Hostname: page-component-cd9895bd7-q99xh Total loading time: 0 Render date: 2024-12-25T05:20:06.529Z Has data issue: false hasContentIssue false

Nonfluent progressive aphasia and semantic dementia: A comparative neuropsychological study

Published online by Cambridge University Press:  26 February 2009

John R. Hodges
Affiliation:
University Neurology Unit, University of Cambridge, Cambridge CB2 2QQ, UK
Karalyn Patterson
Affiliation:
MRC Applied Psychology Unit, Cambridge CB2 2EF, UK

Abstract

Two patients with nonfluent progressive aphasia, who have been studied longitudinally, are contrasted with a group of 5 patients with fluent progressive aphasia or semantic dementia. The most prominent feature of the nonfluent syndrome is the severe distortion of speech output with phonological errors and agrammatic sentence structure. This contrasts with the fluent, well articulated and syntactically correct, but empty, anomic speech found in semantic dementia. Performance on tests of comprehension separates the patient groups: The nonfluent patients show normal single-word comprehension, but marked impairment on tests of syntactic comprehension, while those with semantic dementia demonstrate the opposite pattern. Category fluency is severely defective in semantic dementia, but initial letter-based fluency is more impaired in the nonfluent syndrome. Performance on nonverbally mediated tests of semantic knowledge is impaired in semantic dementia only. The 2 forms of progressive aphasia have in common the sparing of perceptual and visuospatial skills, nonverbal problem solving abilities, and day-to-day (episodic) memory. Neuroradiological investigations have shown marked selective and striking inferolateral left temporal lobe atrophy in all 5 patients with semantic dementia. The changes in nonfluent progressive aphasia appear to be less focal and involve left perisylvian structures more diffusely. These 2 forms of progressive aphasia are, we argue, distinct in their manifestations. (JINS, 1996, 2, 511–524.)

Type
Research Article
Copyright
Copyright © The International Neuropsychological Society 1996

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

REFERENCES

Bayles, K.A, & Tolmoeda, D.K. (1983). Confrontational naming impairment in dementia. Brain and Language. 19, 98114.CrossRefGoogle ScholarPubMed
Beeker, J.T., Huff, R.J., Nebes, R.D., Holland, A., & Boller, F. (1988). Neuropsychological function in Alzheimer's disease: pattern of impairment and rates of progression. Archives of Neurology. 45, 263268.CrossRefGoogle Scholar
Benton, A.L., deS Hamsher, K., Varney, N.R., & Spreen, O. (1983a). Judgment of Line Orientation. New York: Oxford University Press.Google Scholar
Benton, A.L., deS Hamsher, K., Varney, N.R., & Spreen, O. (1983b). Contributions to Neuropsychological Assessment: A Clinical Manual. New York: Oxford University Press.Google Scholar
Bishop, D.V.M. (1989). Test for the Reception of Grammar (2nd ed.). Medical Research Council.Google Scholar
Butters, N., Granholm, E., Salmon, D.P., Grant, I., & Wolfe, J. (1987). Episodie and semantic memory: A comparison of amnesic and demented patients. Journal of Clinical and Experimental Neuropsychology, 9, 479497.CrossRefGoogle Scholar
Butters, N., Salmon, D.P., Cullum, C.M., Cairns, P., Troster, A.I., Jacobs, D., Moss, M., & Cermak, L.S. (1988). Differentiation of amnesic and demented patients with the Wcchslcr Memory Scale-Revised. Clinical Neuropsychology, 2, 133148.CrossRefGoogle Scholar
Caselli, R.J., Jack, C.R., Petersen, R.C., Wahner, H.W., & Yanagihara, T. (1992). Asymmetric cortical degenerative syndromes: Clinical and radiologic correlations. Neurology, 42, 14621468.CrossRefGoogle ScholarPubMed
Chertkow, H. & Bub, D. (1990). Semantic memory loss in dementia of Alzheimer's type. Brain, 113, 397417.CrossRefGoogle ScholarPubMed
Croot, K., Patterson, K.E., Hodges, J.R. (1996). Single word production in non-fluent progressive aphasia. Brain and Language (in press).Google Scholar
Crystal, H.A., Horoupian, D.S., Katzman, R., & Jotkowitz, S. (1982). Biopsy-proved Alzheimer disease presenting as a right parietal lobe syndrome. Annals of Neurology, 12, 186188.CrossRefGoogle ScholarPubMed
De Renzi, E. & Faglioni, P. (1978). Normative data and screening power of a shortened version of the Token Test. Cortex, 14, 4149.CrossRefGoogle ScholarPubMed
Folstein, M.F., Folstein, S.E., & McHugh, P.R. (1975). “Minimental state.” A practical method for grading the mental state of patients for the clinician. Journal of Psychiatric Research, 12, 189198.CrossRefGoogle Scholar
Foster, J.R. & Haggard, M.P. (1987). The four alternative auditory feature test (FAAF)—Linguistic and psychometric properties of the material with normative data in noise. British Journal of Audiology, 21, 165174.CrossRefGoogle ScholarPubMed
Franklin, S., Turner, J.E., & Ellis, A.W. (1992). The Action for Dysphasic Adults Comprehension Battery. York, UK: Human Neuropsychology Laboratory, University of York.Google Scholar
Goodglass, H. & Kaplan, E. (1976). The assessment of aphasia and related disorders. Philadelphia: Lea & Febiger.Google Scholar
Graff-Radford, N.R., Damasio, A.R., Hyman, B.T., Hart, M.N., Tranel, D., Damasio, H., Van Hoesen, G.W., & Rezai, K. (1990). Progressive aphasia in a patient with Pick's disease: A neuropsychological, radiologic, and anatomic study. Neurology, 40, 620626.CrossRefGoogle Scholar
Green, J., Morris, J.C., Sandson, J., MeKcel, D.W., & Miller, J.W. (1990). Progressive aphasia: A precursor of global dementia? Neurology, 40, 423429.CrossRefGoogle ScholarPubMed
Green, J.D.W., Patterson, K., Xuereb, J., & Hodges, J.R. (1996). Alzheimer's disease and nonfluent progressive aphasia. Archives of Neurology (in press).CrossRefGoogle Scholar
Hart, S. (1988). Language and dementia: A review. Psychological Medicine, 18, 99112.CrossRefGoogle ScholarPubMed
Hodges, J.R., Graham, N., & Patterson, K. (1995). Charting the progression in semantic dementia: Implications for the organization of semantic memory. Memory, 3, 463495.CrossRefGoogle ScholarPubMed
Hodges, J.R. & Patterson, K. (1995). Is semantic memory consistently impaired early in the course of Alzheimer's disease? Neuroanatomical and diagnostic implications. Neuropsychologia, 33, 441459.CrossRefGoogle ScholarPubMed
Hodges, J.R., Patterson, K., Oxbury, S., & Funnell, E. (1992a). Semantic dementia: Progressive fluent aphasia with temporal lobe atrophy. Brain, 115, 17831806.CrossRefGoogle ScholarPubMed
Hodges, J.R., Patterson, K., & Tyler, L.K. (1994). Loss of semantic memory: Implications for the modularity of mind. Cognitive Neuropsychology, 11, 505542.CrossRefGoogle Scholar
Hodges, J.R., Salmon, D.P., & Butters, N. (1990). Differential impairment of semantic and episodic memory in Alzheimer's and Huntington's diseases: A controlled prospective study. Journal of Neurology, Neurosurgery and Psychiatry, 53, 10891095.CrossRefGoogle Scholar
Hodges, J.R., Salmon, D.P., & Butters, N. (1991). The nature of the naming deficit in Alzheimer's and Huntington's disease. Brain, 114, 15471558.CrossRefGoogle ScholarPubMed
Hodges, J.R., Salmon, D.P., & Butlers, N. (1992b). Semantic memory impairment in Alzheimer's disease: Failure of access or degraded knowledge? Neuropsychologia. 30, 310314.CrossRefGoogle ScholarPubMed
Hof, P.R., Bouras, C., Constantinidis, J., & Morrison, J.H. (1990). Selective disconnection of specific visual association pathways in cases of Alzheimer's disease presenting with Balint's syndrome. Journal of Neuropathology and Experimental Neurology, 49, 168184.CrossRefGoogle ScholarPubMed
Howard, D. & Franklin, S. (1988). Missing the meaning? Cambridge, MA: MIT Press.Google Scholar
Howard, D. & Patterson, K. (1992). Pyramids and palm trees: A test of semantic access from pictures and words. Bury St Edmunds, UK: Thames Valley Test Company.Google Scholar
Huff, F.J., Corkin, S., & Growden, J.H. (1986). Semantic impairment and anomia in Alzheimer's disease. Brain and Language, 28, 235249.CrossRefGoogle ScholarPubMed
Humphreys, G.W. & Riddoeh, M.J. (1984). Routes to object constancy: Implications for neurological impairment of object constancy. Quarterly Journal of Experimental Psychology, 36, 384415.Google Scholar
Kaplan, E., Goodglass, H., & Weintraub, S. (1983). Boston Naming Test. Philadelphia: Lea and Febiger.Google Scholar
Karbe, H., Kertesz, A., & Polk, M. (1993). Profiles of language impairment in primary progressive aphasia. Archives of Neurology. 50, 193201.CrossRefGoogle ScholarPubMed
Kay, J., Lesser, R., & Coltheart, M. (1992). Psycholinguistic assessments of language processing in aphasia. Hove, UK: Lawrence Erlbaum Associates.Google Scholar
Kempler, D., Metter, E.J., Riege, W.H., Jackson, C.A., Benson, D.F., & Hanson, W.R. (1990). Slowly progressive aphasia: Three cases with language, memory, CT and PET data. Journal of Neurology, Neurosurgery and Psychiatry, 53, 987993.CrossRefGoogle ScholarPubMed
Kirshner, H.J., Webb, W.G., Kelly, M.P., & Wells, C.E. (1984). Language disturbance: An initial symptom of cortical degenerations and dementia. Archives of Neurology, 41, 491496.CrossRefGoogle ScholarPubMed
Mattis, S. (1988). Dementia Rating Scale. Windsor, UK: NFER-Nelson.Google Scholar
MeKenna, P. & Warrington, E.K. (1983). Graded Naming Test. Windsor, UK: NFER-Nelson.Google Scholar
Mesulam, M.M. (1982). Slowly progressive aphasia without generalized dementia. Annals of Neurology, 11, 592598.CrossRefGoogle ScholarPubMed
Mesulam, M.M. & Weintraub, S. (1992). Primary progressive aphasia. In Boiler, F. (Ed.), Heterogeneity of Alzheimer's disease (pp. 4366). Berlin: Springer-Verlag.CrossRefGoogle Scholar
Morris, J., McKcel, D.W.J., Fulling, K., Torack, R.M., & Berg, L. (1988). Validation of clinical diagnostic criteria for Alzheimer's disease. Annals of Neurology, 24, 1722.CrossRefGoogle ScholarPubMed
Morris, J.C., Heyman, A., Mohs, R.C., Hughes, J.P., van Belle, G., Fillcnbaum, G., Mellits, E.D., & Clark, C. (1989). The Consortium to Establish a Registry for Alzheimer's Disease (CERAD), 1: Clinical and neuropsychological assessment of Alzheimer's disease. Neurology, 39, 11591165.Google Scholar
Moss, H.E., Tyler, L.K., Hodges, J.R., & Patterson, K.E. (1995). Exploring the loss of semantic memory in semantic dementia: Evidence from a primed monitoring study. Neuropsychology, 9, 1626.CrossRefGoogle Scholar
Neary, D., Snowden, J.S., Bowen, D.M., Sims, N.R., Mann, D.M.A., Benton, J.S., Northen, B., Yates, P.O., & Davison, A.N. (1986). Neuropsychological syndromes in prcscnile dementia due to cerebral atrophy. Journal of Neurology, Neurosurgery and Psychiatry, 49, 163174.CrossRefGoogle ScholarPubMed
Ober, B.A., Dronkers, N.F., Koss, E., Delis, D.C., & Friedland, R.P. (1986). Retrieval from semantic memory in Alzheimertype dementia. Journal of Clinical and Experimental Neuropsychology, 8, 7592.CrossRefGoogle ScholarPubMed
Parkin, A.J. (1993). Progressive aphasia without dementia—A clinical and cognitive neuropsychological analysis. Brain and Language, 44, 201220.CrossRefGoogle ScholarPubMed
Patterson, K. & Hodges, J.R. (1992). Deterioration of word meaning: Implications for reading. Neuropsychologia, 30, 10251040.CrossRefGoogle ScholarPubMed
Patterson, K.E., Graham, N., & Hodges, J.R. (1994). The impact of semantic memory loss on phonological representations. Journal of Cognitive Neuroscience, 6, 5769.CrossRefGoogle ScholarPubMed
Pietrini, V., Nertempi, P., Vaglia, A., Revelio, M.G., Pinna, V., & Ferro-Milone, F. (1988). Recovery from herpes simplex encephalitis: Selective impairment of specific semantic categories with neuroradiological correlation. Journal of Neurology, Neurosurgery and Psychiatry, 51, 12841293.CrossRefGoogle ScholarPubMed
Poeck, K. & Luzzatti, C. (1988). Slowly progressive aphasia in three patients: The problem of accompanying neuropsychological deficit. Brain, 111, 151168.CrossRefGoogle Scholar
Pogacar, S. & Williams, R.S. (1984). Alzheimer's disease presenting as slowly progressive aphasia. Rhode Island Medical Journal, 67, 181185.Google ScholarPubMed
Price, B.H., Gurvit, H., Weintraub, S., Geuia, C., Leimkuhler, E., & Mesulam, M. (1993). Neuropsychological patterns and language deficits in 20 consecutive cases of autopsy-confirmed Alzheimer's disease. Archives of Neurology, 50, 931937.CrossRefGoogle ScholarPubMed
Randolph, C., Braun, A.R., Goldberg, T.E., & Chase, T.N. (1993). Semantic fluency in Alzheimer's, Parkinson's, and Huntington's disease: Dissociation of storage and retrieval failures. Neuropsychology, 7, 8288.CrossRefGoogle Scholar
Raven, J.C. (1962). Coloured Progressive Matrices Sets A. AB. B. London: H.K. Lewis.Google Scholar
Rey, A. (1941). L'examen psychologique dans les cas d'encephalopathie traumatique. Arch Psychologic, 28, 286340.Google Scholar
Riddoch, M.J. & Humphreys, G.W. (1987). Visual object processing in optic aphasia: A case of semantic access agnosia. Cognitive Neuropsychology, 4, 131185.CrossRefGoogle Scholar
Rosser, A. & Hodges, J.R. (1994). Initial letter and semantic category fluency in Alzheimer's disease, Huntington's disease and progressive supranuclear palsy. Journal of Neurology, Neurosurgery & Psychiatry, 57, 13891394.CrossRefGoogle ScholarPubMed
Saffran, E.M. & Schwartz, M.F. (1994). Of cabbages and things: semantic memory from a neuropsychological perspective—a tutorial review. In Umilta, C. & Moscovitch, M. (Eds.), Attention and performance XV (pp. 507536). Hove and London, UK: Lawrence Erlbaum.Google Scholar
Sagar, H.J. & Sullivan, E.V. (1988). Patterns of cognitive impairment in dementia. In Kennard, C. (Ed.), Recent advances in clinical neurology (Vol. 5, pp. 4786). Edinburgh, UK: Churchill Livingstone.Google Scholar
Sartori, G. & Job, R. (1988). The oyster with four legs: A neuropsychological study on the interaction of visual and semantic information. Cognitive Neuropsychology, 5, 105132.CrossRefGoogle Scholar
Scheltens, P., Ravid, R., & Kamphorst, W. (1994). Pathologic findings in a case of primary progressive aphasia. Neurology, 44, 279282.CrossRefGoogle Scholar
Snodgrass, J.G. & Vanderwart, M. (1980). A standardized set of 260 pictures: Norms for name agreement, familiarity and visual complexity. Journal of Experimental Psychology: Human Learning and Memory, 6, 174215.Google ScholarPubMed
Snowden, J.S., Goulding, P.J., & Neary, D. (1989). Semantic dementia: A form of circumscribed cerebral atrophy. Behavioural Neurology, 2, 167182.Google Scholar
Snowden, J.S., Neary, D., Mann, D.M.A., Goulding, P.J., & Testa, H.J. (1992). Progressive language disorder due to lobar atrophy. Annals of Neurology, 31, 174183.CrossRefGoogle ScholarPubMed
Spinnler, H. & Delia Sala, S. (1988). The role of clinical neuropsychology in the neurological diagnosis of Alzheimer's disease. Journal of Neurology, 235, 258271.CrossRefGoogle ScholarPubMed
Tyrrell, P.J., Warrington, E.K., Frackowiak, R.S.J., & Rossor, M.N. (1990). Heterogeneity in progressive aphasia due to focal cortical atrophy. A clinical and PET study. Brain, 113, 13211336.CrossRefGoogle ScholarPubMed
Warrington, E.K. (1975). Selective impairment of semantic memory. Quarterly Journal of Experimental Psychology, 27, 635657.CrossRefGoogle ScholarPubMed
Warrington, E.K. (1984). Recognition Memory Test. Windsor, UK: NFER Nelson.Google Scholar
Warrington, E.K. & James, M. (1991). The Visual Object and Space-Perception Battery. Bury St. Edmunds, UK: Thames Valley Test Company.Google Scholar
Warrington, E.K. & Shallice, T. (1984). Category specific semantic impairments. Brain, 107, 829854.Google ScholarPubMed
Wechsler, D.A. (1981). Wechsler Adult Intelligence Scale-Revised. New York: Psychological Corporation.Google Scholar
Wechsler, D.A. (1987). Wechsler Memory Scale-Revised. San Antonio, TX: Psychological Corporation.Google Scholar
Weintraub, S., Rubin, N.P., & Mesulam, M.-M. (1990). Primary progressive aphasia: Longitudinal course, profile, and language features. Archives of Neurology, 47, 13291335.CrossRefGoogle ScholarPubMed
Welsh, K., Butters, N., Hughes, J., Mohs, R., & Heyman, A. (1991). Detection of abnormal memory decline in mild cases of Alzheimer's disease using CERAD ncuropsychological measures. Archives of Neurology, 48, 278281.CrossRefGoogle ScholarPubMed
Welsh, K.A., Butters, N., Hughes, J.P., & Mohs, R.C. (1992). Detection and staging of dementia in Alzheimer's disease: Use of the neuropsychological measures developed for the Consortium to Establish a Registry for Alzheimer's disease. Archives of Neurology, 49, 448452.CrossRefGoogle ScholarPubMed