Frontotemporal Dementias Flashcards
What are the CORE Criteria for POSSIBLE bvFTD diagnosis?
Note: 3 of 6 must be present
1) Early behavioral disinhibition ( a hallmark feature of bvFTD)
2) Early apathy or inertia in bvFTD
3) Early loss of sympathy or empathy
4) Early perseverative, stereotyped or compulsive/ritualistic behavior
5) Hyperorality and dietary changes
6) Neuropsychological profile with Executive dysfunction prominent
Describe some symptoms associated with the following CORE criteria of FTD:
Early behavioral disinhibition ( a hallmark feature of bvFTD)
- Socially inappropriate behavior
- Loss of manners or decorum
- Impulsive, rash or careless actions
Describe some symptoms associated with the following CORE criteria of FTD:
Early apathy or inertia in bvFTD
Patients with bvFTD also present with a general loss of interest, drive or motivation. In extreme cases, patients may need prompts to initiate or continue basic activities (e.g., bathing, dressing).
Describe some symptoms associated with the following CORE criteria of FTD:
Early loss of sympathy or empathy
- Diminished responsiveness to other people’s needs and feelings
- Diminished social interest, interrelatedness, or personal warmth
Describe some symptoms associated with the following CORE criteria of FTD:
Early perseverative, stereotyped or compulsive/ritualistic behavior
- Simple repetitive movements
- Complex, compulsive, or ritualistic behaviors
- Stereotypy of speech
Describe some symptoms associated with the following CORE criteria of FTD:
Hyperorality and dietary changes
- Altered food preferences
- Binge eating, increased consumption of alcohol or cigarettes
- Oral exploration or consumption of inedible objects
Describe some symptoms associated with the following CORE criteria of FTD:
Neuropsychological profile
Executive/generation deficits with relative sparing of memory and visuospatial functions
What are the criteria for meeting a PROBABLE bvFTD diagnosis?
1) meet criteria for possible bvFTD
2) exhibit significant functional decline
AND
3) have structural or functional neuroimaging results that are consistent with the disorder
MRI/CT –> frontal and/or anterior temporal lobe atrophy
or
SPECT/PET –> frontal and/or anterior temporal hypoperfusion or hypometabolism
What are the criteria for meeting a DEFINITE bvFTD diagnosis?
1) must meet criteria for possible or probable
AND
2) have histopathological evidence of FTLD on biopsy or at postmortem examination or have a known genetic mutation
MAPT, CHMP2B, VCP, PGRN
All of the following genetic mutations are typically associated with bvFTD except ________.
(A) Microtubule-associated protein tau [MAPT]
(B) Charged multi-vesicular body protein 2B [CHMP2B]
(C) Presenilin 1 [PS1]
(D) Valosin-containing protein [VCP]
(E) Progranulin [PGRN]
(C) Presenilin 1 [PS1]
What name is given to the subset of individuals living with bvFTD that do not progress to dementia and have non-progressive neuroimaging results (i.e., no atrophy or minimal atrophy)?
(A) bvFTD non-progressive subtype
(B) bvFTD phenocopy syndrome
(C) bvFTD static syndrome
(D) bvFTD remitting subtype
(B) bvFTD phenocopy syndrome
unknown cause of this subtype but is associated with mood disorder, autism spectrum disorders and psychological decompensation due to psychosocial stressors.
DEFINITION:
Agrammatism
The inability to speak grammatically, typically because of simplified sentence structure and errors in tense, number, and gender. Speech may include omission of function words (known as telegraphic speech)
DEFINITION:
Alien Limb Syndrome
Unintended but purposeful and autonomous movements of the upper limb
DEFINITION:
Argyrophilic
Having an affinity for silver; binding to silver salts
Argyrophilic grain disease (AGD) is a frequent late-onset, 4-repeat tauopathy reported in Caucasians with high educational attainment.
DEFINITION:
Fasciculation
A small, local, involuntary muscle contraction and relaxation (twitch)
