Term
| What are most neurodeg diseases linked to? |
|
Definition
| pathological protein misfolding and disease causing genes |
|
|
Term
| what are the 2 key neuropath signs of AD? |
|
Definition
| tau rich intraneuronal neurofibrillary tangles and A-beta amyloid or senile plaques in EXTRACELLULAR SPACES |
|
|
Term
| What does the endstage AD brain show? |
|
Definition
| diffuse cerebral atrophy with enlarged ventricles, narrowed gyri, and widened sulci |
|
|
Term
| How are neurofibrillary tangles thought to be made? |
|
Definition
| hyperphosphorylation of tau--aggregation of paired helical filaments into neurofib tangles |
|
|
Term
| What's Tau's normal role? |
|
Definition
| Tau binds to and stabilizes microtubules --facillitating axonal transport. This is negatively regulated by phosphorylation |
|
|
Term
| How are senile plaques formed? |
|
Definition
| Extracellular deposition of aggregated straight bibrils composed of A-Beta peptides cleaved from the precursor protein APP |
|
|
Term
| Are diffuse plaques implicated in AD? |
|
Definition
| No, found in health individuals 65+. |
|
|
Term
| What type of plaques are specific for AD? |
|
Definition
|
|
Term
| What does the diag of AD require? |
|
Definition
| dementia and the pathology |
|
|
Term
| What is FTDP-17 characterized by? |
|
Definition
| frontotemp dementia and parkinsonism is characterized by prom. intracellular neuronal and glial inclusions formed by tau-filaments. This is a tauopathy! |
|
|
Term
| Pick's disease is a prototypical what? |
|
Definition
| neurogenerative FrontoTemp Dementia (FTD) |
|
|
Term
| What are the symptoms of Pick's disease? |
|
Definition
| amnestic cogn dificits, apathy, abulia, difficult with language. |
|
|
Term
| What type of neuropathy is shown in Pick's |
|
Definition
| "knife blade atrophy," swollen neurons with intracytoplasmic Pick bodies |
|
|
Term
| What is the filamentous Pick body formed from? |
|
Definition
| abnormal tau filaments--but no plaques |
|
|
Term
| What are the clinical signs of endstage CJD? |
|
Definition
| severe dementia, ataxia, visual disturbances, myoclonus |
|
|
Term
|
Definition
| intraneuronal and intra-astrocytic accumulation of membrane bound vacuoles |
|
|
Term
| What is the diagnostic hallmark of CJD? |
|
Definition
| abnormal prion proteins accumulating within the vacuoles, resistant to protease digestion. |
|
|
Term
| What is the diagnostic hallmark of PD? |
|
Definition
|
|
Term
| How is PSNP different that AD or Pick's Disease? |
|
Definition
| Progressive supranuclear palsy IS a tauopathy, but with a prediliction for distinct brain regions like the BS/Basal ganglia |
|
|
Term
| What is the pathologic of ALS associated with? |
|
Definition
| degen of upper and lower motor neurons and the connecting corticospinal tract |
|
|
Term
| what is the clinical triad of atrophic weakness seen in ALS? |
|
Definition
| atrophic weakness of the hands and forearms, spasticity of the legs, and generaluize hyperreflexia. Muscles are characterized by amyotrophy (denervation atrophy) |
|
|
Term
| Which classes of neurons are vulnerable to degen in ALS? |
|
Definition
| motor nuerons of the cortex, BS, and spinal cord |
|
|
Term
| Although most ALS/FALS Neuropathy occurs in the neuron-rich gray matter, degen also occurs where? |
|
Definition
| in WM regions containing the CST |
|
|
Term
| Where are tauopathies usually found? |
|
Definition
| they are inclusions in neurons, oligodendrocytes, and astrocytes |
|
|
Term
| what are lewy bodies composed of? |
|
Definition
|
|
Term
|
Definition
| FTD, progressive supranuclear palsy, AD |
|
|
Term
| Name major synucleinopathies |
|
Definition
| PD, demetial with LB, multisystemic actrophy |
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