Question 1

Frontotemporal dementias

Accepted Answer

group of neurodegenerative disorders that leads to deterioration of the anterior temporal and/or frontal lobes resulting in behavioral changes, language dysfunction, and/or motor deficits.

Question 2

Biomarkers of frontotemporal dementias

Accepted Answer

40% caused by an abnormal accumulation of tau protein BUT more than half of FTDs are tau negative and are caused by an abnormal accumulation of TDP-43 protein

Question 3

Age of diagnosis of frontotemporal dementias

Accepted Answer

Known for being a younger-onset dementia syndrome FTD = onset 40s - 50s Alzheimer's = onset after 65

Question 4

FTDs are commonly divided into three variants according to the symptom pattern, which are?

Accepted Answer

Behavioral variant language variant motor variant

Question 5

FTD Behavioral Variant

Accepted Answer

previously referred to as Pick's disease progressive, focal degeneration of the bilateral frontal and temporal brain regions, and/or the presence of argyrophilic globular inclusions (Pick bodies; clumps of protein that builds up) and swollen, achromatic neurons (Pick cells). Pick bodies and Pick cells can be found in frontal/temporal neocortex, amygdala, globes pallidus, etc. bvFTD of three FTD variants is the most common (about half of all FTD cases) and occurs more frequently in men than women.

Question 6

Presentation and course of FTD Behavioral Variant

Accepted Answer

Initial changes in personality and emotion regulation, two subtypes: apathetic subtype - reduced motivation, lack of interest in prior activities, progressive social isolation, decreased empathy, disinhibited subtype - disinhibition (e.g., overspending), impulsivity (e.g., inappropriate comments in public), hyperorality (especially for sweet foods), repetitive motor movements, and perseverative behaviors. rarely results in hallucinations/delusions (compared to schizophrenia) Executive/generation deficits with relative sparing of memory and visuospatial functions generally lack awareness of deficits

Question 7

pathophysiology and incidence of FTD Language Variant

Accepted Answer

Most cases of PPA have tau-positive, ubiquitin/TDP-43-positive frontotemporal lobar degeneration (FTLD) pathology or AD pathology

Question 8

The language variant of FTD is commonly referred to as...?

Accepted Answer

primary progressive aphasia (PPA)

Question 9

FTD language variant/primary progressive aphasia symptoms

Accepted Answer

characterized by initial and prominent language deterioration with relative preservation of other cognitive abilities until late in the disease process presents with marked word-finding difficulty, decreased performance on word list generation tasks, problems with verbal comprehension, and dysarthria. Reading and writing abilities typically remain preserved longer than speech.

Question 10

distinguishing the logopenic from the semantic variant.

Accepted Answer

Logopenic has relatively spared grammar, object knowledge, and single word comprehension

Question 11

distinguishing the logopenic from the progressive nonfluent variant

Accepted Answer

Logopenic has spared motor speech

Question 12

Progression of FTD semantic variant in terms of language functions

Accepted Answer

deficits in object knowledge begin with loss of the ability to finely distinguish between types of objects (e.g., types of animals such as a tiger and a lion) progresses to a broader difficulty making such distinctions (e.g., the difference between a tiger and an alligator) progresses to a loss of knowledge of the word and the category (e.g., a loss of knowledge of what tigers are and what animals are).

Question 13

FTD Motor Variant subtypes

Accepted Answer

progressive supranuclear palsy (PSP) - most common corticobasal syndrome (CBS) FTD with motor neuron disease (FTD-MND)

Question 14

progressive supranuclear palsy (PSP)

Accepted Answer

most common FTD Motor Variant subtype d/t astrocytic lesions, tau-positive neurofibrillary tangles, and neuropil threads within the brainstem and basal ganglia. Supranuclear vertical gaze palsy (impaired downward and upward gaze) which may be perceived as blurred vision Bradykinesia Rigidity Swallowing problems Frequent falls

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Frontotemporal Dementias Flashcards

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