DEGENERATIVE DISEASES AFFECTING THE CEREBRAL CORTEX-Frontotemporal Dementias

Question 1

What are Frontotemporal dementias (FTDs)

Answer 1

These are a group of disorders that were first classified together
because they shared clinical features(progressive deterioration of language and changes in
personality)corresponding to degeneration and atrophy oftemporal and frontal lobes. [49]

Question 2

What is that same feature being shared by frontotemporal dementias?

Answer 2

progressive deterioration in language and personality

Question 3

What is that accumulated deposit being shared the same by FTDs which is the characteristic finding?

Answer 3

tau-containing deposits as their characteristic finding,
giving rise to the term tauopathy.

Question 4

This is a genetically determined disorder in which the clinical syndrome of a FTD is often
accompanied by parkinsonian symptoms.

Answer 4

Frontotemporal Dementia with Parkinsonism Linked to Tau Mutations

Question 5

What is the pathogenesis of FTD?

Answer 5

The study of families with FTD led to the recognition that in some, but not all, pedigrees there
are mutations in the MAPT gene encoding tau.

The mutations fall into several broad
categories: coding-region mutations and intronic mutations.

Question 6

What is the morphology of FTD?

Answer 6

• There is evidence of atrophy of frontal and temporal lobes in various combinations and to various degrees.
• The pattern of atrophy can often be predicted in partby the clinical symptomatology.
• The atrophic regions of cortex are marked by neuronal loss, gliosis, and the presence of tau-containing neurofibrillary tangles.
• These tangles may contain either 4R tau or a mixture of 3R and 4R tau.
• Nigral degeneration may also occur.
• Inclusions can also be found in glial cells in some forms of the disease.