Review Article

Acta Neuropathologica

, Volume 113, Issue 2, pp 107-117

First online:

Neurofibrillary tangle-predominant dementia: comparison with classical Alzheimer disease

  • K. A. JellingerAffiliated withInstitute of Clinical Neurobiology Email author 
  • , J. AttemsAffiliated withDepartment of Pathology, OWS Hospital (JA)

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Abstract

Neurofibrillary tangle predominant dementia (NFTPD) is a subset of late onset dementia, clinically different from traditional “plaque and tangle” Alzheimer disease (AD): later onset, shorter duration, less severe cognitive impairment, and almost absence of ApoE ε4. Neuropathology reveals abundant allocortical neurofibrillary pathology with no or few isocortical tau lesions, absence of neuritic plaques, absence or scarcity of amyloid deposits, but neurofibrillary changes comprising both 3 and 4 repeat (3R and 4R) tau immunohistochemistry are not significantly different from those in classical AD. Comparing 51 autopsy cases of NFTPD with 244 classical AD subjects, the nosology of NFTPD and its differences from AD are discussed.

Keywords

Senile dementia with tangles Alzheimer disease 3 and 4 repeat tau isoforms Tauopathy Differential diagnosis Neuropathology