Developmental and Degenerative Disorders

Question 1

Define

Compensation

Answer 1

local brain pathology where there is increased function in unaffected areas

Question 2

Definition

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

Answer 2

Spatial profile

Question 3

Definition

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

Answer 3

Neurodegenerative disorders

Question 4

Define

Neurodevelopmental disorders

Answer 4

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

Question 5

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

Answer 5

The age of onset gets earlier

Question 6

Define

Transneuronal degeneration

Answer 6

local brain pathology where there is degeneration of remote regions

Question 7

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

Answer 7

Friedreich’s ataxia

Question 8

True or False:

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

Answer 8

True

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

Question 9

Prolonged neural reserve often lead to what pathology?

Answer 9

Diaschisis

Question 10

How can we investigate pre-onset brain changes?

Answer 10

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

Question 11

Definition

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

Answer 11

Neural reserve

Question 12

What does this illustrate?

Answer 12

Compensation

Question 13

Definition

local brain pathology where there is increased function in unaffected areas

Answer 13

Compensation

Question 14

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

Answer 14

Neurodevelopmental disorders

Question 15

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

Answer 15

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

Question 16

What are the two maladaptive spatial consequences?

Answer 16

Diaschisis

Transneuronal degeneration

Question 17

What does this illustrate?

Answer 17

Transneuronal degeneration

Question 18

Definition

the nature of how the disease progresses over time

Answer 18

Temporal profile

Question 19

What are the neurodegenerative abnormalities in Friedreich ataxia?

Answer 19

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

Question 20

Definition

local brain pathology where there is dysfunction in remote regions

Answer 20

Diaschisis

Question 21

What does this illustrate?

Answer 21

Diaschisis

Question 22

Define

Friedreich’s ataxia

Answer 22

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

Question 23

What are the neurodevelopmental abnormalities in Friedreich ataxia?

Answer 23

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

Question 24

What are some examples of degenerative disorders?

Answer 24

• Alzheimer’s
• Parkinson’s
• Huntington’s
• MND
• Spinocerebellar ataxias

Question 25

# Define Neural reserve

Answer 25

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

Question 26

# Define Diaschisis

Answer 26

local brain pathology where there is dysfunction in remote regions

Question 27

How do developmental disorders change the inverted U?

Answer 27

Changes on the left side of the inverted U ("delayed development" * The brain never reaches "normal" maturity or follows an abnormal trajectory

Question 28

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

Answer 28

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

Question 29

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

Answer 29

Autism: developmental Alzheimer's: degenerative Schizophrenia: mixed

Question 30

# Define Neurodegenerative disorders

Answer 30

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

Question 31

List some examples of developmental disorders:

Answer 31

* Autism spectrum disorder * ADHD * Mental illness * Schizophrenia * Major depressive disorder

Question 32

How can neural reserve and compensation be enhanced?

Answer 32

Non-invasive neurostimulation * Transcranial magnetic stimulation (TMS) * Transcranial direct current stimulation (tDCS) Cognitive training Exercise and physical training

Question 33

What does this illustrate?

Answer 33

Neural reserve

Question 34

# Define Temporal profile

Answer 34

the nature of how the disease progresses over time

Question 35

# Definition local brain pathology where there is degeneration of remote regions

Answer 35

Transneuronal degeneration

Question 36

What are the two adaptive spatial consequences?

Answer 36

Compensation Neuronal reserve

Question 37

How do degenerative disorders change the inverted U?

Answer 37

Changes on the right side of the inverted U ("accelerated aging") * The brain was once fully mature, but goes through pathological decline

Question 38

# Define Spatial profile

Answer 38

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

Question 39

Friedreich ataxis is characterised by extensive damage in which regions?

Answer 39

Dorsal columns of the spinal cord and cerebellar dentate nucleus