Frontotemporal Dementia - NPch.20 Flashcards
General Info
What is Frontotemporal dementia?
Several types of dementia involving the progressive degeneration of the brain’s frontal and temporal lobes
What are the 4 main types of FTD?
- BV-FTD
- FTD-MND
- SD
- PNFA
On what do clinicians base their diagnosis?
Based in info from clinical interviews, clinical manifestations of a problem, and neuropsychological assessment
(neurological assessment and imaging can be supportive of a diagnosis but aren’t required)
What is the value of Neuropsychological Assessment?
- Helpful in early phases of behavioral and cognitive changes: monitor further deterioration and provides support in first years
- Neuropsychological findings are useful to find and train for compensatory strategies
- Many patients have fear of dementia -> Neuropsychological assessment and psycho-education provides relief
(Epidemiology)
What is some general info on the epidemiology of FTD?
(In general) 3rd most common cause of ‘cortical dementia’ behind Alzheimer’s an Lewy’s bodies
What is the prevalence of FTD?
- 15-22/100.000 for ages 45-65
- other studies: 9.4/100.000 for ages 60-69
What is the onset of FTD?
Usually before 65, with a peak between 50-60
- 10% of FTD patients are >70
- onset can also occur before 50
!!! The older the age of onset, the slower the rate of decline.
BUT, if somebody has high cognitive reserves, that person usually has a later onset, but a faster rate of decline as well !!!
What is the duration of FTD?
Average duration >8 years, with a range of 2-20 years
- shortest duration is FTF-MND, with 3 years
Is there any gender differences for FTD?
No
(Genetics)
What are the findings regarding FTD and family history?
in 40% of patients there’s a positive family history (at least one relative has dementia/ motor disorder)
What is the Autosomal dominance inheritance pattern?
How a single copy of a mutated gene on one of the non-sex chromosomes causes a genetic disorder.
Found in:
- 35% of BV-FTD cases
- 25% of FTD-MND cases
- 5% of SD cases
- 5% of PNFA cases
What role do genes play in FTD?
- MAPT, GRN and C90RF72 genes
- 10% of FTD cases: mutation in MAPT gene
- 7% of FTD cases: mutation in GRN gene
- 9% of FTD cases: mutation in C90RF72 gene
(Neuropathology)
What is our current knowledge on the neuropathology of FTD?
Knowledge on causes is very limited, apart from genetic deficits
How are some proteins associated with FTD?
3 specific proteins have been found that are associated with FTD
- Are found in Temporal, Frontal an Parietal Cortices
- Also found in Hippocampus, and especially basal ganglia
- Each specific protein has been shown to be associated with one of the aforementioned genetic mutations
What is the treatment for FTD?
Currently there is no curative treatment for FTD.
- Trazodone (serotonin antagonist and reuptake inhibitor) helps a bit with some symptoms:
~ irritability, agitation
~ depressive symptoms
- Current treatment entails good counselling for relatives and friends of patient
- Likely that protein-specific therapies will be developed in the near future
BV-FTD
What is Behavioral Variation - Frontotemporal dementia?
It is the most common manifestation of FTD. Characterized by:
- Changes in Personality
- Changes in Social cognition
- Impairments in Cognition
- Impairments in Language
What are the personality changes specific to BV-FTD?
- Decreased emotional involvement
- Emotional blunting
- Loss of empathy
- Apathy
- (Hyperactivity and restlessness)
What are the changes in Social Cognition?
- Personal neglect
- Act irresponsibly (due to inability to determine consequences of behavior)
- stereotypical/cliched behavior
- compulsive and inflexible behavior (stick to routines)
How does language gradually deteriorate in patients with BV-FTD?
1) Initially, language is fluent, maybe due to echolalia (repetition of speech immediately after hearing somebody say something) or perseverations (similar to echolalia)
2) Disease advances, patients speak less (economy of speech)
- Initiate conversation less often
- In advanced stages, answer only with yes or no
3) In the end, patients become mute
What are the specific language impairments of BV-FTD?
- Gradual naming impairments
- Can’t connect all components of an object into one
- Word fluency deteriorates until spontaneous language is lost
What are the 4 main types of cognitive impairments in patients with BV-FTD?
- Impairments in Attention/concentration
- Impairments in Executive Functions
- Impairments in Memory
- Impairments in abstract thinking
Impairments in Attention/Concentration
- Extremely easy for patients to be distracted (both due to external and internal reminders)
- Make minimal effort (economy of effort)
~ Encouragement doesn’t help
~ Patients usually don’t persevere due to no need for achievement
~ Lack of insight into own performance and errors
Impairments in Executive Functions
Problems in:
- Mental flexibility:
~ Impaired task switching
~ Very strong perseverance in actions they’ve already initiated
- planning and organizing: fail to work according to a specific plan/strategy/instructions
- Responses: responses are often arbitrary, impulsive, and lacking in any basis/arguments
Impairments in Memory
Although usually there aren’t any signs of clinical memory impairments, sometimes assessment of memory performance results in abnormal performance. This can be due to:
- lack of interest or motivation: If material is meaningful, patient remembers it a lot better compared to material that is meaningless or incoherent
- Insufficient use of memory in everyday life
What are the 3 profiles of BV-FTD?
- Disinhibition, Distractibility, hyperactivity profile
- Apathy, Lack of initiative, behavioral withdrawal profile
- Stereotypy and compulsive behavior profile
What symptoms are required for the diagnosis of BV-FTD
- Deterioration in social behavior
- Impairment of the regulation of personal behavior at an early stage of the disease
- emotional blunting
- loss of insight into disease early on
(speech/language impairments are supportive of a diagnosis, but not required for it)
(Other symptoms: not required)
What is observed in patients with BV-FTD through neuroimaging?
- Frontal and/or temporal atrophy
- (sometimes) asymmetrical atrophy in hippocampus
MND
What is FTD - Motor Neuron Disease?
A group of rare neurodegenerative disorders that affect motor neurons which control voluntary muscles of the body.
What is the prevalence of MND?
About 5-15% of FTD patients suffer from MND
What are the symptoms for MND?
- Similar language impairments to BV-FTD
- Dysarthria (can’t articulate words)
- Muscular atrophy
- Fasciculations (minor muscle contractions)
- Loss of strength in arms and legs
What is the most common MND?
Amyotrophic Lateral Sclerosis (ALS). Patients with ALS also develop cognitive and behavioral symptoms, therefore some ALS patients meet criteria for BV-FTD
PNFA
What is Progressive Non-Fluent Aphasia?
A gradually progressive deterioration in language production, but language comprehension and the rest of cognition is intact. This can be seen by patients distress about their impairments and through attempts to self-correction
What are the symptoms of PNFA?
- Problems with phonology and syntax
- Dysarthria
- Impaired reading/writing
- Comprehension of complex sentences may be imparied
What symptoms arise in the patient due to the patient’s reaction to his/her impairments?
1) Frustration, irritability, depression (immediate reaction to impairment)
2) Anxiety, insecure, avoid social contact
3) Apathy, motivation decreases, neglect of personal care, involvement decreases, egocentrism
4) Insight into dysfunctions and impairments also deteriorates
Because of all these other cognitive symptoms might develop as well
What are the requirements to diagnose PNFA?
- Agrammatism
~ Speech contains only content words and no function words
~ Wrong grammar - Phonemic Paraphasia: Speech error regarding speech sounds/phonemes with words
- Word-finding problems
- No other cognitive/behavioral dysfunctions initially
What is observed in patients with PNFA through neuroimaging?
Asymmetrical atrophy in left frontal temporal regions
SD
What is Semantic Dementia?
Multimodal and progressive breakdown of semantic knowledge (language comprehension is impaired, production is intact) but without phonological or syntactical errors
What are the language symptoms of SD?
- Semantic paraphasias and generalizations (a word is replaced by a better-known word from the same category)
- Loss of knowledge about the meaning of a word
- Strong naming impairments (patients might even know the components of an object but won’t be able to name the object as a whole)
- Neologisms: Use of a non-real word in place of the intended word
- Phonemic Paraphasia: When a sound substitution is made, but the stated word still resembles the intended word (e.g. saying “that” instead of “hat”, or “tephelone” instead of “telephone”
What are some other symptoms of SD?
- Compulsive Behavior
- Stereotypy: Patients dislike any deviation from fixed routines
- More egocentric
- Interests diminish
- Emotional involvement decreases
- Deficits in executive functions, but in later stages of the disease
What symptoms are required to diagnose SD?
- Visual/verbal semantic impairments, manifested as fluent, empty speech and word-finding problems
- Also visuo-perceptual impairments: prosopagnosia and impaired object recognition (object agnosia): Assessed through Visual Object and Space perception Battery
- initially, no other cognitive/behavioral dysfunctions
What can be observed in patients with SD through Neuroimaging?
- Bilateral or asymmetrical atrophy of temporal lobes. If asymmetrical, affects left side
- Hippocampal atrophy
Neurocognitive disease due to Vascular Disease (VaD)
What are the symptoms of VaD?
- Mental & Psychomotor slowing
- Problems with initiating, planning and mental flexibility
- Relative intact memory and language skills
- Personality changes (maybe due to denial, or loss of insight that comes about from pathology)
What two symptoms are important when it comes to differentiating VaD from AD?
- Mental & Psychomotor slowing
- Relative intact memory and language skills
What is a problem when it comes to differentiating between VaD and AD?
Problems with memory, language or visuo-percpetual and visuospatial functioning might develop after strokes/further progression of SVD
What is Small Vessel Disease (SVD)?
Abnormalities related to small blood vessels in the brain
What is the manifestation of VaD?
Dementia can start suddenly or begin slowly over time
What is the prevalence of Vascular Disease and AD?
!!! Most common causes for Dementia !!!
- 3-12 months after a stroke, approximately 25% of patients are classified with a major NCD
- Mainly diagnosed in young patients
- Prevalence of VaD differs between cultures (different obesity levels, smoking levels etc.)
What are the risk factors for VaD?
Similar to the risk factors for heart disease and strokes:
- Increasing age (small risk <65, large risk in 90’s)
- History of cardiac problems, strokes or TIA
- Vascular Damage, associated with:
~ High Blood pressure
~ High cholesterol
~ Smoking: directly damages blood vessels
~ Obesity
~ (Comorbid) Somatic Diseases associated with increased risk of vascular damage (e.g. Diabetes)
NCD due to substance abuse
What are the two main NCD’s due to substance abuse?
- Wernicke’s Encephalopathy
- Korsakoff’s syndrome
Wernicke’s encephalopathy
Double Vision & Cerebellar disturbances that affect motor coordination problems
Korsakoff’s syndrome
- Deficits in learning
- Memory retrieval, deficits for both recent and long-term memory
- Problems in Executivw functions (e.g. disinhibition) and personal changes (apathy)
- Lack of insight into problems or denial or confabulation (production of fabricated, distorted, or misinterpreted memories about oneself or the world)
How can these two NCD from susbtance abuse be treated?
- Alcohol use is stopped
- Vitamins are given
In some cases, deterioration can even be reversed
