Developmental and Degenerative Disorders Flashcards
Define
Compensation
local brain pathology where there is increased function in unaffected areas
Definition
the nature of how the disease progresses with regards to brain location
Spatial profile
Definition
disorders causes by the progressive loss of structure or function of neurons, including death of neurons
Neurodegenerative disorders
Define
Neurodevelopmental disorders
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
What happens to the age of onset in Friedreich ataxia as the intron expansions gets longer?
The age of onset gets earlier
Define
Transneuronal degeneration
local brain pathology where there is degeneration of remote regions
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
Friedreich’s ataxia
True or False:
There is no strong evidence for developmental effects on Huntington’s disease
True
However, the brain changes manifest up to 20 years before motor symptoms start
Prolonged neural reserve often lead to what pathology?
Diaschisis
How can we investigate pre-onset brain changes?
- Large cohort studies
- ‘High-risk’ cohorts
- Autosomal dominant disorders
Definition
local brain pathology where there is intact tissue sufficient to support function
Neural reserve
What does this illustrate?
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Compensation
Definition
local brain pathology where there is increased function in unaffected areas
Compensation
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
Neurodevelopmental disorders
Individuals with less impaired motor abilities recruit greater __________ cerebral systems and rely less on _________ cerebral networks
Individuals with less impaired motor abilities recruit greater bottom-up cerebral systems and rely less on top-down cerebral networks
What are the two maladaptive spatial consequences?
Diaschisis
Transneuronal degeneration
What does this illustrate?
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Transneuronal degeneration
Definition
the nature of how the disease progresses over time
Temporal profile
What are the neurodegenerative abnormalities in Friedreich ataxia?
Progressive atophy of the dentate nuclei (and other cerebellar structures) is evident across the life of the illness
Definition
local brain pathology where there is dysfunction in remote regions
Diaschisis
What does this illustrate?
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Diaschisis
Define
Friedreich’s ataxia
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
What are the neurodevelopmental abnormalities in Friedreich ataxia?
Spinal cord cross-sectional area, and dorsal root ganglion (DRG) neruons never reach mature size, but don’t deteriorate over time
What are some examples of degenerative disorders?
- Alzheimer’s
- Parkinson’s
- Huntington’s
- MND
- Spinocerebellar ataxias
Define
Neural reserve
local brain pathology where there is intact tissue sufficient to support function
Define
Diaschisis
local brain pathology where there is dysfunction in remote regions
How do developmental disorders change the inverted U?
Changes on the left side of the inverted U (“delayed development”
- The brain never reaches “normal” maturity or follows an abnormal trajectory
What happens to cerebrum activation in low severity and high severity Friedreich ataxia? What does this suggest?
Increased activation in low severity
Decreased activation in high severity
Suggests neural reserve present in low severity that progresses to diaschisis in high severity
Describe the temporal trajectories for Autism, Alzheimer’s and schizophrenia
Autism: developmental
Alzheimer’s: degenerative
Schizophrenia: mixed
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Define
Neurodegenerative disorders
disorders causes by the progressive loss of structure or function of neurons, including death of neurons
List some examples of developmental disorders:
- Autism spectrum disorder
- ADHD
- Mental illness
- Schizophrenia
- Major depressive disorder
How can neural reserve and compensation be enhanced?
Non-invasive neurostimulation
- Transcranial magnetic stimulation (TMS)
- Transcranial direct current stimulation (tDCS)
Cognitive training
Exercise and physical training
What does this illustrate?
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Neural reserve
Define
Temporal profile
the nature of how the disease progresses over time
Definition
local brain pathology where there is degeneration of remote regions
Transneuronal degeneration
What are the two adaptive spatial consequences?
Compensation
Neuronal reserve
How do degenerative disorders change the inverted U?
Changes on the right side of the inverted U (“accelerated aging”)
- The brain was once fully mature, but goes through pathological decline
Define
Spatial profile
the nature of how the disease progresses with regards to brain location
Friedreich ataxis is characterised by extensive damage in which regions?
Dorsal columns of the spinal cord and cerebellar dentate nucleus