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Forms of Frontotemporal Dementia

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Semantic dementia

Semantic dementia, which has also been called "temporal variant FTD," accounts for 20% of FTD cases. Language difficulty, the predominant complaint of people with SD, is due to the disease damaging the left temporal lobe, an area critical for assigning meaning to words. The language deficit is not in producing speech but is a loss of the meaning, or semantics, of words. At first, you might notice someone substituting a word like "thingy" for more unusual words, but eventually a person with SD will lose the meaning of more common words as well. For example, early in the illness a patient might lose the word for a falcon, later-on forget the word for a chicken, then call all winged creatures "bird" and eventually call all animals "things." Not only do they lose the ability to recall the word, but the concept of these words is also lost. "What is a bird?" might be a typical response for a patient with advanced SD. Reading and spelling usually decline as well, but the person may still be able to do arithmetic and use numbers, shapes or colors well. Names of people, even good friends, can become quite difficult for people with SD. Like the behavioral variant, memory, an understanding of where they are, and sense of day and time tend to function as before. Muscle control for daily life and activities tends to remain good until late in the disease. Some of these skills may seem worse than they actually are because of the language difficulty people with SD have when they try to express themselves.

When SD starts in the right temporal lobe, people in the early stages have more trouble remembering the faces of friends and familiar people. Additionally, these people show profound deficits in understanding the emotions of others. The loss of empathy is an early, and often initial, symptom of patients  with this right-sided form of SD. Eventually people with right-sided onset progress to the left side and then develop the classical language features of SD. Similarly, left-sided cases progress to involve the right temporal lobe and then the person experiences difficulty recognizing faces, foods, animals and emotion. SD patients eventually develop classical bvFTD behaviors including disinhibition, apathy, loss of empathy and diminished insight. The time from diagnosis to the end is longer than for those with bvFTD, typically taking about six years.

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