HC9: Frontotemporal dementia
Arnold Pick (1892) is a 71-year-old man with a progressive decline in speech, paraphasia’s,
difficulties recognizing objects
Autopsy: atrophy frontal and temporal lobes
Neuropathology (Pick’s bodies), described by Alois Alzheimer (1911)
Pick’s disease: progressive dementia with prominent early changes in
behavior
A pick body is a bundle of protein Tau, the same tau we see in the
tangles in AD.
Frontotemporal dementia (FTD) or frontotemporal lobar
degeneration (FTLD)
Epidemiology:
.02% general population (all ages)
FTD +- 5% of the dementia cases
Under 60 FTD is more common than AD. People who develop dementia at a young
age are more likely to be diagnosed with frontotemporal dementia than with AD.
There is also a strong genetic factor:
30-50% of patients with FTD have a family history
Relatives of patients with FTD also have an enhanced risk of 3.5x than general
population
FTD – variations with different symptoms and underlying neuropathology several
classifications proposed
Neary et al. (1998):
Behavioral variant: behavioral changes most prominent symptom
Language variant (primary progressive aphasia): language disturbances most
prominent symptoms. The earlier classification makes a distinction between:
o Semantic dementia – word comprehension
o Progressive nonfluent aphasia – word production difficulties
The most recent classification is:
Behavioral variant
Language variant – primary progressive aphasia
o Semantic variant (comparable SD)
o Agrammatic variant (overlap progressive nonfluent aphasia)
o Logopenic variant – word findings difficulties, no impairments in
comprehension
The main distinction is between the so called behavioral and language variant of FTD.
Etiology
Typical neuropathology FTD is focal damage in frontal and anterior temporal lobes
The damage is caused by abnormal aggregation of proteins
, o Tau, TARDNA binding protein (TDP), fused in sarcoma (FUS) protein
o Also associated other CNS diseases (e.g., amyotrophic lateral sclerosis) =
The same abnormal proteins are also associated with other
neurodegenerative disease
Because of the variation and the different symptoms there is not a single neuropsychology of
FTD.
Hutchinson & Mathias (2007) – meta-analysis 94 studies comparing FTD (n=1748) and AD
(n=2936)
All subtypes FTD
Best discrimination FTD and AD:
o Memory (e.g., AVLT, Rey): FTD > AD
o Language/verbal ability (e.g., naming, fluency): FTD < AD
Visual spatial/construction (e.g., Beery): FTD > AD
EF tasks: no difference between FTD and AD
There were some differences in the overall pattern of main cognitive impairments in the two
groups, but all the different variations were put together in this study.
Behavioral variant (bvFTD)
- Most common (50-70% FTD cases)
- More common in men
- Insidious beginning, slow progression symptoms
- Earliest signs subtle personality, behavioral changes
6 major symptoms:
Behavioral disinhibition
Apathy
Loss of empathy: egocentric behavior
Compulsive behavior: compulsive checking, rigorous time keeping, having to eat your
meal at fixed times and not being able to eat at other times
Hyperorality: exploring objects in the mouths, changing eating habits (a woman who
never ate apple pie now had to eat apple pie with every meal and refused to eat
something else) = very specific eating habits
Deficits EF in the presence of relatively preserved memory and visuospatial functions
This is in the earlier stages with progression of the disease, more functions become
affected
For diagnosis: at least three of these should be present
Neuropathology
Initially there is degeneration in the anterior temporal and frontal areas anterior cingulate
gyrus, dorsal anterior insula, lateral orbitofrontal cortex.
Arnold Pick (1892) is a 71-year-old man with a progressive decline in speech, paraphasia’s,
difficulties recognizing objects
Autopsy: atrophy frontal and temporal lobes
Neuropathology (Pick’s bodies), described by Alois Alzheimer (1911)
Pick’s disease: progressive dementia with prominent early changes in
behavior
A pick body is a bundle of protein Tau, the same tau we see in the
tangles in AD.
Frontotemporal dementia (FTD) or frontotemporal lobar
degeneration (FTLD)
Epidemiology:
.02% general population (all ages)
FTD +- 5% of the dementia cases
Under 60 FTD is more common than AD. People who develop dementia at a young
age are more likely to be diagnosed with frontotemporal dementia than with AD.
There is also a strong genetic factor:
30-50% of patients with FTD have a family history
Relatives of patients with FTD also have an enhanced risk of 3.5x than general
population
FTD – variations with different symptoms and underlying neuropathology several
classifications proposed
Neary et al. (1998):
Behavioral variant: behavioral changes most prominent symptom
Language variant (primary progressive aphasia): language disturbances most
prominent symptoms. The earlier classification makes a distinction between:
o Semantic dementia – word comprehension
o Progressive nonfluent aphasia – word production difficulties
The most recent classification is:
Behavioral variant
Language variant – primary progressive aphasia
o Semantic variant (comparable SD)
o Agrammatic variant (overlap progressive nonfluent aphasia)
o Logopenic variant – word findings difficulties, no impairments in
comprehension
The main distinction is between the so called behavioral and language variant of FTD.
Etiology
Typical neuropathology FTD is focal damage in frontal and anterior temporal lobes
The damage is caused by abnormal aggregation of proteins
, o Tau, TARDNA binding protein (TDP), fused in sarcoma (FUS) protein
o Also associated other CNS diseases (e.g., amyotrophic lateral sclerosis) =
The same abnormal proteins are also associated with other
neurodegenerative disease
Because of the variation and the different symptoms there is not a single neuropsychology of
FTD.
Hutchinson & Mathias (2007) – meta-analysis 94 studies comparing FTD (n=1748) and AD
(n=2936)
All subtypes FTD
Best discrimination FTD and AD:
o Memory (e.g., AVLT, Rey): FTD > AD
o Language/verbal ability (e.g., naming, fluency): FTD < AD
Visual spatial/construction (e.g., Beery): FTD > AD
EF tasks: no difference between FTD and AD
There were some differences in the overall pattern of main cognitive impairments in the two
groups, but all the different variations were put together in this study.
Behavioral variant (bvFTD)
- Most common (50-70% FTD cases)
- More common in men
- Insidious beginning, slow progression symptoms
- Earliest signs subtle personality, behavioral changes
6 major symptoms:
Behavioral disinhibition
Apathy
Loss of empathy: egocentric behavior
Compulsive behavior: compulsive checking, rigorous time keeping, having to eat your
meal at fixed times and not being able to eat at other times
Hyperorality: exploring objects in the mouths, changing eating habits (a woman who
never ate apple pie now had to eat apple pie with every meal and refused to eat
something else) = very specific eating habits
Deficits EF in the presence of relatively preserved memory and visuospatial functions
This is in the earlier stages with progression of the disease, more functions become
affected
For diagnosis: at least three of these should be present
Neuropathology
Initially there is degeneration in the anterior temporal and frontal areas anterior cingulate
gyrus, dorsal anterior insula, lateral orbitofrontal cortex.
