What is Frontotemporal Degeneration?

Frontotemporal Degeneration
Significant gray matter (GM) atrophy in behavioral variant frontotemporal degeneration (bvFTD) and CBS, and regression analysis relating performance to reduced GM density. (A) Regions of reduced GM density in patients with bvFTD relative to healthy seniors (red and green) and regions related to the adjusted accuracy of ambiguous anaphoric reference in bvFTD patients (green). The green cluster is in the ventromedial prefrontal cortex (vmPFC) (BA 10/11; peak: 2 60 −8). (B) Regions of reduced GM density in patients with CBS relative to healthy seniors (blue).Processing ambiguity in a linguistic context: decision-making difficulties in non-aphasic patients with behavioral variant frontotemporal degeneration. Spotorno N, Healey M, McMillan CT, Rascovsky K, Irwin DJ, Clark R, Grossman M - Frontiers in human neuroscience (2015). Not Altered. CC.

Frontotemporal degeneration is a group of disorders that occur when nerve cells in the frontal and temporal lobes of the brain are lost. 

What is the Pathology of Frontotemporal Degeneration?

Etiology: The cause of Frontotemporal degeneration is unknown. 

Genes involved: Mutations in the MAPT gene on chromosome 17. 

Pathogenesis: Frontal lobe neuronal degeneration. 

Histology: The histology associated with Frontotemporal degeneration shows four major types of features microvacuolation without neuronal inclusions, and gliosis. 

How does Frontotemporal Degeneration Present?

Patients with frontotemporal degeneration typically are typically older. It is equally common in males and females. The signs and symptoms include abnormal social behavior, lack of inhibition, lack of judgement, and tremors. 

How is Frontotemporal Degeneration Diagnosed?

Frontotemporal degeneration is diagnosed by hitsory and physical exam. 

How is Frontotemporal Degeneration Treated?

Frontotemporal degeneration has no cure. Antidepressants and antipsychotics may be helpful. 

What is the Prognosis of Frontotemporal Degeneration?

The prognosis of Frontotemporal degeneration is poor.