Behavioural variant frontotemporal dementia (bvFTD) – originally known as Pick’s disease – can cause early and progressive changes in personality, emotional ‘blunting’ and loss of empathy. The underlying pathology is similar in nature to that found in Alzheimer’s disease 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 40-60 years.
In bvFTD, the neurodegenerative process progresses faster than is seen in Alzheimer’s disease, 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.
Tau and TDP-43 aggregates each account for about 50% of patients with bvFTD. In a pilot series of cases, TauRx’s first generation TAI, rember®, was found to arrest the progression of the disease. The active moiety at the heart of our second generation TAI has also been found to act on the aggregation of TDP-43 protein in a similar way to its action on aggregation of tau protein. Because the disease is relatively rare, TauRx was granted Orphan Designation in the EU for our drug in 2010.
TauRx has now completed a Phase 3 clinical trial of this compound in bvFTD. Read a summary of the Phase 3 programme and trial results.
Vuksanovic V, Staff RT, Ahearn T, et al. (2016) Frontotemporal atrophy and clinical estimates of disease severity in behavioural variant frontotemporal dementia: The role of cognitive reserve. Presented at the Alzheimer’s Research UK Conference, Manchester, UK.