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Neuropsych Board Prep > Frontotemporal Dementias > Flashcards

Frontotemporal Dementias Flashcards

1
Q

What are the FTD variants?

A

Behavioral, language, motor

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2
Q

What was the behavioral variant of FTD (bvFTD) previously called?

A

Pick disease

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3
Q

How do Pick bodies differ from pathology in AD?

A
  • Pick bodies have straight, fibrous appearance of tangled tau protiens.
  • Neurofibrillary tangles in AD have a paired and coiled construction
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4
Q

Neuropathology of bvFTD

A
  • Tau proteins are mutated and bind to microtubles, produce toxic inclusions
  • Argyrophilic circular inclusions are known as Pick bodies, co-occur with balloon-shaped Pick cells in neuronal cytoplams
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5
Q

Where are common cerebral locations of Pick bodies?

A
  • Amygdala
  • Dentate gyrus
  • Hippocampus pyramidal cells- CA1 section and subiculum
  • Hypothalamic lateral tuberal nucleus
  • Dorsomedial region of the putamen
  • Globus pallidus
  • Locus ceruleus
  • Mossy fibers, monodendritic brush cells in granule cell layer of the cerebellum
  • Frontal and temporal neocortex
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6
Q

What is the most common FTD variant?

A

bvFTD

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7
Q

bvFTD age of onset and gender trends

A
  • More common in men than women

- Onset between 40-65, average age 54

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8
Q

Early symptom presentation in bvFTD

A
  • behavioral disinhibition: begins with changes in personality, interpersonal conduct, emotional regulation
  • apathy or inertia
  • loss of sympathy or empathy
  • perseverative, stereotyped, or compulsive/ritualistic behavior
  • Hyperorality, dietary changes
  • Executive/generation deficits, sparing of relative memory and visuospatial functions
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9
Q

What are 3 subtypes of primary progressive aphasia (PPA)

A
  • nonfluent/agrammatic (progressive nonfluent aphasia)
  • semantic dementia (temporal variant FTD)
  • logopenic variant (logopenic progressive aphasia and phonological variant PPA)
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10
Q

What neuropathology underlies most cases of PPA?

A

tau-positive, ubiquitin/TDP43-positive frontotemporal lobar degeneration or AD pathology

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11
Q

What neuroanatomic damage is most associated with nonfluent PPA?

A

-Left posterior frontal and insular regions

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12
Q

What neuroanatomic damage is most associated with semantic PPA?

A

-Anterior temporal region

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13
Q

What neuroanatomic damage is most associated with logopenic PPA?

A

-left temporoparietal regions

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14
Q

Average length from PPA onset to death

A

12 years

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15
Q

Core features of logopenic variant PPA?

A
  • Impaired single-word retrieval in spontaneous speech and naming
  • Impaired repetition of sentences and phrases
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16
Q

Core features of semantic variant PPA?

A
  • Impaired confrontation naming

- Impaired single-word comprehension

17
Q

Core features of nonfluent variant PPA?

A
  • Agrammatism in language production

- Effortful, halting speech with inconsistent speech sound errors

18
Q

What characterizes the motor variant of FTD?

A

-progressive deterioration of motor functions with cognitive and psychological sx

19
Q

What are 3 subtypes of motor FTD, and which is most common?

A
  • Progressive supranuclear palsy (most common)
  • Corticobasal degeneration
  • FTD with motor neuron disease
20
Q

Neuropathology of PSP?

A

-Astrocytic lesions, tau-postive neurofibrillary tangles, neuropil threads within the brainstem and basal ganglia

21
Q

Neuropathology of CBD?

A

-Asymmetrical atrophy of bilateral premotor cortex, superior parietal lobules, and striatum

22
Q

Neuropathology of FTD-MND?

A

-ubiquitin-based pathology rather than tau pathology, involving frontal and temporal lobes

23
Q

Average time from onset of PSP to death?

A

5 years, with clinically significant motor problems typically merging about 4 years after disease onset

24
Q

FTD-MND age of onset and time to death?

A
  • 55 years, equal male to female ratio

- Rapid progression from diagnosis to death, typically occurring in late 50’s

25
Q

CBD age of onset?

A

Ranges from 50’s to 70’s

26
Q

Features of FTD-MND?

A

Core:

  • Dementia, impaired memory and EF
  • Speech production deficits
  • Significant disinhibition
  • Personality change

Additional:

  • Muscle atrophy, weakness, cramps, clumsiness
  • slowed vertical saccades
  • weak respiratory muscles
  • hyperflexia
  • fasciculations
  • dysphagia and dysarthia
27
Q

Core features of CBD?

A
  • Limb apraxia
  • Alien limb syndrome
  • Cortical reflex myoclonus
  • Cortical sensory impairment
  • Exec. dysfunction is also common
28
Q

Core features PSP?

A
  • Supranuclear vertical gaze palsy (impaired downward gaze)
  • Bradykinesia
  • Rigidity
  • Swallowing problems
  • Frequent falls
  • Usually involves behavioral and cognitive changes, especially exec. dysfunction
29
Q

What is agrammatism?

A

-inability to speak grammatically, typically because of simplified sentence structure and errors in tense, number, and gender, omission of function words (telegraphic speech)

30
Q

What does argyrophilic mean?

A
  • Affinity for silver; binding to silver salts

- in Picks bodies

31
Q

What is surface dyslexia?

A
  • Common feature of semantic FTD
  • Inability to recognize words as a whole, resulting in failure to read irregularly spelled words that cannot be sounded out phonetically
  • Dyseideic dyslexia
32
Q

What are vertical saccades?

A
  • Very short, rapid vertical movement of both eyes at the same time
  • Saccades are the fastest movements produced by the human body; once underway, they cannot be altered by will
  • Slowed in FTD-MND

Neuropsych Board Prep (32 decks)

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