Frontotemporal dementia

Question 1

Is Pick disease the same thing as bvFTD?

Answer 1

Yes

Question 2

Describe the neuropathology of behavioral variant FTD.

Answer 2

Tau protein accumulates in frontal-temporal areas. Left hemisphere is more likely to be affected relative to right.

Question 3

Which variant of FTD is most common?

Answer 3

The behavioral variant accounts for more than half of FTD cases.

Question 4

Cardinal symptoms of bvFTD are…

Answer 4

1) Florid personality changes, dramatic loss of insight into behavior. May appear more OCD, mentally rigid, emotionally blunted, apathetic, disinhibited, depressed, etc.
2) Socially inappropriate (hyperoral [binge eating;diet changes - eating only sweets] and disinhibited),
3) Memory and visuospatial functions spared
4) Poor planning, perseverative and stereotyped behavior, and utilization behavior.

Changes are insidious and have gradual progression. Early decline in social interpersonal conduct and personal conduct (decline in personal hygiene/grooming).

Question 5

For bvFTD:

What is disease age onset? What sex does it affect more? What is survival rate?

Answer 5

Onset between 35-75 years; average age is around 55. Onset is very rare after age 75. More likely to affect men than women. Median survival is 3 to 8+ years. Patients with bvFTD have shorter survival than patients with other FTD variants.

Question 6

Is bv FTD the second most common cause of early onset dementia?

Answer 6

Yes, after early onset AD.

Question 7

What are common neuroimaging findings for bvFTD?

Answer 7

Atrophy of the orbitofrontal, mesial frontal, and anterior insula cortices.
Basically frontal and/or anterior temporal atrophy on MRI.

Question 8

What do functional neuroimaging results show?

Answer 8

Frontal hypometabolism on PET.

Question 9

Which neurotransmitters are affected by bvFTD?

Answer 9

Serotonin and dopamine. Cholinergic system not affected.

Question 10

What is neuropsychological profile of bvFTD?

Answer 10

Typically neurocognitive presentation is spared early on in process. Deficits with executive functions and episodic memory can be found.

Question 11

What are treatments for bvFTD?

Answer 11

There is no cure. Because cholinergic system is not affected, actelycholinesterase inhibitors have little value for bvFTD. Similarly Namenda has not shown to be effective. SSRIs/SNRIs may be helpful for symptom reduction.

Question 12

Is FTD heritable?

Answer 12

In at least half of affected individuals, the answer is “no” – their FTD is said to be sporadic, meaning that none of their relatives are known to have FTD. However, approximately 40% of affected individuals with FTD do have a family history that includes at least one other relative diagnosed with a neurodegenerative disease. Their FTD is described as familial.

Question 13

What is the language variant of FTD also known as?

Answer 13

Primary progressive aphasia.

Question 14

What are the 3 subtypes of PPA?

Answer 14

1) Nonfluent/agrammatic variant/Progressive nonfluent aphasia
2) Semantic dementia/temporal variant FTD
3) Logopenic variant/logopenic progressive aphasia

Question 15

What is PPA characterized by?

Answer 15

Language deterioration with relative preservation of cognitive abilities until late in the disease process.

Commonly presents with marked word finding problems, as well as decreased verbal fluency, problems with verbal comprehension, and dysarthria.

Reading and writing abilities typically persist longer than speech.
Swallowing problems also are present.

Question 16

Which features should be examined to assess kind of PPA?

Answer 16

Grammar
Motor speech
Sound errors
Wf px or delays
Repetition
Single word and syntax comprehension
Naming
Semantic knowledge
Reading
Spelling

Question 17

What is the neuropathology of PPA?

Answer 17

It is heterogeneous - each variant has different pathological processes. Most cases of PPA are tau positive or have AD pathology.

Question 18

What is the neuropathology of nonfluent/agrammatic variant?

Answer 18

Damage to left posterior frontal or insula areas.

Question 19

What is the neuropathology of semantic dementia?

Answer 19

Damage to anterior temporal area.

Question 20

What is the neuropathology of logopenic variant?

Answer 20

Damage to left temporoparietal area.

Question 21

Can PPA be inherited?

Answer 21

Yes, it can be in an autosomal dominant manner.

Question 22

What is the incidence rate for PPA?

Answer 22

Unknown.

Question 23

What is survival rate for PPA?

Answer 23

Likely 12 years.

Question 24

What is the course of PPA?

Answer 24

Begins with disordered language, and progressively worsens to include more widespread neuropsychological problems. There is little evidence that PPA can be slowed and there is no cure.

Question 25

What are features of logopenic variant?

Answer 25

1) Impaired single word retrieval in conversation
2) Impaired repetition
3) Speech errors in spontaneous speech and naming

Question 26

What are features of semantic dementia?

Answer 26

1) Impaired confrontation naming
2) Impaired single word comprehension
3) Impaired object knowledge
4) Dyslexia, difficulties reading irregularly spelled words
5) Dysgraphia/poor spelling and writing skills
Spared repetition

Question 27

What are features of nonfluent/agrammatic variant?

Answer 27

1) Agrammatism
2) Effortful halting speech
3) Impaired comprehension of syntactically complex sentences

Question 28

What would you expect for npsych profile for PPA?

Answer 28

Impaired performance on language measures, with relatively strong performance in other cognitive domains.

Question 29

What is the last FTD variant?

Answer 29

FTD motor variant, characterized by progressive deterioration of motor functions.

Question 30

What are the 3 subtypes of FTD motor variant?

Answer 30

1) progressive supranuclear palsy (PSP)
2) corticobasal degeneration (CBD)
3) FTD with motor neuron disease (FTD-MND)

Question 31

Which subtype of FTD motor variant is most common?

Answer 31

PSP

Question 32

What is the neuropathology of PSP?

Answer 32

Tau in brainstem and basal ganglia

Question 33

What is neuropathology of corticobasal degeneration?

Answer 33

Assymetrical atrophy of the bilateral premotor cortex, superior parietal lobules, and striatum.

Question 34

What is neuropathology of FTD with motor neuron disease?

Answer 34

Ubiquitin (not tau) pathology involving frontal and temporal poles.

Question 35

What is the course and features of PSP?

Answer 35

Age of onset typically in 60s. Average life expectancy is 5 years. Develop vertical gaze palsy (difficulties looking up and down - reported as blurred vision; this can be helpful in distinguishing from PD), bradykinesia, swallowing difficulties, and frequent falls (falling backwards). Inappropriate emotional expression such as forced laughing or prolonged crying may occur.

Question 36

What is the course and the features of corticobasal degeneration?

Answer 36

Age of onset typically in 50s to 70s.

Asymmetric ideomotor and limb kinetic apraxia and executive dysfunction (disinhibition, apathy, inattention, and perseveration) are present.

Clumsy and slow movements of left arm and leg, followed by impairment of right limbs, with extreme impairment of voluntary movements and resting tremor. Alien hand syndrome. Myoclonus. Cortical sensory impairment.

NP features include impaired visuospatial skills, poor math skills, apraxia, and alien hand syndrome.

Question 37

What are core features across FTD motor variant presentations?

Answer 37

Gait change and poor balance. May look like PD.
Core NP features are impaired executive functions (e.g., mental flexibility, planning, problem solving, judgment and impulse control). Language and memory might be affected. Apathy, indifference, and reduced insight into changes might be seen.

Question 38

What is agrammatism?

Answer 38

Inability to speak grammatically - speech may include omission of critical words, leading to telegraphic speech.