Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-76fb5796d-skm99 Total loading time: 0 Render date: 2024-04-27T17:26:01.134Z Has data issue: false hasContentIssue false

8 - Clinical symptoms and neuropathology in organic dementing disorders affecting the frontal lobes

Published online by Cambridge University Press:  11 September 2009

By
Arne Brun  and
Lars Gustafson
Edited by
Jarl Risberg  and
Jordan Grafman
Jarl Risberg
Affiliation:
Lunds Universitet, Sweden
Jordan Grafman
Affiliation:
National Institute of Health, Bethesda, MD, USA
Get access

Summary

Introduction

Historically the clinical importance of localized cortical atrophy within the frontal and the temporal lobes was first described by Arnold Pick, who pointed out the relationship between these lesions and aphasia and behavioral changes, “thereby bringing neuropathology and psychiatry into closer union … so that the latter may be brought nearer to understanding” (Pick, 1892). Pick's first cases showed mainly temporal lobe involvement and the association with frontal lobe atrophy appeared in later publications. The histopathology of this lobar atrophy was given by Alzheimer (1911) and the clinico-pathological entity of Pick's disease (PiD) was established in the 1920s (Onari & Spatz, 1926; Stertz, 1926). Van Mansvelt (1954) reviewed 196 published cases and found an important variation of the distribution of pathology with frontotemporal atrophy in 54%, mainly frontal atrophy in 25% and mainly temporal atrophy in 17% of the cases. Moreover, the cortical atrophy was asymmetric with left-sided predominance in 46% and right-sided predominance in 17%. The different degenerative patterns were also revealed as variations of the clinical manifestations. Since then there has been an increasing interest in brain disorders specifically affecting the frontal lobes and their often devastating effects on a variety of mental functions.

In this chapter we will first discuss some possible explanations why the frontal lobes are such frequent targets for a variety of damaging processes. After that some general mechanisms of importance for understanding the relationship between symptoms and brain changes will be described.

Type
Chapter
Information
The Frontal Lobes
Development, Function and Pathology
 , pp. 199 - 221
Publisher: Cambridge University Press
Print publication year: 2006

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

Alzheimer, A. (1911). Über eigenartige Krankheitsfälle der späteren Alters. Zeitschrift für die Gesamte Neurologie und Psychiatrie, 4, 356–85.CrossRefGoogle Scholar
Andin, U., Gustafson, L., Passant, U. & Brun, A. (2005). A clinico-pathological study of heart and brain lesions in vascular dementia. Dementia and Geriatric Cognitive Disorders, 19, 222–8.CrossRefGoogle ScholarPubMed
Andrew, J. & Nathan, P. W. (1964). Lesions on the anterior frontal lobes and disturbances of micturition and defaecation. Brain, 87, 233–62.CrossRefGoogle ScholarPubMed
Aylward, E. H., Roberts-Twillie, J. V., Barta, P. E., et al. (1994). Basal ganglia volumes and white matter hyperintensities in patients with bipolar disorder. American Journal of Psychiatry, 151, 687–93.Google ScholarPubMed
Bennett, D. A., Gilley, D. W., Lee, S. & Cochran, E. J. (1994). White matter changes: neuronal manifestations of Binswanger's disease and clinical correlates in Alzheimer's disease. Dementia, 5, 148–52.Google Scholar
Binswanger, O. (1894). Die Abgrenzung der allgemeinen progressiven Paralyse. Berliner Klinische Wochenschrift, 31, 1103–5, 1137–9, 1180–6.Google Scholar
Brun, A. (1965). The subpial granular layer of the foetal cerebral cortex in man. Its ontogeny and significance in congenital cortical malformations. Thesis. Acta Pathologica et Microbiologica Scandinavica, 179 (Suppl.), 1–98.Google Scholar
Brun, A. (1987). Frontal lobe degeneration of non-Alzheimer type. 1. Neuropathology. Archives of Gerontology and Geriatrics, 6, 193–208.CrossRefGoogle Scholar
Brun, A. (1999). The emergence of the frontal lobe and its morbidity, as opposed to the central lobe. Dementia and Geriatric Cognitive Disorders, 10 (Suppl.), 3–5.CrossRefGoogle ScholarPubMed
Brun, A. & Englund, E. (1981). Regional pattern of degeneration in Alzheimer's disease: neuronal loss and histopathological grading. Histopathology, 5, 549–64.CrossRefGoogle ScholarPubMed
Brun, A., Englund, B.Gustafson, L.et al. (1994). Clinical and neuropathological criteria for frontotemporal dementia. Journal of Neurology, Neurosurgery and Psychiatry, 57, 416–18.Google Scholar
Brun, A. & Gustafson, L. (1976). Distribution of cerebral degeneration in Alzheimer's disease. A clinico-pathological study. Archiv für Psychiatrie und Nervenkrankheiten, 223, 15–33.CrossRefGoogle ScholarPubMed
Brun, A. & Gustafson, L. (1991). Psychopathology and frontal lobe involvement in organic dementia. In Alzheimer's Disease: Basic Mechanisms, Diagnosis and Therapeutic Strategies, ed. Iqbal, K., McLachlan, D. R. C., Winblad, B.et al. London: John Wiley & Sons, pp. 27–33.Google Scholar
Brun, A. & Gustafson, L. (1993). I. The Lund longitudinal dementia study: A 25-year perspective on neuropathology, differential diagnosis and treatment. In Alzheimer's Disease: Advances in Clinical and Basic Research, ed. Corain, B., Iqbal, K., Nicolini, M.et al. London: John Wiley & Sons, pp. 4–18.Google Scholar
Chui, H. C., Teng, E. L., Henderson, V. W. & Moy, A. C. (1985). Clinical subtypes of dementia of the Alzheimer type. Neurology, 35, 1544–50.CrossRefGoogle ScholarPubMed
Corbin, J. G., Nery, S. & Fishell, G. (2001). Telencephalic cells take a tangent: non-radial migration in the mammalian forebrain. Nature Neuroscience, 4, 1177–82.CrossRefGoogle Scholar
Cummings, J. L. (1994). Vascular subcortical dementias: clinical aspects. Dementia, 5, 177–80.Google ScholarPubMed
Groot, J. C., Leeuw, F. E., Oudkerk, M., et al. (2000). Cerebral white matter lesions and depressive symptoms in elderly adults. Archives of General Psychiatry, 57, 1071–76.CrossRefGoogle ScholarPubMed
Elfgren, C., Passant, U. & Risberg, J. (1993). Neuropsychological findings in frontal lobe dementia. Dementia, 4, 214–19.Google ScholarPubMed
Elfgren, C., Ryding, E. & Passant, U. (1996). Performance on neuropsychological tests related to single photon emission computerised tomography findings in frontotemporal dementia. British Journal of Psychiatry, 169, 416–22.CrossRefGoogle ScholarPubMed
Escourolle, R. (1958). La Maladie de Pick. Étude Critique d'Ensemble et Synthèse Anatomo-Clinique, Paris: R Foulon.Google Scholar
Eslinger, P. J., Warner, G. C., Grattan, L. M. & Easton, J. D. (1991). “Frontal lobe” utilization associated with paramedian thalamic infarction. Neurology, 41, 450–2.CrossRefGoogle ScholarPubMed
European Concerted Action on Pick's Disease Consortium, European Concerted Action on Pick's Disease (ECAPD) Consortium (1998). Provisional clinical and neuroradiological criteria for the diagnosis of Pick's disease. European Neurology, 5, 519–20.CrossRefGoogle Scholar
Floel, A. H., Lohmann, S. & Knecht, S. (2002). Neuropsychological disorders in amyotrophic lateral sclerosis. Nervenartz, 73, 1144–52.Google ScholarPubMed
Foster, N. L., Wilhelmsen, K., Sima, A. A. F., et al., and conference participants (1997). Frontotemporal dementia and parkinsonism linked to chromosome 17: A consensus conference. Annals of Neurology, 41, 706–15.CrossRefGoogle ScholarPubMed
Fredriksson, K., Brun, A. & Gustafson, L. (1992). Pure subcortical arteriosclerotic encephalopathy (Binswanger's disease): A clinicopathological study. Part 1: Clinical features. Cerebrovascular Diseases, 2, 82–6.CrossRefGoogle Scholar
Frisoni, G. B., Rozzini, L., Gozzetti, A., et al. (1999). Behavioral syndromes in Alzheimer's disease: description and correlates. Dementia and Geriatric Cognitive Disorders, 10, 130–8.CrossRefGoogle ScholarPubMed
Green, J., Morris, J. C., Sandson, J., McKeel, D. W. Jr & Miller, J. W. (1990). Progressive aphasia: a precursor of global dementia?Neurology, 40, 423–9.CrossRefGoogle ScholarPubMed
Greenwald, B. S., Kramer-Ginsberg, E., Krishnan, K. R., et al. (1998). Neuroanatomic localization of magnetic resonance imaging signal hyperintensities in geriatric depression. Stroke, 29, 613–17.CrossRefGoogle ScholarPubMed
Gregory, C. A. & Hodges, J. R. (1996). Clinical features of frontal lobe dementia in comparison to Alzheimer's disease. Journal of Neural Transmission, 47 (Suppl.), 103–23.CrossRefGoogle ScholarPubMed
Groen, J. J. & Endtz, L. J. (1982). Hereditary Pick's disease: second re-examination of the large family and discussion of other hereditary cases, with particular reference to electroencephalography, and computerized tomography. Brain, 105, 443–59.CrossRefGoogle ScholarPubMed
Guiraud, P. (1956). Psychiatrie Clinique. Paris: Le Francois.Google Scholar
Gustafson, L. (1987). Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Archives of Gerontology and Geriatrics, 6, 209–23.CrossRefGoogle ScholarPubMed
Gustafson, L. (1993). Clinical picture of frontal lobe degeneration of non-Alzheimer type. Dementia, 4, 143–8.Google ScholarPubMed
Gustafson, L., Passant, U., Gräsbeck, A. & Brun, A. (2001). Clinical variability of frontotemporal dementia. In Contemporary Neuropsychiatry, ed. Miyoschi, K., Shapiro, C., Gavilia, M. & Morika, Y.. Tokyo: Springer-Verlag, pp. 152–7.CrossRefGoogle Scholar
Gustafson, L. & Risberg, J. (1993). Deceptions and delusions in Alzheimer's disease and frontal lobe dementia. In Delusions and Hallucinations in Old Age, ed. Katona, C. & Levy, R.. London: Gaskell, pp. 216–25.Google Scholar
Hagberg, B. (1987). Behaviour correlates to frontal lobe dysfunction. Archives of Gerontology and Geriatrics, 6, 311–21.CrossRefGoogle ScholarPubMed
Harwood, D. G., Sultzer, D. L., Feil, D., et al. (2005). Frontal lobe hypometabolism and impaired insight in Alzheimer disease. American Journal of Geriatric Psychiatry, 13, 934–41.CrossRefGoogle ScholarPubMed
Hashimoto, R., Yoshida, M. & Tanaka, Y. (1995). Utilization after right thalamic infarction. European Neurology, 35, 58–62.CrossRefGoogle ScholarPubMed
Hodges, J. R., Davies, R. R., Xuereb, J. H., et al. (2004). Clinicopathological correlates in frontotemporal dementia. Annals of Neurology, 56, 399–406.CrossRefGoogle ScholarPubMed
Janota, I. (1981). Dementia, deep white matter damage and hypertension: ‘Binswanger's disease’. Psychological Medicine, 11, 39–48.CrossRefGoogle ScholarPubMed
Jellinger, K. & Neumayer, E. (1964). Binswanger's progressive subcortical vascular encephalopathy. A clinico-neuropathological study. Archiv für Psychiatrie und Nervenkrankheiten, 205, 523–54.CrossRefGoogle ScholarPubMed
Jimenez, D., Rivera, R., Lopez-Mascaraque, L. & DeCarlos, J. A. (2003). Origin of the cortical layer 1 in rodents. Developmental Neuroscience, 23, 105–15.CrossRefGoogle Scholar
Johanson, A., Gustafson, L., Smith, G. J. W., et al. (1990). Adaptation in different types of dementia and in normal elderly subjects. Dementia, 1, 95–101.Google Scholar
Johanson, A. & Hagberg, B. (1989). Psychometric characteristics in patients with frontal lobe degeneration of non-Alzheimer type. Archives of Gerontology and Geriatrics, 8, 129–37.CrossRefGoogle ScholarPubMed
Johnson, J. K., Head, E., Kim, R., Starr, A. & Cotman, C. W. (1999). Clinical and pathological evidence for a frontal variant of Alzheimer disease. Archives of Neurology, 56, 1233–9.CrossRefGoogle ScholarPubMed
Kertesz, A. & Martinez-Lage, P. (1998). Cognitive changes in corticobasal degeneration. In Pick's Disease and Pick Complex, ed. Kertesz, A. and Munoz, D. G.. New York: Wiley-Liss, Inc., pp. 121–128.Google ScholarPubMed
Knopman, D. S., Mastri, A. R., Frey, W. H., 2nd, , Sung, J. H. & Rustan, T. (1990). Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology, 40, 251–6.CrossRefGoogle ScholarPubMed
Lhermitte, F., Pillon, B. & Serdaru, M. (1986). Human autonomy and the frontal lobes. Part I: Imitation and utilization: a neuropsychological study of 75 patients. Annals of Neurology, 19, 326–34.CrossRefGoogle ScholarPubMed
Lomen-Hoerth, C., Anderson, T. & Miller, B. (2002). The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology, 59, 1077–9.CrossRefGoogle ScholarPubMed
Luria, A. R. (1958). Brain disorders and language analysis. Language and Speech, 1, 14–34.CrossRefGoogle Scholar
Luria, A. R. (1973). The Working Brain. An Introduction to Neuropsychology. London: Allen Lane, The Penguin Press.Google Scholar
Mackenzie, I. R. & Feldman, H. (2003). Neuronal intranuclear inclusions distinguish familial FTD-MND type from sporadic disease. Acta Neuropathologica, 105, 543–8.Google Scholar
Mesulam, M. M. (1982). Slowly progressive aphasia without generalized dementia. Annals of Neurology, 11, 592–8.CrossRefGoogle ScholarPubMed
Miller, B. L., Ikonte, C., Ponton, M., et al. (1997). A study of the Lund-Manchester research criteria for frontotemporal dementia: clinical and single-photon emission CT correlations. Neurology, 48, 937–42.CrossRefGoogle ScholarPubMed
Miller, B. L., Lesser, I. M., Boone, K. B., et al. (1991). Brain lesions and cognitive function in late-life psychosis. British Journal of Psychiatry, 158, 76–82.CrossRefGoogle ScholarPubMed
Mitsuyama, Y. (1984). Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. Journal of Neurology, Neurosurgery and Psychiatry, 47, 953–9.CrossRefGoogle ScholarPubMed
Morita, K., Kaiya, H., Ikeda, T. & Namba, M. (1987). Presenile dementia combined with amyotrophy. A review of 34 Japanese cases. Archives of Gerontology and Geriatrics, 6, 263–77.CrossRefGoogle ScholarPubMed
Neary, D., Snowden, J. S., Gustafson, L., et al. (1998). Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology, 51, 1546–54.CrossRefGoogle ScholarPubMed
Neary, D., Snowden, J. S. & Mann, D. M. A. (1993). The clinical pathological correlates of lobar atrophy. Dementia, 4, 154–9.Google ScholarPubMed
Neary, D., Snowden, J. & Mann, D. (2005). Frontotemporal dementia. Lancet Neurology, 4, 771–80.CrossRefGoogle ScholarPubMed
Neary, D., Snowden, J. S., Mann, D. M., et al. (1990). Frontal lobe dementia and motor neuron disease. Journal of Neurology, Neurosurgery and Psychiatry, 53, 23–32.CrossRefGoogle ScholarPubMed
Neary, D., Snowden, J. S., Northen, B. & Goulding, P. (1988). Dementia of frontal lobe type. Journal of Neurology, Neurosurgery and Psychiatry, 51, 353–61.CrossRefGoogle ScholarPubMed
Nimchinsky, E. A., Vogt, B. A., Morrison, J. H. & Hof, P. R. (1995). Spindle neurons of the human anterior cingulate cortex. Journal of Comparative Neurology, 355, 27–37.CrossRefGoogle ScholarPubMed
Nyatsanza, S., Shetty, T., Gregory, C., et al. (2003). A study of stereotypic behaviours in Alzheimer's disease and frontal and temporal variant frontotemporal dementia. Journal of Neurology, Neurosurgery and Psychiatry, 74, 1398–402.CrossRefGoogle ScholarPubMed
O'Brien, J., Ames, D., Chiu, E., et al. (1998). Severe deep white matter lesions and outcome in elderly patients with major depressive disorder: follow up study. British Medical Journal, 317, 982–4.CrossRefGoogle ScholarPubMed
Onari, K. & Spatz, H. (1926). Anatomische Beiträge zur Lehre von der Pickschen umschriebenen Grosshirnrinden-Atrophie (‘Picksche Krankheit’). Zentralblad für Neurologie, 101, 470–511.Google Scholar
Pachana, N. A., Boone, K. B., Miller, B. L., Cummings, J. L. & Berman, N. (1996). Comparison of neuropsychological functioning in Alzheimer's disease and frontotemporal dementia. Journal of the International Neuropsychological Society, 2, 505–10.CrossRefGoogle ScholarPubMed
Pasquier, F., Lebert, F. & Petit, H. (1995). Dementia, apathy, and thalamic infarcts. Behavioral Neurology, 8, 208–14.Google Scholar
Pasquier, F., Richard, F. & Lebert, F. (2004). Natural history of frontotemporal dementia: comparison with Alzheimer's disease. Dementia and Geriatric Cognitive Disorders, 17, 253–7.CrossRefGoogle ScholarPubMed
Passant, U., Elfgren, C., Englund, E. & Gustafson, L. (2005a). Psychiatric symptoms and their psychosocial consequences in frontotemporal dementia. Alzheimer Disease and Associated Disorders, 19 (Suppl. 1), S15–S18.CrossRefGoogle Scholar
Passant, U., Rosen, I., Gustafson, L. & Englund, E. (2005b). The heterogeneity of frontotemporal dementia with regard to initial symptoms, qEEG and neuropathology. International Journal of Geriatric Psychiatry, 20, 983–8.CrossRefGoogle Scholar
Pick, A. (1892). Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medizinische Wochenschrift, 17, 165–7.Google Scholar
Poirier, J., Barbizet, J., Gaston, A. & Meyrignac, C. (1983). Thalamic dementia. Expansive lacunae of the thalamo-paramedian mesencephalic area. Hydrocephalus caused by stenosis of the aqueduct of Sylvius. Revue Neurologique (Paris), 139, 349–58.Google ScholarPubMed
Richfield, E. K., Twyman, R. & Berent, S. (1987). Neurological syndrome following bilateral damage to the head of the caudate nuclei. Annals of Neurology, 22, 768–71.CrossRefGoogle ScholarPubMed
Risberg, J. & Gustafson, L. (1988). Regional cerebral blood flow in psychiatric disorders. In Handbook of Regional Cerebral Blood Flow, ed. Knezevic, S., Maximilian, V., Mubrin, Z., Prohovnik, I. & Wade, J.. Hillsdale, New Jersey: Lawrence Erlbaum, pp. 219–40.Google Scholar
Risberg, J., Passant, U., Warkentin, S. & Gustafson, L. (1993). Regional cerebral blood flow in frontal lobe dementia of non-Alzheimer type. Dementia, 4, 186–7.Google ScholarPubMed
Robinson, R. G., Kubos, K. L., Starr, L. B., et al. (1983). Mood changes in stroke patients: relationship to lesion location. Comprehensive Psychiatry, 24, 555–66.CrossRefGoogle ScholarPubMed
Salazar, A. M., Masters, C. L., Gajdusek, D. C. & Gibbs, C. J., Jr. (1983). Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Annals of Neurology, 14, 17–26.CrossRefGoogle ScholarPubMed
Schneider, C. (1927). Über Picksche Krankheit. Monatschrift für Psychiatrie und Neurologie, 65, 230–75.CrossRefGoogle Scholar
Segarra, J. M. (1970). Cerebral vascular disease and. I. The syndrome of the mesencephalic artery (basilar artery bifurcation). Archives of Neurology, 22, 408–18.CrossRefGoogle Scholar
Shallice, T., Burgess, P. W., Schon, F. & Baxter, D. M. (1989). The origins of utilization behaviour. Brain, 112, 1587–98.CrossRefGoogle ScholarPubMed
Snowden, J. S., Bathgate, D., Varma, A., et al. (2001). Distinct behavioural profiles in frontotemporal dementia and semantic dementia. Journal of Neurology, Neurosurgery and Psychiatry, 70, 323–32.CrossRefGoogle ScholarPubMed
Snowden, J. S. & Neary, D. (1993). Progressive language dysfunction and lobar atrophy. Dementia, 4, 226–31.Google ScholarPubMed
Snowden, J. S., Neary, D. & Mann, D. M. A. (1996). Frontotemporal Lobar Degeneration: Fronto-Temporal Dementia, Progressive Aphasia, Semantic Dementia. Edinburgh: Churchill Livingstone.Google Scholar
Stertz, G. (1926). Über die Picksche Atrophie. Zentralblad für Neurologie, 101, 729–47.Google Scholar
Swanberg, M. M., Tractenberg, R. E., Mohs, R., Thal, L. J. & Cummings, J. L. (2004). Executive dysfunction in Alzheimer disease. Archives of Neurology, 61, 556–60.CrossRefGoogle ScholarPubMed
Tomonaga, M., Yamanouchi, H., Tohgi, H. & Kameyama, M. (1982). Clinicopathologic study of progressive subcortical vascular encephalopathy (Binswanger type) in the elderly. Journal of the American Geriatric Society, 30, 524–9.CrossRefGoogle Scholar
Mansfelt, J. (1954). Pick's Disease. A Syndrome of Lobar, Cerebral Atrophy; Its Clinico-Anatomical and Histopathological Types [Thesis] Enschede, Utrecht.Google Scholar
Wikström, J., Pateau, A., Palo, J., Sulkava, R. & Haltia, M. (1982). Classic amyotrophic lateral sclerosis with dementia. Archives of Neurology, 38, 681–3.CrossRefGoogle Scholar
Wilhelmsen, K. C., Lynch, T., Pavlou, E., Higgins, M. & Nygaard, T. G. (1994). Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21–22. The American Journal of Human Genetics, 55, 1159–65.Google ScholarPubMed
Zhukareva, V., Mann, D., Pickering-Brown, S., et al. (2002). Sporadic Pick's disease: a tauopathy characterized by a spectrum of pathological tau isoforms in gray and white matter. Annals of Neurology, 51, 730–9.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×