Clinical Perspectives - Basal Ganglia Disorders Flashcards

1
Q

Parkinson’s disease is associated with the buildup of what protein in the brain?

A

Lewy Bodies - composed of alpha-synuclein protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Wilson’s disease presents with neurologic and psychiatric symptoms, which sometimes are mistaken for substance abuse or mental illness. What symptom is unique to Wilson’s disease and can help with diagnosis?

A

Kayser-Fleischer rings in the corneas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

In what manner is Huntington’s disease passed genetically?

A

Dominant trait due to a defective gene on chromosome 4 that is usually passed paternally (sperm are prone to more genetic mutations than eggs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

An attending is describing a patient they saw who has a movement disorder. The attending describes a “pill-rolling” tremor, “cogwheel” rigidity, and micrographia as symptoms. What disorder best matches with these symptoms?

A

Parkinson’s Disease (TRAPSS - Tremor, Rigidity, Akinesia, Postural Instability, Shuffling Gait, Small Handwriting)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How does the symptom presentation of males and females differ in Tourette’s disease?

A

Males are more likely to present with tics. Females are more likely to present with obsessive-compulsive symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

The striatum is made up of what two structures?

A

Caudate nucleus, putamen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is a common cause of Sydenham Chorea?

A

Autoimmune disorders due to rheumatic fever or streptococcus infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

True/False. The presence of any tic is indicative of Tourette’s disease.

A

False. A person may have a tic without having Tourette’s disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

A patient feels the need to repeatedly clear his throat. What type of tic is this?

A

Simple vocal tic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Mutations of the ATP7B gene are associated with what disorder?

A

Wilson’s Disease - copper accumulation due to decreased excretion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What movements are most associated with Sydenham Chorea?

A

Tongue darting and milkmaids sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How do the symptoms of Parkinson’s and Huntington’s diseases differ?

A

Parkinson’s involves the gradual loss of the ability to initiate movement. Huntington’s, on the other hand, is characterized by an inability to prevent the body from moving involuntarily.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the most commonly used drug for the treatment of Parkinson’s disease?

A

Carbidopa-levodopa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Neurons of the substantia nigra are lost due to the pathophysiology of what disease?

A

Parkinson’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

This disease most commonly affects young girls more than boys and presents after infection with rheumatic fever or streptococcus.

A

Sydenham Chorea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What medication is used to treat Huntington’s disease?

A

There is no drug to cure Huntington’s or stop its progression. Antidepressants, mood stabilizers, and medicines to reduce movement may be used to manage symptoms

17
Q

Sudden, repetitive, rapid, and unwanted movements or vocal sounds are associated with what basal ganglia disorder?

A

Tourette’s Disease

18
Q

What is the inhibitory neurotransmitter?

A

GABA

19
Q

What types of drugs are most commonly used to suppress tics associated with Tourette’s disease?

A

Medications that block dopamine - other drugs, such as alpha-adrenergic agonists, stimulants, and antidepressants are also used

20
Q

The loss of what neurotransmitters specifically cause Huntington’s chorea?

A

Loss of ACh and GABA

21
Q

The caudate and putamen atrophy due to Huntington’s disease. What other pathological findings are present in the brain?

A

Enlargement of the ventricles

22
Q

What gene is mutated in Huntington’s disease?

A

Huntingtin gene - CAG repeat expansion on chromosome 4

23
Q

Wilson’s disease is associated with the accumulation of what compound in the liver and nervous system?

A

Copper - due to decreased movement of copper into bile for excretion

24
Q

What is the excitatory neurotransmitter?

A

Glutamate

25
Q

What is the primary neurotransmitter functioning in the basal ganglia?

A

GABA - this is compared to the cortex, where glutamate is primarily present

26
Q

This movement disorder is unique in that it has both hypo- and hyper-kinetic symptoms.

A

Parkinson’s Disease

27
Q

What is the effect of dopamine? (excitatory/inhibitory)

A

Both excitatory and inhibitory, depending upon the receptor

28
Q

What comorbidities are associated with Tourette’s disease?

A

ADHD, OCD, anxiety, learning disabilities, behavioral issues, sleep issues, social deficits, sensory processing issues

29
Q

What treatment is used for Sydenham Chorea?

A

There is no treatment. Symptoms are generally self-resolving within three to six weeks

30
Q

What brain structure has one of the highest concentrations of acetylcholine?

A

Striatum (caudate, putamen)

31
Q

True/False. Constipation, orthostatic hypotension, smell dysfunction, and sexual dysfunction are complications of Parkinson’s disease.

A

True - Fatigue, pain, bladder problems, sleep disturbances, and problems with eating/swallowing are other complications