Frontotemporal dementia

A faster neurodegenerative process

Behavioural variant frontotemporal dementia (bvFTD), originally known as Pick’s disease, is the most common type of frontotemporal dementia, and can cause early and progressive changes in personality, emotional ‘blunting’ and loss of empathy. The underlying pathology is similar to that found in Alzheimer’s disease (AD) but affects a different region of the brain and also tends to affect people at a younger age. 

The average age of onset of FTD is between 40 and 60 years. In bvFTD, the neurodegenerative process progresses faster than in AD, initially affecting the frontal and temporal lobes, which govern behaviour and emotion. Atrophy in these regions of the brain was recently found to be associated with core clinical symptoms in patients with bvFTD.¹ As the disease progresses, other parts of the brain are affected, eventually producing a global dementia.

A potential treatment for rare diseases

Protein aggregates of tau and TDP-43 are the two most common underlying pathologies in bvFTD, with TDP-43 pathology accounting for approximately 50% of cases and tau pathology accounting for roughly 40–45% of cases¹. The active component at the heart of our second generation TAI has been found to act on the aggregation of the TDP-43 protein in a similar way to its action on aggregation of tau protein. In 2010, our drug was granted Orphan Designation. We completed a Phase III clinical trial of our lead compound in bvFTD.