Dementia differs from other disorders of cognitive function, such as coma or confusional states, in that ...?
The level of consciousness (wakefulness or arousal) is preserved in dementia.
The term "mild cognitive impairment" is sometimes used to describe ...?
Deficits that are more severe than are customarily seen with normal aging but are insufficiently pronounced to warrant a diagnosis of dementia.
Patients with MCI have an increased risk (approx. 10% per year) of ...?
Neurologic changes in normal aging - 5 main categories:
1. Cognitive. 2. Neuro-ophthalmologic. 3. Motor. 4. Sensory. 5. Reflexes.
Neurologic changes in normal aging - Cognitive:
1. Slowed information processing. 2. Impaired learning and recall of new information. 3. Reduced spontaneous word finding and verbal fluency. 4. Incr. reaction time.
Neurologic changes in normal aging - Neuro-ophthalmologic:
1. Small, sluggishly reactive pupils. 2. Impaired upgaze. 3. Impaired convergence.
Neurologic changes in aging - Motor:
1. Atrophy of intrinsic hand and foot muscles. 2. Incr. muscle tone. 3. Flexion (stooped) posture. 4. Small-stepped or broad-based gait.
Neurologic changes in normal aging - Sensory:
1. Reduced visual acuity. 2. Reduced auditory acuity. 3. Reduced gustatory acuity. 4. Reduced olfactory acuity. 5. Reduced vibration sense.
Neurologic changes in normal aging - Reflexes:
1. Primitive reflexes. 2. Absent ankle reflexes. 3. Absent abdominal reflexes.
At present, ...% of dementias are reversible.
Dementia implies deterioration in cognitive ability. It is important to establish that the patient's level of functioning has declined:
1. Data that can help to establish the cause of dementia include the time course of deterioration. 2. Associated symptoms - Headache, gait disturbances, or incontinence. 3. Family history of a similar condition. 4. Concurrent medical illnesses. 5. The use of alcohol and therapeutic or recreational drugs.
Acute confusional state vs dementia - Level of consciousness:
Acute confusional state --> Impaired. Dementia --> Not impaired, except occasionally late in course.
Acute confusional state vs Dementia - Course:
Acute confusional state --> Acute to subacute; fluctuating. Dementia --> Chronic; steadily progressive.
Acute confusional state vs Dementia - Autonomic hyperactivity:
Acute confusional state - Often present. Dementia - Absent.
Acute confusional state vs Dementia - Prognosis:
Acute confusional state - Usually reversible. Dementia - Usually irreversible.
What is the usefulness of the mental status exam:
1. Helps to determine the level or the content of consciousness that is impaired. 2. Whether the cognitive dysfunction is global or circumscribed.
A disorder of the level of consciousness is suggested by ...?
1. Sleepiness. 2. Inattention. 3. Impairment of immediate recall. 4. Disorientation regarding place or time. --> Abnormalities in these areas are unusual in dementia until the disorder is far advanced.
To determine the scope of the cognitive dysfunction (global or circumscribed) ...?
Different spheres of cognition are tested in turn: 1. Memory. 2. Language. 3. Parietal lobe functions (pictorial construction, R-L discrimination, localization of objects in space). 4. Frontal lobe or diffuse cerebral cortical functions (judgement, abstraction, thought content, the ability to perform previously learned acts). --> Multiple areas of cognitive function are impaired in dementia.
Dementia from different causes may preferentially impair ...?
Different spheres of cognition --> This can provide diagnostic clues. --> e.g. Alzheimer disease affects memory disproportionately, whereas language function is often impaired in frontotemporal dementia.
Clinical features helpful in the DDx of dementia - History - Unprotected sexual intercourse, IVDA, hemophilia, blood transfusions:
Clinical features helpful in the DDx of dementia - History - Family history:
1. Huntington disease. 2. Wilson disease.
Clinical features helpful in the DDx of dementia - History - Headache:
1. Brain tumor. 2. Chronic subdural hematoma.
Clinical features helpful in the DDx of dementia - Vital signs:
Hypothermia - Hypothyroidism. HTN - Vascular dementia. Hypotension - Hypothyroidism. Bradycardia - Hypothyroidism.
Clinical features helpful in the DDx of dementia - General examination:
1. Meningismus --> Chronic meningitis. 2. Jaundice --> Frontotemporal dementia (semantic dementia, progressive nonfluent aphasia). 3. Kayser-Fleischer rings --> Lewy body disease.
Clinical features helpful in the DDx of dementia - Cranial nerves - Papilledema:
1. Brain tumor. 2. Chronic subdural hematoma.
Clinical features helpful in the DDx of dementia - Cranial nerves - Argyll-Robertson pupils:
Clinical features helpful in the DDx of dementia - Cranial nerves - Ophthalmoplegia:
Progressive supranuclear palsy.
Clinical features helpful in the DDx of dementia - Cranial nerves - Pseudobulbar palsy:
1. Vascular dementia. 2. Progressive supranuclear palsy.
Clinical features helpful in the DDx of dementia - Motor - Limb apraxia:
Clinical features helpful in the DDx of dementia - Motor - Tremor:
1. Lewy body disease. 2. Corticobasal degeneration. 3. Acquired hepatocerebral degeneration. 4. Wilson disease. 5. HIV-associated dementia.
Clinical features helpful in the DDx of dementia - Motor - Asterixis:
Acquired hepatocerebral degeneration.
Clinical features helpful in the DDx of dementia - Motor - Myoclonus:
1. CJD. 2. HIV-associated dementia.
Clinical features helpful in the DDx of dementia - Motor - Rigidity:
1. Lewy body disease. 2. Corticobasal degeneration. 3. Acquired hepatocerebral degeneration. 4. CJD. 5. Progressive supranuclear palsy. 6. Wilson disease.
Clinical features helpful in the DDx of dementia - Motor - Chorea:
1. Huntington. 2. Wilson.
Clinical features helpful in the DDx of dementia - Other:
1. Gait apraxia - NPH. 2. Hyporeflexia (from associated polyneuropathy) - Neurosyphilis, B12, HIV-dementia.
Minimental status examination (MME) - Main categories:
1. Orientation. 2. Registration. 3. Attention and calculation. 4. Recall. 5. Language. 6. Construction.
MME - Orientation:
1. Time (1 point each for year, season, month, date, day of the week) - 5. 2. Place (1 point each for state, country, city, building, floor or room) - 5.
MME - Registration:
Repeat names of three objects (1 point per object) - 3.
MME - Attention and calculation:
Serial 7s or spell "world" backwards (1 point per substraction or letter) - 5.
MME - Language:
1. Name pencil and watch (1 point each) - 2. 2. Repeat "No ifs, ands, or buts" - 1. 3. Follow three-step command (1 point per step) - 3. 4. Read and follow: "Close your eyes" - 1. 5. Write a complete sentence - 1.
MME - Construction:
Copy 2 intersecting pentagons - 1.
MME - A total score of ...?
A wide variety of diseases can produce dementia, but only a few do so commonly. In its most typical presentation - with gradual cognitive decline in an elderly (>65y) patient - the MCCs of dementia are:
1. Alzheimer's. 2. Vascular (formerly "multi-infarct") dementia. 3. Frontotemporal dementia. 4. Lewy body disease. 5. Parkinson disease.
Patients who present with dementia before
A wider range of neurodegenerative (Huntington, corticobasal degeneration), inflammatory (MS, SLE, vasculitis), and infective (prion) causes.
Dementia that progresses rapidly over weeks to months results most often from ...?
Prion (Creutzfeldt-Jakob) disease.
Reversible causes of dementia:
1. NPH. 2. Intracranial mass lesions. 3. B12. 4. Hypothyroidism. 5. Neurosyphilis. --> RARE.
Approx. ...% of patients referred for evaluation of possible dementia instead have other disorders (pseudodementias), such as depression.
In several neurodegenerative diseases, the production of ...?
Misfolded proteins and their association to form insoluble aggregates appears to play an important role in pathogenesis.
The abnormally misfolded proteins can arise from ...?
Either genetic or acquired modifications, and their pathologic effects may result from: 1. Loss of normal protein function. 2. Gain of toxic function. 3. Combination of these factors.
Except in rare inherited or infectious cases, the underlying cause of neurodegenerative proteinopathies is unknown. However these disease share several features:
In addition to protein misfolding and aggregation, which sometimes produces characteristic histopathologic findings, these diseases may be associated with cell-to-cell prionic transmission.
Alzheimer affects approximately ...% of individuals over 65, and ...% of individuals over 85.
15% over 65. 45% over 85.
>5million cases in the USA. 30million cases worldwide.
Why Alzheimer's is more common in women?
Because they live longer!
What is dementia?
An acquired + generalized + progressive impairment of cognitive function.
Neuritic plaques are ...?
EXTRACELLULAR deposits that contain β-amyloid (Aβ) and other proteins: 1. Presenilin 1, 2. 2. Alpha-1-antichymotrypsin. 3. Apolipoprotein E. 4. Alpha-2 macroglobulin. 5. Ubiquitin.
Neuritic plaques are ...?
Extracellular deposits that contain β-amyloid (Aβ) and other proteins, including:
- Presenilin 1.
- Presenilin 2.
- Alpha-1 antichymotrypsin.
- Apolipoprotein E.
- Alpha-2 macroglobulin.