Frontotemporal Dementia _____________________________________________________________________
FORMS OF FRONTOTEMPORAL DEGENERATION
Behavioral presentation • Behavioral variant FTD (bvFTD) Language presentation, variants of primary progressive aphasia (PPA) • Nonfluent/agrammatic PPA (nfvPPA), previously called progressive non-fluent aphasia (PNFA) • Semantic variant PPA (svPPA), previously called semantic dementia (SD) • Logopenic variant PPA (lvPPA) (often found to have Alzheimer disease pathology at autopsy) Associated movement disorders (not classified as FTD, but have shared symptoms) • Corticobasal syndrome (CBS) • Progressive supranuclear palsy (PSP) • Motor neuron disease, also called amyotrophic lateral sclerosis (FTD/MND or FTD/ALS) Source: Compiled by Author
Table 1
BEHAVIORAL VARIANT FTD
Major Clinical Features
Examples
Disinhibition
Making inappropriate comments, taking food off someone else’s table at a restaurant, telling sexual jokes, shoplifting, hitting Less involved in old hobbies or activities, deterioration in personal hygiene
Apathy
Loss of empathy
Indifferent when a family member is hurt
Compulsive behaviors
Repeating the same phrase, clapping hands in the same pattern repeatedly, checking the time repeatedly
Hyper-oral behaviors Overeating, eating one certain type of food, eating excessive sweets Impaired executive function Poor performance at work, poor financial decisions, difficulty planning and preparing a meal Source: Compiled by Author Table 2
LANGUAGE PRESENTATION Nonfluent/Agrammatic Variant PPA
disease (MND). Some patients may present with symptoms that overlap the different subtypes of FTD or may develop symptoms of other subtypes of FTD as the disease evolves. As more is learned about FTD, the terminology and classification of the subtypes may be revised.
Nonfluent/agrammatic variant PPA, also referred to as progres- sive non-fluent aphasia (PNFA), accounts for about 25% of all FTD cases and involves problems with language expression [13]. People with nfvPPA have difficulty producing speech but retain the meaning of words and know what they want to say. As a result, speech may become hesitant, slow, and labored. Speech patterns may be “agrammatic” or “telegraphic,” mean- ing that only the most important content words are used, without connecting words. For example, a patient might say “Tuesday…hospital…sister.” Patients with nfvPPA have dif- ficulty talking on the telephone and tend to talk progressively less. Eventually, some may become mute. While in the early stages, these patients continue to understand the speech of others, but this comprehension is eventually lost also. Reading and writing skills are better preserved than speech, although these abilities are also eventually lost. As the disease progresses, patients may develop behavioral symptoms. Some individuals with nfvPPA may develop extrapyramidal symptoms of rigidity and tremors, as seen in CBS and PSP.
BEHAVIORAL PRESENTATION Behavioral Variant FTD
As noted, bvFTD is the most common type of FTD, estimated to account for more than half of all cases [13]. The prominent features include disinhibition, apathy/inertia, loss of empathy, compulsive behaviors, hyper-orality, and impaired executive function ( Table 2 ) [14]. People with FTD may become socially withdrawn, inflexible, and impulsive. They may have a short- ened attention span and a tendency to be easily distracted. Behavior may become socially inappropriate. People with bvFTD are usually unaware of the changes in their personality and behavior and the impact these changes have on others. Memory and visual-spatial functioning are initially relatively spared in bvFTD. Some individuals with bvFTD may develop symptoms similar to Parkinson disease, such as bradykinesia, rigidity, postural instability, and masked face.
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MDRI2026
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