Pathology I Flashcards
What is dementia?
A decrease in cognative ability, memory, or function with intact consciousness (p. 443)
What proteins are altered in early onset familial Alzheimer’s disease?
APP (Ch. 21), Presenilin-1 (Ch. 14), Presenilin-2 (Ch. 1) (p. 443)
What protein is altered in late onset familial Alzheimer’s disease?
ApoE4 (Ch. 19) (p. 443)
What protein is thought to be protective against Alzheimer’s?
ApoE2 (Ch. 19) (p. 443)
What gross findings are characteristic of Alzheimer’s disease?
Widespread cortical atrophy (p.443)
What histologic findings are characteristic of Alzheimer’s disease?
Senile plaques (extracellular B-amyloid core; amyloid beta (p.443)
How is amyloid beta protein synthesized?
By cleaving amyloid precursor protein (p.443)
What are neurofibrilary tangles?
Intracellular, abnormally phosphorylated tau proteins. They contain insoluble cytoskeletal elements and tangles correlate with degree of dementia (p.443)
What is Pick’s disease?
Frontotemporal dementia characterised by dementia, aphasia, Parkinsonisms, personality changes (p.443)
What areas of the brain are typically sparred in Pick’s disease?
Parietal lobe, posterior 2/3 of superior temporal gyrus (p.443)
What histologic findings are characteristic of Pick’s disease?
Pick bodies- spherical tau protein aggregates (p.443)
What gross findings are characteristic of Pick’s disease?
Frontotemporal atrophy (p.443)
What is Lewy body dementia?
Parkinsonism with dementia and hallucinations (p.443)
What defect is associated with Lewy Body dementia?
a-synuclein defect (p.443)
What is Crutzfeldt-Jakob disease (CJD)?
Rapidly progressive (weeks to months) dementia with myoclonus (startle myoclonus) (p.443)
What histologic findings are characteristic of Crutzfeldt-Jakob disease?
Spongiform cortex with prions (proteins containing beta pleated sheets resistant to proteases) (p.443)
Name six non-conventional causes of dementia.
Multi-infarct dementia (2nd most common cause in the elderly); syphilis, HIV, Vitamins B1, B3, B12 deficiency, Wilson’s disaese, Normal Pressure Hydrocephalus (p.443)
What is multiple sclerosis?
An autoimmune condition characterized by inflammation and demyelination of the CNS (brain and spinal cord) (p.444)
How do patients with MS typically present?
Optic neuritis (sudden loss of vision), MLF syndrome, hemiparesis, hemisensory symptoms, bladder/bowel incontinence (p.444)
Name four classic symptoms (Charcot’s classic triad) of MS.
Scanning speech, Intention tremor (also incontinence, internuclear opthalmoplegia), Nystagmus (p.444)
What lab findings are classic for MS?
Increased protein IgG in CSF with oligoclonal bands (p.444)
Describe the MRI findings in a patient with MS.
Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with axonal destruction (p.444)
What is the treatment for MS?
B-interferon, immunosuppression, natalizumab; symptomatic treatment for neurogenic bladder, spasticity, pain (p.444)
What is acute inflammatory demyelinating polyradiculopathy?
The most common variant of Guillain-Barre syndrome; an autoimmune condition that destroys Schwann cells causing inflammation and demyelination of peripheral nerves and motor fibers (p.444)