Questions and Answers
Dementia is characterized by an impairment of consciousness.
False
Alzheimer's disease is the most common cause of dementia.
True
Only 10% of dementia cases are currently considered potentially reversible.
False
ICD-10 requires impairment in only one cognitive domain for dementia diagnosis.
Signup and view all the answers
Mild cognitive impairment is defined as deficits that do not interfere with social or occupational functioning.
Signup and view all the answers
Vascular dementia and dementia with Lewy bodies are considered separate from Alzheimer's disease.
Signup and view all the answers
Dementia typically begins with widespread cognitive disturbances.
Signup and view all the answers
The management of dementia is discussed in Chapter 20.
Signup and view all the answers
Alzheimer's disease is characterized by stepwise progression.
Signup and view all the answers
Visual hallucinations are a prominent symptom of frontotemporal dementia.
Signup and view all the answers
Normal-pressure hydrocephalus is the commonest reversible dementia.
Signup and view all the answers
Behavioral changes are not significant in the assessment of dementia.
Signup and view all the answers
Prion disease typically has a slow onset and progression.
Signup and view all the answers
The CAMCOG is primarily a clinical tool used for case finding in the general population.
Signup and view all the answers
Expressive dysphasia is a prominent symptom of Alzheimer's disease.
Signup and view all the answers
Dementia with Lewy bodies is often associated with frequent adverse reactions to antipsychotics.
Signup and view all the answers
Subcortical dementia is characterized by severe memory impairment occurring early in the disease.
Signup and view all the answers
Dysphasias are an early language impairment associated with subcortical dementia.
Signup and view all the answers
Cortical dementias commonly feature preserved mathematical skills.
Signup and view all the answers
Patients with subcortical dementia often display apathetic and inert personality traits.
Signup and view all the answers
Individuals with cortical dementia generally show flat and depressed mood.
Signup and view all the answers
Aggressive behavior in dementia patients can be a risk factor for those around them.
Signup and view all the answers
Fitness to drive is not a concern for patients in the early stages of dementia.
Signup and view all the answers
Mild cognitive impairment (MCI) is recognized as an intermediate category introduced in ICD-10 for early detection of dementia.
Signup and view all the answers
The Clinical Dementia Rating (CDR) is utilized solely for assessing cognitive function in dementia.
Signup and view all the answers
MCI patients have a high probability of converting to dementia, with 40-70% progressing within 5 years.
Signup and view all the answers
The Montreal Cognitive Assessment (MoCA) is part of the screening tests for dementia.
Signup and view all the answers
Thyroid function tests are classified as secondary care investigations for dementia.
Signup and view all the answers
Olfactory deficits can be detected up to 20 years before the onset of dementia symptoms.
Signup and view all the answers
Neuropsychiatric Inventory is used exclusively to assess cognitive impairments.
Signup and view all the answers
The Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) solely focuses on daily living activities.
Signup and view all the answers
Genetic testing is considered a routine examination for dementia diagnosis.
Signup and view all the answers
Alzheimer's disease is classified as a primary neurodegenerative disorder.
Signup and view all the answers
Cushing's syndrome is a primary neurodegenerative cause of dementia.
Signup and view all the answers
Dementia with Lewy bodies is caused exclusively by prion diseases.
Signup and view all the answers
The most conspicuous feature of dementia is the difficulty with new learning.
Signup and view all the answers
Systemic lupus erythematosus is an inflammatory cause of dementia.
Signup and view all the answers
HIV is mentioned as a potential cause of vascular dementia.
Signup and view all the answers
Recent memory loss is typically more evident than remote memory loss in dementia patients.
Signup and view all the answers
Disturbed episodic memory is manifested as forgetfulness of recent day-to-day events.
Signup and view all the answers
Neurosarcoidosis is considered a metabolic cause of dementia.
Signup and view all the answers
Carbon monoxide poisoning can lead to post-anoxia dementia.
Signup and view all the answers
Subcortical dementia is characterized by early, prominent impairments of memory and word finding.
Signup and view all the answers
Presenile dementia occurs in individuals under 70 years of age.
Signup and view all the answers
The Mini-Mental State Examination (MMSE) is a benchmark for assessing cognitive impairment with a sensitivity of around 80%.
Signup and view all the answers
Vascular dementia is more commonly associated with presenile dementia than late-onset dementia.
Signup and view all the answers
Depression can coexist with dementia, potentially worsening functioning.
Signup and view all the answers
In presenile dementia, cases due to genetic diseases are relatively less common compared to late-onset dementia.
Signup and view all the answers
Investigations for presenile dementia are generally less intensive than for late-onset dementia.
Signup and view all the answers
The Montreal Cognitive Assessment (MoCA) is a commonly used test that takes longer than 10 minutes to complete.
Signup and view all the answers
Frontotemporal dementia and prion disease are relatively more common in senile dementia.
Signup and view all the answers
Screening tests for dementia may also help monitor the progression of the disease.
Signup and view all the answers
Over 50% of Alzheimer's disease patients experience psychotic symptoms such as delusions and hallucinations.
Signup and view all the answers
Aggression in Alzheimer's patients primarily manifests as physical violence towards others.
Signup and view all the answers
The median survival from the diagnosis of Alzheimer's disease is typically longer for women than for men.
Signup and view all the answers
Anosmia, or loss of smell, is identified as one of the core features of Alzheimer's disease.
Signup and view all the answers
Routine genetic testing for the apoE4 allele is recommended for all patients suspected of having Alzheimer's disease.
Signup and view all the answers
Cognitive impairments in the later stages of Alzheimer's disease increasingly dominate the clinical picture.
Signup and view all the answers
Sundowning refers specifically to an increase in disorientation during the daytime.
Signup and view all the answers
All patients with Alzheimer's disease will undergo personalized care strategies regardless of severity.
Signup and view all the answers
Neurofibrillary tangles and senile plaques are the principal diagnostic features identified in Alzheimer's disease through microscopic examination.
Signup and view all the answers
Depression occurs in less than 25% of Alzheimer's disease patients.
Signup and view all the answers
The strongest neuropathological correlate of cognitive impairment in Alzheimer's disease is the proliferation of astrocytes.
Signup and view all the answers
Senile plaques are deposits of insoluble proteins and are always pathologically less significant than neurofibrillary tangles.
Signup and view all the answers
Neurofibrillary tangles are primarily formed of the protein tau, which is usually soluble and aids in axonal transport.
Signup and view all the answers
The aetiology of most Alzheimer's disease cases can be attributed to Mendelian inheritance.
Signup and view all the answers
The presence of amyloid deposits in blood vessel walls is referred to as vascular amyloid or congophilic angiopathy.
Signup and view all the answers
Alzheimer's disease neuropathology begins in the hippocampus before spreading to the entorhinal cortex.
Signup and view all the answers
Apolipoprotein E (apoE) polymorphisms are unequivocally associated with all forms of Alzheimer's disease.
Signup and view all the answers
Familial forms of Alzheimer's disease involve mutations primarily in four genes including PSEN2 on chromosome 2.
Signup and view all the answers
The Braak stages of Alzheimer's disease focus on the deposition of tau proteins rather than β-amyloid.
Signup and view all the answers
Neuritic plaques possess a loose structure and are considered pathologically significant.
Signup and view all the answers
The APP gene is located on chromosome 21.
Signup and view all the answers
The first APP mutation related to Alzheimer's disease was discovered in 1984.
Signup and view all the answers
APP mutations promote the formation of β-amyloid by affecting APP metabolism.
Signup and view all the answers
Presenilin mutations are found in familial Alzheimer's disease and affect γ-secretase activity.
Signup and view all the answers
The APP gene mutations explain the majority of sporadic Alzheimer's disease cases.
Signup and view all the answers
Transgenic mice with a mutated APP gene exhibit cognitive impairment and deposit β-amyloid.
Signup and view all the answers
APP and PSEN mutations account for the majority of Down's syndrome cases.
Signup and view all the answers
A point mutation changing isoleucine to valine at position 717 in APP is linked to Alzheimer's disease.
Signup and view all the answers
The prevalence of Alzheimer's disease in individuals aged 60 or older is approximately 10-12%.
Signup and view all the answers
Olfactory deficits are considered a clinical feature of preclinical Alzheimer's disease.
Signup and view all the answers
Amyloid PET scans are currently in clinical use for diagnosing Alzheimer's disease.
Signup and view all the answers
Total Tau levels correlate with the severity of neurodegeneration in Alzheimer's disease.
Signup and view all the answers
Hippocampal atrophy occurs later in Alzheimer's disease compared to other forms of dementia.
Signup and view all the answers
The first symptom of Alzheimer's disease is often significant memory loss experienced all at once.
Signup and view all the answers
Major depression is found in roughly 20% of Alzheimer's disease cases.
Signup and view all the answers
Phosphorylated Tau correlates with neurofibrillary changes in the brain.
Signup and view all the answers
The rate of Alzheimer's disease doubles every 3 years in the elderly.
Signup and view all the answers
Neuropsychological deficits in Alzheimer's disease typically enhance verbal episodic memory.
Signup and view all the answers
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.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
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.
