Advertisement

Cognitive deficits in non-Alzheimer’s degenerative diseases

  • B. Pillon
  • B. Dubois
  • Y. Agid
Part of the Journal of Neural Transmission Supplement book series (NEURAL SUPPL, volume 47)

Summary

Although observed in various brain disorders, dementia is particularly frequent in neurodegenerative diseases. Alzheimer’s disease is characterized by the association of progressive amnesia with either instrumental (aphasia, apraxia, agnosia) or behavioral (apathy, indifference, anosognosia) disorders, depending upon the location of the underlying neuronal lesions. By contrast, memory, linguistic, praxic, visuo-spatial or comportemental impairments are dissociated in more focal “lobar” atrophies, while planning and retrieval deficits predominate in movement disorders with dementia. Alzheimer’s and non-Alzheimer’s neurodegenerative diseases can therefore be distinguished insofar as the severity and location of the associated neuronal lesions differ.

Dementia may be observed in various brain diseases, either vascular, metabolic, demyelinating, traumatic, infectious, inflammatory, neoplastic or hydrocephalus (Chui, 1989). It is particularly frequent in neurodegenerative diseases. The recent clinical description of focal lobar atrophies (Weintraub and Mesulam, 1993) and the analysis of cognitive impairment observed in diseases with movement disorders (Cummings and Benson, 1984) have changed the conception of dementia, that may no more be defined as a global deterioration of higher cortical functions. The relative specificity of the cognitive picture of each disease depends on the location of the underlying neuronal lesions. Together with other tools, such as the neurological examination or the functional imagery, the neuropsychological exam may contribute to characterize the clinical picture of a patient with non-Alzheimer’s degenerative disease and therefore to determine a clinical diagnosis, that remains probable till the neuropathological confrontation.

Keywords

Multiple System Atrophy Neurol Neurosurg Psychiatry Progressive Supranuclear Palsy Corticobasal Degeneration Lewy Body Dementia 
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.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Albert ML, Feldman RG, Willis AL (1974) The subcortical dementia of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37: 121–130PubMedCrossRefGoogle Scholar
  2. Agid Y, Ruberg M, Dubois B, Pillon B (1987) Anatomo-clinical and biochemical concepts of subcortical dementia. In: Stahl SM, Iversen SD, Goodman EC (eds) Cognitive neurochemistry. Oxford University Press, New York Oxford, pp 248–271Google Scholar
  3. American Psychiatric Association (1994) Diagnostic and statistical manual of mental disorders, 4th edn. DSM-IVTM. American Psychiatric Association, Washington, DCGoogle Scholar
  4. Benson DF, Davis RJ, Snyder BD (1988) Posterior cortical atrophy. Arch Neurol 45: 789–793PubMedCrossRefGoogle Scholar
  5. Brandt J (1991) Cognitive impairments in Huntington’s disease: insights into the neuropsychology of the striatum. In: Boller F, Grafman J (eds) Handbook of neuropsychology, vol 5. Elsevier Sciences Publishers BV, Amsterdam, pp 241–264Google Scholar
  6. Burkhardt CR, Filley CM, Kleinschmidt-DeMasters BK, De La Monte S, Norenberg MD, Schneck SA (1988) Diffuse Lewy body disease and progressive dementia. Neurology 38; 1520–1528PubMedGoogle Scholar
  7. Byrne EJ, Lennox G, Lowe J, Godwin-Austen RB (1989) Diffuse Lewy body disease: clinical features in 15 cases. J Neurol Neurosurg Psychiatry 52: 709–717PubMedCrossRefGoogle Scholar
  8. Cambier J, Masson M, Viader F, Limodin J, Strube A (1895) Le syndrome frontal de la paralysie supranucléaire progressive. Rev Neurol 141: 528–536Google Scholar
  9. Celsis P, Agniel A, Puel M, Le Tinnier A, Viallard G, Démonet JF, Rascol A, Marc-Vergnes JP (1990) Lateral asymmetries in primary degenerative dementia of the Alzheimer type. A correlative study of cognitive, haemodynamic and EEG data, in relation with severity, age of onset and sex. Cortex 26: 585–596PubMedGoogle Scholar
  10. Chawluk JB, Mesulam MM, Hurtig H, Kushner M, Weintraub S, Saykin A, Rubin N, Alavi A, Reivitch M (1986) Slowly progressive aphasia without generalized dementia: studies with positron emission tomography. Ann Neurol 19: 68–74PubMedCrossRefGoogle Scholar
  11. Chui HC (1989) Dementia. A review emphasizing clinico-pathologic correlation and brain-behavior relationship. Arch Neurol 46: 806–814PubMedCrossRefGoogle Scholar
  12. Cogan DG (1985) Visual disturbances with focal progressive dementing disease. Am J Ophthalmol 100: 68–72PubMedGoogle Scholar
  13. Cooper JA, Sagar HJ, Jordan N, Harvey NS, Sullivan EV (1991) Cognitive impairment in early untreated Parkinson’s disease and its relationship to motor disability. Brain 114: 2095–2122.PubMedCrossRefGoogle Scholar
  14. Croisile B, Trillet M, Hibert O, Cinotti L, Le Bars D, Mauguière F, Aimard G (1991) Désordres visuo-constructifs et alexie-agraphie associés à une atrophie corticale postérieure. Rev Neurol 147: 138–143PubMedGoogle Scholar
  15. Cummings JL (1993) Psychosis in basal ganglia disorders. In: Wolters E, Scheltens P (eds) Mental dysfunction in Parkinson’s disease. Vrije Universiteit, Amsterdam, pp 257–268Google Scholar
  16. Cummings JL, Benson DF (1984) Subcortical dementia. Review of an emerging concept. Arch Neurol 41: 874–879PubMedCrossRefGoogle Scholar
  17. De Renzi E (1986) Slowly progressive visual agnosia or apraxia without dementia. Cortex 22: 171–180PubMedGoogle Scholar
  18. Dick JP, Snowden J, Northern B, Goulding PT, Neary D (1989) Slowly progressive apraxia. Behav Neurol 2: 101–114Google Scholar
  19. Dubois B, Boller F, Pillon B, Agid Y (1991) Cognitive deficits in Parkinson’s disease. In: Boller F, Grafman J (eds) Handbook of neuropsychology, vol 5. Elsevier Sciences Publishers BV, Amsterdam, pp 195–240Google Scholar
  20. Dubois B, Défontaines B, Deweer B, Malapani C, Pillon B (1995) Cognitive and behavioral changes in patients with focal lesions of the basal ganglia. Adv Neurol 65: 29–41PubMedGoogle Scholar
  21. Durr A, Dodé C, Hahn V, Pêcheux C, Pillon B, Feingold J, Kaplan JC, Agid Y, Brice A (1995) Diagnosis of “sporadic” Huntington’s disease. J Neurol Sci 129: 51–55PubMedCrossRefGoogle Scholar
  22. Gibb WR, Luthert PJ, Janota I, Lantos PL (1989) Cortical Lewy body dementia: clinical features and classification. J Neurol Neurosurg Psychiatry 52: 185–192PubMedCrossRefGoogle Scholar
  23. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192PubMedCrossRefGoogle Scholar
  24. Grafman J, Litvan I, Gomez C, Chase T (1990) Frontal lobe function in progressive supranuclear palsy. Arch Neurol 47: 553–558PubMedCrossRefGoogle Scholar
  25. Grober E, Buschke H (1987) Genuine memory deficits in dementia. Dev Neuropsychol 3: 13–36CrossRefGoogle Scholar
  26. Gustafson L (1987) Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 6: 209–223PubMedCrossRefGoogle Scholar
  27. Hauw JJ, Delaère P (1991) Topographie des lésions. In: Signoret JL, Hauw JJ (eds) Maladie d’Alzheimer et autres démences. Flammarion Médecine-Sciences, Paris, pp 22–38Google Scholar
  28. Haxby JV, Grady CL, Koss E, Horwitz B, Schapiro M, Friedland RP, Rapoport SI (1988) Heterogeneous anterior-posterior metabolic patterns in dementia of the Alzheimer type. Neurology 38: 1853–1863PubMedGoogle Scholar
  29. Jellinger KA, Bancher C (1992) Neuropathology. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy: clinical and research approaches. Oxford University Press, New York Oxford, pp 44–88Google Scholar
  30. Karbe H, Kertesz A, Polk M (1993) Profiles of language impairment in primary progressive aphasia. Arch Neurol 50: 193–201PubMedCrossRefGoogle Scholar
  31. Knopman DS, Christensen KJ, Schut LJ, Harbaugh RE, Reeder T, Ngo T, Frey W (1989) The spectrum of imaging and neuropsychological findings in Pick’s disease. Neurology 39: 362–368PubMedGoogle Scholar
  32. Knopman DS, Mastric AR, Frey WH, Sung JH, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40: 251–256PubMedGoogle Scholar
  33. Leiguarda R, Lees AJ, Merello M, Starkstein S, Marsden CD (1994) The nature of apraxia in corticobasal degeneration. J Neurol Neurosurg Psychiatry 57: 455–459PubMedCrossRefGoogle Scholar
  34. Litvan I, Mohr E, Williams J, Gomez C, Chase TN (1991) Differential memory and executive functions in demented patients with Parkinson’s and Alzheimer’s disease. J Neurol Neurosurg Psychiatry 54: 25–29PubMedCrossRefGoogle Scholar
  35. Maher ER, Smith EM, Lees AJ (1985) Cognitive deficits in the Steele-RichardsonOlszewski syndrome (progressive supranuclear palsy). J Neurol Neurosurg Psychiatry 48: 1234–1239PubMedCrossRefGoogle Scholar
  36. McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM (1984) Clinical diagnosis of Alzheimer’s disease: report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Servicies task force on Alzheimer’s disease. Neurology 34: 939–944PubMedGoogle Scholar
  37. Mendez MF, Selwood A, Mastri AR, Frey WH (1993) Pick’s disease versus Alzheimer’s disease: a comparison of clinical characteristics. Neurology 43: 289–292PubMedGoogle Scholar
  38. 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 41: 1374–1382PubMedGoogle Scholar
  39. Neary D (1990) Non Alzheimer’s disease form of cerebral atrophy. J Neurol Neurosurg Psychiatry 53: 929–931PubMedCrossRefGoogle Scholar
  40. Neary D, Snowden JS, Northen B, Goulding P (1988) Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry 51: 353–361PubMedCrossRefGoogle Scholar
  41. Olichney JM, Galasko D, Corey-Bloom J, Thal LJ (1995) The spectrum of diseases with diffuse Lewy bodies. Adv Neurol 65: 159–170PubMedGoogle Scholar
  42. Owen AM, Robbins TW (1993) Comparative neuropsychology of Parkinsonian syndromes. In: Wolters EC, Scheltens P (eds) Mental dysfunction in Parkinson’s disease. Vrije Universiteit, Amsterdam, pp 221–241Google Scholar
  43. Paulus W, Jellinger K (1991) The neuropathologie basis of different clinical subgroups of Parkinson’s disease. J Neuropathol Exp Neurol 50: 473–755CrossRefGoogle Scholar
  44. Perry RH, Irving D, Blessed G, Fairbairn A, Perry EK (1990) Senile dementia of Lewy body type. A clinically and neuropathologically distinct form of Lewy body dementia in the elderly. J Neurol Sci 95: 119–139PubMedCrossRefGoogle Scholar
  45. Pillon B, Blin J, Vidailhet M, Deweer B, Sirigu A, Dubois B, Agid Y (1995) The neuropsychological pattern of corticobasal degeneration. Comparison with progressive supranuclear palsy and Alzheimer’s disease. Neurology 45: 1477–1483PubMedGoogle Scholar
  46. Pillon B, Deweer B, Agid Y, Dubois B (1993) Explicit memory in Alzheimer’s, Huntington’s, and Parkinson’s diseases. Arch Neurol 50: 374–379PubMedCrossRefGoogle Scholar
  47. Pillon B, Deweer B, Michon A, Malapani C, Agid Y, Dubois B (1994) Are explicit memory disorders of progressive supranuclear palsy related to damage to striatofrontal circuits? Comparison with Alzheimer’s, Parkinson’s, and Huntington’s diseases. Neurology 44: 1254–1270Google Scholar
  48. Pillon B, Dubois B (1992) Cognitive and behavioral impairments. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy; clinical and research approaches. Oxford University Press, New York Oxford, pp 223–239Google Scholar
  49. Pillon B, Gouider-Khouja N, Deweer B, Vidhailhet M, Malapani C, Dubois B, Agid Y (1995) Neuropsychological pattern of striatonigral degeneration: comparison with Parkinson’s disease and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 58: 174–179PubMedCrossRefGoogle Scholar
  50. Richard J, Constantinidis J (1970) Les démences de la vieillesse; notions acquises et problèmes cliniques actuels. Confront Psychiat 3: 39–61Google Scholar
  51. Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewicz O, Black S (1990) Corticobasal ganglionic degeneration. Neurology 40: 1203–1212PubMedGoogle Scholar
  52. Robbins TW, James M, Lange KW, Owen AM, Quinn NP, Marsden CD (1992) Cognitive performance in multiple System Atrophy. Brain 115: 271–291PubMedCrossRefGoogle Scholar
  53. Sawle GV, Brooks DJ, Marsden CD, Frackoviack RJ (1991) Corticobasal degeneration. A unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography. Brain 114: 541–556PubMedCrossRefGoogle Scholar
  54. Snowden JS, Neary D, Mann DW, Goulding PJ, Testa HJ (1992) Progressive language disorder due to lobar atrophy. Ann Neurol 31: 174–183PubMedCrossRefGoogle Scholar
  55. Stern Y, Richards M, Sano M, Mayeux R (1993) Comparison of cognitive changes in patients with Alzheimer’s and Parkinson’s disease. Arch Neurol 50: 1040–1045PubMedCrossRefGoogle Scholar
  56. Sulkava R, Haltia M, Paetau A, Wilkstrom J, Palo J (1983) Accuracy of clinical diagnosis in primary degenerative dementia: correlation with neuropathological findings. J Neurol Neurosurg Psychiatry 46: 9–13PubMedCrossRefGoogle Scholar
  57. Taylor AE, Saint-Cyr JA, Lang AE (1990) Memory and learning in early Parkinson’s disease: evidence for a “frontal lobe syndrome”. Brain Cogn 13: 211–232PubMedCrossRefGoogle Scholar
  58. Testa D, Fetoni V, Soliveri P, Musicco M, Palazzini E, Girotti F (1993) Cognitive and motor performance in multiple system atrophy and Parkinson’s disease compared. Neuropsychologia 31: 207–210PubMedCrossRefGoogle Scholar
  59. Tissot R, Constantinidis J, Richard J (1975) La maladie de Pick. Masson, ParisGoogle Scholar
  60. Tyrell PJ, Warrington EK, Frackowiak RS, Rossor MN (1990) Progressive degeneration of the right temporal lobe studied with positron emission tomography. J Neurol Neurosurg Psychiatry 53: 1046–1050CrossRefGoogle Scholar
  61. Weintraub S, Mesulam MM (1993) Four neuropsychological profiles in dementia. In: Boller F, Grafman J (eds) Handbook of neuropsychology, vol 8. Elsevier, Amsterdam, pp 253–282Google Scholar
  62. Weintraub S, Rubin N, Mesulam M (1990) Primary progressive aphasia: longitudinal course, neuropsychological profile and language features. Arch Neurol 47: 1329–1335PubMedCrossRefGoogle Scholar
  63. Zetusky WJ, Jankovic J, Pirozzolo FJ (1985) The heterogeneity of Parkinson’s disease; clinical and prognostic implications. Neurology 35: 522–526PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • B. Pillon
    • 1
  • B. Dubois
    • 1
  • Y. Agid
    • 1
  1. 1.INSERM U-289 and Fédération de NeurologieHôpital de la SalpêtrièreParis Cedex 13France

Personalised recommendations