Week 9 Neurodegenerative

Question 1

What is Multiple Sclerosis

Answer 1

Multiple sclerosis (MS) is a condition of the central nervous system, interfering with nerve impulses within the brain, spinal cord and optic nerves.

Question 2

Which of the following is not a Neurodegenerative Disorder?

A. Parkinson’s Disease
B. Multiple Sclerosis
C. Huntington Disease
D. Addison’s disease

Answer 2

D. Addison’s disease

AKA - Primary adrenal insufficiency and hypocortisolism, is a long-term endocrine disorder in which the adrenal glands do not produce enough steroid hormones. Symptoms generally come on slowly and may include abdominal pain, weakness, and weight loss.

Question 3

Multiple Sclerosis affects the central nervous system (CNS). Which part of the CNS is affected?

A. Nerve cells (neurons)
B. Myelin
C. Plaques
D. Vertebrae

Answer 3

B. Myelin

Myelin is the fatty covering that insulates the nerve cell fibres in the brain and spinal cord. MS destroys myelin. Myelin is important because it keeps messages flowing between the brain, spinal cord, and the rest of the body. When MS destroys the myelin, these messages may be slowed or even completely blocked. “Sclerosis” comes from the Greek word for “scarring” or “hardening” of the patchy areas of the CNS where the myelin has been destroyed.

Question 4

Which age group is MS most likely to strike?

A. Children under 12
B. Teens
C. Adults ages 20 to 50
D. Adults ages 65 and older

Answer 4

C. Adults ages 20 to 50

Woman are diagnosed at a rate of 2:1 against men. Whites are more likely to develop MS.
More prevalent in temperate climate vs tropics - ?lack of Vitamin D

Question 5

Although the exact cause of MS is not yet known, which factor may play a role?

A. Environment
B. Viruses
C. Family history
D. All of the above

Answer 5

D. All of the above

MS is considered an autoimmune disease.
Blood Brain Barrier is breached - immune cells enter CNS.
T Cells - Stimulate Pro-Inflammatory Cytokines
B Cells - release antibodies that attack myelin
Macrophages - phagocytose the myelin

Question 6

How does Multiple Sclerosis usually progress?

A. In a series of attacks and remissions
B. In a gradual decline of function with no remission
C. The disease does not progress
D. All of the above

Answer 6

D. All of the above.
There are 3 main subtypes
1. Relapsing-remitting (RR) MS
– unpredictable relapses following remission
2. Secondary Progressive
– relapses and remissions and then becomes progressive neurologic decline
3. Primary Progressive
– never have remission after initial MS symptoms

Question 7

What are some of the symptoms of MS?

A. Blurred or double vision
B. Muscle weakness or partial paralysis
C. Fatigue
D. All of the above

Answer 7

D. All of the above

• Eye problems include red-green color distortion and blindness in one eye (Rx w/steroids and other meds)
• Muscle weakness’ difficulty walking or standing.
• Other symptoms include numbness, prickling, speech problems, tremors, dizziness, loss of bowel or bladder control, difficulty concentrating, memory problems, and, occasionally, hearing loss.

Question 8

Which therapy is often recommended for treating MS?

A. Corticosteroids such as prednisone
B. The hormone ACTH
C. Antibiotics
D. A and B
E. None of the above

Answer 8

A. Corticosteroids such as prednisone

Others include

• Anticonvulsants
• Antidepressants
• Immunosuppressants - corticosteroids (as above)
• Immunomodulators - Copaxans

Question 9

Huntington disease (HD) is also known as:

A. Huntington syndrome
B. Huntington neuritis
C. Huntington chorea
D. Huntington reaction

Answer 9

C. Huntington chorea

“Chorea” comes from the Greek word for “dance” and refers to the uncontrolled movements that mark the HD patient.

Question 10

Huntington disease (HD) primarily affects which body system?

A. Lymphatic system
B. Neurological system
C. Cardiovascular system
D. Respiratory system

Answer 10

B. Neurological system.

HD causes certain brain cells called neurons to die, eventually affecting the way a person feels, thinks, and moves. The death of the neurons causes jerky movements (chorea) and dementia.

Question 11

What causes brain cell damage in Huntington Disease (HD) patients?

A. A lack of oxygen
B. Poor nerve development
C. An abnormal protein
D. None of the above

Answer 11

C. An abnormal protein.

A genetic defect causes the production of an abnormal version of a protein called huntingtin which leads to

• -> sever neurodegeneration of Basal Ganglia, frontal cerebral cortex
• -> depletion of GABA (an inhibitory neurotransmitter)

Question 12

In which age group does Huntington Disease (HD) usually first appear?

A. Teens
B. 20s
C. 30s and 40s
D. 60 and older

Answer 12

C. 30s and 40s.

HD most often strikes in midlife, but it can also show up in children and in older adults.

Men and women have an equal risk of HD.
Affects every country in the world.

Question 13

Huntington Disease (HD) is an inherited illness, passed on from one generation to another. If a parent has HD, what’s the chance of a child developing HD?

A. 100%
B. 75%
C. 50%
D. 10%

Answer 13

C. 50%.

A single abnormal gene on chromosome number 4 causes HD. Chromosome number 4 is one of the 22 chromosomes that aren’t linked to gender, which means both men and women have equal chances of inheriting HD (called autosomal inheritance).

Question 14

Early symptoms of Huntington’s Disease include which of the following?

A. Mood swings
B. Depression
C. Irritability
D. All of the above

Answer 14

D. All of the above

Others SxSx from the lecture slide =

• Emotional, Cognitive and motor disturbances
• Hypertonia and hyperkinesia
• Loss of control of bodily functions
• Dementia
• Mood Imbalances

Question 15

How is HD diagnosed?

A. Through a genetic test
B. With a physical exam
C. With a family history
D. All of the above

Answer 15

D. All of the above.

A brain imaging test may also be ordered for diagnosis. For those at risk of developing HD, a blood test is also available to see whether the HD gene has been inherited. Not all people at risk choose to have this test.

Question 16

Parkinson disease is marked by a lack of which chemical in the brain?

A. Serotonin
B. GABA
C. Dopamine
D. Norepinephrine
E. None of the above

Answer 16

C. Dopamine.

Hallmark pathology of Parkinson’s Disease - Death of dopaminergic neurons in the midbrain/top of the brain stem - cells of the basal ganglia and the presence of Intraneuronal inclusions known as Lewy Bodies

Or

Death of Dopaminergic Neurons in the Substantia NIgra

Question 17

What is the average age when Parkinson disease first appears?

A. 25
B. 50
C. 60
D. 75

Answer 17

C. 60 (50 - 75 is the full range from the lecture slides)

20% of cases are people between the age of 30 - 50

Question 18

What is often the first symptom of Parkinson disease?

A. Headache
B. Nausea
C. Shaking of a hand or foot
D. Turning of the head

Answer 18

C. Shaking of a hand or foot.

About 7 in 10 people with Parkinson first have a slight tremor in a hand or foot. It usually appears on one side of the body. Later it may spread to the other side of the body, but to a lesser degree.

Question 19

Which of these statements best describes the brain abnormality characteristic of Parkinson’s disease?

A. Neurons in the substantia nigra begin to degenerate
B. CNS neurons become demyelinated
C. Amyloid plaques are deposited in the brain
D. There is a progressive breakdown of the blood-brain barrier

Answer 19

A. Neurons in the substantia nigra begin to degenerate

The basal ganglia, the red nucleus, the substantia nigra, the reticular formation and the cerebellum are all part of the extrapyramidal system, which controls gross autonomic motor movement

Question 20

True or False?

Parkinson Disease has a comparative excess of Ach as Dopamine production is progressively reduced

Answer 20

True

The twitching actions and jerky movement are because of too much Ach activity in skeletal muscles

Ach binds to its receptors in skeletal muscle (opens Na channels….initiates events for muscle contraction

Question 21

In your own words, explain the difference between Primary and Secondary Parkinsonism.

Answer 21

Primary Parkinsonism = Loss of pigmented neurons (die or impaired) in the substantia nigra leading to gliosis (scaring due to damaged cells)

Secondary Parkinsonism = Caused by other disorders e.g. Alzheimer’s Disease, Lewy Body Disease, Multiple Cerebral infarcts, AIDS Dementia Complex

Parkinson’s disease is a neurodegenerative brain disorder that progresses slowly in most people. … Parkinsonism is a general term that refers to a group of neurological disorders that cause movement problems similar to those seen in Parkinson’s disease such as tremors, slow movement and stiffness.

Question 22

Which is a risk factor for Parkinson’s Disease?

A. Rural/Agricultural Occupation
B. Exposure to heavy metals
C. Family Hx
D. All the above

Answer 22

D. All the above

Greater frequency in rural areas - exposure to pesticides and heavy metals
Inherited genetics
Exposure to certain toxins

Question 23

Bradykinesia, Hypokinesia and Akinesia are clinical features of which disease?

A. Huntington’s Disease
B. Multiple Sclerosis
C. Parkinsons Disease
D. None of the above

Answer 23

C. Parkinsons Disease

Brady = slowness and difficulty maintaining movement
Hypo = partial or complete loss of muscle movement
Akinesia = difficulty initiating movements/prolonged reaction times

Question 24

True or False

A late stage of Parkinson’s disease is the overall weakening of the muscles

Answer 24

False

Parkinson’s Disease is not a disease of the motor cortex or corticospinal

Therefore, muscle weakness is used to help differentially diagnose the disease.

Question 25

What are the 4 movement symptoms of Parkinson’s Disease?

HINT: PPRR

Answer 25

Resting Tremor
Rigidity
Problems Initiating Movement
Postural Instability

Question 26

Why is Levodopa considered the gold standard for pharmaceutical Rx of Parkinson’s Disease?

A. Contains synthetic Dopamine
B. An amino acid that crosses the Blood-Brain Barrier and is converted to Dopamine
C. Is an Anticholinergic Drug lowering ACh levels
D. Dopamine Agonist

Answer 26

B. An amino acid that crosses the Blood-Brain Barrier and is converted to Dopamine

Levodopa is combined with a decarboxylase inhibitor, carbidopa, to prevent conversion in the peripheral bloodstream

Question 27

Amyotrophic Lateral Sclerosis is also known as?

A. Motor Neuron Hypertonia
B. Autonomic Neuron Infection
C. Huntington’s Disease
D. Motor Neuron Disease

Answer 27

D. Motor Neuron Disease

Amyotrophic Lateral Sclerosis (ALS) is a disease of the nerve cells (neurons) in the brain and spinal cord that controls voluntary muscle movement. This progressive disorder is also known as Lou Gehrig’s Disease.

Question 28

At what age does ALS generally occur?

A. 20 - 30
B. 30 - 40
C. 40 - 60
D. 60 +

Answer 28

C. 40 - 60

Men at greater risk
Death usually occurs at 3 - 5 years post dianosis

Question 29

True or False

Humans can only be diagnosed with ALS if they have Family Hx.

Answer 29

False

Two Types

Familial and Sporadic

Sporadic accounts for 90 to 95% of cases and generally have no known cause

Question 30

Which is not true regarding Amyotrophic Lateral Sclerosis and Multiple Sclerosis?

A. ALS is more common in men than women and vice versa for MS
B. MS cannot be inherited but ALS can be inherited
C. Unlike ALS, MS can affect your motor control and autonomic dysfunction such as bladder control
D. All the above are true

Answer 30

D. All the above are true

Question 31

Multiple sclerosis is a demyelinating disease affecting the Central Nervous System.

What type of cell would be the most likely target of this disease? Why?

Answer 31

Myelin sheaths are surrounding the axons of the neurons in the PNS = (produced by Schwann cells) and in the CNS = (produced by Oligodendrocytes).