Continuing our series on dementia, this article focuses on a less common type called frontotemporal dementia, which accounts for up to 10% of all dementia cases. This type of dementia primarily affects two lobes of the brain, the frontal and temporal lobes, hence its name. The degeneration which occurs can result in changes in personality and behavior, and/or difficulties with language. This condition usually occurs in individuals in their fifties or early sixties, and while for some, specific genetic mutations are the cause of the disease, others who are affected have no family history of dementia. There are no other risk factors.
As with all of the other types of dementia that we have discussed, the abnormal behavior of specific proteins in the brain results in damage to nerve cells, which leads to dementia symptoms. Diagnosis is made by observation of symptoms, neurological testing, and testing to rule out other disease processes. There is no cure or method to slow the progression of the disease, and treatment focuses on symptom management.
Frontotemporal dementia can be divided into three main categories, based on the primary symptoms that are present. The first category is behavior type. Individuals in this category may experience dramatic personality changes and become socially inappropriate, impulsive, or emotionally indifferent. They demonstrate distractibility, a loss of empathy and tact, a lack of judgment and inhibition, an increased interest in sex, repetitive or compulsive behaviors, a neglect of personal hygiene, and a lack of insight into their own behavior as well as the behavior of others. When apathy is experienced, a misdiagnosis of depression may be made due to decreased energy and motivation. Weight gain may occur due to impulsive eating and decreased activity. When sleep disturbances accompany the behavior type, symptoms may be exacerbated and progress more quickly. These individuals are living in the moment, and in conversation they may have difficulty transitioning between the past, present, and future.
A second category of frontotemporal dementia is primary progressive aphasia. In the early stages, impairment of language skills is the primary symptom, but behavioral symptoms may develop as the condition progresses. There are two subtypes of primary progressive aphasia: semantic dementia and progressive nonfluent aphasia. In semantic dementia, a person’s actual speech is fluent and grammatically correct, but the content of that speech does not relate to the conversation taking place or make sense. Vocabulary becomes increasingly limited, and broad terms may be used in place of more specific ones (for example, “building” instead of “house”). Difficulty understanding spoken and written language may also be present. The second subtype, progressive nonfluent aphasia, manifests itself in language that contains short halting phrases, spelling errors, and incorrect grammar. Individuals have some sense of understanding and remain rational, but they become concrete in their thinking and their language deficits make answering questions difficult. The ability to read and write may be impaired.
The third category is movement disorders, in which certain automatic muscle functions are affected. Two different subtypes exist, the first exhibiting symptoms of shakiness, lack of coordination, and muscle rigidity and spasms. The second causes balance problems and difficulty walking, frequent falls, and muscle stiffness, especially in the neck and upper body. Slowing and reduced control of eye movements may be another symptom.
To read the poignant story of a 48 year old woman affected by frontotemporal dementia, visit www.nextavenue.org/article/2014-01/special-series-under-60-and-living-dementia.
Frontotemporal dementia used to be known as Pick’s disease, and is still referred to by that name by some professionals. The progression of the disease is rapid in some, occurring over two to three years, while in others it may stretch over ten years. As with other types of dementia, the physical and emotional toll on affected individuals and their caregivers can be overwhelming. Are you ready to join the fight against dementia and Alzheimer’s disease? The opportunity to start a new team or join an existing one for the Walk to End Alzheimer’s is still available! We hope to see you on September 20th at City Island. For details, give us a call at 717-697-3223.
Karen Kaslow, RN