Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients. These include: progressive behavior/personality decline, progressive language decline, and progressive motor skills decline.
Frontotemporal disorders are the result of damage to neurons (nerve cells) in parts of the brain called the frontal and temporal lobes. As neurons die in the frontal and temporal regions, these lobes atrophy, or shrink.
Gradually, this damage causes difficulties in thinking and behaviors controlled by these parts of the brain.
Many possible symptoms can result, including strange behaviors, emotional problems, trouble communicating, or difficulty with walking and other basic movements.
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A Form of Dementia
People can live with frontotemporal disorders for 2 to 10 years, sometimes longer, but it is difficult to predict the time course for an individual patient. The disorders are progressive, meaning symptoms get worse over time. In the early stages, people have one type of symptom. As the disease progresses, other types of symptoms appear as more parts of the brain are affected.
No cure or treatments for frontotemporal disorders are available today. However, research is improving awareness and understanding of these challenging conditions. This progress is opening doors to better diagnosis, improved care, and, eventually, possible new treatments.
Types of Frontotemporal Disorders
Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients.
- Progressive behavior/personality decline—characterized by changes in personality, behavior, emotions, and judgment (e.g., behavioral variant frontotemporal dementia).
- Progressive language decline—marked by early changes in language ability, including speaking, understanding, reading, and writing (e.g., primary progressive aphasia).
- Progressive motor decline—characterized by various difficulties with physical movement, including shaking, difficulty walking, frequent falls, and poor coordination.
It can be hard to know which of these disorders a person has because symptoms and the order in which they appear can vary widely from one person to the next. Also, the same symptoms can appear in different disorders. For example, language problems are most typical of primary progressive aphasia but can also appear in the course of behavioral variant frontotemporal dementia. The table below summarizes the three types of frontotemporal disorders and lists the various terms that could be used when clinicians diagnose these disorders.
Trouble with words
Alicia’s first symptom was trouble talking. She spoke more slowly and thought she sounded stilted. She could understand people well enough, but finding the right words when she was talking became harder and harder. Also, Alicia, 49, could not write words like "and" and "it" but could write words like "alligator." Her doctor recommended a neurological exam, which helped diagnose agrammatic PPA.
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