Developmental and Degenerative Disorders Flashcards

1
Q

Define

Compensation

A

local brain pathology where there is increased function in unaffected areas

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2
Q

Definition

the nature of how the disease progresses with regards to brain location

A

Spatial profile

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3
Q

Definition

disorders causes by the progressive loss of structure or function of neurons, including death of neurons

A

Neurodegenerative disorders

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4
Q

Define

Neurodevelopmental disorders

A

a group of Disorders which affect the development of the nervous system, leading to abnormal brain function which may affect emotion, learning ability, self-control and memory

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5
Q

What happens to the age of onset in Friedreich ataxia as the intron expansions gets longer?

A

The age of onset gets earlier

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6
Q

Define

Transneuronal degeneration

A

local brain pathology where there is degeneration of remote regions

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7
Q

Definition

a rare genetic disease that causes difficulty walking, a loss of sensation in the arms and legs, and impaired speech. It’s also known as spinocerebellar degeneration. The disease causes damage to parts of your brain and spinal cord and can also affect your heart

A

Friedreich’s ataxia

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8
Q

True or False:

There is no strong evidence for developmental effects on Huntington’s disease

A

True

However, the brain changes manifest up to 20 years before motor symptoms start

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9
Q

Prolonged neural reserve often lead to what pathology?

A

Diaschisis

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10
Q

How can we investigate pre-onset brain changes?

A
  • Large cohort studies
  • ‘High-risk’ cohorts
  • Autosomal dominant disorders
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11
Q

Definition

local brain pathology where there is intact tissue sufficient to support function

A

Neural reserve

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12
Q

What does this illustrate?

A

Compensation

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13
Q

Definition

local brain pathology where there is increased function in unaffected areas

A

Compensation

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14
Q

Definition

a group of Disorders which affect the development of the nervous system, leading to abnormal brain function which may affect emotion, learning ability, self-control and memory

A

Neurodevelopmental disorders

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15
Q

Individuals with less impaired motor abilities recruit greater __________ cerebral systems and rely less on _________ cerebral networks

A

Individuals with less impaired motor abilities recruit greater bottom-up cerebral systems and rely less on top-down cerebral networks

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16
Q

What are the two maladaptive spatial consequences?

A

Diaschisis

Transneuronal degeneration

17
Q

What does this illustrate?

A

Transneuronal degeneration

18
Q

Definition

the nature of how the disease progresses over time

A

Temporal profile

19
Q

What are the neurodegenerative abnormalities in Friedreich ataxia?

A

Progressive atophy of the dentate nuclei (and other cerebellar structures) is evident across the life of the illness

20
Q

Definition

local brain pathology where there is dysfunction in remote regions

A

Diaschisis

21
Q

What does this illustrate?

A

Diaschisis

22
Q

Define

Friedreich’s ataxia

A

a rare genetic disease that causes difficulty walking, a loss of sensation in the arms and legs, and impaired speech. It’s also known as spinocerebellar degeneration. The disease causes damage to parts of your brain and spinal cord and can also affect your heart

23
Q

What are the neurodevelopmental abnormalities in Friedreich ataxia?

A

Spinal cord cross-sectional area, and dorsal root ganglion (DRG) neruons never reach mature size, but don’t deteriorate over time

24
Q

What are some examples of degenerative disorders?

A
  • Alzheimer’s
  • Parkinson’s
  • Huntington’s
  • MND
  • Spinocerebellar ataxias
25
Q

Define

Neural reserve

A

local brain pathology where there is intact tissue sufficient to support function

26
Q

Define

Diaschisis

A

local brain pathology where there is dysfunction in remote regions

27
Q

How do developmental disorders change the inverted U?

A

Changes on the left side of the inverted U (“delayed development”

  • The brain never reaches “normal” maturity or follows an abnormal trajectory
28
Q

What happens to cerebrum activation in low severity and high severity Friedreich ataxia? What does this suggest?

A

Increased activation in low severity

Decreased activation in high severity

Suggests neural reserve present in low severity that progresses to diaschisis in high severity

29
Q

Describe the temporal trajectories for Autism, Alzheimer’s and schizophrenia

A

Autism: developmental

Alzheimer’s: degenerative

Schizophrenia: mixed

30
Q

Define

Neurodegenerative disorders

A

disorders causes by the progressive loss of structure or function of neurons, including death of neurons

31
Q

List some examples of developmental disorders:

A
  • Autism spectrum disorder
  • ADHD
  • Mental illness
    • Schizophrenia
    • Major depressive disorder
32
Q

How can neural reserve and compensation be enhanced?

A

Non-invasive neurostimulation

  • Transcranial magnetic stimulation (TMS)
  • Transcranial direct current stimulation (tDCS)

Cognitive training

Exercise and physical training

33
Q

What does this illustrate?

A

Neural reserve

34
Q

Define

Temporal profile

A

the nature of how the disease progresses over time

35
Q

Definition

local brain pathology where there is degeneration of remote regions

A

Transneuronal degeneration

36
Q

What are the two adaptive spatial consequences?

A

Compensation

Neuronal reserve

37
Q

How do degenerative disorders change the inverted U?

A

Changes on the right side of the inverted U (“accelerated aging”)

  • The brain was once fully mature, but goes through pathological decline
38
Q

Define

Spatial profile

A

the nature of how the disease progresses with regards to brain location

39
Q

Friedreich ataxis is characterised by extensive damage in which regions?

A

Dorsal columns of the spinal cord and cerebellar dentate nucleus