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22 Cards in this Set
- Front
- Back
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Huntington’s Disease:
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genetic condition caused by mutation on chromosome 4 (the more repeated it is the worse the disease is)
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Basic features of Huntington's disease (3)
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* Onset around 40 years old after reproductive age, problematic b/c it is strongly genetic and will have already passed on 25% to children
* Progresses over 10-15 years * Far less common than PD |
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Clinical features of Huntington's disease (5 points)
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* Behavioral changes: Depression, mood swings, anger
Abnormal movements: * Chorea: rhythmic movements of head, arms, other parts of body, cannot control these. If person tries to control them they get worse * Athetosis: writhing of the hands, continuous, nonstop, cannot control it * Global cognitive impairment (dementia): cognitive impairment that impairs memory and other aspects of cognition * Basal ganglia is destroyed in Huntington’s, as well as other parts of the cortex. * Caused by parts of the basal ganglia dying, thalamus is no longer inhibited at all (or very little), no regulatory mechanism to control the thalamus, becomes over active and person moves in an uncontrolled way. |
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Huntington's disease treatment (4)
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* Medication to manage the chorea
* Excitotoxicity * Defect in the mitochondria of these neurons that is genetic and results in these neurons dying * Free radicals |
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Excitotoxicity
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overactive neuron depletes itself and dies
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free radicals
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naturally produced by body, in excess the are linked to neurological pathology and aging (degrade and destroy cells) -> in Huntington’s may kill neurons in the brain somewhat selectively
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Motor weakness and paralysis: injury involving the corticospinal tract may result in:
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Hemiparesis
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Hemiparesis
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half of the body becomes weak
Can use lokomat to help restrengthen parts of the body with less body weight (physical therapy) Often use other side of the body to compensate… But weak limb will get worse because it is not being used |
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Hemiplegia
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half of the body is paralyzed
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Amyotrophic Lateral Sclerosis (ALS)
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Motor neuron disease: kills upper and lower motor neurons
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ALS symptoms
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very debilitating, at first clumsiness, as it progresses lose the ability to walk, speak, eventually will require full support even to breath, paralysis, can cause death
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ALS features
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Does not affect cognition, it is a loss of control over the body
No cure, no way to slow the disease |
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Research on ALS
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Mutations in SOD1 gene
- usually resolves free radicals and gets rid of them, mutations impair the ability to resolve free radicals and results in them destroying motor neurons
Finding a reliable biological marker (currently diagnosis is through the process of elimination) |
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Cerebellar ataxia
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dramatically impairs the coordination of skilled movement
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Role of cerebellum in movement
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The cerebellum integrates sensory inputs describing body position and motor commands to modify motor outflow for smooth coordinated movements
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Damage in cerebellum effects
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Damage to the cerebellum: stroke, traumatic brain injury, tumors, agent orange -> impairment in smooth coordinated movement
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What is agent orange?
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used as a herbicide in war, sprayed on vegetation and humans… People exposed had children with birth defects, people developed strange neurological conditions decades down the line (could no longer walk or talk) -> cerebellum is getting destroyed
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What do people with cerebellum problems do?
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People w/ cerebellum problems spread legs when walking, lumber
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What test shows cerebellum problems?
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Test cerebellum problems using the Romberg test
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Myasthenia gravis basic features
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*autoimmune disorder, acetylcholine cannot bind and people have difficulty controlling their muscles
* Referred to as “Grave muscle weakness” |
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Myasthenia gravis symptoms
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Clinical symptoms: fatigability, cannot control muscles
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Myasthenia Future research
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Autoimmune response
Link with the thymus gland: thymus gland is bigger then would be expected, associated w/ an elevated immune response |
