neurodegeneration_flashcards
Who is the speaker and what is the focus of the lecture?
The speaker is Prof. Steve Gentleman, and the focus is on neurodegenerative diseases, their pathology, diagnostic protocols, and underlying mechanisms. This lecture is part of a broader series on neuropathology.
What are neurodegenerative diseases?
Neurodegenerative diseases are progressive disorders characterized by the degeneration of the structure and function of the nervous system.
What are common features of neurodegenerative diseases?
Common features include involvement of specific dysfunctional proteins, accumulation of these proteins leading to neuronal death and brain atrophy, and clinical overlap among different diseases, making diagnosis challenging.
What is the diagnostic protocol for neurodegenerative diseases?
Routine brain bank procedures and CNS autopsies are essential for understanding and diagnosing neurodegenerative diseases by identifying key pathological features and differentiating diseases.
Why is handling CNS autopsies important?
CNS autopsies provide critical insights into the underlying pathology of neurodegenerative diseases, which is crucial for accurate diagnosis.
Who first described Alzheimer’s Disease and what were the key pathological features observed?
Alzheimer’s Disease was first described by Alois Alzheimer in 1906, observing a patient with senile dementia and identifying key pathological features: amyloid plaques and neurofibrillary tangles.
What are the pathological features of Alzheimer’s Disease?
Pathological features include amyloid plaques (extracellular deposits of amyloid beta), neurofibrillary tangles (hyperphosphorylated tau), vascular involvement (amyloid deposits around blood vessels), and neuronal loss leading to brain shrinkage.
How is Alzheimer’s Disease diagnosed?
Alzheimer’s Disease diagnosis involves imaging (MRI) to show cortical and hippocampal atrophy, pathological assessment including amyloid and tau staging (Braak staging), and the ABC Score which combines amyloid distribution, tangle staging, and plaque density.
What is the clinical presentation of Alzheimer’s Disease?
The clinical presentation includes progressive memory loss, cognitive decline, and behavioral changes.
What characterizes the pathology of Parkinson’s Disease?
Parkinson’s Disease is characterized by the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (alpha-synuclein inclusions).
What are the symptoms of Parkinson’s Disease?
Symptoms include motor symptoms such as tremor, rigidity, bradykinesia, and postural instability, as well as cognitive decline in later stages.
What are Lewy Body Diseases?
Lewy Body Diseases include Lewy Body Dementia (progressive cognitive decline with Lewy bodies in cortical neurons) and Parkinson’s Disease Dementia (cognitive decline in patients with long-standing Parkinson’s Disease).
How is Parkinson’s Disease diagnosed?
Parkinson’s Disease is diagnosed based on clinical findings supported by imaging and post-mortem examination. Alpha-synuclein immunohistochemistry reveals extensive pathology beyond the substantia nigra.
What is Multiple System Atrophy (MSA)?
Multiple System Atrophy is a disorder affecting autonomic functions and motor control, characterized by alpha-synuclein accumulation in oligodendrocytes, with subtypes MSA-P (Parkinsonian) and MSA-C (Cerebellar).
What are the pathological features of Multiple System Atrophy (MSA)?
Pathological features include glial cytoplasmic inclusions (GCIs) in oligodendrocytes, affecting the basal ganglia, cerebellum, and brainstem.
What are the symptoms and pathology of Progressive Supranuclear Palsy (PSP)?
Symptoms include postural instability, vertical gaze palsy, and cognitive decline. Pathology involves tau accumulation in neurons and glial cells, with atrophy of the midbrain and cerebellum.
What characterizes Corticobasal Degeneration (CBD)?
CBD is characterized by asymmetric motor dysfunction, apraxia, and the alien limb phenomenon. Pathology includes ballooned neurons and tau pathology in cortical and basal ganglia regions.
What are the pathological features of Corticobasal Degeneration (CBD)?
Pathological features include cortical and basal ganglia atrophy, astrocytic plaques, and tau-positive inclusions.
What are Frontotemporal Dementias (FTDs)?
Frontotemporal Dementias are a diverse group of disorders affecting the frontal and temporal lobes, with various subtypes involving different proteinopathies like tau, TDP-43, or FUS proteins.
What characterizes Pick’s Disease?
Pick’s Disease is characterized by Pick bodies (intracellular inclusions of tau protein) and affects the frontal and temporal lobes, leading to personality changes and language deficits.
What are the pathological features of Pick’s Disease?
Pathological features include Pick bodies (round, basophilic inclusions within neurons) and ballooned neurons (swollen, pale-staining neurons).
What are tauopathies and proteinopathies involved in Frontotemporal Dementias (FTDs)?
Tauopathies involve tau protein (e.g., PSP, CBD, Pick’s Disease), TDP-43 proteinopathies involve TDP-43 protein (e.g., some forms of FTD), and FUS proteinopathies involve FUS protein (rare).
What are prion diseases?
Prion diseases are rapidly progressive and fatal neurodegenerative diseases caused by prions (misfolded proteins), with the most common being Creutzfeldt-Jakob disease (CJD).
What is the pathology of prion diseases?
Pathology includes spongiform changes in the brain (neuronal loss and vacuolation) and the accumulation of prion protein (PrP^Sc) in the brain.
