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Stage 2: Ageing > Parkinson's Disease > Flashcards

Parkinson's Disease Flashcards

1
Q

What do you need to formally diagnose PD?

A

Motor symptoms

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2
Q

What is rigidity?

A

Muscle stiffness

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3
Q

What is bradykinesia?

A

Reduction/slowness of movements

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4
Q

What fraction of PD patients have tremor of the hand/leg?

A

⅔ (so not essential but useful for diagnosis)

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5
Q

Describe the distribution of motor symptoms in PD

A
  • Start unilaterally
  • As the disease progresses they spread to the other side (after 10-15 years) but one side is always more affected than the other
  • If have a strictly unilateral or bilateral disease it is probably not PD (many diseases that present similarly)
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6
Q

What is the mean age of onset of PD?

A

65 (50-75) - 10% diagnosed < 40

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7
Q

Which gender are more likely to get PD?

A

Men (1.5x more likely)

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8
Q

What are risk factors for PD?

A
  • Family history of PD (if 2 or more family members affected)
  • Repeated head injury
  • Pesticide exposure (countryside)
  • Smoking and drinking protective?
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9
Q

Which neurotransmitter are PD patients deficient in?

A

Dopamine

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10
Q

What is the abnormality that is present in the brain of PD patients and what is it composed of?

A

Lewy bodies - alpha synuclein accumulation, causing neuronal damage

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11
Q

When do motor symptoms develop?

A

When the pathology of PD reaches the midbrain

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12
Q

In what order are areas of the brain affected by PD?

A

Medulla, rest of brain stem, cortex

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13
Q

What sense do people with PD typically have a disorder of and why?

A
  • Smell (hyposmia) - one of the earliest sites affected in addition to the medulla is the olfactory bulb
  • Majority of people who develop a reduced sense of smell will go on to develop PD in a few years but can’t diagnose without motor symptoms (can’t smell pizza)
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14
Q

What nervous system is affected in PD?

A

Autonomic nervous system, leading to non-motor symptoms

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15
Q

What NT systems are affected in PD?

A

1) Dopamine
2) Noradrenaline (autonomic problems and sleep dysfunction)
3) Serotonin (depression, fatigue)
4) Acetylcholine (dementia and apathy)

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16
Q

What does the variable amount of systems affected mean for presentation?

A

That there is a lot of heterogeneity of non-motor and to a lesser extent motor symptoms between presenting PD patients

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17
Q

What is the most important site affected by neurodegeneration in PD?

A

Substantia nigra pars compacta - the origin of the dopaminergic nigrostriatal tract (to the caudate nucleus and putamen)

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18
Q

What is the main cause of the classic clinical motor features of PD?

A

Dopamine deficiency in the nigrostriatal pathway

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19
Q

When do you see dementia and cognitive problems in PD patients?

A

After 5-15 years, never an early sign of PD

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20
Q

How do you test for hyposmia?

A

Sniffing stick test (sticks with different smells) - useful bc many people won’t be aware that their sense of smell is reduced

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21
Q

What sleep problem is typical of PD?

A

RBD (REM sleep behaviour disorder) - acting out dreams physically while asleep

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22
Q

Describe what happens in RBD in PD

A
  • People lose the atony/paralysis that normally occurs in REM sleep to prevent you moving about when dreaming
  • Therefore during REM sleep they tend to move a lot and can hurt themselves or their partner e.g. if they are having a scary dream or fighting something
  • If have this before PD, v likely that you will develop PD in 5-10 years
  • Most sleep centres located in brain stem so makes sense that this would be affected in PD
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23
Q

What are the key motor symptoms used to diagnose PD?

A

1) Shaking (tremor at rest)
2) Stiffness (rigidity on passive movement)
3) Slowness (bradykinesia)
4) Poverty of movement (hypokinesia)

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24
Q

What type of tremor occurs in PD?

A

Resting tremor

25
Q

How do you examine rigidity?

A

Feel the patient to see if muscle tone is increased

26
Q

What are clinical signs/features of rigidity in PD?

A

1) Lead pipe increase in tone - takes effort to make a movement that would normally be easy
2) Stiffness in limbs
3) Reduced arm swing - arm is kept still
4) Postural change

27
Q

What are the clinical signs/features of bradykinesia (cardinal feature, need for diagnosis) in PD?

A

1) Slowness of movement
2) Expressionless faces
3) Soft, quiet speech (slurred = dysarthria)
4) Slowness of thought

28
Q

What is a test you can do for bradykinesia?

A
  • Ask patient to open and close hand

- In PD the amplitude is decreased (movements get smaller and smaller) and movement is slow

29
Q

Describe freezing of gait in PD

A
  • Patient is ‘stuck’ to floor, difficult to initiate gait and move forward
  • If give them a visual cue e.g. line on floor suddenly find it much easier to walk
  • So with a cue they can go but without a cue there is freezing of gait
  • This is a v typical feature of PD (specific)
  • Can also have freezing of other movements or voice
30
Q

Describe using drawing to test PD

A
  • e.g. ask to draw spiral and in PD this will be normal shape but small e.g. not shaky
  • This is called micrographia
  • Also when writing letter will be small and will get smaller as they write more/towards the end of the sentence
  • V typical of PD
31
Q

Describe the Hoehn and Yahr stages of PD motor symptoms

A
  • Stage 1 → unilateral involvement only usually with minimal or no functional disability
  • Stage 2 → bilateral or midline involvement without impairment of balance
  • Stage 3 → bilateral disease, mild to moderate disability with impaired postural reflexes, physically independent, problem with balance, tend to fall
  • Stage 4 → severely disabling disease, still able to walk or stand unassisted
  • Stage 5 → confinement to bed or wheelchair unless aided, usually patients who have had disease for 15-20 years or more
32
Q

What type of symptoms are often present before motor symptoms?

A

Non-motor symptoms

33
Q

What are the non-motor symptoms of PD?

A

1) Fatigue
2) Sleep disturbance - insomnia, sleep fragmentation
3) Anxiety/depression
4) Psychosis, hallucinations (later on)
5) Hyposmia
6) Dementia/apathy
7) Constipation (obstipation)
8) Pain
9) Excessive daytime sleepiness
10) Urinary symptoms - nocturia, urgency
11) Difficulties concentrating
12) Restless leg syndrome
13) Sialorrea
14) Diplopia
15) Changed libido/sexual capacity

34
Q

Which non-motor symptom is v typical as a prodromal feature and common/universal in PD patients and why is it important to treat?

A

Constipation (muscles of bowel and anal sphincter affected)

  • If v constipated PD medication isn’t absorbed properly so if improve constipation can improve other symptoms as well if medication is absorbed better
  • Constipation can also disrupt sleep and cause bladder dysfunction (rarely leads to serious complications)
35
Q

Which symptoms of PD get worse over time?

A

Motor and non-motor symptoms

36
Q

Describe daytime somnolence in PD

A
  • Excessive daytime sleepiness as a result of poor sleep at night and medication e.g. levodopa, dopamine agonists
  • Management of sleep disturbances may also result in improving symptoms during the day
37
Q

Describe pain in PD

A
  • Dystonic spasms are one of the most painful symptoms

- Linked to depression

38
Q

What are the 4 most prevalent prodromal symptoms of PD (2-5 years)?

A

1) Hyposmia
2) RBD (sleep disruption)
3) Depression
4) Constipation

39
Q

What are symptoms at the early motor stage of PD (3-6 years)?

A

1) Fatigue
2) Pain
3) Diplopia
4) Motor symptoms mainly unilateral (progress to bilateral involvement as disease develops)

40
Q

What are symptoms at the early-mid stage of PD (4-12 years)?

A

1) Anxiety
2) Hypophonia
3) Dysphagia
4) Sleep disturbance e.g. fragmentation
5) After several years of levodopa treatment, pts are at increased risk of motor complications

41
Q

What are symptoms at the late stage of PD (8 years)?

A

1) Dementia
2) Cognitive dysfunction
3) Hallucinations/psychosis
4) Incontinence
5) Sexual dysfunction
6) Orthostatic hypotension
7) Instability and falls more frequent

42
Q

Describe the prodromal phase of PD

A
  • Variably long

- Can be mixed with some motor symptoms

43
Q

What % of dopaminergic neutrons are lost at diagnosis before any treatment can be given and what does this mean?

A

> 50%
- Can’t really treat disease bc by the time we see them, it is too late as most of their dopamine and probably the other NTs have already disappeared

44
Q

What is used to assess non-motor symptoms?

A
  • PD NMS questionnaire
  • Asking whether these symptoms are present or not
  • About 30 questions
  • These NMS can be grouped into domains which the questions ask about so all of these domains can be affected in PD
45
Q

What can be used to visualise dopamine loss in the brain that can be useful in diagnosis?

A

DaT scan (dopamine transporter scan) - inject tracer which is v specific for the dopamine receptor and can then visualise this using a PET scan

46
Q

Describe the difference between a normal and PD brain in a DaT scan

A

1) Normal - can see accumulation of DaT ligand in basal ganglia (caudate and striatum)
2) PD - less DaT (weaker signal) and one side is more affected than the other (asymmetry), less DaT on one side than the other, reflecting unilateral symptoms, side with less dopamine is side with more symptoms

47
Q

What % of PD patients have a genetic mutation causing their PD?

A

< 1% - only use in v young patients (25-30) bc risk of this being related to a genetic defect increases to 5-10%

48
Q

What mutations in genes linked to what increase your risk of PD?

A

Dopamine, DaT or alpha synuclein (about 50-60 genes identified)

49
Q

Is genetic testing routinely done in PD?

A

No

50
Q

What is Parkinsonism?

A
  • Tremor
  • Rigidity
  • Bradykinesia
  • Postural instability/gait difficulty
51
Q

What are differential diagnoses of Parkinsonism (90% will be PD)?

A

1) Degenerative diseases e.g. multiple system atrophy (related to alpha synuclein) or progressive supranuclear palsy - rare, present bilateral symptoms, more rapid progression (die after 5-8 years from complications rather than PD 20 years) and usually don’t respond to PD treatment
2) Drugs can induce Parkinsonism - neuroleptics e.g. haloperidol, olanzapine
3) Vascular lesions e.g. repeated strokes can cause PD like syndromes
4) Infections, toxins

52
Q

Why are people with tremor disorders with tremors that look like PD tremor easy to differentiate from PD?

A

These patients will lack all of the other Parkinsonian features e.g. rigidity, bradykinesia

53
Q

What causes motor symptoms in PD?

A

There is a lot less dopamine in the pre-synaptic area so the rate of stimulation is a lot less than healthy control

54
Q

Which enzymes break down dopamine?

A

MAO-B and COMT

55
Q

What is the prodromal stage of PD?

A

Prior to the appearance of recognised signs and symptoms (motor symptoms/diagnosis?)

56
Q

Describe the basal ganglia direct pathway in PD

A
  • When activated it facilitates movement
  • Activated by glutamate and dopamine via D1 receptors
  • Therefore in PD, as less dopamine is released from the SNc, the direct pathway is underactive
57
Q

Describe the basal ganglia indirect pathway in PD

A
  • When activated it inhibits movement
  • Activated by glutamate BUT inhibited by dopamine via D2 receptors (overrides) to make sure that in the presence of dopamine, you will always be able to move
  • Therefore in absence of dopamine this pathway is overactive so movement is inhibited
58
Q

What is the consequence of the direct pathway being underactive and the direct pathway being overactive?

A

Thalamocortical feedback is reduced, evoking motor deficits

Stage 2: Ageing (10 decks)

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