FTD Flashcards
Which of the following best describes Frontotemporal Dementia (FTD)?
A) A disorder caused primarily by vascular pathology
B) A group of clinical syndromes associated with frontotemporal lobar degeneration (FTLD) pathology
C) A late-onset neurodegenerative disease primarily affecting memory
D) A disease exclusively characterized by amyloid beta deposition
Correct Answer: B) A group of clinical syndromes associated with frontotemporal lobar degeneration (FTLD) pathology
Rationale: FTD is an umbrella term for syndromes that share a common underlying FTLD pathology, which involves degeneration of the frontal and temporal lobes. It is distinct from Alzheimer’s disease (AD), which primarily affects memory and involves amyloid beta plaques.
What are the primary abnormal protein aggregations found in FTD?
A) Alpha-synuclein and amyloid beta
B) Tau and transactive response DNA-binding protein of 43 kDa (TDP-43)
C) Beta-amyloid and prion proteins
D) Huntingtin and tau
Correct Answer: B) Tau and transactive response DNA-binding protein of 43 kDa (TDP-43)
Rationale: FTD is associated with pathological protein aggregations, primarily tau and TDP-43, which contribute to neuronal dysfunction and degeneration. This differs from AD, which is mainly associated with amyloid beta plaques and tau tangles.
At what age range is FTD most commonly diagnosed?
A) First to third decades of life
B) Fifth to seventh decades of life
C) Seventh to ninth decades of life
D) Any age group equally
Correct Answer: B) Fifth to seventh decades of life
Rationale: FTD typically manifests between the ages of 40-70 and is nearly as prevalent as Alzheimer’s disease in this younger age group. It is a leading cause of early-onset dementia.
What are the three core clinical syndromes of FTD?
A) Behavioral variant (bvFTD), Primary progressive aphasia (PPA), Corticobasal syndrome
B) Behavioral variant (bvFTD), Primary progressive aphasia (PPA), Progressive supranuclear palsy
C) Behavioral variant (bvFTD), Primary progressive aphasia (PPA), Alzheimer’s disease
D) Behavioral variant (bvFTD), Primary progressive aphasia (PPA), Semantic dementia
Correct Answer: A) Behavioral variant (bvFTD), Primary progressive aphasia (PPA), Corticobasal syndrome
Rationale: The three core clinical syndromes of FTD include bvFTD, which affects social and emotional behavior; PPA, which includes semantic and nonfluent/agrammatic variants affecting language; and corticobasal syndrome, a movement disorder that can sometimes be linked to FTLD pathology.
Which of the following is a hallmark symptom of behavioral variant FTD (bvFTD)?
A) Predominant short-term memory loss
B) Early visual hallucinations
C) Social disinhibition, apathy, compulsive behaviors, and loss of empathy
D) Gradual loss of episodic memory with visuospatial deficits
Correct Answer: C) Social disinhibition, apathy, compulsive behaviors, and loss of empathy
Rationale: BvFTD primarily affects social and emotional functioning. Patients often exhibit disinhibited behavior, compulsions, overeating, and diminished empathy, whereas memory loss is more characteristic of Alzheimer’s disease.
Frontotemporal dementia (FTD) is primarily associated with the accumulation of which abnormal proteins?
A) Beta-amyloid and alpha-synuclein
B) Tau and TDP-43
C) Alpha-synuclein and ubiquitin
D) Beta-amyloid and tau
Answer: B) Tau and TDP-43
Rationale: FTD is considered a disease of abnormal protein aggregation, primarily involving tau or transactive response DNA-binding protein of 43 kDa (TDP-43).
Which of the following is the most common clinical syndrome of FTD?
A) Corticobasal syndrome (CBS)
B) Behavioral variant FTD (bvFTD)
C) Progressive supranuclear palsy–Richardson syndrome (PSP-RS)
D) Semantic variant primary progressive aphasia (PPA)
Answer: B) Behavioral variant FTD (bvFTD)
Rationale: bvFTD is the most common form of FTD and is characterized by social and emotional dysfunction, including apathy, disinhibition, and compulsivity.
Which of the following is NOT a characteristic feature of the behavioral variant of FTD (bvFTD)?
A) Apathy
B) Loss of empathy
C) Early prominent visuospatial dysfunction
D) Disinhibition
Answer: C) Early prominent visuospatial dysfunction
Rationale: Unlike Alzheimer’s disease, FTD typically spares visuospatial processing and arithmetic functions until very late in the disease.
Which form of primary progressive aphasia (PPA) is characterized by a gradual loss of word and object meaning?
A) Nonfluent/agrammatic variant
B) Logopenic variant
C) Semantic variant
D) Mixed variant
Answer: C) Semantic variant
Rationale: The semantic variant of PPA is associated with progressive loss of word comprehension and object recognition due to anterior temporal lobe degeneration.
Which neurodegenerative syndrome is commonly associated with motor neuron disease (MND)?
A) Corticobasal syndrome (CBS)
B) Behavioral variant FTD (bvFTD)
C) Progressive supranuclear palsy–Richardson syndrome (PSP-RS)
D) Dementia with Lewy bodies (DLB)
Answer: B) Behavioral variant FTD (bvFTD)
Rationale: FTD-MND refers to cases where FTD coexists with motor neuron disease, suggesting an overlap in neurodegenerative mechanisms.
Which brain regions are primarily affected in bvFTD?
A) Medial temporal lobe and hippocampus
B) Posterior parietal cortex and occipital lobes
C) Medial and orbital frontal lobes and anterior insula
D) Substantia nigra and basal ganglia
Answer: C) Medial and orbital frontal lobes and anterior insula
Rationale: These areas control social behavior, executive function, and emotional regulation, which are commonly impaired in bvFTD.
Which of the following is a hallmark feature of the nonfluent/agrammatic variant of PPA?
A) Impaired word meaning and object recognition
B) Fluent but empty speech with paraphasias
C) Difficulty with speech production and grammar
D) Visual hallucinations
Answer: C) Difficulty with speech production and grammar
Rationale: The nonfluent/agrammatic variant is characterized by difficulty forming grammatically correct sentences and motor speech impairment.
What is the pathological hallmark of Frontotemporal Lobar Degeneration (FTLD)?
A) Widespread beta-amyloid plaques
B) Focal atrophy of the frontal, insular, and/or temporal cortex
C) Lewy body accumulation in the substantia nigra
D) Increased cholinergic innervation in the cerebral cortex
Answer: B) Focal atrophy of the frontal, insular, and/or temporal cortex
Rationale: FTLD is characterized by focal atrophy in the frontal, insular, and temporal lobes, often visualized through neuroimaging. This distinguishes it from Alzheimer’s disease (AD), which prominently affects the hippocampus and medial temporal lobe.
What is the significance of serotonergic innervation in FTLD?
A) Loss of serotonergic innervation is common in FTLD
B) Serotonergic innervation is increased in FTLD
C) FTLD primarily affects dopamine rather than serotonin
D) The serotonergic system is relatively spared in FTLD
Answer: A) Loss of serotonergic innervation is common in FTLD
Rationale: FTLD often exhibits a loss of cortical serotonergic innervation, which may contribute to behavioral and mood symptoms.
Why are acetylcholinesterase inhibitors generally ineffective in FTLD?
A) The cholinergic system is relatively spared in FTLD
B) FTLD patients cannot tolerate cholinesterase inhibitors
C) Acetylcholinesterase inhibitors accelerate neurodegeneration in FTLD
D) FTLD patients have too much acetylcholine in their brains
Answer: A) The cholinergic system is relatively spared in FTLD
Rationale: Unlike AD, FTLD does not significantly affect cholinergic neurons, explaining why cholinesterase inhibitors show poor efficacy in treating its symptoms.
Which of the following microscopic features is common across all FTLD cases?
A) Beta-amyloid plaques
B) Neurofibrillary tangles only
C) Gliosis, microvacuolation, and neuronal loss
D) Lewy bodies
Answer: C) Gliosis, microvacuolation, and neuronal loss
Rationale: These microscopic findings are present in all FTLD cases, but the specific type of protein accumulation (tau, TDP-43, or FUS) determines the subtype.
What histopathologic subtype of FTLD is associated with Pick bodies?
A) FTLD-TDP
B) FTLD-FET
C) Pick’s disease (FTLD-tau)
D) Lewy body dementia
Answer: C) Pick’s disease (FTLD-tau)
Rationale: Pick’s disease is a specific FTLD-tau subtype characterized by argyrophilic Pick bodies that stain positively with Bielschowsky silver but not with the Gallyas method.
What is the primary goal of treatment in patients with Frontotemporal Dementia (FTD)?
A) Slowing disease progression
B) Enhancing cholinergic neurotransmission
C) Symptomatic management
D) Reversing neurodegeneration
Answer: C) Symptomatic management
Rationale: There are no disease-modifying treatments for FTD. Management focuses on alleviating symptoms such as depression, compulsions, and irritability to improve the quality of life for both patients and caregivers.
Which class of medication is commonly used to manage behavioral symptoms in FTD?
A) Acetylcholinesterase inhibitors
B) Selective serotonin reuptake inhibitors (SSRIs)
C) Dopamine agonists
D) Anticholinergics
Answer: B) Selective serotonin reuptake inhibitors (SSRIs)
Rationale: SSRIs are often used to treat behavioral symptoms such as depression, compulsions, and irritability in FTD. Acetylcholinesterase inhibitors are ineffective because the cholinergic system is relatively spared in FTD.
Why should antipsychotics be used with caution in FTD patients?
A) They can accelerate cognitive decline
B) They may worsen parkinsonism symptoms
C) They have no effect on behavioral symptoms
D) They increase cholinergic activity
Answer: B) They may worsen parkinsonism symptoms
Rationale: Many FTD patients exhibit parkinsonian features, and antipsychotics can exacerbate these motor symptoms. If necessary, atypical antipsychotics with a lower risk of motor side effects should be considered.
