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Neurobiology > Neurodegenerative Disorders > Flashcards

Neurodegenerative Disorders Flashcards

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
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1
Q

What are the main indications of neurodegenerative disorders ?

A

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

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2
Q

What is the most common neurodegenerative disorder ?

A

Alzheimer’s

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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

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4
Q

Why are the pathological markers of AD?

A

Cortical extracellular senile plaques and neurofibrillary tangles

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5
Q

What are the pathological markers of PD?

A

Lewy bodies- intracellular

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6
Q

What are the pathological markers of PSP?

A

Subcortical neurofibrillary tangles

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7
Q

What are the pathological markers of CBGD?

A

Cortical and subcortical achromatic inclusion bodies

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8
Q

What are the pathological markers of Huntingtons disease ?

A

Selective loss or neurons and gliosis

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9
Q

What are the pathological markers of picks disease ?

A

Selective temporal lobe atrophy and picks bodies

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10
Q

What was PD first described as ?

A

Shaking palsy in 1817

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11
Q

What does PD cause ?

A

A kinetic rigid syndrome

  • loss of movement
  • increased muscle tone
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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

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13
Q

What are the main symptoms of Parkinson disease ?

A 
Tremor
Rigidity 
Speech difficulty 
Postural changes 
Akinesia
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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

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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

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16
Q

What are the causes of Parkinson’s disease ?

A

Unknown - idiopathic

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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

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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

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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
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20
Q

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

A

Irritability
Moodiness
Antisocial behaviour- aggressive, angry and unpleasant

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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

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22
Q

Where are neurons lost in huntingtons disease?

A

Cerebral cortex

Corpus striatum

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23
Q

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

A

Medium sized spiny neurons containing GABA and enkephalins

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24
Q

What happens to neurotransmitters in huntingtons disease ?

A

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

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25
Q

What happens to the pathway in huntingtons disease ?

A

Lose some of the negative feedback control on dopamine causing increased dopamine output

26
Q

What are the causes of huntingtons disease?

A

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
Q

Describe progressive supranuclear palsy

A 
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
Q

What are the main symptoms of PSP?

A

Progressive dementia causing exaggerated emotional response
Motor disorders
- opthalmoplegia, pseudobulbar palsy and balance/posture impaired
Sometimes causes limb rigidity, bradykinesia and hyperreflexia

29
Q

What is opthalmoplegia ?

A

Loss of eye movements

First the vertical gaze and then horizontal

30
Q

What balance/postural impairments occur in PSP?

A

Unexplained falls

Neck extended and rigid to flexion so it’s difficult to move

31
Q

What are he pseudobulbar palsy symptoms of PSP?

A

Facial weakness
Dysarthria
Dysphagia
Exaggerated gag reflex

32
Q

What are the pathologies of PSP?

A

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
Q

What are neurofibrillary tangles ?

A

Aggregates of tau protein in micro tubules

34
Q

Describe cortical basal ganglionic degeneration

A

Rare
Non inherited
Onset is Middle age/elderly

35
Q

What are the symptoms of CBGD??

A 
Bradykinesia/rigidity 
Clumsiness 
Speech- apraxia, asphasia and dysarthria 
Myoclonus 
Postural instability
36
Q

What is dysarthia?

A

Inability to vocalise

37
Q

What are the pathologies of CBGD ?

A

Atrophy of frontal and parietal cortex

Cell loss and gliosis in cortex, thalamus, red nucleus, lentiform nucleus, substantia nigra and locus coerulus

38
Q

What are the different disorders in motor neurons disease ?

A

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
Q

Describe ALS

A 
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
Q

What are the symptoms on motor neurons disease ?

A

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
Q

What is ALS-PDC?

A

Occured in chaumoro people of Guam

They ate flying foxes which act cycads that contain toxic amino acid beta-methylamino l-Alanine

42
Q

What is picks disease ?

A

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
Q

What are the symptoms of picks disease ?

A

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
Q

What are the pathologies of picks disease ?

A

Neuronal cell loss causing astrogliosis

Swollen ballooned neurons - picks cells

45
Q

What is the cause of picks disease ?

A

Unknown
- decrease in serotonin receptors and serotonin
Decrease in somatostatin in brain and CSF

46
Q

Describe Alzheimer’s disease

A 
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
Q

What are the pathologies of ad ?

A

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
Q

What are the plaques in ad ?

A 
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
Q

What are neurofibrillary tangles in ad ?

A

Flame like

Intracellular inclusions of hyperphosphorylated tau protein

50
Q

What neurons are lost in ad ?

A

Cholinergic neurons

Lose nAChR and ChAT

51
Q

What are the earliest symptoms of ad ?

A 
Short term memory loss 
Disorientation- time then place
Visuospatial orientation 
Apraxia 
Aphasia, anomia acalculia
52
Q

What are the later symptoms of ad ?

A

Loss of social skills
Psychosis and paranoia/hallucinations/delusions
Bradykinesia/rigidity

53
Q

What are the latest symptoms of ad ?

A

Mutism
Bedridden
Incontinence

54
Q

How is ad assessed ?

A

Mini mental state examination

55
Q

What is mild cognitive impairment ?

A

Can precede ad
Complaint of memory loss
Have intact activities of daily living

56
Q

What is mild ad ?

A 
Forgetfulness 
Short term memory loss 
Impaired daily actives 
Loss of hobbies and interests 
Repetitive questions
57
Q

What is moderate ad ?

A

Progression do cognitive deficits
Dysexecutive syndrome
Further impaired daily activities
Emergence of behavioural and psychological symptoms

58
Q

What is severe ad ?

A

Agitation
Altered sleep pattern
Assistance required for feeding, dressing and bathing
Established behavioural and psychological symptoms

59
Q

What is very severe ad ?

A

Bed bound
No speech
Incontinent
Basic psychomotor skills lost

60
Q

What are the causes of ad ?

A

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
Q

What pathway forms amyloid beta proteins ?

A

The beta pathway not the alpha

62
Q

What is the amyloid hypothesis ?

A

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)

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