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What is Frontotemporal Dementia?
Frontotemporal Dementia (FTD) is a neurodegenerative disease characterized by atrophy in the frontal and temporal lobes. It is a highly heterogenous disease with several variants that are closely related in clinical presentation to other diseases such as Corticobasal syndrome and Motor Neuron Disease. The disease is roughly seperated into two categories, the behavioral variant and the language variant. The symptoms are progressive and debilitating, severely impacting the patient’s personality, behavior, and language skills depending on the variant.
The pathophysiology of FTD involves a variety of mechanisms present in most neurodegenerative disorders; amongst them, dysfunctional mitochondria, misfolded proteins, toxic RNA, impaired autophagy, and neuroinflammation. Anavex Life Sciences is developing therapeutics that can possibly modify the progression of FTD.
Signs & Symptoms
The behavioral variant of FTD (bvFTD) present symptoms that are atypical in most other dementias, while memory and spatial skills remain intact. Progressive changes to social conduct (inappropriate, aggressive behaviors) and emotional processing (apathy, inability to gauge emotions in others and one’s self) are some of the hallmarks of bvFTD.
Inability to recognize common words
Loss of vocabulary
Neuro-developmental delays are typically the first signs of FTD.
The language variant can have an even more complex clinical presentation including loss of vocabulary, inability to understand previously known words, loss of the mental connection between words, objects, and concepts. Other forms of the language variant can lead to impaired speech and sentence production. As the disease progresses, these symptoms begin to overlap with each other.
FTD is a form of dementia found most frequently in individuals less than 65 years old at time of diagnosis. FTD affects roughly 50,000 to 60,000 individuals in the United States and roughly 110,000 individuals in the European Union. FTD is a rapidly progressing degenerative syndrome characterized by prominent cognitive dysfunction, behavioral and personality changes, and language deficits. The average life expectancy in FTD patients is seven to 10 years after the onset of symptoms.