# frontotemporal dementia | Symptoms, Causes, Tests and Treatment | OSANG > Frontotemporal dementia is a degenerative dementia caused by selective degeneration of the frontal and temporal lobes. It is a common cause of early-onset dementia in pe… - Official page: https://osns.co.kr/en/encyclopedia/frontotemporal-dementia - Organization: OSANG Neurosurgery ## Page content Dementia/Cognition ## frontotemporal dementia Frontotemporal Dementia · G31.0 Frontotemporal dementia is a degenerative dementia in which personality and behavioral changes and language disorders appear before memory due to neural degeneration in the frontal and temporal lobes. It is a common cause of early-onset dementia in people under 65 years of age. ## At a glance Frontotemporal dementia is a degenerative dementia caused by selective degeneration of the frontal and temporal lobes. It is a common cause of early-onset dementia in people under 65 years of age, and the average age of onset is about 58 years. In the beginning, personality/behavioral changes or language impairment are more noticeable than memory decline. It is divided into behavioral variant (bvFTD) and primary progressive aphasia (PPA), which are caused by tau or TDP-43 protein pathology and some are hereditary. The effectiveness of anti-dementia drugs is limited, so symptom management becomes the mainstay of treatment. - 01Definition and Overview - 02Subtype - 03Causes and Pathophysiology - 04symptoms - 05diagnosis - 06treatment ## Definition and Overview Frontotemporal dementia is a group of degenerative dementias caused by selective degeneration of the frontal and temporal lobes. Unlike Alzheimer's disease, which begins with a decline in recent memory, personality and behavioral changes and language disorders first appear in the early stages while memory is relatively maintained. It is a common cause of early-onset dementia that occurs in people under the age of 65, and the average age of onset is about 58 years, which is earlier than Alzheimer's disease. It starts at a relatively young age and has a significant impact on career and family life. In ICD-10, it is classified as G31.0 (localized brain atrophy) and includes the disease previously called Pick's disease. ## Subtype ### Behavioral variant (bvFTD) Behavioral variant frontotemporal dementia (FTD) is the most common subtype, and its key focus is changes in personality and behavior due to damage to the frontal lobe. Disinhibition, apathy, decreased empathy, perseverative and compulsive behavior, and changes in eating habits appear, and memory is relatively preserved in the early stages. ### Primary Progressive Aphasia (PPA) Primary progressive aphasia is a subtype in which language function declines first. It is divided into the non-fluent type, where words cannot be found, speech becomes slurred, or grammar breaks down, and the semantic type, where the meaning of words is lost. Speech impairment increasingly interferes with everyday communication. ## Causes and Pathophysiology Frontotemporal dementia is based on frontotemporal lobar degeneration, in which abnormal proteins accumulate in nerve cells. The main pathological proteins are tau and TDP-43, and the clinical pattern and course vary depending on which protein is accumulated. Many cases occur without a clear family history, but some are hereditary and involve genetic mutations such as MAPT, GRN, and C9orf72. In particular, C9orf72 mutation appears together with amyotrophic lateral sclerosis (ALS). ## symptoms Early symptoms of frontotemporal dementia vary depending on the area of damage. - Personality/behavioral changes: disinhibition, apathy, decreased empathy, impulsive or socially inappropriate behavior - Changes in eating habits: preference for sweet foods, overeating, compulsive eating - Speech disorders: Difficulty finding words, decreased fluency in speech, loss of word meaning - Decreased executive functioning: Decreased ability to plan, make judgments, and solve problems. Memory decline is often not noticeable in the beginning but appears as it progresses, so it can easily be mistaken for a psychiatric disease. ## diagnosis Diagnosis is a combination of medical history, neuropsychological testing, and brain imaging. - History taking: Check the time of onset and patterns of behavioral and language changes through guardians - Neuropsychological testing: assessing selective decline in executive function, behavior, and language domains. - Behavioral variant diagnostic criteria: Core items such as disinhibition, apathy, and decreased empathy are evaluated using the Rascovsky criteria revised in 2011. - Brain MRI: confirms focal atrophy of the frontal and temporal lobes - Functional imaging: Evaluation of decreased metabolism and blood flow in the relevant area ## treatment There is no cure, and symptom management and staying safe are the mainstays of treatment. Cholinesterase inhibitors used for Alzheimer's disease have limited effectiveness and may worsen behavioral symptoms in some people. For behavioral symptoms, environmental adjustments and non-pharmacological approaches are given priority, and drugs are used cautiously when necessary. Speech rehabilitation is helpful for primary progressive aphasia. Parental education and support are also important. Because the disease occurs at a relatively young age, the burden of care is high, so understanding behavioral symptoms and linking social support is necessary. ## Frequently asked questions ### Q01How is frontotemporal dementia different from Alzheimer's disease? While Alzheimer's disease begins with a decline in recent memory, in frontotemporal dementia, memory is relatively maintained in the early stages, and personality and behavioral changes and language disorders first appear. Additionally, the average age of onset is about 58 years, which is relatively young. ### Q02My personality is changing and I am behaving rudely. Could it be dementia? In behavioral variant frontotemporal dementia (bvFTD), early symptoms include disinhibition, apathy, decreased empathy, impulsive or inappropriate behavior, and changes in eating habits. It can easily be mistaken for a simple personality change or psychiatric problem, so if the changes persist, a neurological evaluation is necessary. ### Q03Is this a disease that causes slurred speech and inability to come up with words? Primary progressive aphasia (PPA) is a subtype in which language function deteriorates before memory, and is manifested by inability to find words, slurred speech, and breakdown in grammar. If your language disorder progresses slowly, it is best to get evaluated. ### Q04Is frontotemporal dementia inherited? It appears familial in some patients, and genetic mutations such as MAPT, GRN, and C9orf72 are known. However, not all patients have hereditary disease, and if there is a clear family history, genetic counseling is considered. ### Q05How is it diagnosed? Behavioral and language changes are evaluated through medical history and neuropsychological tests, and Rascovsky's criteria are applied to behavioral variants. Brain MRI shows localized atrophy of the frontal and temporal lobes, and when necessary, functional imaging tests are used to evaluate metabolic deterioration in those areas. ### Q06Is there any cure? Cholinesterase inhibitors used for Alzheimer's disease have limited effectiveness and may worsen symptoms in some cases. Therefore, treatment focuses on symptom management, including behavioral symptoms, safety management, speech rehabilitation, and guardian education. ## Related articles - Dementia Overview - Alzheimer's disease - vascular dementia - Lewy body dementia This article provides general medical information and does not replace an individual diagnosis or treatment plan. Please seek a medical assessment if symptoms persist. ## Important notice This Markdown alternative helps search and AI tools understand the public page accurately. It provides general health information and does not replace an individual diagnosis or treatment.