Frontotemporal dementia, or FTD, is a term used to describe a collection of related brain disorders that involve the neurodegeneration of the frontal and temporal lobes. In this video, I discuss the symptoms and subtypes of FTD as well as the pathological brain mechanisms underlying FTD.

TRANSCRIPT:

Frontotemporal dementia, or FTD, is a term used to describe a collection of related brain disorders that involve the neurodegeneration of the frontal and temporal lobes. The neurodegeneration in FTD is associated with a variety of symptoms, and different presentations of symptoms have been used to establish multiple subtypes of FTD. The three main subtypes are: behavioral variant FTD, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia. In behavioral variant FTD, patients display a variety of changes in behavior, emotions, personality, and executive control. For example, they may exhibit compulsive behavior, apathy, and/or socially inappropriate behavior. In primary progressive aphasias, the main impairment is with language. Patients who have the semantic variant of primary progressive aphasia display deficits in word comprehension as well as trouble recalling certain words, along with other language difficulties. Patients who have the nonfluent variant primary progressive aphasia have difficulty producing fluent speech, which might involve trouble speaking at all or problems with the use of grammar to produce understandable speech.

FTD is associated with a pathological process called frontotemporal lobar degeneration, which involves the death of neurons and other pathological changes in the frontal and temporal lobes, as well as other areas of the brain such as the anterior cingulate cortex and insular cortex. In FTD, the degeneration of neurons is associated with the accumulation of structurally abnormal proteins, which might contribute to cell death through multiple mechanisms. The protein accumulation and associated damage spreads, causing the symptoms of FTD to get progressively worse over time. While initially a patient may display symptoms linked to just one of the FTD subtypes, the spreading neurodegeneration may eventually lead to the manifestation of symptoms from multiple subtypes. Unfortunately at this point there are no drugs approved to treat FTD, so treatment primarily involves managing symptoms.

REFERENCES:

Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet. 2015 Oct 24;386(10004):167282. doi: 10.1016/S01406736(15)004614. PMID: 26595641; PMCID: PMC5970949.

Olney NT, Spina S, Miller BL. Frontotemporal Dementia. Neurol Clin. 2017 May;35(2):339374. doi: 10.1016/j.ncl.2017.01.008. PMID: 28410663; PMCID: PMC5472209.

Young JJ, Lavakumar M, Tampi D, Balachandran S, Tampi RR. Frontotemporal dementia: latest evidence and clinical implications. Ther Adv Psychopharmacol. 2018