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Frontotemporal dementia (FTD) is a clinical syndrome caused by degeneration of the frontal lobe of the brain and may extend back to the temporal lobe. It is one of three syndromes caused by frontotemporal lobar degeneration.
Symptoms can be classified (roughly) into two groups which underlie the functions of the frontal lobe: behavioural symptoms (and/or personality change) and symptoms related to problems with executive function.
Behavioural symptoms include lethargy and aspontaneity or oppositely disinhibition. Apathetic patients may become socially withdrawn and stay in bed all day or no longer take care of themselves. Disinhibited patients can make inappropriate (sometimes sexual) comments or perform inappropriate acts. Patients with FTD can sometimes get into trouble with the police because of inappropriate behaviour such as stealing. Recent findings indicate that psychotic symptoms are rare in FTD, possibly due to limited temporal-limbic involvement in this disorder. Among the FTD patients, only 2 (2.3%) had delusions, 1 of whom had paranoid ideation; no FTD patient had hallucinations. This was significantly less than the AD patients, 4 (17.4%) of whom had delusions and paranoia. [1] See ref. “Psychotic symptoms.”
Executive function is the cognitive skill of planning and organizing. Patients become unable to perform skills that require complex planning or sequencing.
Language skills can be affected in a number of ways with two broad patterns. Some patients remain fluent with normal phonology and syntax but increasing difficulty with naming and word comprehension, known as semantic dementia in which there is atrophy of the anterior temporal lobes, typically with an asymmetric pattern. Other patients, by contrast, present with a breakdown in speech fluency due to articulation difficulty, phonological and/or syntactic errors but preservation of word comprehension, referred to as progressive nonfluent aphasia.
In addition to the characteristic cognitive dysfunction, a number of primitive reflexes known as frontal release signs are often able to be elicited. Usually the first of these frontal release signs to appear is the palmomental reflex which appears relatively early in the disease course whereas the palmar grasp reflex and rooting reflex appear late in the disease course.
FTD can occur in patients with motor neurone disease (also known in the US as Lou Gehrig’s disease or amyotrophic lateral sclerosis) in a small number of cases. The prognosis for people with MND is worse when combined with FTD, shortening survival by about a year.[2]
A number of case series have now been published looking at the pathological basis of frontotemporal dementia. As with other syndromes associated with FTLD, a number of different pathologies are associated with FTD:
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