Frontotemporal Dementia Flashcards
What’s the percentage of FTD’s been caused by abnormal accumulation of tau protein
40%
Are all FTD cases tau positive?
No, more than half of the cases are TAU negative
For cases that are TAUnegative, FTD is caused by abnormal accumulation of what
TDP 43 protein
Name two contributions that lead to frontal temporal dementia
TAU abnormal accumulation
TDP 43 Abnormal accumulation
Percentage of people with frontal temporal dementia having a family history of dementia
40%
What age does cognitive and behavioral symptoms start emerging in frontotemporal dementia
40 to 50
How many variants are there in frontal temporal dementia
Behavioral variant
Language variant
Motor variant
What is another term for frontal temporal dementia behavior variant
Picks disease
What happens in behavioral variant of FTD
Initial changes in social behavior, personality.
Progressive for focal de generation of frontal temporal brain regions
Presence of argyrophilic globular Inclusions (picks bodies)
Swollen acrobatic cells known as picks cells
Common locations in the brain that are affected by picks disease
Frontal temporal neo cortex Amygdala Dentate gyrus Piramidal cells of CA one section of hippocampus Hypothalamic lateral to pronucleus Dorsal medial region of putamen Globus pallidus Lucas cerulus
50% of patients with picks present with greater left or right hemisphere involvement
Left Hemisphere involvement is greater than right hemisphere involvement
Right hemisphere in five minutes about 20%
Other neurological contributions for picks disease
Neuro inflammation
Non thyroid Spectrum auto immune disorders
Altered metabolism of serotonin and lower levels of CSF dopamine
Most common variant of FTD
Behavior variant accounts for half
Age and gender
Average age 54 years,
onset between 40 to 65,
Onset is very rare after age 75
Median survival rate from 3 to 8 years
Onset of FTD behavior variant
Insidious onset
2 subtypes
Apathy type
Disinhibited type
Other conditions to be considered
OCD, ritualistic behaviors, psychosis could be mistaken for schizophrenia. Hallucinations and delusions are not common in behavior FTD.
Diagnostic criteria using what language for probabilistic diagnosis
Possible probable definite
Probable diagnosis requires confirmed neural imaging, functional decline, and all criteria met for possible
Definite diagnosis of FTD behavior in variant
Probable diagnosis also with histopathological evidence on biopsy or post Mortem exam or non-genetic mutation
Behavioral variant frontal temporal dementia phenocopy syndrome
Does not progressed to dementia
Nonprogressive neural imaging results
No atrophy, normal scans
Neuropsychological findings with behavior variant FTD
Mostly everything is preserved early in the disease process except some deficits in executive functions in episodic memory.
Cognitive screening is typically insensitive to deficits early in the course.
Medication for behavior variant frontal temporal dementia
Acetylcholinesterase inhibitor’s typically prescribed for Alzheimer’s, do not work for behavior variant FTD
SSRI, SNRI are used for emotional symptoms
Frontal temporal language variant
Also known as primary progressive aphasia PPA
Three subtypes
Nonfluent agrammatic variant
Semantic dementia
Logopenic variant
PPA Characteristics
Initial and prominent language deterioration with relative preservation of other cognitive abilities until late
Marked word finding difficulties
Decreased performance on worthless generation
Problems with verbal comprehension
Dysarthria
Reading and writing abilities typically remain preserved longer than speech
Swallowing problems
Semantic dementia characteristics
Naming deficits with loss of single word knowledge and comprehension
loss of person recognition
Surface dyslexia or dysgraphia
Repetition OK, speech production OK
PPA nonfluent agrramatic variant
Wrong grammar in language production
effortful halting speech
impaired comprehension of complex sentences
Single word comprehension OK
object knowledge OK
Logo penic Variant
Impaired single word retrieval in speech and naming
Impaired repetition
Motor speech OK grammar OK
Neuropathology of PPA non fluent type
Deterioration in the left posterior frontal and insular regions associated with nonfluent forms of the disease
Strongly associated with TAU pathology
Predominant left posterior frontal insular pathology
Neuropathology of PPA, semantic dementia
Bilateral anterior temporal region
Most present with left hemisphere atrophy, 25% have right atrophy
ALL semantic dementia cases are caused by abnormal accumulation of TDP 43
 Least likely to be inherited
Neuropathology of PPA- logopenic variant
Left temporal parietal
Epidemiology of PPA
20 to 25% of people have semantic dementia
males more likely to have semantic dementia,
females more likely to have non-fluent in variant
There is more non-right handedness in semantic dementia
Average length of time for months at a death in language variant FTD
12 years
How to differentiate between nonfluent PPA and semantic dementia
Nonfluent PPA are known to have more problems with word list generation
Semantic dementia patients have extremely poor confrontation naming. Deficits in object knowledge begin with loss of the ability to distinguish between types of object and progressed to losing knowledge of the word
