Neurodegenerative & Prion Diseases Overview

<p>Anterior horn of the spinal cord. (D)</p> Signup and view all the answers

<p>Progressive muscle weakness, atrophy, and paralysis. (D)</p> Signup and view all the answers

<p>Induction of a chronic inflammatory response mediated by microglia and astrocytes. (C)</p> Signup and view all the answers

<p>Disruption of microtubule assembly and stability due to reduced tau binding affinity. (D)</p> Signup and view all the answers

<p>Extracellular tangles serve as a reservoir of misfolded tau, which can seed the formation of new tangles in neighboring cells. (C)</p> Signup and view all the answers

<p>Aβ plaques induce oxidative stress and inflammation, which exacerbate tau hyperphosphorylation and tangle formation. (A)</p> Signup and view all the answers

<p>Aβ peptides exhibit abnormal folding propensity due to aberrant cleavage of APP, promoting self-assembly into oligomers and fibrils. (A)</p> Signup and view all the answers

<p>Presence of extracellular deposits of aggregated abnormal PrP, often Congo red- and PAS-positive, particularly in the cerebellum forming Kuru plaques. (D)</p> Signup and view all the answers

<p>Proteinase K-resistant PrPsc in tissue. (A)</p> Signup and view all the answers

<p>Overproduction and reduced clearance of Aβ and tau proteins. (A)</p> Signup and view all the answers

<p>Infection by a slow or latent virus directly causing the disease. (C)</p> Signup and view all the answers

<p>GABA (C)</p> Signup and view all the answers

<p>The medial temporal lobe, including the hippocampus and amygdala. (D)</p> Signup and view all the answers

<p>Autosomal dominant (B)</p> Signup and view all the answers

<p>Fibrillary gliosis and huntingtin protein aggregation in neurons (A)</p> Signup and view all the answers

<p>Cortical Kuru plaques surrounded by spongiform change. (A)</p> Signup and view all the answers

<p>Dysregulation of the basal ganglia leading to excessive thalamocortical pathway excitation (C)</p> Signup and view all the answers

<p>Insidious impairment of intellectual function. (B)</p> Signup and view all the answers

<p>Mutation in a DNA repair gene (C)</p> Signup and view all the answers

<p>Aggregation of misfolded prion protein (PrPsc). (C)</p> Signup and view all the answers

<p>Loss of axons and gliosis in the posterior columns of the spinal cord (D)</p> Signup and view all the answers

<p>Hypoplasia of lymph nodes, gonads, and thymus (C)</p> Signup and view all the answers

<p>Friedreich Ataxia (B)</p> Signup and view all the answers

<p>It serves as a (+) (-) regulatory pathway, critical for modulating voluntary movement. (A)</p> Signup and view all the answers

<p>Loss of dopaminergic neurons from the substantia nigra. (A)</p> Signup and view all the answers

<p>Rapid, uncontrolled movements. (D)</p> Signup and view all the answers

<p>It receives dopaminergic projections from the substantia nigra and is affected by dopamine reduction in PD. (B)</p> Signup and view all the answers

<p>It reflects the loss of pigmented, dopamine-producing neurons. (C)</p> Signup and view all the answers

<p>Catalyzing the conversion of normal α-helical PrPc into the misfolded β-sheet PrPsc form, leading to aggregation. (B)</p> Signup and view all the answers

<p>It accumulates and aggregates, leading to mitochondrial abnormalities and neuronal loss. (C)</p> Signup and view all the answers

<p>It makes up the majority (~90%) of CJD cases and arises without a clear identifiable cause. (D)</p> Signup and view all the answers

<p>Lewy bodies. (D)</p> Signup and view all the answers

<p>PrPsc exhibits a higher content of β-sheet structures, which confers resistance to protease digestion. (D)</p> Signup and view all the answers

<p>Deep implantation of electrodes in the brain that were previously used on a patient with CJD. (D)</p> Signup and view all the answers

<p>Cytoplasmic, eosinophilic, round to elongated inclusions that often have a dense core surrounded by a pale halo. (A)</p> Signup and view all the answers

<p>It involves the development of numerous small, empty vacuoles in the cerebral cortex and deep gray matter. (A)</p> Signup and view all the answers

<p>Rapidly progressive dementia accompanied by myoclonus and a uniformly fatal outcome. (C)</p> Signup and view all the answers

<p>Inflammation (C)</p> Signup and view all the answers

<p>7 months (D)</p> Signup and view all the answers

A degenerative disease affecting motor neurons in the spinal cord and brainstem, leading to muscle atrophy.

Nerve cells that transmit signals from the brain and spinal cord to muscles, controlling movement.

Neurons located in the anterior horn of the spinal cord that directly innervate muscles.

Genetic mutations in superoxide dismutase 1, linked to reduced detoxification of free radicals, causing neuronal death in ALS.

Muscle wasting caused by loss of motor neuron input, commonly seen in ALS patients.

Deposits of aggregated Aβ peptides in the neuropil from abnormal APP cleavage.

Fragments derived from the abnormal cleavage of the APP protein that can aggregate and are neurotoxic.

Intracellular aggregates of hyperphosphorylated tau protein that disrupt neuronal function.

A microtubule-associated protein that stabilizes microtubules in axons; becomes hyperphosphorylated in AD.

Inflammatory reaction from microglia and astrocytes due to toxicity from aggregated Aβ peptides.

A normal cellular prion protein that can convert into a pathogenic form.

The abnormal prion protein that leads to disease by aggregating and inducing damage.

The process by which PrPsc causes disease through interaction with PrPc and resistance to digestion.

The most common prion disease characterized by rapid dementia and neurological decline.

A form of CJD occurring with no known genetic link, making up 90% of cases.

CJD caused by genetic mutations in the PRNP gene encoding prion protein.

Rapidly progressive dementia, behavioral changes, and muscle contractions are key symptoms.

Characterized by spongiform transformation in the brain, forming vacuoles due to neuron loss.

A prion disease causing rapid neurodegeneration and dementia, marked by spongiform changes in the brain.

Extracellular deposits of aggregated abnormal PrP found typically in the cerebellum of CJD patients.

Proteinase K–resistant form of prion protein, indicative of prion diseases.

The most common cause of dementia in the elderly, leading to intellectual decline and memory loss.

Degeneration leading to widening of cerebral sulcus, commonly found in Alzheimer’s patients.

Progressive accumulation of Aβ and tau proteins, leading to neurodegeneration and cognitive decline.

Stiffness of muscles and reduced mobility, often seen in Parkinson's disease.

Neurodegenerative disease leading to hypokinetic movement due to loss of dopaminergic neurons.

Condition characterized by reduced voluntary movement.

Neural pathway connecting the substantia nigra to the striatum, important for movement control.

A rhythmic movement of the fingers resembling rolling a pill, common in Parkinson's disease.

Eosinophilic inclusions in neurons, associated with Parkinson's disease pathology.

Protein that accumulates in Parkinson’s disease, forming Lewy bodies.

Brain region critical for movement, where dopaminergic neurons are lost in Parkinson’s disease.

An autosomal dominant disorder characterized by progressive movement disorders and dementia, due to degeneration of striatal neurons.

Degeneration of the caudate nucleus in Huntington disease leads to motor dysfunction and cognitive decline.

Involuntary, jerky movements associated with increased motor output in Huntington disease.

Group of disorders causing neuronal loss affecting the cerebellum, spinal cord, and peripheral nerves, leading to ataxia.

A specific type of spinocerebellar degeneration, characterized by gait ataxia and loss of sensory functions in early life.

A syndrome that includes ataxic dyskinetic movements and immune deficiency, caused by a mutation in the ATM gene.

Loss of GABA-producing neurons, particularly in the context of Huntington disease, leading to an imbalance in neurotransmitters.

The proliferation of glial cells in response to neuronal injury, often seen in degeneration conditions like Huntington disease.