Friday, December 12, 2008

Dementia Syndromes


1. Alzheimer's disease: A very common form of dementia in elderly patients. Characterized by progressive short term memory loss and cognitive decline. It is a clinical diagnosis, however classic histology reveals amyloid deposition in the brain (in beta-pleated sheets) and neurofibrillary tangles. A great link on AD here.

2. Vascular dementia: Manifested by cognitive decline after a stroke. There is a "stepwise" deterioration in function with subsequent ischemic injuries. Imaging of the brain will reveal ischemic changes.

3. Fronto-Temporal dementia: Atrophy of frontal and temporal lobes, but without histologic evidence of Alzheimer disease. This classically occurs in a slightly younger cohort...50-60 year old males and females. One of the hallmarks of this disease are personality changes - inappropriate personal or social behaviours. Aphasia may also be seen. You may hear of "Pick's Disease", a subset of fronto-temporal dementia with a progressive, non-fluent aphasia component to it.

4. Lewy-Body dementia: manifested by progressive cognitive decline, vivid visual hallucinations, delusions, syncope, and occasionally Parkinson-like symptoms (see below).

5. Dementia associated with Parkinsonism: This is an umbrella term for dementia associated with primary Parkinson's disease, and the Parkinson Plus Syndromes. Note that the Parkinson Plus syndromes typically have a poor response to dopaminergic therapy - that with their individual clinical features, this often helps clinicians distinguish them from primary Parkinson's disease.

  • Progressive supranuclear palsy: Parkinsonian symptoms, dementia, syncope, and vertical gaze abnormalities. Many have bulbar symptoms like dysarthria and dysphagia. It is sometimes referred to as the Steele-Richardson-Olszewski syndrome.


  • Multisystem Atrophy: A more mild progressive dementia, with mild features of Parkinson's disease (although tremor is mostly absent), and autonomic dysfunction manifested by syncope. You may also hear this condition referred to as the Shy-Drager syndrome. An excellent link here.

  • Corticobasilar Degeneration: A progressive dementia, with motor features of bradykinesia, rigidity, and postural instability. Patients may have sensory disturbances, apraxia, aphasia, or even alien limb syndrome.


6. Reversible Dementias: these include conditions where we can remove/modify an offending agent. Think about drug/toxin exposure, space occupying lesion, depression, or a metabolic abnormality.

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