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65 Cards in this Set

  • Front
  • Back
Causes of dementia:
8
-Vascular disease (including multi-infarct dementia)

-Parkinson’s disease

-Pick’s disease

-Huntington’s disease

-Normal pressure hydrocephalus

-Metabolic diseases, including Vitamin B12 deficiency, Chronic drug intoxication, hypothyroidism, and alcoholism

-Infectious causes, including HIV, neurosyphilis, and bacterial meningitis

-Major depression

*They all reduce the number of functional neurons/synapses below a critical level.
Cardinal signs of dementia:
2
-Lost short term memory
-Difficulty in ADLs
How many people have AD at age 85?
50% of people that age.
Neurofibrillary tangles:
shape
composition
-Intracellular inclusion bodies consisting of paired helical filaments that appear in a characteristic double-helix shape

-Filaments appear to be composed of a hyperphosphorylated microtubule-associated protein called tau

-Remains of damaged neuronal microtubules
Particularly affected regions of brain in AD?
Hc, frontal lobes, association cortical areas.
hirano bodies
intracellular cigar-shaped inclusions made of actin
found in AD
Granulo-vacuolar Degeneration:
-inclusions made of tubilin
found in AD
Brain in Down's:
Small brain with abnormal gyral pattern

Decreased neuronal populations

Abnormality of neuronal structure (simplification of dendritic arbor)
-most successful drugs to date in treating AD?
-cholinergic drugs targeting the NBM. gives you about another 6 months.
Chromosomes associated with AD:
-ones linked with early-onset AD?
-genes linked to late-onset AD?
-Research has produced evidence of a link between Alzheimer’s disease and chromosomes 1,10,14,19 and 21

-Chromosomes 1,14,and 21 have been linked with early-onset Alzheimer’s disease (familial AD), an extremely rare form of the disease (10%)

-ApoE4 gene located on chromosome 19 has been associated with late-onset (sporadic) Alzheimer’s disease, the most common form of the disease, so has Beta-Catenin gene on chromosome 10. (90%)
Pick's Disease:
-Progressive dementia in middle or late life

-Higher incidence in males

-Cerebral gyral atrophy of FRONTAL and TEMPORAL lobes, sparing posterior 2/3 of the superior temporal gyrus

-Pick bodies and “balloon” cells
Pathology of PD:
-Lewy bodies
-Loss of neuromelanin-bearing nerve cells
Parkinsonism caused by:
6
-Carbon monoxide,

-drugs (e.g.phenothiazines),

-manganese,

-arteriosclerosis (?),

-encephalitis (esp. von Economo’s disease),

-ALS-PD
Diffuse Lewy Body Disease:
-AKA dementia w/ LBs
-LBs not just in SN, but throughout the whole brain
-2nd most common cause of dementia
Progressive Supranuclear Palsy:
Postural instability, dysphagia/dysarhria, supranuclear gaze palsy, marked axial rigidity

-Neurofibrillary tangles in many subcortical structures (accumulation of tau protein) DEEP BRAIN

-Resistant to treatment with antiparkinsonian agents, but clinically resembles PD.
HD:
-Chorea, neuropsychiatric disturbances, progressive dementia

-Gene linked to chromosome 4 and identified (huntingtin); “unstable repeat disease”

-Atrophy of caudate and dorsal putamen

-Treatment is symptomatic
ALS:
-Atrophic weakness of hands and forearms, slight spasticity of the legs, and generalized hyperreflexia, with normal sensation

-Degeneration of anterior horn cells and lower brainstem motor nuclei with gliosis
-Loss of large myelinated fibers in motor nerves
-Skeletal muscle shows denervation atrophy

-Corticospinal tract degeneration
Olivopontocerebellar atrophy:
-Ataxia: first in legs, then in arms/hands and bulbar musculature

-Some develop symptoms of Parkinsonism

-Degeneration of MCP’s, cerebellar white matter, and the pontine, olivary, and arcuate nuclei; Purkinje cell loss variable

-“Dying-back” of axons; secondary myelin loss

-Part of “multiple system atrophy”
Friedreich's Ataxia:
-Most common spastic hereditary ataxia

-Gene mapped chromosome 9 but not identified (autosomal recessive inheritance)

-Degeneration of posterior columns, Clarke’s nucleus, spinocerebrellar tracts (especially dorsal), distal corticospinal tracts

-50% die from cardiac arrhythmias