Download PDF
Semin Neurol 2000; 20(4): 447-454
DOI: 10.1055/s-2000-13177
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Alzheimer's Disease and Its Focal Variants

Joel H. Kramer, Bruce L. Miller
  • University of California, San Francisco Medical Center, San Francisco, California
Further Information

Publication History

Publication Date:
03 March 2004 (online)

    Permissions and Reprints

ABSTRACT

Alzheimer's disease (AD) is the most common form of dementia in elderly individuals. Although the diagnosis of AD requires pathological confirmation, several common clinical features of AD have been identified. These include insidious onset, gradual progression, medial temporal atrophy, temporoparietal hypoperfusion, early and prominent deficits in episodic memory and mental tracking, and later deficits in semantic memory. These characteristic features of AD have enabled excellent in vivo diagnostic sensitivity. It has become increasingly clear, however, that AD is a heterogeneous disorder. The left and right cerebral hemispheres can be affected to varying degrees, and variability on the anterior-posterior axis has also been reported. AD can also present as a focal degenerative disease, and clinicians must be aware that there may be diagnostic overlap between AD, primary progressive aphasia, posterior cortical atrophy, corticobasal degeneration, and frontotemporal dementia.

KEYWORD

Alzheimer's disease - primary progressive aphasia - frontotemporal dementia - posterior cortical atrophy - focal variants - corticobasal degeneration

REFERENCES

  • 1 Kolb B, Wishaw I. Fundamentals of Human Neuropsychology.  3rd ed. New York: Freeman; 1991
    Search in Google Scholar
  • 2 Katzman R, Kawas C. The epidemiology of dementia and Alzheimer's disease. In: Terry RD, Katzman R, Bick KL, eds. Alzheimer's Disease New York: Raven Press 1994: 104-122
    Search in Google Scholar
  • 3 Weiner M F, Vega G, Risser R C. Apolipoprotein E epsilon 4, other risk factors, and course of Alzheimer's disease.  Biol Psychiatry . 1999;  45 633-638
    CrossrefPubMedSearch in Google Scholar
  • 4 Slooter A J, Cruts M, Kalmijn S. Risk estimates of dementia by apolipoprotein E genotypes from a population-based incidence study: the Rotterdam Study.  Arch Neurol . 1998;  55 964-968
    CrossrefPubMedSearch in Google Scholar
  • 5 Saunders A M, Hulette O, Welsh-Bohmer K A. Specificity, sensitivity, and predictive value of apolipoprotein-E genotyping for sporadic Alzheimer's disease.  Lancet . 1996;  348 90-93
    CrossrefPubMedSearch in Google Scholar
  • 6 Craft S, Teri L, Edland S D. Accelerated decline in apolipoprotein E-epsilon 4 homozygotes with Alzheimer's disease.  Neurology . 1998;  51 149-153
    PubMedSearch in Google Scholar
  • 7 van Duijm M C, Hofman A, Kay D W. Risk factors for Alzheimer's disease: a collaborative re-analysis of case-control studies.  Int J Epidemiol . 1991;  20 4-12
    PubMedSearch in Google Scholar
  • 8 Davis D G, Schmitt F A, Wekstein D R, Markesbery W R. Alzheimer neuropathologic alterations in aged cognitively normal subjects.  J Neuropathol Exp Neurol . 1999;  58 376-388
    PubMedSearch in Google Scholar
  • 9 Mirra S S. The CERAD neuropathology protocol and consensus recommendations for the postmortem diagnosis of Alzheimer's disease: a commentary.  Neurobiol Aging . 1997;  18(suppl 4) S91-S94
    CrossrefPubMedSearch in Google Scholar
  • 10 Braak H, Braak E, Yilmazer D, de Vos A R, Jansen E N, Bohl J. Pattern of brain destruction in Parkinson's and Alzheimer's diseases.  J Neural Transm . 1996;  103 455-490
    CrossrefPubMedSearch in Google Scholar
  • 11 Delacourte A, David J P, Sergeant N. The biochemical pathway of neurofibrillary degeneration in aging and Alzheimer's disease.  Neurology . 1999;  52 1158-1165
    PubMedSearch in Google Scholar
  • 12 McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan E M. Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease.  Neurology . 1984;  34 939-944
    CrossrefPubMedSearch in Google Scholar
  • 13 Nagy Z, Esiri M M, Hindley N J. Accuracy of clinical operational diagnostic criteria for Alzheimer's disease in relation to different pathological diagnostic protocols.  Dement Geriatr Cogn Disord . 1998;  9 219-226
    CrossrefPubMedSearch in Google Scholar
  • 14 Jobst K A, Barnetson L P, Shepstone B J. Accurate prediction of histologically confirmed Alzheimer's disease and the differential diagnosis of dementia: the use of NINCDS-ADRDA and DSM-III-R criteria, SPECT, X-ray CT, and Apo E4 in medial temporal lobe dementias. Oxford Project to Investigate Memory and Aging. [corrected and republished article originally printed in Int Psychogeriatr 1997;9(suppl 1):191-222; discussion 247-252].  Int Psychogeriatr . 1998;  10 271-302
    CrossrefPubMedSearch in Google Scholar
  • 15 DeCarli C, Kaye J A, Horwitz B, Rapoport S I. Critical analysis of the use of computer-assisted transverse axial tomography to study human brain in aging and dementia of the Alzheimer type.  Neurology . 1990;  40 872-883
    PubMedSearch in Google Scholar
  • 16 Sandor T, Jolesz F, Tieman J, Kikinis R, Jones K, Albert M. Comparative analysis of computed tomographic and magnetic resonance imaging scans in Alzheimer patients and controls.  Arch Neurol . 1992;  49 381-384
    PubMedSearch in Google Scholar
  • 17 Foundas A L, Eure K F, Seltzer B. Conventional MRI volumetric measures of parietal and insular cortex in Alzheimer's disease.  Prog Neuropsychopharmacol Biol Psychiatry . 1996;  20 1131-1144
    CrossrefPubMedSearch in Google Scholar
  • 18 Smith C D, Malcein M, Meurer K, Schmitt F A, Markesbery W R, Pettigrew L C. MRI temporal lobe volume measures and neuropsychologic function in Alzheimer's disease.  J Neuroimaging . 1999;  9 2-9
    PubMedSearch in Google Scholar
  • 19 Braak H, Braak E. Staging of Alzheimer's disease-related neurofibrillary changes.  Neurobiol Aging. 1995;  16 271-278 discussion, 278-discussion, 284
    CrossrefPubMedSearch in Google Scholar
  • 20 Squire L R. Memory and Brain.  New York: Oxford University Press; 1987
    Search in Google Scholar
  • 21 Mori E, Ikeda M, Hirono N, Kitagaki H, Imamura T, Shimomura T. Amygdalar volume and emotional memory in Alzheimer's disease.  Am J Psychiatry . 1999;  156 216-222
    PubMedSearch in Google Scholar
  • 22 Schuff N, Amend D L, Meyerhoff D J. Alzheimer disease: quantitative H-1 MR spectroscopic imaging of frontoparietal brain.  Radiology . 1998;  207 91-102
    PubMedSearch in Google Scholar
  • 23 Juottonen K, Laakso M P, Partanen K, Soininen H. Comparative MR analysis of the entorhinal cortex and hippocampus in diagnosing Alzheimer disease.  AJNR . 1999;  20 139-144
    PubMedSearch in Google Scholar
  • 24 Visser P J, Scheltens P, Verhey F R. Medial temporal lobe atrophy and memory dysfunction as predictors for dementia in subjects with mild cognitive impairment.  J Neurol . 1999;  246 477-485
    CrossrefPubMedSearch in Google Scholar
  • 25 Zakzanis K K. Quantitative evidence for neuroanatomic and neuropsychological markers in dementia of the Alzheimer's type.  J Clin Exp Neuropsychol . 1998;  20 259-269
    PubMedSearch in Google Scholar
  • 26 Pantel J, Schroder J, Essig M. In vivo quantification of brain volumes in subcortical vascular dementia and Alzheimer's disease: an MRI-based study.  Dement Geriatr Cogn Disord . 1998;  9 309-316
    CrossrefPubMedSearch in Google Scholar
  • 27 Schuff N, Amend D, Ezekiel F. Changes of hippocampal N-acetyl aspartate and volume in Alzheimer's disease: a proton MR spectroscopic imaging and MRI study.  Neurology . 1997;  49 1513-1521
    PubMedSearch in Google Scholar
  • 28 Haxby J V, Duara R, Grady C L, Cutler N R, Rapoport S I. Relations between neuropsychological and cerebral metabolic asymmetries in early Alzheimer's disease.  J Cereb Blood Flow Metab . 1985;  5 193-200
    PubMedSearch in Google Scholar
  • 29 Collette F, Van der Linden M, Salmon E. Executive dysfunction in Alzheimer's disease [published erratum appears in Cortex 1999;35:451].  Cortex . 1999;  35 57-72
    PubMedSearch in Google Scholar
  • 30 Jacobs D M, Sano M, Dooneief G, Marder K, Bell K L, Stern Y. Neuropsychological detection and characterization of preclinical Alzheimer's disease.  Neurology . 1995;  45 957-962
    PubMedSearch in Google Scholar
  • 31 Folstein M F, Blysma F W. Noncognitive symptoms of Alzheimer's disease. In: Terry RD, Katzman R, eds. Alzheimer's Disease New York: Raven Press 1994: 27-40
    Search in Google Scholar
  • 32 Martin A, Brouwers P, Lalonde F. Towards a behavioral typology of Alzheimer's patients.  J Clin Exp Neuropsychol . 1986;  8 594-610
    PubMedSearch in Google Scholar
  • 33 Fisher N J, Rourke B P, Bieliauskas L A, Giordani B, Berent S, Foster N L. Unmasking the heterogeneity of Alzheimer's disease: case studies of individuals from distinct neuropsychological subgroups.  J Clin Exp Neuropsychol . 1997;  19 713-754
    PubMedSearch in Google Scholar
  • 34 Friedland R P, Budinger T F, Koss E, Ober B A. Alzheimer's disease: anterior-posterior and lateral hemispheric alterations in cortical glucose utilization.  Neurosci Lett . 1985;  53 235-240
    CrossrefPubMedSearch in Google Scholar
  • 35 Haxby J V, Grady C L, Koss E. Longitudinal study of cerebral metabolic asymmetries and associated neuropsychological patterns in early dementia of the Alzheimer type.  Arch Neurol . 1990;  47 753-760
    PubMedSearch in Google Scholar
  • 36 Grady C L, Haxby J V, Schapiro M B. Subgroups in dementia of the Alzheimer type identified using positron emission tomography.  J Neuropsychiatry Clin Neurosci . 1990;  2 373-384
    PubMedSearch in Google Scholar
  • 37 Becker J T, Huff F J, Nebes R D, Holland A, Boller F. Neuropsychological function in Alzheimer's disease: pattern of impairment and rates of progression.  Arch Neurol . 1988;  45 263-268
    PubMedSearch in Google Scholar
  • 38 Mesulam M M. Slowly progressive aphasia without generalized dementia.  Ann Neurol . 1982;  11 592-598
    CrossrefPubMedSearch in Google Scholar
  • 39 Westbury C, Bub D. Primary progressive aphasia: a review of 112 cases.  Brain Lang . 1997;  60 381-406
    CrossrefPubMedSearch in Google Scholar
  • 40 Black S E. Focal cortical atrophy syndromes.  Brain Cogn . 1996;  31 188-229
    PubMedSearch in Google Scholar
  • 41 Greene J D, Miles K, Hodges J R. Neuropsychology of memory and SPECT in the diagnosis and staging of dementia of Alzheimer type.  J Neurol . 1996;  243 175-190
    PubMedSearch in Google Scholar
  • 42 Ross S J, Graham N, Stuart-Green L. Progressive biparietal atrophy: an atypical presentation of Alzheimer's disease.  J Neurol Neurosurg Psychiatry . 1996;  61 388-395
    PubMedSearch in Google Scholar
  • 43 Benson D F, Davis R J, Snyder B D. Posterior cortical atrophy.  Arch Neurol . 1988;  45 789-793
    CrossrefPubMedSearch in Google Scholar
  • 44 Victoroff J, Ross G W, Benson D F, Verity M A, Vinters H V. Posterior cortical atrophy: neuropathologic correlations.  Arch Neurol . 1994;  51 269-274
    CrossrefPubMedSearch in Google Scholar
  • 45 Hof P R, Vogt B A, Bouras C, Morrison J H. Atypical form of Alzheimer's disease with prominent posterior cortical atrophy: a review of lesion distribution and circuit disconnection in cortical visual pathways.  Vision Res . 1997;  27 3609-3625
    PubMedSearch in Google Scholar
  • 46 Hof P R, Archin N, Osmand A P. Posterior cortical atrophy in Alzheimer's disease: analysis of a new case and re-evaluation of a historical report.  Acta Neuropathol . 1993;  86 215-223
    CrossrefPubMedSearch in Google Scholar
  • 47 Levine D N, Lee J M, Fisher C M. The visual variant of Alzheimer's disease: a clinicopathologic case study.  Neurology . 1993;  43 305-313
    PubMedSearch in Google Scholar
  • 48 Heilman K M, Rothi L J. Apraxia. In: Heilman KM, Valenstein E, eds. Clinical Neuropsychology 3rd ed. New York: Oxford University Press 1993: 141-163
    Search in Google Scholar
  • 49 Beatty W W, Scott J G, Wilson D A, Prince J R, Williamson D J. Memory deficits in a demented patient with probable corticobasal degeneration.  J Geriatr Psychiatry Neurol . 1995;  8 132-136
    PubMedSearch in Google Scholar
  • 50 Kaida K, Takeda K, Nagata N, Kamakura K. Alzheimer's disease with asymmetric parietal lobe atrophy: a case report.  J Neurol Sci . 1998;  160 96-99
    CrossrefPubMedSearch in Google Scholar
  • 51 Johnson J K, Head E, Kim R, Starr A, Cotman C W. Clinical and pathological evidence for a frontal variant of Alzheimer disease.  Arch Neurol . 1999;  56 1233-1239
    CrossrefPubMedSearch in Google Scholar
  • 52 Neary D, Snowden J S, Gustafson L. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.  Neurology . 1998;  51 1546-1554
    CrossrefPubMedSearch in Google Scholar
  • 53 Gregory C A, Serra-Mestres J, Hodges J R. Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?.  Neuropsychiatry Neuropsychol Behav Neurol . 1999;  12 128-135
    PubMedSearch in Google Scholar
  • 54 Hodges J R, Patterson K, Ward R. The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study.  Neuropsychology . 1999;  13 31-40
    CrossrefPubMedSearch in Google Scholar