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Fundamentals of neuroscience > Neurodegenerative disorders (diseases of NS) > Flashcards

Neurodegenerative disorders (diseases of NS) Flashcards

1
Q

What is Huntington’s disease?

A

A hereditary movement disorder typically characterised by choeric (involuntary) movements

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2
Q

What is Huntington’s disease a result of?

A

The death of the basal ganglia neurones

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3
Q

Who first described Huntington’s disease?

A

George Huntington in 1872 in ‘Essay in Choera’

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4
Q

When is the typical onset of Huntington’s disease?

A

30-50 years old

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5
Q

What is the prognosis for Huntington’s disease?

A

15-20 years from diagnosis to death

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6
Q

What is the incidence of Huntington’s disease?

A

Rare = 1 in 10,000

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7
Q

What is the Huntingtin gene?

A
  • Autosomal dominant gene mutation

50% chance of passing it on

  • CAG repeat in the Huntingtin gene
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8
Q

What lenght of CAG repeat can lead to Huntington’s disease?

A

7-34 = Normal

35-39 = Unlikely but could be more likely to pass it onto children than normal

> 40 = HD

> 70 = Juveline HD

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9
Q

What is the roles of the Huntingtin protein?

A
  • Intracellular transport
  • Intracellular signalling
  • Matabolism
  • Neurogenesis
  • Synaptic activity
  • Transcription regulation
  • Anti-apoptotic
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10
Q

What are the motor deficit symtoms in the early stage of Huntington’s disease?

A
  • Abnormal eye movements
  • Inappropriate hand and toes movements
  • General restlessness
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11
Q

What are the motor deficit symtoms in the mid stage of Huntington’s disease?

A

Onset of involutary movements (chorea)

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12
Q

What are the motor deficit symtoms in the impaired voluntary movements stage of Huntington’s disease?

A
  • Rigidity
  • Bradykinesia
  • Dystonia
  • Convulsions
  • Weight loss
  • Leading to death from pneumonia, choking, nutritional deficits
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13
Q

Which areas of cognition are affected wiht Huntington’s disease?

A
  • Executive function
  • Learning new skills
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14
Q

Which areas of cognition are not affected with Huntington’s disease?

A

Declarative memory

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15
Q

What is the gross pathology of Huntington’s disease?

A
  • 10-20% reduction in brain volume
  • Dec striatal volume
  • Inc ventricle size
  • Dec cortical volume
  • Protein inclusions throughout the brain
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16
Q

What is the overall cellular pathology of Huntington’s disease

A
  • Cell death
  • Primarily loss of GABAergic neurones in the striatum
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17
Q

What is the inital cellular pathology of Huntington’s disease?

A
  • Losss of GABAergic neurones which express D2 recpetors & project to globus pallidus external
  • Damage to indirect pathway
  • Excessive involuntary movements
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18
Q

What is the later stage cellular pathology of Huntington’s disease?

A
  • Loss of GABAergic neurones which express D1 receptors & project to globus pallid us internal
  • Damage to direct pathways
  • Paucity of movements
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19
Q

What are the treatments for Huntington’s disease?

A
  • Currently no cure
  • Treatment limited to symptom relief
  • Supportive care is essential
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20
Q

What are the types of supportive care provided as a treatment for Huntington’s?

A
  • Psychotherapy
  • Physiotherapy
  • Speech and swallowing therapy
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21
Q

What is Parkinson’s diesease?

A

Movement disorder typically characterised by tremors and bradykinesia

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22
Q

What is the cause of Parkinson’s disease?

A

As a result of death of the substantial nigra neurones

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23
Q

When is the typical onset of Parkinson’s disease?

A

60s - disease of ageing

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24
Q

What is the incidence of Parkinson’s disease?

A

1 in 500 in the UK

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25
Q

What are the 3 main causes of Parkinson’s disease?

A
  • Idiopathic
  • Familial
  • Parkinsonism
26
Q

How do idiopathic reasons cause Parkinson’s disease?

A
  • Majority of cases
  • Unknown cause
27
Q

What are the familial causes of Parkinson’s disease?

A

Multuple mutations can cause Parkinson’s disease

28
Q

How does parkinsonism cause Parkinson’s disease?

A
  • Maybe caused by DISEASES e.g. encephalitis and syphilis
  • Maybe caused by DRUGS e.g. MPTP
  • Maybe caused by HERBICIDES e.g. cypberquat
29
Q

What are the primary symptoms of Parkinson’s disease?

A
  • Tremor
  • Bradykinesia (slowness of movement)
  • Rigidity
  • Postural instability

Must display 3 or 4 of these to be diagnosed

30
Q

What are the other motor symptoms of Parkinson’s disease?

A
  • Altered posture
  • Shuffling gait
31
Q

What are the cognitive and psychological symptoms of Parkinson’s disease?

A

Vary from person to person

32
Q

What is the gross pathology of Parkinson’s disease?

A
  • Degeneration of the substantia nigra
  • Loss of dopaminergic neurones in the substantia seen as a loss of dark melanin staining of the substantia nigra
33
Q

What is the cellular pathology of Parkinson’s disease?

A
  • Death of dopaminergic neurones in the substantia nigra that project to the striatum (putamena nd caudate nucleus) of the basal ganglia
  • Normally these neurones promote the direct pathway
  • Their death results in reduced activation of the direct pathway & therefore reduced voluntary movement
34
Q

What are the 3 main treatments for Parkinson’s disease?

A
  • Increase levels of dopamine
  • Physiotherapy
  • Deep brain stimulation (DBS) or lesioning of the internal globus pallidus
35
Q

How is increasing levels of dopamine used to treat Parkinson’s disease?

A

Dopamine precursons e.g. L-dopa

36
Q

How is physiotherapy used to treat Parkinson’s disease?

A

To help with rigidity and stiffness

37
Q

How is Deep brain stimulation (DBS) or lesioning of the internal globus pallidus used to treat Parkinson’s disease?

A

To help counteract the imbalance in the basal ganglia circuitry

38
Q

How is Alzheimer’s disease characterised?

A

By a progressive deterioration in cognitive function

Occurs without an antecedent cause

39
Q

What is the most prevalent form of dementia?

A

Alzheimer’s disease

~65% of all dementia cases

40
Q

Who first described Alzheimer’s disease?

A

Alois Alzheimer in 1906

41
Q

When does Alzheimer’s disease tend to onset?

A

Late onset ~95% of cases

Early onset (before 65) tends to be familial

42
Q

What is the incidence of Alzheimer’s disease?

A

1 in 14 aged 65+

43
Q

What is the prognosis of Alzheimer’s disease?

A

5-8 years from diagnosis

44
Q

What are the suggested cause of Alzheimer’s disease?

A
  • Genetic predisposition
  • ENvironemental toxins
  • Reduced blood flow to the hemispheres

Cause is unkown

45
Q

What is the main symptom of Alzheimer’s disease?

A

Memory dysfunction:

Declarative memory which requires the temporal lobe is most commonly affected

46
Q

To be diagnosed with Alzheimer’s disease what are the cognitive deficits that must be displayed? (on top of memory dysfunction)

A
  • Aphasia
  • Apraxia
  • Agnosia
  • Executive dysfunction
47
Q

What is Aphaisa?

A

Inability to produce or understand language

48
Q

What is Apraxia?

A

Poor motor planning

49
Q

What is Agnosia?

A

Inability to recognise things
(Can have a number of forms: ibjects, faces, sounds or smells

50
Q

What is the gross pathology of AZ?

A
  • Degeneration of cells in the hippocampus
  • Cortical degeneration
  • Enlarged ventricles
51
Q

What is the degeneration of cells in the hippocampus in Alzheimer’s disease?

(Gross pathology)

A
  • Earliest and most severe changes
  • Memory dysfunction
52
Q

What is cortical degeneration in Alzheimer’s disease?

(Gross pathology)

A
  • Most affected areas are in the limbic cortex, inferior temoral cortex & posterior parietal ccortex
  • Decline in language & judgement
  • Emotional outbursts
53
Q

What areas of the brain change with Alzheimer’s disease?

(Gross pathology)

A
  • Severely enlarged ventricles

Extreme shrinkage of:
- Cerebral cortex
- Hippocampus
- Entorhinal cortex

54
Q

What are the two pain changes that occur in cellular pathology with Alzheimer’s disease?

A
  • Amyloid plaques
  • Neurofibrillary tangles
  • Cortical degeneration
55
Q

What are amyloid plaques in Alzheimer’s disease like?

(Cellular pathology)

A
  • Main pathological marker
  • EXTRACELLULAR deposits
  • Located mostly in teh CEREBRAL CORTEX
  • Associated w neuronal cell death
56
Q

What are neurofibrillary tangles?

(Cellular pathology)

A

Disorganised bundles of filaments in the neuronal cytoplasm formed by hyperphosphorylated tau proteins

57
Q

What are Amyloid plaques?

A

Large neurotoxic aggregates of insoluble amyloid protein

58
Q

What are neurofibrillary tangles like in Alzheimer’s disease?

(Cellular pathology)

A
  • INTRACELLULAR
  • Located in the CEREBRAL CORTEX & HIPPOCAMPUS
  • Inc as the disease progresses
  • Associated w neuronal cell death
59
Q

What is cortical degeneration like in Alzheimer’s disease?

(Cellular pathology)

A
  • Cerebral atrophy (dec in size) may be largely due to the loss of dendritic arborisation (branching at the end of a nerve fibre)
  • Reduction in synapses leads to loss of neurones and atrophy
60
Q

What NT loss happens in Alzheimer’s disease?

A
  • Cholinergic (ACh) pathways most affected
  • Predominant in the hippocampus and cerebral cortex
61
Q

What is the treatment for Alzheimer’s disease?

A

Stimulate cholinergic function

62
Q

How is stimulating cholinergic function used to treat Alzheimer’s disease?

A
  • Acetylcholinesterase inhibitors
  • In ACh at synapses
  • Slows progression of the disease
  • Only a TEMPORARY SOLUTION since neurones die

Fundamentals of neuroscience (38 decks)

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