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Questions and Answers
Dementia is characterized by an impairment of consciousness.
Dementia is characterized by an impairment of consciousness.
False
Alzheimer's disease is the most common cause of dementia.
Alzheimer's disease is the most common cause of dementia.
True
Only 10% of dementia cases are currently considered potentially reversible.
Only 10% of dementia cases are currently considered potentially reversible.
False
ICD-10 requires impairment in only one cognitive domain for dementia diagnosis.
ICD-10 requires impairment in only one cognitive domain for dementia diagnosis.
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Mild cognitive impairment is defined as deficits that do not interfere with social or occupational functioning.
Mild cognitive impairment is defined as deficits that do not interfere with social or occupational functioning.
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Vascular dementia and dementia with Lewy bodies are considered separate from Alzheimer's disease.
Vascular dementia and dementia with Lewy bodies are considered separate from Alzheimer's disease.
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Dementia typically begins with widespread cognitive disturbances.
Dementia typically begins with widespread cognitive disturbances.
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The management of dementia is discussed in Chapter 20.
The management of dementia is discussed in Chapter 20.
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Alzheimer's disease is characterized by stepwise progression.
Alzheimer's disease is characterized by stepwise progression.
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Visual hallucinations are a prominent symptom of frontotemporal dementia.
Visual hallucinations are a prominent symptom of frontotemporal dementia.
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Normal-pressure hydrocephalus is the commonest reversible dementia.
Normal-pressure hydrocephalus is the commonest reversible dementia.
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Behavioral changes are not significant in the assessment of dementia.
Behavioral changes are not significant in the assessment of dementia.
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Prion disease typically has a slow onset and progression.
Prion disease typically has a slow onset and progression.
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The CAMCOG is primarily a clinical tool used for case finding in the general population.
The CAMCOG is primarily a clinical tool used for case finding in the general population.
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Expressive dysphasia is a prominent symptom of Alzheimer's disease.
Expressive dysphasia is a prominent symptom of Alzheimer's disease.
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Dementia with Lewy bodies is often associated with frequent adverse reactions to antipsychotics.
Dementia with Lewy bodies is often associated with frequent adverse reactions to antipsychotics.
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Subcortical dementia is characterized by severe memory impairment occurring early in the disease.
Subcortical dementia is characterized by severe memory impairment occurring early in the disease.
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Dysphasias are an early language impairment associated with subcortical dementia.
Dysphasias are an early language impairment associated with subcortical dementia.
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Cortical dementias commonly feature preserved mathematical skills.
Cortical dementias commonly feature preserved mathematical skills.
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Patients with subcortical dementia often display apathetic and inert personality traits.
Patients with subcortical dementia often display apathetic and inert personality traits.
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Individuals with cortical dementia generally show flat and depressed mood.
Individuals with cortical dementia generally show flat and depressed mood.
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Aggressive behavior in dementia patients can be a risk factor for those around them.
Aggressive behavior in dementia patients can be a risk factor for those around them.
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Fitness to drive is not a concern for patients in the early stages of dementia.
Fitness to drive is not a concern for patients in the early stages of dementia.
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Mild cognitive impairment (MCI) is recognized as an intermediate category introduced in ICD-10 for early detection of dementia.
Mild cognitive impairment (MCI) is recognized as an intermediate category introduced in ICD-10 for early detection of dementia.
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The Clinical Dementia Rating (CDR) is utilized solely for assessing cognitive function in dementia.
The Clinical Dementia Rating (CDR) is utilized solely for assessing cognitive function in dementia.
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MCI patients have a high probability of converting to dementia, with 40-70% progressing within 5 years.
MCI patients have a high probability of converting to dementia, with 40-70% progressing within 5 years.
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The Montreal Cognitive Assessment (MoCA) is part of the screening tests for dementia.
The Montreal Cognitive Assessment (MoCA) is part of the screening tests for dementia.
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Thyroid function tests are classified as secondary care investigations for dementia.
Thyroid function tests are classified as secondary care investigations for dementia.
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Olfactory deficits can be detected up to 20 years before the onset of dementia symptoms.
Olfactory deficits can be detected up to 20 years before the onset of dementia symptoms.
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Neuropsychiatric Inventory is used exclusively to assess cognitive impairments.
Neuropsychiatric Inventory is used exclusively to assess cognitive impairments.
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The Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) solely focuses on daily living activities.
The Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) solely focuses on daily living activities.
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Genetic testing is considered a routine examination for dementia diagnosis.
Genetic testing is considered a routine examination for dementia diagnosis.
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Alzheimer's disease is classified as a primary neurodegenerative disorder.
Alzheimer's disease is classified as a primary neurodegenerative disorder.
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Cushing's syndrome is a primary neurodegenerative cause of dementia.
Cushing's syndrome is a primary neurodegenerative cause of dementia.
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Dementia with Lewy bodies is caused exclusively by prion diseases.
Dementia with Lewy bodies is caused exclusively by prion diseases.
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The most conspicuous feature of dementia is the difficulty with new learning.
The most conspicuous feature of dementia is the difficulty with new learning.
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Systemic lupus erythematosus is an inflammatory cause of dementia.
Systemic lupus erythematosus is an inflammatory cause of dementia.
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HIV is mentioned as a potential cause of vascular dementia.
HIV is mentioned as a potential cause of vascular dementia.
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Recent memory loss is typically more evident than remote memory loss in dementia patients.
Recent memory loss is typically more evident than remote memory loss in dementia patients.
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Disturbed episodic memory is manifested as forgetfulness of recent day-to-day events.
Disturbed episodic memory is manifested as forgetfulness of recent day-to-day events.
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Neurosarcoidosis is considered a metabolic cause of dementia.
Neurosarcoidosis is considered a metabolic cause of dementia.
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Carbon monoxide poisoning can lead to post-anoxia dementia.
Carbon monoxide poisoning can lead to post-anoxia dementia.
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Subcortical dementia is characterized by early, prominent impairments of memory and word finding.
Subcortical dementia is characterized by early, prominent impairments of memory and word finding.
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Presenile dementia occurs in individuals under 70 years of age.
Presenile dementia occurs in individuals under 70 years of age.
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The Mini-Mental State Examination (MMSE) is a benchmark for assessing cognitive impairment with a sensitivity of around 80%.
The Mini-Mental State Examination (MMSE) is a benchmark for assessing cognitive impairment with a sensitivity of around 80%.
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Vascular dementia is more commonly associated with presenile dementia than late-onset dementia.
Vascular dementia is more commonly associated with presenile dementia than late-onset dementia.
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Depression can coexist with dementia, potentially worsening functioning.
Depression can coexist with dementia, potentially worsening functioning.
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In presenile dementia, cases due to genetic diseases are relatively less common compared to late-onset dementia.
In presenile dementia, cases due to genetic diseases are relatively less common compared to late-onset dementia.
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Investigations for presenile dementia are generally less intensive than for late-onset dementia.
Investigations for presenile dementia are generally less intensive than for late-onset dementia.
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The Montreal Cognitive Assessment (MoCA) is a commonly used test that takes longer than 10 minutes to complete.
The Montreal Cognitive Assessment (MoCA) is a commonly used test that takes longer than 10 minutes to complete.
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Frontotemporal dementia and prion disease are relatively more common in senile dementia.
Frontotemporal dementia and prion disease are relatively more common in senile dementia.
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Screening tests for dementia may also help monitor the progression of the disease.
Screening tests for dementia may also help monitor the progression of the disease.
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Over 50% of Alzheimer's disease patients experience psychotic symptoms such as delusions and hallucinations.
Over 50% of Alzheimer's disease patients experience psychotic symptoms such as delusions and hallucinations.
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Aggression in Alzheimer's patients primarily manifests as physical violence towards others.
Aggression in Alzheimer's patients primarily manifests as physical violence towards others.
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The median survival from the diagnosis of Alzheimer's disease is typically longer for women than for men.
The median survival from the diagnosis of Alzheimer's disease is typically longer for women than for men.
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Anosmia, or loss of smell, is identified as one of the core features of Alzheimer's disease.
Anosmia, or loss of smell, is identified as one of the core features of Alzheimer's disease.
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Routine genetic testing for the apoE4 allele is recommended for all patients suspected of having Alzheimer's disease.
Routine genetic testing for the apoE4 allele is recommended for all patients suspected of having Alzheimer's disease.
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Cognitive impairments in the later stages of Alzheimer's disease increasingly dominate the clinical picture.
Cognitive impairments in the later stages of Alzheimer's disease increasingly dominate the clinical picture.
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Sundowning refers specifically to an increase in disorientation during the daytime.
Sundowning refers specifically to an increase in disorientation during the daytime.
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All patients with Alzheimer's disease will undergo personalized care strategies regardless of severity.
All patients with Alzheimer's disease will undergo personalized care strategies regardless of severity.
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Neurofibrillary tangles and senile plaques are the principal diagnostic features identified in Alzheimer's disease through microscopic examination.
Neurofibrillary tangles and senile plaques are the principal diagnostic features identified in Alzheimer's disease through microscopic examination.
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Depression occurs in less than 25% of Alzheimer's disease patients.
Depression occurs in less than 25% of Alzheimer's disease patients.
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The strongest neuropathological correlate of cognitive impairment in Alzheimer's disease is the proliferation of astrocytes.
The strongest neuropathological correlate of cognitive impairment in Alzheimer's disease is the proliferation of astrocytes.
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Senile plaques are deposits of insoluble proteins and are always pathologically less significant than neurofibrillary tangles.
Senile plaques are deposits of insoluble proteins and are always pathologically less significant than neurofibrillary tangles.
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Neurofibrillary tangles are primarily formed of the protein tau, which is usually soluble and aids in axonal transport.
Neurofibrillary tangles are primarily formed of the protein tau, which is usually soluble and aids in axonal transport.
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The aetiology of most Alzheimer's disease cases can be attributed to Mendelian inheritance.
The aetiology of most Alzheimer's disease cases can be attributed to Mendelian inheritance.
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The presence of amyloid deposits in blood vessel walls is referred to as vascular amyloid or congophilic angiopathy.
The presence of amyloid deposits in blood vessel walls is referred to as vascular amyloid or congophilic angiopathy.
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Alzheimer's disease neuropathology begins in the hippocampus before spreading to the entorhinal cortex.
Alzheimer's disease neuropathology begins in the hippocampus before spreading to the entorhinal cortex.
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Apolipoprotein E (apoE) polymorphisms are unequivocally associated with all forms of Alzheimer's disease.
Apolipoprotein E (apoE) polymorphisms are unequivocally associated with all forms of Alzheimer's disease.
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Familial forms of Alzheimer's disease involve mutations primarily in four genes including PSEN2 on chromosome 2.
Familial forms of Alzheimer's disease involve mutations primarily in four genes including PSEN2 on chromosome 2.
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The Braak stages of Alzheimer's disease focus on the deposition of tau proteins rather than β-amyloid.
The Braak stages of Alzheimer's disease focus on the deposition of tau proteins rather than β-amyloid.
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Neuritic plaques possess a loose structure and are considered pathologically significant.
Neuritic plaques possess a loose structure and are considered pathologically significant.
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The APP gene is located on chromosome 21.
The APP gene is located on chromosome 21.
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The first APP mutation related to Alzheimer's disease was discovered in 1984.
The first APP mutation related to Alzheimer's disease was discovered in 1984.
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APP mutations promote the formation of β-amyloid by affecting APP metabolism.
APP mutations promote the formation of β-amyloid by affecting APP metabolism.
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Presenilin mutations are found in familial Alzheimer's disease and affect γ-secretase activity.
Presenilin mutations are found in familial Alzheimer's disease and affect γ-secretase activity.
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The APP gene mutations explain the majority of sporadic Alzheimer's disease cases.
The APP gene mutations explain the majority of sporadic Alzheimer's disease cases.
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Transgenic mice with a mutated APP gene exhibit cognitive impairment and deposit β-amyloid.
Transgenic mice with a mutated APP gene exhibit cognitive impairment and deposit β-amyloid.
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APP and PSEN mutations account for the majority of Down's syndrome cases.
APP and PSEN mutations account for the majority of Down's syndrome cases.
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A point mutation changing isoleucine to valine at position 717 in APP is linked to Alzheimer's disease.
A point mutation changing isoleucine to valine at position 717 in APP is linked to Alzheimer's disease.
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The prevalence of Alzheimer's disease in individuals aged 60 or older is approximately 10-12%.
The prevalence of Alzheimer's disease in individuals aged 60 or older is approximately 10-12%.
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Olfactory deficits are considered a clinical feature of preclinical Alzheimer's disease.
Olfactory deficits are considered a clinical feature of preclinical Alzheimer's disease.
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Amyloid PET scans are currently in clinical use for diagnosing Alzheimer's disease.
Amyloid PET scans are currently in clinical use for diagnosing Alzheimer's disease.
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Total Tau levels correlate with the severity of neurodegeneration in Alzheimer's disease.
Total Tau levels correlate with the severity of neurodegeneration in Alzheimer's disease.
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Hippocampal atrophy occurs later in Alzheimer's disease compared to other forms of dementia.
Hippocampal atrophy occurs later in Alzheimer's disease compared to other forms of dementia.
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The first symptom of Alzheimer's disease is often significant memory loss experienced all at once.
The first symptom of Alzheimer's disease is often significant memory loss experienced all at once.
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Major depression is found in roughly 20% of Alzheimer's disease cases.
Major depression is found in roughly 20% of Alzheimer's disease cases.
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Phosphorylated Tau correlates with neurofibrillary changes in the brain.
Phosphorylated Tau correlates with neurofibrillary changes in the brain.
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The rate of Alzheimer's disease doubles every 3 years in the elderly.
The rate of Alzheimer's disease doubles every 3 years in the elderly.
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Neuropsychological deficits in Alzheimer's disease typically enhance verbal episodic memory.
Neuropsychological deficits in Alzheimer's disease typically enhance verbal episodic memory.
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Study Notes
Major Causes of Dementia
- Alzheimer's Disease: Characterized by short-term memory loss, dysphasia, early smell impairment, behavioral changes, and mood instability. Progressive, with an average survival of 5-8 years.
- Vascular Dementia: Features stepwise progression and signs of cerebrovascular disease; associated with hypertension, more common in men and smokers.
- Dementia with Lewy Bodies: Fluctuations in alertness, Parkinsonism, visual hallucinations, and falls. High rate of adverse reactions to antipsychotics.
- Frontotemporal Dementia: Marked behavioral changes, expressive dysphasia, loss of insight, and primitive reflexes. Onset typically under 70 years, with various clinical subtypes.
- Prion Disease: Symptoms include myoclonic jerks, seizures, ataxia, and psychiatric issues. Generally presents early with rapid progression and transmissibility.
- Normal-Pressure Hydrocephalus: Features include cognitive slowing, inattention, gait apraxia, and urinary incontinence. Most common reversible dementia in ages 50-70.
Assessment of Dementia Causes
- Definitive diagnosis often requires neuropathological methods or rare genetic tests.
- Experienced clinicians can make probable diagnoses based on symptom profiles.
- Monitoring treatment response involves biochemical, radiological, and genetic investigations.
- CAMCOG used mainly for research; not for general case finding.
- Behavioral symptoms should be monitored as they fluctuate and significantly affect caregivers.
Clinical Features of Dementia
- Dementia is a global impairment of memory, intellect, and personality without consciousness loss.
- Often progressive and most cases are due to Alzheimer's disease.
- Symptoms may begin with focal cognitive changes, such as memory loss, language issues, or behavioral disturbances.
- ICD-10 criteria require impairments in two or more cognitive domains, impacting social or occupational functioning.
Types of Dementia and Their Features
- Cortical Dementia: Includes Alzheimer's and Frontotemporal Dementias; severe memory loss and early language difficulties are common.
- Subcortical Dementia: Includes Huntington’s and Parkinson’s Diseases; memory impairment is moderate, language skills are generally preserved.
- Mixed Causes: Conditions like vascular dementia and dementia with Lewy bodies show features from both categories.
Risk Assessment in Dementia
- Patients exhibit risks such as self-neglect, poor judgment, wandering, and potential aggression.
- Assessing fitness to drive is crucial, with UK regulations permitting driving if skills are maintained despite dementia.
- Good history from caregivers, alongside functional ability assessments, is vital for risk management.
Early Detection and Mild Cognitive Impairment (MCI)
- Increased focus on early dementia diagnosis, especially in those showing MCI features.
- MCI is an intermediate category; its clinical significance is uncertain, with a small percentage converting to full dementia annually.
Assessment of Dementia Severity
- Involves ruling out other causes like delirium and depression.
- Severity assessments utilize screening tests (MMSE, MoCA, etc.) and tallied symptoms for monitoring progression.
- Various tests assess cognitive function, daily living activities, and mood.
Biomarkers and Presymptomatic Diagnosis
- Alzheimer’s disease may be presymptomatic; research is ongoing for early detection markers.
- Neuropsychological, olfactory, and biochemical indicators can suggest early pathology.
Alzheimer's Disease Overview
- Most prevalent cause of dementia; affects around 60% of cases.
- Initial symptoms often include slight forgetfulness leading to greater memory disturbance.
- Significant cognitive impairments occur with language, memory, and visuospatial skills affected early on.
Symptoms of Alzheimer's Disease
- Core Features: Pronounced memory impairment, aphasia, executive function disturbances, and apraxia.
- Other Features: Depression, agitation, personality changes, and psychotic symptoms.
- 10-25% experience hallucinations; delusions—particularly persecutory—may arise from memory lapses.
Conclusion
- Alzheimer's disease dominates dementia cases, but various identifiable causes and symtompcatological features exist.
- Early detection and understanding of behavioral changes are crucial for effective management and care.### Behavioral and Physical Changes in Dementia
- Evening agitation, known as sundowning, can disrupt the sleep-wake cycle.
- Aggressive behaviors, both verbal and physical, are common, often as resistance to personal care.
- Serious physical violence is rare; varied activity levels and purposefulness can be observed.
- Wandering behaviors may expose patients to unsafe situations.
- Eating patterns can fluctuate, leading to weight changes and nutritional issues.
- Sexual behavior typically decreases, although some may experience sexual disinhibition.
- Declines in self-care and social interactions occur, though some maintain a facade with caregiver support.
Progression of Alzheimer's Disease
- Early stages are influenced by the patient's premorbid personality.
- Cognitive impairments become more pronounced in middle and later stages of the disease.
- Delirium can occur with incidental physical illness, causing sudden cognitive decline.
- Median survival post-diagnosis is 5-7 years, generally shorter in men and with an older age of onset.
Investigative Procedures
- Investigations for suspected Alzheimer's mirror those for general dementia.
- Genetic testing is recommended for rare familial early-onset cases.
- The apoE4 allele is a significant risk factor but does not warrant routine testing in dementia diagnosis.
Neuropathological Changes
- Characteristic features include shrunken brain appearance, widened sulci, and enlarged ventricles.
- Diagnosis relies on neurofibrillary tangles and senile (amyloid) plaques identified microscopically.
- Selective neuron loss occurs in the hippocampus and entorhinal cortex with synaptic loss being strongly correlated to cognitive impairment.
Neuropathological Progression
- The disease process begins in the entorhinal cortex, moving to the hippocampus and cortical areas.
- Braak stages identify six stages of β-amyloid deposition correlating with disease progression.
- Neuropathology manifests well before clinical symptoms, disrupting connections between brain regions.
Senile (Amyloid) Plaques
- Composed of insoluble proteins, degenerating neurites, and proliferated glial cells found in neuropil.
- Neuritic plaques are more dangerous compared to diffuse plaques, with β-amyloid key to plaque formation.
Neurofibrillary Tangles
- Found within neuron cell bodies, particularly in cerebral cortex and hippocampus.
- Composed of hyperphosphorylated tau protein affecting axonal transport and leading to neuron death.
Genetic Factors of Alzheimer's Disease
- Autosomal dominant inheritance characterizes rare early-onset cases.
- Causative genes include APP (chromosome 21), PSEN1 (chromosome 14), and PSEN2 (chromosome 1).
- Total familial cases are primarily linked to mutations in these genes; sporadic cases are more common.
- Genetic predisposition is significant for first-degree relatives, especially with relationships to the apoE gene.
Discovery of APP Gene Mutations
- The β-amyloid peptide linked to Alzheimer's was identified in 1984, with the APP gene localized to chromosome 21.
- Research into familial cases led to the discovery of a pathogenic APP mutation in 1991.
- APP mutations lead to altered APP metabolism, promoting β-amyloid formation.
- PSEN mutations contribute similarly by influencing APP processing via the γ-secretase enzyme.
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Explore this quiz on the prominent symptoms and signs of various major causes of dementia, including Alzheimer's disease and vascular dementia. Understand the distinctions between these conditions and their clinical features to enhance your knowledge of neurodegenerative disorders.
