Dementia Flashcards
Early changes in personality and behavior, or aphasia. May have associated movement disorders
Frontotemporal dementia (previously known as Pick disease) Atrophy of FRONTAL lobe
Initially dementia and visual hallucinations followed by parkinsonian features
Lewy body dementia (haLEWYcinations)
Lewy bodies in cortex, basal nucleus of Meynert, and substantia nigra (alpha synuclein or ubiquitin)
Note: lewy bodies are accum of aggregated protein within the cytoplasm of the neurons
Step-wise decline in cognitive ability with late-onset memory impairment.
Vascular dementia
due to multiple arterial infarcts and/or chronic ischemia
Rapidly progressive (weeks to months) dementia with myoclonus (startle myoclonus)
Creutzfeldt-Jakob disease (spongiform cortex)
Slowly progressive dementia, difficulty with language, disorientation, mood swings
Alzheimer’s disease
Neurofibrillary tangles in hippocampus, neocortex and cholinergic nuclei of basal forebrain
What is the only agent shown to extend the survival of patients and/or time until tracheotomy in ALS patients?
MOA?
Riluzole - glutamate release inhibitor and glutamate receptor blocker, and sodium channel blocker
NO diffuses into smooth mm, activates _____ making ____, triggering smooth mm relaxation
- guanylate cyclase
2. cGMP
Prion diseases cause spongiform encephalopathy (dementia, loss of motor function) and are unusual infectious agents why?
NOT viruses bc NO nucleic acids. They are abnormal variants of normal cellular proteins.
They are the ONLY pathogenic agent that has NO NUCLEIC acids and are therefore insensitive to nucleases.
Causes of early vs late onset Alzhemiers disease?
Early - amyloid precursor protein gene mutation, presenilin 1 and 2 mutations
Late - apolipoprotein E4 genotype
What biochemical process is defective in patients with accumulation of misfolded proteins (eg alzheimers or parkinsons?)
ubiquitination
Any impairment of the ubiquitin-proteasome system can cause these diseases
First line treatment for essential tremor?
Propranolol - nonselective b-adrenergic antagonist that lessons tremor via CNS effects
Most important biochemical abnormality in Alzheimer disease?
Decrease in acetylcholine - decline of Ach in basal nucleus of Meynert
ie: dec choline acetyltransferase activity in nucleus basalis
HIV-assoc dementia (memory problems, executive dysfunction, slow info processing) affects what part of the brain? Pathogenesis?
Subcortical/deep gray matter structures
D/t inflam activation of microglial cells (macrophages). May fuse to form multinucleated giant cells
What area of the brain demonstrates the greatest degree of atrophy in Alzheimer’s disease
hippocampus
Alzheimer’s can be treated with a cholinesterase inhibitor to improve memory because of its ability to target synaptic connections between which structures?
The basal forebrain and neurons in the cerebral cortex
Note: The basal forebrain is a collection of structures located to the front of and below the striatum. It includes the nucleus accumbens, nucleus basalis, diagonal band of Broca, substantia innominata, and medial septal nuclei.
