Cognitive and behavioral profile in a case of right anterior temporal lobe neurodegeneration
Open accessAbstract
Semantic dementia (SD) is a clinical variant of frontotemporal lobar degeneration (FTLD) characterized by progressive deterioration of semantic memory with relative sparing of other cognitive functions. It is associated with mainly left anterior temporal atrophy, and is also referred to as "left-temporal lobe variant" of FTLD. Recently, patients with mainly right-sided atrophy, or "right-temporal lobe variant"(RTLV), have been described. While some authors have reported that the initial and most significant deficit in these right-sided cases is a difficulty in recognizing famous people, others have observed that major behavioral abnormalities are the presenting symptoms. Here we report a detailed neuropsychological, language, behavioral and neuroimaging assessment of JT, a case of right temporal lobe variant of FTLD. JT showed early and prominent behavioral changes accompanied by a severe impairment in recognizing foods by their look, flavor or name. Later she also developed a difficulty in recognizing familiar people and objects. Standardized caregiver questionnaires of JT's pre- and post-morbid personality and interpersonal functioning showed that she went from being a flexible, dominant, extraverted, person to showing rigid, submissive and introverted behaviors. Her levels of neuroticism significantly increased, while her scores on agreeableness and cognitive and emotional empathy dropped. Voxel-based morphometry (VBM) showed most significant atrophy in the right amygdala/anterior hippocampal complex and collateral sulcus, extending to the right insula. We discuss the atypical cognitive and behavioral features of this case of RTLV of FTLD and stress the importance of behavioral changes and atypical semantic deficits for early diagnosis.
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?
Frontotemporal dementia with orbitofrontal cortex pathology is marked by compulsive consummatory behaviors including hyperphagia, gambling, and substance abuse.
“Cases of frontotemporal dementia with OFC pathology are also marked by compulsive consummatory behaviors, including hyperphagia, gambling, and substance abuse (Gorno-Tempini et al., 2004; Ikeda et al., 2002; Rosen et al., 2005; Thompson et al., 2003; Whitwell et al., 2007; Williams et al., 2005; Woolley et al., 2007).”
From Impulsivity, frontal lobes and risk for addiction (2009) · cites this paper as Gorno-Tempini et al., 2004
Cites
Folstein, M. F., Folstein, S. E., & McHugh, P. R. (1975) · Journal of Psychiatric Research · doi:10.1016/0022-3956(75)90026-6 · Cited by 88,282 · see citing works
Measuring individual differences in empathy: Evidence for a multidimensional approach.
Davis, M. H. (1983) · Journal of Personality and Social Psychology · doi:10.1037/0022-3514.44.1.113 · Cited by 10,272 · see citing works
The Clinical Dementia Rating (CDR): Current version and scoring rules
Morris, J. C. (1993) · Neurology · doi:10.1212/wnl.43.11.2412-a · Cited by 9,882 · see citing works
Voxel-Based Morphometry—The Methods
Ashburner, J., & Friston, K. J. (2000) · NeuroImage · doi:10.1006/nimg.2000.0582 · Cited by 8,676 · see citing works
The fusiform face area: a module in human extrastriate cortex specialized for face perception
Kanwisher, N., McDermott, J., & Chun, M. M. (1997) · The Journal of Neuroscience · doi:10.1523/jneurosci.17-11-04302.1997 · Cited by 7,738 · see citing works
Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria
Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., Freedman, M., Kertesz, A., Robert, P. H., Albert, M., et al. (1998) · Neurology · doi:10.1212/wnl.51.6.1546 · Cited by 5,047 · see citing works
Self-citationNormal personality assessment in clinical practice: the NEO personality inventory
Costa, P. T., & McCrae, R. R. (1992) · Psychological Assessment · doi:10.1037/1040-3590.4.1.5 · Cited by 3,414 · see citing works
Wisconsin Card Sorting Test Manual – Revised and Expanded
Heaton, R. K., Chelune, C., Talley, J., Kay, G., & Curtiss, G. (1981) · Medical Entomology and Zoology · Cited by 2,975 · see citing works
Delis-Kaplan Executive Function System
Delis, D. C., Kaplan, E., & Kramer, J. H. (2012) · PsycTESTS Dataset · doi:10.1037/t15082-000 · Cited by 2,683 · see citing works
The Neuropsychiatric Inventory
Cummings, J. L. (1997) · Neurology · doi:10.1212/wnl.48.5_suppl_6.10s · Cited by 2,017 · see citing works
A differential neural response in the human amygdala to fearful and happy facial expressions
Morris, J. S., Frith, C. D., Perrett, D. I., Rowland, D., Young, A. W., Calder, A. J., & Dolan, R. J. (1996) · Nature · doi:10.1038/383812a0 · Cited by 2,013 · see citing works
SEMANTIC DEMENTIA: PROGRESSIVE FLUENT APHASIA WITH TEMPORAL LOBE ATROPHY
HODGES, J. R., PATTERSON, K., OXBURY, S., & FUNNELL, E. (1992) · Brain · doi:10.1093/brain/115.6.1783 · Cited by 1,710 · see citing works
Hécaen, H. (1983) · Neuropsychologia · doi:10.1016/0028-3932(83)90089-1 · Cited by 1,700 · see citing works
Preliminary analysis of functions of the temporal lobes in monkeys
KLÜVER, H. (1939) · Archives of Neurology And Psychiatry · doi:10.1001/archneurpsyc.1939.02270240017001 · Cited by 1,696 · see citing works
Patterns of brain atrophy in frontotemporal dementia and semantic dementia
Rosen, H. J., Gorno–Tempini, M. L., Goldman, W. P., Perry, R. J., Schuff, N., Weiner, M., Feiwell, R., Kramer, J. H., & Miller, B. L. (2002) · Neurology · doi:10.1212/wnl.58.2.198 · Cited by 841 · see citing works
Self-citationPatterns of temporal lobe atrophy in semantic dementia and Alzheimer's disease
Chan, D., Fox, N. C., Scahill, R. I., Crum, W. R., Whitwell, J. L., Leschziner, G., Rossor, A. M., Stevens, J. M., Cipolotti, L., & Rossor, M. N. (2001) · Annals of Neurology · doi:10.1002/ana.92 · Cited by 749 · see citing works
California Verbal Learning Test
Templeton, D. M., Schwenk, M., & Duffus, J. H. (2016) · IUPAC Standards Online · doi:10.1515/iupac.87.0120 · Cited by 693 · see citing works
Representation of Pleasant and Aversive Taste in the Human Brain
O’Doherty, J. P., Rolls, E. T., Francis, S., Bowtell, R. W., & McGlone, F. P. (2001) · Journal of Neurophysiology · doi:10.1152/jn.2001.85.3.1315 · Cited by 674 · see citing works
Psychometric and Geometric Characteristics of the Revised Interpersonal Adjective Scales (IAS-R)
Wiggins, J. S., Trapnell, P. D., & Phillips, N. (1988) · Multivariate Behavioral Research · doi:10.1207/s15327906mbr2304_8 · Cited by 589 · see citing works
Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia
Galton, C. J., Patterson, K., Graham, K., Lambon-Ralph, M. A., Williams, G., Antoun, N., Sahakian, B. J., & Hodges, J. R. (2001) · Neurology · doi:10.1212/wnl.57.2.216 · Cited by 582 · see citing works
Impairment in facial recognition in patients with cerebral disease.
Benton, A., & van Allen, M. V. (1968) · Transactions of the American Neurological Association · doi:10.1016/s0010-9452(68)80018-8 · Cited by 551 · see citing works
Ikeda, M. (2002) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.73.4.371 · Cited by 499 · see citing works
Neural Systems Underlying the Recognition of Familiar and Newly Learned Faces
Leveroni, C. L., Seidenberg, M., Mayer, A. R., Mead, L. A., Binder, J. R., & Rao, S. M. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-02-00878.2000 · Cited by 494 · see citing works
The neural systems sustaining face and proper-name processing
Tempini, M. L. G. (1998) · Brain · doi:10.1093/brain/121.11.2103 · Cited by 461 · see citing works
Human cortical gustatory areas: A review of functional neuroimaging data
Small, D. M., Zald, D. H., Jones-Gotman, M., Zatorre, R. J., Pardo, J. V., Frey, S., & Petrides, M. (1999) · NeuroReport · doi:10.1097/00001756-199901180-00002 · Cited by 431 · see citing works
Progressive prosopagnosia associated with selective right temporal lobe atrophy
Evans, J. J., Heggs, A. J., Antoun, N. M., & Hodges, J. R. (1995) · Brain · doi:10.1093/brain/118.1.1 · Cited by 413 · see citing works
Good, C. D., Scahill, R. I., Fox, N. C., Ashburner, J., Friston, K. J., Chan, D., Crum, W. R., Rossor, M. N., & Frackowiak, R. S. J. (2002) · NeuroImage · doi:10.1006/nimg.2002.1202 · Cited by 410 · see citing works
The temporal variant of frontotemporal dementia
Edwards-Lee, T. (1997) · Brain · doi:10.1093/brain/120.6.1027 · Cited by 373 · see citing works
Renzi, E. D., Perani, D., Carlesimo, G. A., Silveri, M. C., & Fazio, F. (1994) · Neuropsychologia · doi:10.1016/0028-3932(94)90041-8 · Cited by 333 · see citing works
Naming in semantic dementia—what matters?
Ralph, M. A. L., Graham, K. S., Ellis, A. W., & Hodges, J. R. (1998) · Neuropsychologia · doi:10.1016/s0028-3932(97)00169-3 · Cited by 307 · see citing works
Alzheimer Disease and Frontotemporal Dementias
Levy, M. L. (1996) · Archives of Neurology · doi:10.1001/archneur.1996.00550070129021 · Cited by 277 · see citing works
Distributional assumptions in voxel-based morphometry
Salmond, C. H., Ashburner, J., Vargha-Khadem, F., Connelly, A., Gadian, D. G., & Friston, K. J. (2002) · NeuroImage · doi:10.1006/nimg.2002.1153 · Cited by 256 · see citing works
Emotion comprehension in the temporal variant of frontotemporal dementia
Rosen, H. J. (2002) · Brain · doi:10.1093/brain/awf225 · Cited by 250 · see citing works
Self-citationGorno‐Tempini, M. (2001) · Brain · doi:10.1093/brain/124.10.2087 · Cited by 250 · see citing works
Self-citationPerry, R., Rosen, H. R., Kramer, J., Beer, J. S., Levenson, R., & Miller, B. L. (2001) · Neurocase · doi:10.1093/neucas/7.2.145 · Cited by 235 · see citing works
Self-citationScoring Reliability on the Wechsler Adult Intelligence Scale-Third Edition (WAIS-III)
Ryan, J. J., & Schnakenberg‐Ott, S. D. (2003) · Assessment · doi:10.1177/1073191103010002006 · Cited by 224 · see citing works
Miller, B. L., Chang, L., Mena, I. G., Boone, K. B., & Lesser, I. M. (1993) · Dementia and Geriatric Cognitive Disorders · doi:10.1159/000107324 · Cited by 206 · see citing works
Self-citationGainotti, G., Barbier, A., & Marra, C. (2003) · Brain : a journal of neurology · doi:10.1093/brain/awg092 · Cited by 204 · see citing works
Ralph, M. A. L., Graham, K. S., Patterson, K. E., & Hodges, J. R. (1999) · Brain and Language · doi:10.1006/brln.1999.2143 · Cited by 154 · see citing works
A Role for the Right Anterior Temporal Lobe in Taste Quality Recognition
Small, D. M., Jones‐Gotman, M., Zatorre, R. J., Petrides, M., & Evans, A. C. (1997) · Journal of Neuroscience · doi:10.1523/jneurosci.17-13-05136.1997 · Cited by 150 · see citing works
Double dissociation of social functioning in frontotemporal dementia
Rankin, K. P., Kramer, J. H., Mychack, P., & Miller, B. L. (2003) · Neurology · doi:10.1212/01.wnl.0000041497.07694.d2 · Cited by 149 · see citing works
Self-citationHodges, J. R., & Graham, K. S. (1998) · Neuropsychologia · doi:10.1016/s0028-3932(97)00126-7 · Cited by 139 · see citing works
Slowly progressive semantic impairment with category specificity
Barbarotto, R., Capitani, E., Spinnler, H. R., & Trivelli, C. (1995) · Neurocase · doi:10.1080/13554799508402355 · Cited by 134 · see citing works
Welleford, E. A., Harkins, S. W., & Taylor, J. R. (1995) · Experimental Aging Research · doi:10.1080/03610739508253986 · Cited by 122 · see citing works
Concordance between observers in descriptions of personality change in Alzheimer's disease.
Strauss, M. E., Pasupathi, M., & Chatterjee, A. K. (1993) · Psychology and Aging · doi:10.1037/0882-7974.8.4.475 · Cited by 114 · see citing works
Concordance between observers in descriptions of personality change in Alzheimer's disease.
Strauss, M. E., Pasupathi, M., & Chatterjee, A. K. (1993) · Psychology and Aging · doi:10.1037//0882-7974.8.4.475 · Cited by 112 · see citing works
Progressive degeneration of the right temporal lobe studied with positron emission tomography.
Tyrrell, P. J., Warrington, E. K., Frackowiak, R. S. J., & Rossor, M. N. (1990) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.53.12.1046 · Cited by 110 · see citing works
Hodges, J. R., & Miller, B. L. (2001) · Neurocase · doi:10.1093/neucas/7.2.113 · Cited by 107 · see citing works
Self-citationRe‐examination of the human taste region: a positron emission tomography study
Frey, S., & Petrides, M. (1999) · European Journal of Neuroscience · doi:10.1046/j.1460-9568.1999.00738.x · Cited by 92 · see citing works
Kurtz, J. E., Lee, P. A., & Sherker, J. L. (1999) · Assessment · doi:10.1177/107319119900600201 · Cited by 79 · see citing works
Progressive defective recognition of familiar people
Gentileschi, V., Sperber, S. A., & Spinnler, H. R. (1999) · Neurocase · doi:10.1080/13554799908402735 · Cited by 66 · see citing works
Agnosia for familiar faces and odors in a patient with right temporal lobe dysfunction
Mendez, M. F., & Ghajarnia, M. (2001) · Neurology · doi:10.1212/wnl.57.3.519 · Cited by 54 · see citing works
Meningeal cells can communicate with astrocytes by calcium signaling
Grafstein, B., Liu, S., Cotrina, M. L., Goldman, S. A., & Nedergaard, M. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200001)47:1 · Cited by 50 · see citing works
Kitchener, E. G., & Hodges, J. R. (1999) · Cognitive Neuropsychology · doi:10.1080/026432999380744 · Cited by 44 · see citing works
Novel Applications of Social-Personality Measures to the Study of Dementia.
Mychack, P., Rosen, H. J., & Miller, B. L. (2001) · Neurocase · doi:10.1093/neucas/7.2.131 · Cited by 29 · see citing works
Self-citation(1944) · Archives of Internal Medicine · doi:10.1001/archinte.1944.00210170068013 · Cited by 27 · see citing works
Agreement between spouses and children in descriptions of personality change in Alzheimer's disease.
Heinik, J., Keren, P., Vainer-Benaiah, Z., Lahav, D., & Bleich, A. (1999) · PubMed · Cited by 9 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
The Enigmatic temporal pole: a review of findings on social and emotional processing
Olson, I. R., Plotzker, A., & Ezzyat, Y. (2007) · Brain · doi:10.1093/brain/awm052 · Cited by 1,370 · see citing works
Impulsivity, frontal lobes and risk for addiction
Crews, F. T., & Boettiger, C. A. (2009) · Pharmacology Biochemistry and Behavior · doi:10.1016/j.pbb.2009.04.018 · Cited by 679 · see citing works
“…dementia with OFC pathology are also marked by compulsive consummatory behaviors, including hyperphagia, gambling, and substance abuse (Gorno-Tempini et al., 2004; Ikeda et al., 2002; Rosen et al., 2005; Thompson et al., 2003; Whitwell et al., 2007; Williams et al., 2005; Woolley et…”
BackgroundWhat the left and right anterior fusiform gyri tell us about semantic memory
Mion, M., Patterson, K., Acosta-Cabronero, J., Pengas, G., Izquierdo-Garcia, D., Hong, Y. T., Fryer, T. D., Williams, G. B., Hodges, J. R., & Nestor, P. J. (2010) · Brain · doi:10.1093/brain/awq272 · Cited by 425 · see citing works
The clinical profile of right temporal lobe atrophy
Chan, D., Anderson, V., Pijnenburg, Y., Whitwell, J., Barnes, J., Scahill, R., Stevens, J. M., Barkhof, F., Scheltens, P., Rossor, M. N., et al. (2009) · Brain · doi:10.1093/brain/awp037 · Cited by 377 · see citing works
The Kuleshov Effect: the influence of contextual framing on emotional attributions
Mobbs, D., Weiskopf, N., Lau, H. C., Featherstone, E., Dolan, R. J., & Frith, C. D. (2006) · Social Cognitive and Affective Neuroscience · doi:10.1093/scan/nsl014 · Cited by 178 · see citing works
A clinical-radiological framework of the right temporal variant of frontotemporal dementia
Ulugut Erkoyun, H., Groot, C., Heilbron, R., Nelissen, A., van Rossum, J., Jutten, R., Koene, T., van der Flier, W. M., Wattjes, M. P., Scheltens, P., et al. (2020) · Brain · doi:10.1093/brain/awaa225 · Cited by 161 · see citing works
Younes, K., Borghesani, V., Montembeault, M., Spina, S., Mandelli, M. L., Welch, A. E., Weis, E., Callahan, P., Elahi, F. M., Hua, A. Y., et al. (2022) · Brain · doi:10.1093/brain/awac217 · Cited by 152 · see citing works
Self-citationDuval, C., Desgranges, B., de La Sayette, V., Belliard, S., Eustache, F., & Piolino, P. (2012) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2011.11.019 · Cited by 138 · see citing works
Brain structure correlates of emotion-based rash impulsivity
Muhlert, N., & Lawrence, A. D. (2015) · NeuroImage · doi:10.1016/j.neuroimage.2015.04.061 · Cited by 113 · see citing works
The Language Network Is Recruited but Not Required for Nonverbal Event Semantics
Ivanova, A. A., Mineroff, Z., Zimmerer, V., Kanwisher, N., Varley, R., & Fedorenko, E. (2021) · Neurobiology of Language · doi:10.1162/nol_a_00030 · Cited by 65 · see citing works
Hurlemann, R., Rehme, A. K., Diessel, M., Kukolja, J., Maier, W., Walter, H., & Cohen, M. X. (2008) · Journal of Neuroscience Methods · doi:10.1016/j.jneumeth.2008.04.004 · Cited by 51 · see citing works
Toller, G., Mandelli, M. L., Cobigo, Y., Rosen, H. J., Kramer, J. H., Miller, B. L., Gorno-Tempini, M. L., & Rankin, K. P. (2022) · NeuroImage: Clinical · doi:10.1016/j.nicl.2022.102994 · Cited by 23 · see citing works
Self-citationVonk, J. M. J., Borghesani, V., Battistella, G., Younes, K., DeLeon, J., Welch, A., Hubbard, H. I., Miller, Z. A., Miller, B. L., & Gorno-Tempini, M. L. (2020) · Aphasiology · doi:10.1080/02687038.2019.1659935 · Cited by 16 · see citing works
“Scores from controls were derived from Gorno-Tempini et al. (2004) for the MMSE, Benson complex figure tasks, CVLT, Digit Span tasks, BNT, verbal fluency tasks (semantic and letter), Western Aphasia Battery tests, and Pyramids & Palm Trees test, from Watson et al. (2018) for the Stroop and PPVT,…”
BackgroundMethodInfluentialSelf-citation
- Authors
- Gorno-Tempini, M. L., Rankin, K. P., Woolley, J. D., Rosen, H. J., Phengrasamy, L., & Miller, B. L.
- Affiliations
- University of California, San Francisco (US)
- Venue
- Cortex
- Publisher
- Elsevier BV
- Published
- 2004-01
- Volume
- 40
- Issue
- 4-5
- Pages
- 631-644
- ISSN
- 0010-9452 (ISSN-L)
- OpenAlex
- W2018595475
- Cited by
- 164
- References
- 57
- Citations by year
- 2026: 2 · 2025: 6 · 2024: 6 · 2023: 2 · 2022: 8 · 2021: 4 · 2020: 9 · 2019: 10 · 2018: 4 · 2017: 11 · 2016: 10 · 2015: 8 · 2014: 11 · 2013: 9 · 2012: 9
- FWCI
- 4.35
- Type
- journal article
- Language
- en
- Open access
- green
- escholarship.org
- Licence
- Locations
- Cortex (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- Funders
- Sandler Foundation
- Canadian Centre for Applied Research in Cancer Control
- National Institute on Aging (P30AG010129, AG 05142, P50AG016570, AG 16570, P50-AG05142, AG-10129, P50AG005142, P01AG019724)
- National Institute of Mental Health (R25MH060482)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- frontotemporal lobar degeneration, semantic dementia, behavior change, personality change, semantic memory impairment, food recognition, face recognition (cognition), amygdala atrophy, hippocampal complex, collateral sulcus, insula, neuroticism, empathy, early diagnosis
- MeSH terms
- Activities of Daily Living, Aged, Amnesia, Amygdala, Atrophy, Mental Disorders, Dementia, Dominance, Cerebral, Empathy, Female, Hippocampus, Humans, Image Processing, Computer-Assisted, Interpersonal Relations, Magnetic Resonance Imaging, Neuropsychological Tests, Personality, Social Behavior, Temporal Lobe
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
