/01.wnl.000024021...	Majority classification of anomic aphasia (55%) in both groups (cognitive and motor onset), but more motor onset patients were normal and more cognitive onset patients had severe aphasias	WAB (1st section)	Restricted assessment	Moderate
McMillan et al., 2006³²32. McMillan CT, Clark R, Moore P, Grossman M. Quantifier comprehension in corticobasal degeneration. Brain Cogn. 2006;62(3):250-60. https://doi.org/10.1016/j.bandc.2006.06.005 https://doi.org/10.1016/j.bandc.2006.06....	Non-aphasic patients with CBD are significantly impaired in their comprehension of quantifiers	Sentence comprehension task	Restricted assessment	Moderate
Cotelli et al., 2006²⁹29. Cotelli M, Borroni B, Manenti R, Alberici A, Calabria M, Agosti C, et al. Action and object naming in frontotemporal dementia, progressive supranuclear palsy, and corticobasal degeneration. Neuropsychology. 2006;20(5):558-65. https://doi.org/10.1037/0894-4105.20.5.558 https://doi.org/10.1037/0894-4105.20.5.5...	Action naming is impaired in FTD, PSP and CBS in comparison to object naming	Token Test, phonemic and semantic verbal fluency, action and object naming, Battery for Analysis of the Aphasic Deficits (action–object comprehension tasks)	Restricted assessment	Moderate
Cotelli et al., 2007³⁰30. Cotelli M, Borroni B, Manenti R, Ginex V, Calabria M, Moro A, et al. Universal grammar in the frontotemporal dementia spectrum: evidence of a selective disorder in the corticobasal degeneration syndrome. Neuropsychologia. 2007;45(13):3015-23. https://doi.org/10.1016/j.neuropsychologia.2007.05.012 https://doi.org/10.1016/j.neuropsycholog...	CBS patients present with syntactic knowledge deficits	AAT (repetition, naming, writing and comprehension), BADA (sentence comprehension tasks)	Comprehensive assessment	Moderate
Donovan et al., 2007²³23. Donovan NJ, Kendall DL, Moore AB, Rosenbek JC, Rothi LJG. Why consider impaired social language usage in a case of corticobasal degeneration? Clin Neuropsychol. 2007;21(1):190-203. https://doi.org/10.1080/13854040600634584 https://doi.org/10.1080/1385404060063458...	Aphasia, speech apraxia, alexia, agraphia, social language usage deficits	Pragmatic Protocol, Revised Token Test, WAB, BNT, Battery of Adult Reading Function, Woodcock Reading Mastery Tests, Comprehensive Test of Phonological Processing	Comprehensive assessment	High
Koenig et al., 2007²⁸28. Koenig P, Smith EE, Moore P, Glosser G, Grossman M. Categorization of novel animals by patients with Alzheimer's disease and corticobasal degeneration. Neuropsychology. 2007;21(2):193-206. https://doi.org/10.1037/0894-4105.21.2.193 https://doi.org/10.1037/0894-4105.21.2.1...	CBS patients were impaired in similarity-based categorization process	Semantic decision task	Restricted assessment	Moderate
Silveri and Ciccarelli, 2007³¹31. Silveri MC, Ciccarelli N. The deficit for the word-class “verb” in corticobasal degeneration: Linguistic expression of the movement disorder? Neuropsychologia. 2007;45(11):2570-9. https://doi.org/10.1016/j.neuropsychologia.2007.03.014 https://doi.org/10.1016/j.neuropsycholog...	Hypofluent speech, agrammatism, anomia, word-finding difficulties, agraphia	Confrontation naming task of objects and verbs, semantic and phonemic fluency	Restricted assessment	Moderate
Halpern et al., 2007²⁷27. Halpern C, Clark R, Moore P, Cross K, Grossman M. Too much to count on: Impaired very small numbers in corticobasal degeneration. Brain Cogn. 2007;64(2):144-9. https://doi.org/10.1016/j.bandc.2007.01.006 https://doi.org/10.1016/j.bandc.2007.01....	CBS patients were less accurate and slower at judging smaller Arabic numeral dot array compared to FTD patients and controls	PPT	Restricted assessment	Moderate
Kim et al., 2008⁴⁵45. Kim SK, Park KW, Kang D-Y, Cha JK, Kim S-H, Kim JW. Severe Episodic Memory Impairment in a Patient with Clinical Features Compatible with Corticobasal Degeneration. J Clin Neurol. 2008;4(2):94. https://doi.org/10.3988/jcn.2008.4.2.94 https://doi.org/10.3988/jcn.2008.4.2.94...	Language functions relatively preserved	BNT	Restricted assessment	Low
Shelley et al., 2009¹²12. Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH. Is the pathology of corticobasal syndrome predictable in life? Mov Disord. 2009;24(11):1593-9. https://doi.org/10.1002/mds.22558 https://doi.org/10.1002/mds.22558...	nf-PPA	n/a	No tests or language skills are mentioned	Low
Gross et al., 2010²⁴24. Gross RG, Ash S, McMillan CT, Gunawardena D, Powers C, Libon DJ, et al. Impaired Information Integration Contributes to Communication Difficulty in Corticobasal Syndrome. Gon Bahv Neurol. 2010;23(1):1-7. https://doi.org/10.1097/WNN.0b013e3181c5e2f8 https://doi.org/10.1097/WNN.0b013e3181c5...	CBS patients have a higher-level deficit integrating described events into a coherent narrative	BNT, PPT	Restricted assessment	Moderate
Valverde et al., 2011³⁵35. Valverde AH, Costa S, Timoteo Â, Ginestal R, Pimentel J. Rapidly progressive corticobasal degeneration syndrome. Case Rep Neurol. 2011;3(2):185-90. https://doi.org/10.1159/000329820 https://doi.org/10.1159/000329820...	Aphasic, without details	n/a	No tests or language skills are mentioned	Moderate
Borroni et al., 2011⁴⁷47. Borroni B, Premi E, Agosti C, Alberici A, Cerini C, Archetti S, et al. CSF Alzheimer's disease-like pattern in corticobasal syndrome: Evidence for a distinct disorder. J Neurol Neurosurg Psychiatry. 2011;82(8):834-8. https://doi.org/10.1136/jnnp.2010.221853 https://doi.org/10.1136/jnnp.2010.221853...	The AD-like group showed greater impairment of memory performances, language and psychomotor speed while the nAD-like group had more severe extrapyramidal syndrome	Semantic and phonemic verbal fluency, Token Test,	Restricted assessment	Moderate
Troiani et al., 2011³³33. Troiani V, Clark R, Grossman M. Impaired verbal comprehension of quantifiers in corticobasal syndrome. Neuropsychology. 2011;25(2):159-65. https://doi.org/10.1037/a0021448 https://doi.org/10.1037/a0021448...	CBS patients were significantly impaired in their judgments of quantified statements	Philadelphia Brief Assessment of Cognition (used to exclude aphasic patients), BNT, phonemic verbal fluency (FAS), Oral Sentence Comprehension Test, short sentence comprehension task	Restricted assessment	Moderate
Passov et al., 2011³⁶36. Passov V, Gavrilova RH, Strand E, Cerhan JH, Josephs KA. Sporadic corticobasal syndrome with progranulin mutation presenting as progressive apraxic agraphia. Arch Neurol. 2011;68(3):376-80. https://doi.org/10.1001/archneurol.2011.26 https://doi.org/10.1001/archneurol.2011....	Mild apraxia of speech, mild hypokinetic dysarthria, apraxic agraphia	“Formal speech pathology evaluation”; picture description task; confrontation naming task; comprehension of simple and complex commands; writing; spelling; motor speech disorders	Comprehensive assessment	High
Dopper et al., 2011⁴¹41. Dopper EGP, Seelaar H, Chiu WZ, De Koning I, Van Minkelen R, Baker MC, et al. Symmetrical corticobasal syndrome caused by a novel c.314dup progranulin mutation. J Mol Neurosci. 2011;45(3):354-8. https://doi.org/10.1007/s12031-011-9626-z https://doi.org/10.1007/s12031-011-9626-...	nonfluent speech with perseverations, word-finding difficulties and comprehension deficits, hypokinetic dysarthria	n/a	No tests or language skills are mentioned	Moderate
Caso et al., 2012²¹21. Caso F, Onofrio F, Falautano M, Todeschini P, Migliaccio R, Comi G, et al. From primary progressive aphasia to corticobasal syndrome: two clinical and rCBF functional reports. Neurocase. 2012;19(2):1-7. https://doi.org/https://doi.org/10.1080/13554794.2012.667117 https://doi.org/https://doi.org/10.1080/...	nf-PPA, L-PPA	AAT, Token Test, phonemic and semantic verbal fluency	Comprehensive assessment	High
Assal et al., 2012³⁷37. Assal F, Laganaro M, Remund CD, Paquier CR. Progressive crossed-apraxia of speech as a first manifestation of a probable corticobasal degeneration. Behav Neurol. 2012;25(4):285-9. https://doi.org/10.3233/BEN-2012-110219 https://doi.org/10.3233/BEN-2012-110219...	crossed-PAOS followed by peripheral agraphia	Bachy 90-item battery (confrontation naming), MTL (auditory and written language comprehension, and writing), written descriptions of the Bank Robbery Picture, and the Cookie Theft Picture, and oral spelling with the French version of the WAIS III	Comprehensive assessment	Moderate
Mathew et al., 2012⁴4. Mathew R, Bak TH, Hodges JR. Diagnostic criteria for corticobasal syndrome: a comparative study. J Neurol Neurosurg Psychiatry. 2012;83(4):405-10. https://doi.org/10.1136/jnnp-2011-300875 https://doi.org/10.1136/jnnp-2011-300875...	nf-PPA (60%) and anomic aphasia (40%)	n/a	No tests or language skills are mentioned	Moderate
Sakurai et al., 2013³⁸38. Sakurai Y, Ishii K, Sonoo M, Saito Y, Murayama S, Iwata A, et al. Progressive apraxic agraphia with micrographia presenting as corticobasal syndrome showing extensive Pittsburgh compound B uptake. J Neurol. 2013;260(8):1982-91. https://doi.org/10.1007/s00415-013-6908-0 https://doi.org/10.1007/s00415-013-6908-...	Progressive apraxic agraphia with micrographia, and acalculia	WAB, reading and writing test with 100 single-character kanji and kana transcription	Comprehensive assessment	Moderate
Burrell et al., 2013¹⁵15. Burrell JR, Hornberger M, Villemagne VL, Rowe CC, Hodges JR. Clinical profile of PiB-positive corticobasal syndrome. PLoS ONE. 2013;8(4):e61025. https://doi.org/10.1371/journal.pone.0061025 https://doi.org/10.1371/journal.pone.006...	Impaired single word repetition (61.5%), dysgraphia (58.3%), phonological errors in spontaneous speech (46.2%), impaired sentence repetition (38.5%), and word-finding difficulty (30.8%). Agrammatism and anomia were only occasionally identified. There was a trend for greater impairment of sentence repetition in PiB-positive cases	Motor speech disorder, phonological errors, agrammatism, word-finding difficulty, anomia, word and sentence repetition	Restricted assessment	Moderate
Turaga et al., 2013⁵¹51. Turaga S, Mridula R, Borgohain R. Cerebral glucose metabolism, clinical, neuropsychological, and radiological profile in patients with corticobasal syndrome. Neurol India. 2013;61(1):7. https://doi.org/10.4103/0028-3886.107916 https://doi.org/10.4103/0028-3886.107916...	phonemic verbal fluency impairment	ACE-R (phonemic verbal fluency, semantic verbal fluency, naming)	Restricted assessment	Moderate
Marshall et al., 2015⁴⁰40. Marshall CR, Guerreiro R, Thust S, Fletcher P, Rohrer JD, Fox NC. A novel MAPT mutation causing corticobasal syndrome led by progressive apraxia of speech. J Alzheimer's Dis. 2015;48(4):923-6. https://doi.org/10.3233/JAD-150477 https://doi.org/10.3233/JAD-150477...	PAOS	n/a	No tests or language skills are mentioned	Moderate
Abe et al., 2016¹³13. Abe Y, Kimura N, Goto M, Aso Y, Matsubara E. Brain perfusion in corticobasal syndrome with progressive aphasia. Dement Geriatr Cogn Dis Extra. 2016;6(1):133-41. https://doi.org/10.1159/000443329 https://doi.org/10.1159/000443329...	nf-PPA (34,61%)	Standard Language Test of Aphasia	Comprehensive assessment	High
Di Stefano et al., 2016¹⁶16. Di Stefano F, Kas A, Habert MO, Decazes P, Lamari F, Lista S, et al. The phenotypical core of Alzheimer-related and nonrelated variants of the corticobasal syndrome: a systematic clinical, neuropsychological, imaging, and biomarker study. Alzheimer's Dement. 2016;12(7):786-95. https://doi.org/10.1016/j.jalz.2016.02.005 https://doi.org/10.1016/j.jalz.2016.02.0...	Mixed progressive aphasia, including disorders of L-PPA (anomia, sentence repetition impairment) and S-PPA (deficits in single-word comprehension)	BDAE, picture naming test, single-word comprehension task, semantic and phonemic verbal fluency, sentence repetition test, assessment of motor speech disorders and agrammatism	Comprehensive assessment	High
Ash et al., 2016³⁴34. Ash S, Ternes K, Bisbing T, Min NE, Moran E, York C, et al. Dissociation of quantifiers and object nouns in speech in focal neurodegenerative disease. Neuropsychologia. 2016;89:141-52. https://doi.org/10.1016/j.neuropsychologia.2016.06.013 https://doi.org/10.1016/j.neuropsycholog...	CBS were significantly impaired in the production of quantifiers	BNT, semantic verbal fluency, semi-structured speech sample (description of the Cookie Theft picture from the BDAE)	Restricted assessment	Moderate
Kim et al., 2016²²22. Kim JS, Lee GB, Hong YJ, Park KW. Corticobasal degeneration presenting as non-fluent/agrammatic primary progressive aphasia: a case report. Dement Neurocognitive Disord. 2016;15(2):55. https://doi.org/10.12779/dnd.2016.15.2.55 https://doi.org/10.12779/dnd.2016.15.2.5...	nf-PPA	WAB, BNT, semantic and phonemic verbal fluency	Comprehensive assessment	High
Magdalinou et al., 2018²⁵25. Magdalinou NK, Golden HL, Nicholas JM, Witoonpanich P, Mummery CJ, Morris HR, et al. Verbal adynamia in parkinsonian syndromes: behavioral correlates and neuroanatomical substrate. Neurocase. 2018;24(4):204-12. https://doi.org/10.1080/13554794.2018.1527368 https://doi.org/10.1080/13554794.2018.15...	Impaired verbal fluency and sentence generation	BNT, Graded Naming Test, Verb Naming Task, PALPA (sentence comprehension), Sentence Production Program for Aphasia (expressive grammar), phonemic and semantic verbal fluency, National Adult Reading Test, sentence completion tasks	Restricted assessment	Moderate
Mazzon et al., 2018³⁹39. Mazzon G, Menichelli A, Fabretto A, Cattaruzza T, Manganotti P. A new MAPT deletion in a case of speech apraxia leading to corticobasal syndrome. Neurocase. 2018;24(3):140-4. https://doi.org/https://doi.org/10.1080/13554794.2018.1492729 https://doi.org/https://doi.org/10.1080/...	Apraxia of speech, characterized by slow overall speech rate, mild dysphonia, abnormal prosody, distorted and inconsistent speech sound substitutions, segmentation of syllables in words productions, mild dysgraphia with letter substitutions and omissions	Motor Speech Evaluation, AAT, Cookie Thief Test	Comprehensive assessment	High
Dodich et al., 2019¹⁴14. Dodich A, Cerami C, Inguscio E, Iannaccone S, Magnani G, Marcone A, et al. The clinico-metabolic correlates of language impairment in corticobasal syndrome and progressive supranuclear palsy. Neuroimage Clin. 2019;24:102009. https://doi.org/10.1016/j.nicl.2019.102009 https://doi.org/10.1016/j.nicl.2019.1020...	nf-PPA, other language disorders	Connected speech production (speech apraxia and articulation difficulties, anomia, circumlocutions, agrammatism), CAGI battery (naming and word-picture matching), phonemic and semantic controlled associations, AAT (repetition), Token Test, BADA (sentence comprehension) phonemic (P-F-L) and semantic (animals-fruits-cars) verbal fluency	Comprehensive assessment	High

Review findings	Studies contributing to the review finding	Methodological limitation	Relevance	Coherence	Adequacy of data	Overall CERQual assessment of confidence
Comprehensive language impairments (presence of aphasia)	2; 3; 4; 12; 13; 14; 15; 16; 20; 21; 22; 23; 35; 41; 42; 47; 50	minor methodological limitation (8 studies with moderate methodological quality and 1 study with low methodological quality)	moderate concerns about relevance (only 8 studies carried out a comprehensive language assessment)	moderate concerns about coherence (inconsistent data across studies regarding language outcomes)	substantial concerns about adequacy of data (6 studies are case reports or case series and 4 have up to 15 participants)	Low confidence
Impairments in isolated language processing	24; 25; 26; 27; 28; 29; 30; 31; 32; 33; 34; 36; 37; 38; 39; 40; 51	moderate methodological limitation (15 studies with moderate methodological quality)	moderate concerns about relevance (only 5 studies carried out a comprehensive language assessment)	moderate concerns about coherence (inconsistent data across studies regarding language outcomes)	substantial concerns about adequacy of data (7 studies are case reports or case series)	Low confidence
Absence of language impairments	45	substantial concerns (low methodological quality)	substantial concerns about relevance (restricted language assessment)	not applicable	substantial concerns about adequacy of data (case report)	Very low confidence

Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revistadementia@abneuro.org.br | demneuropsy@uol.com.br

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[1] Isabel Junqueira de Almeida. Rua Cipotânea, 51 – Cidade Universitária – 05360-160 São Paulo SP – Brazil. E-mail: junqueira.bel@gmail.com