Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Neurobiology > Neurodegenerative Disorders > Flashcards

Neurodegenerative Disorders Flashcards

(63 cards)

Start Studying
0
Q

Give examples of neurodegenerative disorders

A 
Alzheimer's 
Parkinson's 
Huntington's
Frontal temporal dementia/picks 
CBGD
Motor neuron disease 
PSP - progressive supra palsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
1
Q

What are the main indications of neurodegenerative disorders ?

A

Loss of neurons in brain and spinal cord
Progressive symptoms
Irreversible symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the most common neurodegenerative disorder ?

A

Alzheimer’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Define dementia:

A

An acquired, persistent intellectual impairment involving at least 3 of these domains:

  • language
  • visuospatial skills
  • emotion/personality
  • memory
  • cognitive/executive function s

It is a failure of higher brain functions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Why are the pathological markers of AD?

A

Cortical extracellular senile plaques and neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the pathological markers of PD?

A

Lewy bodies- intracellular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the pathological markers of PSP?

A

Subcortical neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the pathological markers of CBGD?

A

Cortical and subcortical achromatic inclusion bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the pathological markers of Huntingtons disease ?

A

Selective loss or neurons and gliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the pathological markers of picks disease ?

A

Selective temporal lobe atrophy and picks bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What was PD first described as ?

A

Shaking palsy in 1817

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does PD cause ?

A

A kinetic rigid syndrome

  • loss of movement
  • increased muscle tone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Do patients normally die form PD and what are their life expectancies ?

A

No they normally die of a complication of the disease such as bronchopneumonia or bed sores

10-15 years but some people survive decades - the drug treat,net significantly prolongs life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the main symptoms of Parkinson disease ?

A 
Tremor
Rigidity 
Speech difficulty 
Postural changes 
Akinesia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What do Parkinson’s patients do to try and help initiate movements ?

A

Use visual cues such as a time on a clock

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the pathological effects of Parkinson’s disease ?

A

Loss of neurons in substantia nigra
Lewy bodies - spherical eosinophilic inclusion bodies
It is the dopaminergic neurones that are affected
Loss of the excitatory and inhibitory nigro-striatal pathways
Affects midbrain nuclei

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the causes of Parkinson’s disease ?

A

Unknown - idiopathic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What things have caused associated disorders of Parkinson’s disease ?

A

Drug induced - iatrogenic- dopamine antagonist
MPTP induced - contaminated amphetamines which kills off dopamine neurons in substantia nigra causing rapid onset of Parkinson’s disease
Post encephalitic- viral illness that triggers degeneration of nerve cells in substantia nigra

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What happens to the pathways in Parkinson’s disease ?

A

Lose indirect and direct pathways so it reduces thalamic output causing a reduction in motor output

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Describe huntingtons disease

A 
Gradual onset 
Normal onset is 30-50 years - younger means it's more sever 
Progressive 
Inherited 
Death within 10-20 years 
Main symptoms are chores and dementia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the symptoms that lead to dementia in huntingtons disease ?

A

Irritability
Moodiness
Antisocial behaviour- aggressive, angry and unpleasant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are th symptoms that lead to gross chore inform movements of huntingtons disease ?

A

Fidgeting
Restlessness

Gross choreiform movements cause over reaching and grasping things too tightly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Where are neurons lost in huntingtons disease?

A

Cerebral cortex

Corpus striatum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Which neurons are the first to be affected in huntingtons disease ?

A

Medium sized spiny neurons containing GABA and enkephalins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
What happens to neurotransmitters in huntingtons disease ?
Decrease in GABA and its enzyme GAD Decrease in ACh and its enzyme choline acetyl transferase Either no change to dopamine or a slight increase
25
What happens to the pathway in huntingtons disease ?
Lose some of the negative feedback control on dopamine causing increased dopamine output
26
What are the causes of huntingtons disease?
Autosomal dominant disorder Affects shorts arm of chromosome 4 Locus 4p16.3 codes for protein Huntingtin - gives rise to poly glutamine repeat - CAG More than 36 repeats means you have the disease
27
Describe progressive supranuclear palsy
``` Idiopathic Death within 2-12 years Affects subcortical grey matter- loss of neurons 2x more prevalent in men Causes aspiration and inanition Starts 45-75 years ```
28
What are the main symptoms of PSP?
Progressive dementia causing exaggerated emotional response Motor disorders - opthalmoplegia, pseudobulbar palsy and balance/posture impaired Sometimes causes limb rigidity, bradykinesia and hyperreflexia
29
What is opthalmoplegia ?
Loss of eye movements | First the vertical gaze and then horizontal
30
What balance/postural impairments occur in PSP?
Unexplained falls | Neck extended and rigid to flexion so it's difficult to move
31
What are he pseudobulbar palsy symptoms of PSP?
Facial weakness Dysarthria Dysphagia Exaggerated gag reflex
32
What are the pathologies of PSP?
Neurodegeneration Neurofibrillary tangles in midbrain, pons, basal ganglia, dentate nuclei of cerebellum - in all motor control areas Decrease in da in cerebellum and caudate nucleus
33
What are neurofibrillary tangles ?
Aggregates of tau protein in micro tubules
34
Describe cortical basal ganglionic degeneration
Rare Non inherited Onset is Middle age/elderly
35
What are the symptoms of CBGD??
``` Bradykinesia/rigidity Clumsiness Speech- apraxia, asphasia and dysarthria Myoclonus Postural instability ```
36
What is dysarthia?
Inability to vocalise
37
What are the pathologies of CBGD ?
Atrophy of frontal and parietal cortex | Cell loss and gliosis in cortex, thalamus, red nucleus, lentiform nucleus, substantia nigra and locus coerulus
38
What are the different disorders in motor neurons disease ?
Amyotrophic lateral sclerosis - ALS- loss of both upper and lower Primary lateral sclerosis - PLS - loss of upper Progressive muscular atrophy - PMA - loss of lower Pseudobulbar palsy -PBP- loss of lower Preogressive bulbar palsy -PrBP- loss of upper
39
Describe ALS
``` Begins 30-60 years old Degen of both upper and lower( anterior horn cells) Muscle denervation 90-95% sporadic and 5-10% familial Fatal in 3-5 years ```
40
What are the symptoms on motor neurons disease ?
Bulbar 20% - chewing, swallowing, breathing and speaking Upper extremities and lower extremities 40% Muscle wasting and weakness Affected muscles are easily tired, stiff and twitch
41
What is ALS-PDC?
Occured in chaumoro people of Guam | They ate flying foxes which act cycads that contain toxic amino acid beta-methylamino l-Alanine
42
What is picks disease ?
It's the most common frontotemporal dementia - 20-25% Onset 56-58 years Duration 8-11 years No known genetic factor but 45% have first degree relatives with the disorder
43
What are the symptoms of picks disease ?
Personality changes - inappropriate social behaviours, impulsive, neglect of hygiene Emotional changes- apathy, depression, mania, anger and irritability Speech- no fluency, echolalia, dysarthia, palilalia Fm develop voracious appetites
44
What are the pathologies of picks disease ?
Neuronal cell loss causing astrogliosis | Swollen ballooned neurons - picks cells
45
What is the cause of picks disease ?
Unknown - decrease in serotonin receptors and serotonin Decrease in somatostatin in brain and CSF
46
Describe Alzheimer's disease
``` Most common form of dementia Sporadic - 90% Familial - 10% Onset is usually > 60 Death within 5-10 years - due to infection, inanition or haemorrhagic stroke ```
47
What are the pathologies of ad ?
Atrophy of cortex- gyri shrink and sulci eidden and lose grey matter Plaques and neurofibrillary tangles - start in enorhinal cortex-hippocampus-parahippocampus-cingulate gyrus- cortex
48
What are the plaques in ad ?
``` Proteinacious structure Made up of amyloid beta protein Surround by dystrophic neurites- neuronal processes that show kinking and strange structure around plaques Extracellular Reactive gliosis around them ```
49
What are neurofibrillary tangles in ad ?
Flame like | Intracellular inclusions of hyperphosphorylated tau protein
50
What neurons are lost in ad ?
Cholinergic neurons | Lose nAChR and ChAT
51
What are the earliest symptoms of ad ?
``` Short term memory loss Disorientation- time then place Visuospatial orientation Apraxia Aphasia, anomia acalculia ```
52
What are the later symptoms of ad ?
Loss of social skills Psychosis and paranoia/hallucinations/delusions Bradykinesia/rigidity
53
What are the latest symptoms of ad ?
Mutism Bedridden Incontinence
54
How is ad assessed ?
Mini mental state examination
55
What is mild cognitive impairment ?
Can precede ad Complaint of memory loss Have intact activities of daily living
56
What is mild ad ?
``` Forgetfulness Short term memory loss Impaired daily actives Loss of hobbies and interests Repetitive questions ```
57
What is moderate ad ?
Progression do cognitive deficits Dysexecutive syndrome Further impaired daily activities Emergence of behavioural and psychological symptoms
58
What is severe ad ?
Agitation Altered sleep pattern Assistance required for feeding, dressing and bathing Established behavioural and psychological symptoms
59
What is very severe ad ?
Bed bound No speech Incontinent Basic psychomotor skills lost
60
What are the causes of ad ?
Genetic -trisomy 21- beta amyloid protein resides on this - familial - ApoE4 Amyloid - forms plaques Nd it's thought the build up of these causes death of neurons Trauma - risk with head injury or with hypoxia/anoxic episodes
61
What pathway forms amyloid beta proteins ?
The beta pathway not the alpha
62
What is the amyloid hypothesis ?
States that amyloid beta protein is overproduced in ad and will give rise to aggregates of the protein binding to itself and forming plaques which give rise to the excitotoxcity that causes the symptoms of ad

Neurobiology (30 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions