A.Prof Frank Gaillard et al.

Tauopathies are a heterogeneous group of neurodegenerative diseases characterized by abnormal metabolism of tau proteins leading to intracellular accumulation and formation of neurofibrillary tangles (NFT). These neurofibrillary tangles are deposited in the cytosol of neurons and glial cells. 

Examples of tauopathies include 3:

It should be noted that some texts 2 define tauopathies as a disease characterized by mutations in the tau protein gene itself. If such a strict definition is used, even though the histopathological hallmark of Alzheimer disease is the presence of numerous neurofibrillary tangles (which is also formed by tau proteins), it still would not be strictly a tauopathy, as no defect in the tau protein gene has been identified.

More recently the discovery of the glymphatic pathway and the importance of this in the normal physiological clearance of extracellular solutes including beta-amyloid, suggests that there is also the possibility of reduced clearance, in addition to abnormal metabolism, as the underpinning of some tauopathies including chronic traumatic encephalopathy 4

Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

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Article information

rID: 13021
Section: Gamuts
Synonyms or Alternate Spellings:
  • Tauopathies

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