Clinical and pathological characteristics of primary progressive aphasia and frontal dementia

  • A. Kertesz
  • D. Munoz
Part of the Journal of Neural Transmission Supplement book series (NEURAL SUPPL, volume 47)


The recently described conditions of primary progressive aphasia and frontal lobe dementia overlap clinically and pathologically to a considerable extent with each other and with clinical and pathological descriptions of Pick’s disease. Our clinical and neuropathological experience is summarized, leading us to the conclusion that the degree of overlap justifies the concept of “Pick complex” to include, in addition to the above, corticobasal ganglionic degeneration and some instances motor neuron disease with dementia.


Multiple System Atrophy Neurol Neurosurg Psychiatry Motor Neuron Disease Primary Progressive Aphasia Posterior Cortical Atrophy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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  1. Alzheimer A (1907) Über eine eigenartige Erkrankung der Hirntinde. Allg Z Psychiatrie 64: 146–148Google Scholar
  2. American Psychiatric Association (1987) Diagnostic and statistical manual of mental disorders (DSM-III-R). American Psychiatric Association, Washington DCGoogle Scholar
  3. Benson DF, Davis RJ, Snyder J (1988) Posterior cortical atrophy. Arch Neurol 45: 789–793PubMedCrossRefGoogle Scholar
  4. Benson DF, Zaias BW (1991) Progressive aphasia: a case with postmortem correlation. Neuropsychiatr Neuropsychol Behav Neurol 4: 115–223Google Scholar
  5. Braak H, Braak E (1989) Cortical and subcortical argyrophilic grains characterize a disease associated with adult onset dementia. Neuropathol Appl Neurobiol 15: 13–26PubMedCrossRefGoogle Scholar
  6. Brun A (1987) Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr 6: 193–208PubMedCrossRefGoogle Scholar
  7. Caron M (1934) Etude clinique de la maladie Pick. Vigot, ParisGoogle Scholar
  8. Caselli RJ, Windebank AJ, Petersen RC, Komori T, Parisi JE, Okazaki E, Iverson R, Dinapoli RP, Graff-Radford NR, Stein SD (1993) Rapidly progressive aphasic dementia and motor neuron disease. Ann Neurol 33: 200–207PubMedCrossRefGoogle Scholar
  9. Constantinidis J, Richard J, Tissot R (1974) Pick’s disease. Histological and clinical correlations. Eur Neurol 11: 208–217PubMedCrossRefGoogle Scholar
  10. Gustafson L (1987) Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 6: 209–223PubMedCrossRefGoogle Scholar
  11. Hodges JR, Patterson K, Oxbury S, Funnell E (1992) Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain 115: 1783–1806PubMedCrossRefGoogle Scholar
  12. Holland AL, McBurney DH, Moossy J, Reinmuth OM (1985) The dissolution of language in Pick’s disease with neurofibrillary tangles: a case study. Brain Lang 24: 36–58PubMedCrossRefGoogle Scholar
  13. Jagust WJ, Reed BR, Seab JP, Kramer JH, Budinger TF (1989) Clinical-physiologic correlates of Alzheimer’s disease and frontal lobe dementia. Am J Physiol Imag 4: 89–96Google Scholar
  14. Karbe H, Kertesz A, Polk M (1993) Profiles of language impairment in primary progressive aphasia. Arch Neurol 50: 193–201PubMedCrossRefGoogle Scholar
  15. Kertesz A, Appell J, Fisman M (1986) The dissolution of language in Alzheimer’s disease. Can J Neurol Sci 13: 415–418PubMedGoogle Scholar
  16. Kertesz A, Davidson W, Fox H (1995) Clinical and behavioral criteria in the diagnosis of frontal lobe dementia. Neurology 45 [Suppl 4]: A273Google Scholar
  17. Kertesz A, Hudson L, Mackenzie IRA, Munoz DG (1994) The pathology and nosology of primary progressive aphasia. Neurology 44: 2065–2072PubMedGoogle Scholar
  18. Knopman DS, Mastri AR, Frey II WH, Sung JH, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40: 251–256PubMedGoogle Scholar
  19. Kosaka K, Ikeda K, Kobayashi K, Mehraein P (1991) Striatopallidonigral degeneration in Pick’s disease. J Neurol 238: 151–160PubMedCrossRefGoogle Scholar
  20. Lang AE, Bergeron C, Pollanen MS, et al (1994) Parietal Pick’s disease mimicking corticobasal ganglionic degeneration. Neurology 44: 1436–1440PubMedGoogle Scholar
  21. Lippa CF, Cohen R, Smith TW, Drachman DA (1991) Primary progressive aphasia with focal neuronal achromasia. Neurology 41: 882–886PubMedGoogle Scholar
  22. Lüers T (1947) Ueber den Verfall der Sprache bei der Pickschen Krankheit (umschniebene Atrophie der Grosshirnrinde). Z Ges Neurol Psychiatrie 179: 94–131CrossRefGoogle Scholar
  23. Lynch T, Sano M, Marder KS, et al (1994) Clinical characteristics of a family with chromosome 17 linked disinhibition-dementia-parkinsonism-amyotrophy complex. Neurology 44: 1878–1884PubMedGoogle Scholar
  24. Mandell AM, Alexander MP, Carpenter S (1989) Creutzfeldt-Jakob disease presenting as isolated aphasia. Neurology 39: 55–58PubMedGoogle Scholar
  25. Mesulam MM (1982) Slowly progressive aphasia without dementia. Ann Neurol 11: 592–598PubMedCrossRefGoogle Scholar
  26. Mesulam MM (1987) Primary progressive aphasia — differentiation from Alzheimer’s disease. Ann Neurol 22: 533–534PubMedCrossRefGoogle Scholar
  27. Miller BL, Cummings JL, Villanueva-Meyer J, Boone K, Mehringer CM, Lesser IM, Mena I (1991) Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics. Neurology 14: 1374–1382Google Scholar
  28. Morris JC, Cole M, Banker BQ, Wright D (1984) Hereditary dysphasic dementia and the Pick-Alzheimer spectrum. Ann Neurol 16: 455–466PubMedCrossRefGoogle Scholar
  29. Neary D, Snowden JS, Bowen DM, Sims NR, Mann DMA, Benton JS, Northen B, Yates PO, Davison AN (1986) Neuropsychological syndromes in presenile dementia due to cerebral atrophy. J Neurol Neurosurg Psychiatry 49: 163–174PubMedCrossRefGoogle Scholar
  30. Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, MacDermott N (1990) Frontal lobe dementia and motor neurone disease. J Neurol Neurosurg Psychiatry 53: 23–32PubMedCrossRefGoogle Scholar
  31. Neary D, Snowden JS, Shields RA, Burjan AWI, Northen B, MacDermott N, Prescott MC, Testsa HJ (1987) Single photon emission tomography using 99m Tc-AM-PM in the investigation of dementia. J Neurol Neurosurg Psychiatry 50: 1101–1109PubMedCrossRefGoogle Scholar
  32. Pick A (1892) -Ober die Beziehungen der senilen Hirnatrophie zur Aphasie. Prag Med Wochenschr 17: 165–167Google Scholar
  33. Pogacar S, Williams RG (1984) Alzheimer’s disease presenting as slowly progressive aphasia. RI Med J 67: 181–185Google Scholar
  34. Rebeiz JJ, Kolodny EH, Richardson EP Jr (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18: 20–33PubMedCrossRefGoogle Scholar
  35. Rice GPA, Paty DW, Ball MJ, Tatham R, Kertesz A (1980) Spongiform encephalopathy of long duration: a family study. Can J Neurol Sci 7: 171–175PubMedGoogle Scholar
  36. Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Horneykiewicz O, Black SI (1990) Cortical-basal ganglionic degeneration. Neurology 40: 1203–1212PubMedGoogle Scholar
  37. Rinne JO, Lee MS, Thompson PD, et al (1994) Corticobasal degeneration. A clinical study of 36 cases. Brain 117: 1183–1196PubMedCrossRefGoogle Scholar
  38. Rosenfeld M (1909) Die partielle Grosshirnatrophie. J Psychol Neurol 14: 115–130Google Scholar
  39. Snowden JS, Goulding PJ, Neary D (1989) Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 2: 167–182Google Scholar
  40. Starkstein SE, Migliorelli R, Teson A, Sabe L, Vazquez S, Turjanski M, Robinson RG, Leiguarda R (1994) Specificity of changes in cerebral blood flow in patients with frontal lobe dementia. J Neurol Neurosurg Psychiatry 57: 790–796PubMedCrossRefGoogle Scholar
  41. Tariska I (1970) Circumscribed cerebral atrophy in Alzheimer’s disease: a pathological study. In: Wolstenhome GEW, O’Connor M (eds) Alzheimer disease and related conditions. Churchill, London, pp 51–69Google Scholar
  42. The Lund and Manchester Groups (1994) Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 57: 416–418CrossRefGoogle Scholar
  43. Thompson PD, Marsden CD (1992) Corticobasal degeneration. In: Bailliere’s clinical neurology, vol 1. Bailliere-Tindall, London, pp 677–687Google Scholar
  44. Tissot R, Constantinidis J, Richard J (1985) Pick’s disease. In: Frederiks JAM (ed) Handbook of clinical neurology, vol 2 (46). Neurobehavioural disorders. Elsevier, Amsterdam, pp 233–246Google Scholar
  45. Wechsler AF (1977) Presenile dementia presenting as aphasia. J Neurol Neurosurg Psychiatry 40: 303–305PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • A. Kertesz
    • 2
  • D. Munoz
    • 1
  1. 1.Department of Pathology, Division of NeuropathologyUniversity of Western OntarioLondonCanada
  2. 2.Department of Clinical Neurological Sciences, St. Joseph’s Health CentreUniversity of Western OntarioLondonCanada

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