HLTH 2501: chronic degenerative disorders

Question 1

multiple sclerosis

Answer 1

involves a progressive demyelination of the neurons of the brain, spinal cord, and cranial nerves; there are multiple types

Question 2

what is the result of multiple sclerosis

Answer 2

the loss of myelin interferes with conduction of impulses in the affected fibres (motor, sensory, or autonomic); this causes neural degeneration that varies in its severity

Question 3

how does multiple sclerosis develop?

Answer 3

an inflammatory response creates a lesion that results from cells that do not normally enter the brain, but attack neurons, causing a loss of myelin in the white matter of the brain; this develops into larger areas of inflammation and demyelination termed plaques

Question 4

what might be responsible for the development of multiple sclerosis?

Answer 4

a protein in the body’s blood-clotting mechanism that triggers the immune response; it is also believed to be an autoimmune disorder and linked to genetic and environment conditions

Question 5

where do plaques commonly develop in multiple sclerosis?

Answer 5

in the lateral ventricles, the brainstem and the optic nerves; these appear pinkish and swollen, but later become grey and firm

Question 6

who does multiple sclerosis affect more?

Answer 6

women between the ages of 20 and 40 and often those of European descent

Question 7

signs of multiple sclerosis

Answer 7

blurred vision is the common early sign, weakness in the legs, diplopia (double vision), scotoma (spot in the visual field), dysarthria (poor articulation), paresthesias, and as it progresses, loss of coordination of the bladder and bowel may occur

Question 8

complications arising from multiple sclerosis

Answer 8

those related to immobility like respiratory infection, decubitus ulcers, and contractures

Question 9

diagnosis for multiple sclerosis

Answer 9

no definitive test but includes a history of exacerbations and remissions, involvement of multiple focal areas, and absence of other disorders; MRI’s can be used, CSF analysis, and optical coherence tomography

Question 10

what are CSF serum levels like in those with multiple sclerosis?

Answer 10

elevated protein, gamma globulin, and lymphocytes

Question 11

treatment for multiple sclerosis

Answer 11

interferon beta-1b can reduce frequency of exacerbations, glucocorticoids, muscle relaxants, avoiding fatigue, stress, injury, or infection, PT, OT, and speech therapy

Question 12

parkinson disease other name

Answer 12

paralysis agitans

Question 13

parkinson’s disease

Answer 13

is a progressive degenerative disorder that affects motor function through loss of extrapyramidal activity due to changes in the basal nuclei and substantia nigra, leading to decreased dopamine release

Question 14

what does the lack of dopamine release in Parkinson’s disease cause?

Answer 14

leads to an imbalance between excitation and inhibition of the basal nuclei; this causes affected movement and posture by increasing muscle tone, restless tremors, muscle rigidity, difficulty in initiating movement, and instability

Question 15

what neurons are lacking in Parkinson’s disease?

Answer 15

cortical neurons

Question 16

toxins that may cause parkinson’s disease

Answer 16

manganese, carbon monoxide, carbon disulfide, and some pesticides

Question 16

primary parkinson’s disease

Answer 16

usually develops after age 60 and is associated with genes, toxins, mitochondrial damage, and lewy bodies

Question 17

mitochondrial damage in Parkinson’s patients

Answer 17

changes in the mitochondria due to oxidative stress lead to the accumulation of free radicals within the cell

Question 18

lewy bodies and parkinson’s disease

Answer 18

these are clumps of specific substances in the affected brain cells that contain a protein called alpha-synuclein

Question 19

secondary parkinsonism

Answer 19

may follow encephalitis, trauma, or vascular disease

Question 20

drug-induced parkinson’s disease

Answer 20

is linked to the use of phenothiazines (antipsychotic’s)

Question 21

signs of parkinson’s disease

Answer 21

fatigue, muscle weakness, muscle aching, decreasing flexibility, less spontaneous change in facial expression, hand tremors that progress to feet, face, tongue, and lip tremors, muscle rigidity, difficulty initiating movement, lack of associated involuntary movements, propulsive gait, bradykinesia, low voice, drolling, reduced linking, and increased risk for falls

Question 22

bradykinesia

Answer 22

slow movements

Question 23

propulsive gait

Answer 23

short, shuffling steps with increasing acceleration

Question 24

treatment for parkinson's disease

Answer 24

dopamine replacement (Levodopa), monoamine oxidase B inhibitors, anticholinergic drugs, catechol-O-methyltransferase inhibitors, antidepressant drugs, speech therapy, PT, and OT

Question 25

levodopa

Answer 25

is a precursor of dopamine and used for parkinson's treatment

Question 26

monoamine oxidase B inhibitors

Answer 26

selegiline and rasagiline; these block the breakdown of L-dopa in the brain

Question 27

anticholinergic drugs

Answer 27

include benztropine

Question 28

catechol-O-methyltransferase inhibitors

Answer 28

include entacapone that mildly prolong the effect of L-dopa therapy by blocking an enzyme that breaks down dopamine

Question 29

ALS

Answer 29

amyotrophic lateral sclerosis

Question 30

2 other names for ALS

Answer 30

Lou Gehrig disease and motor neuron disease

Question 31

amyotrophic meaning

Answer 31

muscle wasting

Question 32

sclerosis means

Answer 32

hardening; for ALS it refers to the degenerative hardening of the lateral corticospinal tracts

Question 33

ALS

Answer 33

is a progressive degenerative disease affecting both UMNs in the cerebral cortex and LMNs in the brainstem and spinal cord; sensory neurons are not affected; this is a fatal disease

Question 34

how does ALS develop?

Answer 34

supportive glial cells called astrocytes secrete a neurotoxin, leading to the death of motor neurons

Question 35

what does damage to the UMNs result in?

Answer 35

spastic paralysis and hyperreflexia

Question 36

what does damage to the LMNs result in?

Answer 36

flaccid paralysis, with decreased muscle tone and reflexes

Question 37

what does the progressive muscle weakness in ALS lead to?

Answer 37

affected respiratory function

Question 38

signs of ALS

Answer 38

the upper extremities, particular the hands manifest weakness and muscle atrophy, with a loss of fine motor coordination; stumbling and falls are common, along with muscle cramping and twitching, speech loss, impaired respiration, and swallowing difficulties

Question 39

treatment for ALS

Answer 39

to treatment to reverse the course; Rulutrek slows damage to neurons, allowing for swallowing and ventilation to remain; exercise and rest is also helpful, along with respiratory therapist, nutritionist, speech pathologist, OT, PT, psychologist, and social workers

Question 40

myasthenia gravis

Answer 40

is an autoimmune disorder that impairs the receptors for ACh at the neuromuscular junction; cause is not known but is related to thymus disorders like hyperplasia or benign tumors

Question 41

what happens in mysasthenia gravis

Answer 41

immunoglobulin G autoantibodies to ACh receptors form, blocking and ultimately destroying the receptor site, thus preventing any further stimulation of the the muscle; this leads to skeletal muscle weakness and rapid fatigue of the affected muscles

Question 42

what muscles are often affected in myasthenia gravis

Answer 42

the facial and ocular muscles, followed by the arm and trunk muscles

Question 43

tests for myasthenia gravis

Answer 43

EMG to test muscle fatigue, looking at serum antibodies, and a test that uses edrophonium chloride (Tensilon) which is a shorter-acting anticholinesterase inhibitor to prolong the action of ACh at the myoneural junction

Question 44

signs of myasthenia gravis

Answer 44

noticeable muscle weakness, fatigue, diplopia and ptosis impairs vision, speech becomes monotone, spontaneous facial expressions are lost (droopy face), difficulty chewing and swallowing, head droops, upper respiratory infections are common and myasthenic crisis

Question 45

myasthenic crisis

Answer 45

occurs when there is added stress such as an infection, trauma, or alcohol intake and involves an increase in weakness and fatigue, and respiratory impairment may develop

Question 46

treatment for myasthenia gravis

Answer 46

anticholinesterase agents, glucocorticoids (prednisone) for suppressing the immune system, other immunosuppressants, plasmapheresis, and thymectomy

Question 47

anticholinesterase agents for myasthenia gravis

Answer 47

can be neostigmine and this temporarily improves neuromuscular transmission through prolonging the action of Ach

Question 48

plasmapheresis

Answer 48

is a process that removes antibodies from the blood and can be treatment for myasthenia gravis

Question 49

huntington disease

Answer 49

is an inherited disorder that does not manifest until midlife and is characterized by progressive atrophy of the brain, including degeneration of neurons, particular of the basal ganglia of the frontal cortex, and dilation of the ventricles

Question 50

genetic factors for huntington's disease

Answer 50

is a genetic disorder via an autosomal-dominant trait carried on chromosome 4 (50% probability) and maternal inheritance delays the onset compose to paretrnal

Question 51

what neurotransmitters are reduced in huntington's disease

Answer 51

GABA (inhibitory neurotransmitter) and Ach

Question 52

signs of huntington's disease

Answer 52

mood swings, personality changes, restlessness and choreiform (rapid, jerky) movements in the arms, and possibly early signs on intellectual impairment like loss of problem-solving skills, difficulty learning, and poor judgement

Question 53

how is huntington's disease diagnosed?

Answer 53

DNA analysis

Question 54

treatment for huntington's disease

Answer 54

no therapy but the drug tetrabenazine is used to maintain mobility and other drugs for behavioral changes, as well as PT

Question 55

dementia

Answer 55

is a progressive chronic disease in which cortical function is decreased, there are impaired cognitive skills, decreased motor coordination, loss of memory, and behavioural changes

Question 56

how is normal forgetfulness distinguished from dementia

Answer 56

those with dementia cannot recall after being given clues, the day cannot be identified, calculations are difficult, and reminders are needed for meals and hygiene

Question 57

causes of dementia

Answer 57

vascular disease (ex. atherosclerosis), infections, toxins, and genetic disorders

Question 58

most common dementia disorder

Answer 58

Alzheimer's disease

Question 59

alzheimer's disease

Answer 59

is characterized by a loss of intellectual function, and includes personality changes, lack of initiative, repetitive behaviours, judgement impairment, abstract-thinking, and problem-solving abilities

Question 60

how does Alzheimer's disease develop?

Answer 60

progressive cortical atrophy leads to dilated ventricles and widening of the sulci; neurofibrillary tangles and senile plaques are present, and these disrupt neural conduction; there is also a deficit of Ach

Question 61

what is contained in the plaques in those with Alzheimer's disease?

Answer 61

beta-amyloid precursor protein

Question 62

causes of Alzheimer's

Answer 62

unknown but is genetic, as at least four defective genes are located on different chromosomes (1, 14, and 21 are autosomal dominant traits and 21 in those with down's syndrome); there is another late-onset AD that is associated with chromosome 19

Question 63

early stage of Alzheimer's

Answer 63

gradual loss of memory and lack of concentration; the ability to learn new information and to reason is impaired, along with behavioural changes; this progresses and ADL become impaired, along with wandering

Question 64

late stage of Alzheimer's disease

Answer 64

the person does not recognize his or her family, lacks awareness or interest, it incontinent, cannot function and motor function degenerate

Question 65

treatment for Alzheimer's disease

Answer 65

OT, depression and anxiety medication, anticholinesterase drugs, and providing a safe space, with a daily routine and some light exercise

Question 66

4 types of dementia

Answer 66

Alzheimer's disease, vascular dementia, Creutzfeldt-Jakob disease, and acquired immunodeficiency syndrome dementia

Question 67

vascular dementia

Answer 67

is caused by cerebrovascular disease and frequently is a result of multiple small brain infarctions; common in those with hypertension

Question 68

Creutzfeldt-Jakob disease

Answer 68

is rare, but progresses rapidly; can be cause by infection by a prion, which is an altered, infectious form of the prion protein that has a preference for nervous tissue

Question 69

how does the prion protein cause Creutzfeldt-Jakob disease

Answer 69

it alters the shape of normal host PrPs which has a long incubation period and then is followed by rapid destruction of neurons and the formation of plaques and vacuoles in the neurons

Question 70

how it Creutzfeldt-Jakob disease acquired?

Answer 70

can be genetic (a defect in the human prion protein gene) or have be through surgeries, corneal transplants, or other invasive procedures

Question 71

how is Creutzfeldt-Jakob disease diagnosed?

Answer 71

MRI and EEG

Question 72

treatment for Creutzfeldt-Jakob disease

Answer 72

no treatment but amantadine, steroids, interferon, acyclovir, antiviral agents, and antibiotics may help to alleviate symptoms

Question 73

acquired immunodeficiency syndrome dementia

Answer 73

dementia may develop in later stages on acquired immunodeficiency syndrome and occurs when the virus itself invades brain tissue and may be exacerbated by other infectious agents like Candida, toxoplasma species, and tumors such as lymphoma