Questions and Answers
Early signs of frontotemporal dementia include hyperorality, characterized by a craving for spicy foods.
False
The average survival period of a patient with frontotemporal dementia is between 6-11 years from symptom onset.
True
The presence of motor neuron disease in patients with frontotemporal dementia is believed to improve their prognosis.
False
Echolalia is a feature primarily observed in the non-fluent variant of primary progressive aphasia.
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Behavioral features such as apathy and disinhibition are exclusive to the behavioral variant of frontotemporal dementia.
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Anomia, particularly for verbs, is a hallmark symptom of semantic-variant primary progressive aphasia.
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Structural brain imaging, like MRI or CT, can reveal focal atrophy in patients with frontotemporal dementia.
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Depression and emotional blunting are affective features of frontotemporal dementia.
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Increasing age is considered a protective factor against non-familial Alzheimer's disease.
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The cholinergic hypothesis suggests that acetylcholine loss has a primary causal role in Alzheimer's disease.
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Cerebrovascular disease can lead to a form of dementia known as vascular dementia.
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The use of non-steroidal anti-inflammatory drugs (NSAIDs) is considered a risk factor for Alzheimer's disease.
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High homocysteine levels are listed as a genetic factor in the risk of non-familial Alzheimer's disease.
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Microglial activation is increasingly understood to be important in the disease process of Alzheimer's disease.
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The term 'multi-infarct dementia' was historically used to describe dementia caused solely by single cerebrovascular events.
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Cognitive and physical activity in mid-life are protective factors against Alzheimer's disease.
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Frontotemporal dementias are the leading cause of late-life dementia.
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Recurrent visual hallucinations are a key feature of dementia with Lewy bodies.
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Cognitive impairment is the most prominent feature in frontotemporal dementia.
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The presence of cerebrovascular disease makes the diagnosis of dementia with Lewy bodies more likely.
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Non-visual hallucinations are a supportive feature of dementia with Lewy bodies.
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There is a well-established genetic risk factor for dementia with Lewy bodies.
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Primary progressive aphasia is a recognized subtype of frontotemporal dementia.
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Patients with frontotemporal dementia typically show severe cognitive decline early in the disease.
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A-synuclein protein is not present in Lewy bodies.
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Pick's disease is classified under frontotemporal dementia.
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Vascular dementia is primarily caused by damage to brain tissues due to high cholesterol levels.
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The onset of vascular dementia typically occurs in individuals in their 60s or 70s.
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Emotional changes are not common symptoms of vascular dementia until advanced stages.
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Multi-infarct dementia and small-vessel dementia are considered subtypes of vascular dementia.
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Approximately one third of individuals who experience a first stroke will develop vascular dementia.
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CT imaging is generally more effective than MRI in detecting vascular damage associated with vascular dementia.
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Vascular dementia progresses in a steady linear fashion without periods of recovery.
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CADASIL is a hereditary form of vascular dementia linked to mutations in the Notch gene.
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Increasing age doubles the risk of vascular dementia every 5 years.
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Orthostatic hypotension is not considered a risk factor for vascular dementia.
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Dementia with Lewy bodies is a relatively new classification established in the late 1980s.
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The presence of neurofibrillary tangles is common in dementia with Lewy bodies.
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The average survival time for individuals with dementia with Lewy bodies is estimated to be around 5-7 years.
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Homocysteine levels do not have any association with vascular dementia risk factors.
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Lewy bodies in dementia with Lewy bodies are made primarily of the protein tau.
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Dementia with Lewy bodies can exhibit features similar to Alzheimer's disease.
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Mixed dementia occurs in less than 20% of cases when considering lower thresholds for cerebrovascular disease.
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In individuals over 80, mixed dementia is considered the exception rather than the norm.
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The coexistence of Alzheimer's disease with cerebrovascular disease has no impact on the severity of dementia.
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Molecular classification of neurodegenerative diseases indicates a strong one-to-one correspondence between protein types and syndromes.
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Proteins such as $eta$-amyloid, PrP, and $ ext{a-synuclein}$ are only associated with their respective diseases without overlap.
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Tau is exclusively linked to Alzheimer's disease and does not appear in other neurodegenerative conditions.
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The reclassification of dementias into groups like 'tauopathies' and 'synucleinopathies' is influencing diagnostic practice.
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Risk factors and disease mechanisms for Alzheimer's disease are completely distinct from those for vascular dementia.
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Variant Creutzfeldt-Jakob disease (vCJD) was first identified in Germany in 1996.
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Bovine spongiform encephalopathy (BSE) is linked to variant Creutzfeldt-Jakob disease (vCJD).
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The incidence of vCJD peaked around the year 2005.
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The 'pulvinar' sign on MRI is a common diagnostic characteristic for vCJD.
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Kuru was primarily transmitted through blood transfusions.
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Prion diseases can be caused by autosomal dominant mutations of the prion protein gene.
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Prion proteins (PrP) are encoded by a gene located on chromosome 15.
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PrPSc is more resistant to breakdown by proteases compared to normal PrPC.
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All vCJD patients have been shown to be heterozygous for methionine.
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It is now considered likely that a human epidemic of vCJD will occur in the future.
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Pick's disease was first described by Arnold Pick in 1892.
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Prion diseases are primarily caused by a viral infection.
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The primary pathological feature of Pick's disease includes ballooned neurons known as Pick cells.
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About 50% of cases of frontotemporal dementia are caused by autosomal dominant mutations.
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The mutation in the C9orf72 gene is a repeated sequence of six nucleotides.
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Prion diseases do not share neuropathological features with any other neurodegenerative disorder.
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Sporadic cases of prion diseases are common and well understood.
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Neuropathological features of frontotemporal dementia can vary based on the protein that is deposited.
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The GRN mutations lead to increased production of the encoded protein.
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Creutzfeldt-Jakob disease (CJD) has an incubation period that can exceed 20 years.
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Study Notes
Clinical Features of Frontotemporal Dementias
Behavioural Features
- Insidious onset and slow progression highlight gradual changes in behavior.
- Early loss of insight affects self-awareness and recognition of condition.
- Signs include disinhibition, lack of judgment, mental inflexibility, hyperorality (craving for sweet foods), distractibility, and impulsivity.
- Behaviors often manifest as stereotyped and imitative actions.
Language and Speech Features
- Notable decrease in speech output as the condition progresses.
- Perseveration and echolalia indicate disruption in language control.
Affective Features
- Common emotional disturbances include depression and apathy.
- Emotional blunting reduces overall expression, while hypochondriasis involves excessive worry about health.
Physical Signs
- Early primitive reflexes and incontinence noted, with late-stage features including parkinsonism.
- Patients may experience low and labile blood pressure.
Subtypes of Frontotemporal Dementia
Behavioural-variant
- Characterized by profound personality changes, including apathy and selfishness.
- Additional features: self-neglect, overeating, and rigid mental processes.
Semantic-variant Primary Progressive Aphasia
- Predictable anomia (specifically nouns) and word-finding difficulties.
- Impaired verbal comprehension, increased pain responses, and prosopagnosia (difficulty recognizing faces).
Non-fluent Variant Primary Progressive Aphasia
- Features include slow, labored speech with grammatical misuse and speech sound errors.
- Anomia is prevalent, particularly for verbs, along with irritability.
Diagnosis and Prognosis
- Early diagnosis challenging; symptoms may mimic depression or psychosis.
- Assessment requires thorough review of psychiatric history, family history, and behavioral & language capabilities.
- Physical exams focus on motor system for signs of weakness or parkinsonism.
- Brain imaging, including MRI or CT, typically shows focal atrophy in temporal and frontal areas.
- Average survival post symptom onset is 6-11 years; shorter survival noted with concurrent motor neuron disease.
Dementia with Lewy Bodies (DLB)
Key Features
- Progressive cognitive decline, especially in attention and visuospatial skills.
- Fluctuations in cognitive abilities and recurrent visual hallucinations, alongside parkinsonism.
Supportive Features
- Possible recurrent falls, transient loss of consciousness, and non-visual hallucinations.
- Adverse reactions to antipsychotics can occur.
Diagnosis Considerations
- Diagnosis less likely if evidence of cerebrovascular disease or other disorders is evident.
Pathophysiology of DLB
- Characterized by α-synuclein protein aggregates in Lewy bodies, similar to Parkinson's disease.
- Neuropathological features include Lewy bodies and neurites in the cortex, with notable overlap with Alzheimer’s disease pathology.
Risk Factors for Non-Familial Alzheimer’s Disease
Demographic Factors
- Advancing age and family history of dementia increase risk.
- Individuals with Down's syndrome are also at higher risk.
Genetic Factors
- ApoE4 allele associated with increased risk, along with other genetic polymorphisms.
Environmental and Medical Risks
- Factors including low education, head injury, cerebrovascular disease, prior depression, diabetes, and elevated homocysteine levels contribute to risk.
Protective Factors
- Engagement in cognitive activities, physical exercise, a Mediterranean diet, and use of NSAIDs, HRT, and statins may reduce risk.
Vascular Dementia
Definition
- Cognitive impairment arising from brain blood vessel damage, often post-stroke.
Symptoms
- Typically emerges in late 60s to 70s, with emotional/personality changes preceding cognitive decline.
- Includes depression, confusion, and stepwise progression of cognitive impairment.
Diagnosis
- History of stroke critical for diagnosis; physical examination may reveal signs of vascular disease.
- Imaging aids in assessing severity of vascular damage.
Subtypes
- Multi-infarct dementia and small-vessel dementia (Binswanger's disease) are notable classifications.
Prion Diseases
- Unique neurodegenerative disorders due to the abnormal conformation of prion proteins.
- Variant Creutzfeldt-Jakob disease (vCJD) associated with BSE and has shown to have a genetic susceptibility component.
- Other prion diseases include Kuru, Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia, linked to genetic mutations.
Pick's Disease
- Classic form of frontotemporal dementia characterized by language impairment and focal atrophy of brain regions.
- Neuropathology reveals ballooned neurons called Pick cells, composed mainly of tau proteins.
- Genetic mutations (C9orf72, MAPT, GRN) underpinning familial cases linked to various neuropathological profiles.### PrP and Prion Disease
- PrPSc (infectious) is more protease-resistant than PrPC (normal), leading to its accumulation in the brain.
- PrPSc self-aggregates and spreads trans-synaptically, causing synaptic and neuronal loss, gliosis, and spongiform changes in brain tissue.
- In familial prion diseases, mutated PrP demonstrates a higher propensity for self-aggregation.
- Acquired prion disease involves the conversion of normal PrPC into misfolded PrPSc, propagating the disease. The exact molecular mechanisms remain poorly understood.
- Peripheral routes for PrPSc transmission may include nerves, lymphatics, and bloodstream.
Emerging Concepts in Dementia
- Classification and clinical practice in dementia are evolving due to new epidemiological and biological research findings.
Mixed Dementia
- "Mixed dementia" is increasingly recognized, particularly the coexistence of Alzheimer's disease and vascular dementia, affecting at least 20% of dementia cases.
- In individuals over 80, mixed dementia is often the norm, complicating clinical presentation.
- Alzheimer's disease’s co-occurrence with cerebrovascular disease intensifies dementia severity.
- There is significant overlap in risk factors and disease mechanisms between Alzheimer's and vascular dementia.
Molecular Classification of Neurodegenerative Diseases
- Misfolding, protein accumulation, and neuronal spread are central processes in multiple neurodegenerative disorders, including Alzheimer's, prion disease, and Parkinson's.
- No direct correlation exists between specific proteins and their syndromes; for instance, tau is involved in both Alzheimer's and some frontotemporal dementias.
- There is a shift towards molecular classification, leading to terms such as "tauopathies" and "synucleinopathies," which may impact diagnostics and treatment strategies moving forward.
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Description
Test your knowledge about frontotemporal dementia and its various aspects, including early signs, prognosis, and variants. This quiz covers symptoms like hyperorality, echolalia, and behavioral features. Understand the complexities of this neurological condition and its impact on patients.
