Parkinson's Disease and Basal Ganglia Disorders Flashcards

1
Q

What is the parkinsonian syndrome?

A

Rigidity, akinesia/bradykinesia and resting tremor

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2
Q

What is dystonia?

A

Prolonged muscle spasms and abnormal postures

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3
Q

What is chorea and ballismus?

A

Fragments of movements flow irregularly from one body segment to another
Ballismus is if amplitude of these movements is large

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4
Q

Motor symptom presentation of PD (5)

Clinical syndrome of Parkinsonism

A

rest tremor, rigidity, akinesia/bradykinesia, impairment of gait and posture

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5
Q

What changes in the brain cause PD

A

Loss of normally dark black pigment in substantia nigra and locus coeruleus - pigment loss coreelates with dopaminergic cell loss

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6
Q

Neurohistological hallmark of PD

A

Lewy bodies

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7
Q

Risk factors for PD

A

Old age
Family history
Possibly pesticide exposure

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8
Q

What gene is commonly associated with PD

A

PARK gene

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9
Q

Is smoking a PD risk factor?

A

No - actually shows protection against PD

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10
Q

Bradykinesia in PD

A

Slowness of movement with progressive loss of amplitude or speed during attempted rapid alternating movement of body segments - test by repetitive movements eg foot tapping, closing opening hands.

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11
Q

Signs of bradykinesia

A

Hypomimia - less facial expression and eye blinking
Hypophonia
Micrographia - progressively smaller handwriting

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12
Q

Rest tremor in PD

A

Rhythimic oscillatory involuntary movement of affected body part at rest
Vanishes with active movement
Typically reappears after a few secs when arms outstretched - reemerging tremor

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13
Q

Most distinguishing type or resting tremors in PD

A

Pill rolling tremor

or finger flexion/extension or abduction/adduction

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14
Q

When is it best to observe the tremor?

A

When patient is focused on a particular mental task eg count backwards from 100

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15
Q

Is head tremor typical for PD?

A

no

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16
Q

Describe rigidity and when it is felt

A

Increased muscle tone felt during examination by passive movement
Resistance felt throughout full range or movement
No increase with speed

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17
Q

What is cog wheel rigidity

A

Rigidity + resting tremor

Especially felt at wrist

18
Q

What is the difference in rigidity in PD and rigidity from spasticity owing to UMN lesions

A

Spasticity increases with higher mobilizing speed

19
Q

Describe a positive Froment’s manoeuvre

A

Rigidity increases in examined body segment by voluntary movement of other body parts

20
Q

Describe a parkinsonian gait

A

Slow, occurs at narrow base with short, shuffling steps
Decreased arm swing
Slow turning with multiple steps

21
Q

Describe posture of a typical patient with PD

A

Stooped owing to impaired postural reflexes

22
Q

Describe early nonmotor symptoms of PD (4)

A

Hyposmia
REM
Constipation
Depression

23
Q

Describe late non motor symptoms of PD (2)

A

Dementia (over 80% pts after 20yrs)

Hallucinations

24
Q

Investigations of PD

A

– Rule out treatable conditions of asthenia (hypothyroidism, anaemia)
– Structural brain imaging
– Possibly dopamine funcLonal imaging:
• PET with fluoro-­‐dopa (limited availability and high cost)
• Dopamine transporter (DAT) imaging with single-­‐photon emission CT (DAT-­‐SPECT)
– Dopamine functional imaging is unable to distinguish PD from other causes of degenerative Parkinsonism, but should be normal in essential tremor, dystonic tremor, psychogenic parkinsonism
– Positive levodopa (or s.c.apomorphine) challenge
– Genetic testing where appropriate (if young and if positive family hx)

25
Q

Common causes of Parkinsonism (7)

A

Parkinson’s Disease
Drug induced/iatrogenic
Progressive supranuclear palsy
Multiple system atrophy
Dementia with Lewy bodies
Diffuse white matter ischaemia/ vascular parkinsonism
Lower half parkinsonism with marked gait apraxia

26
Q

Drugs which can induce Parkinsonism (6)

A
Drugs which block the action of dopamine
Metoclopramide
Antipsychotics
Some CCBs
Amiodarone
Chloroquinine
Prochlorperazine
27
Q

Uncommon causes of parkinsonism (6)

A
Infectious and post-infectious
Normal pressure hydrocephalusirone
Toxins
Metabolic and endocrine
Head trauma
Hallervorden-Spatz syndrome and other neurodegenerative disorders with accumulation
28
Q

Describe vascular parkinsonism

A

Affects predominantly lower limbs
Rest tremor uncomon
Other signs of brain vascular lesion may be present eg spasticity, hemiparesis and pseudobulbar palsy
Poor levodopa response

29
Q

Describe drug induced Parkinsonism (5)

A
Symmetrical
Coarse postural tremor
Emergence after drug exposure
Improvement resolution after complete drug withdrawal
Presence of other drug induced disorders
30
Q

How is Essential Tremor distinct from PD tremor

A
  • Symmetrical, higher frequency
  • Infrequently observed at rest
  • Often autosomal dominant with mean outset of 15yrs
  • Alcohol responsiveness
  • Head tremor - if present is mild
31
Q

Describe dementia with lewy body presentation (5)

A

Dementia
Daily fluctuations in alertness and cognition
Visual hallucinations
Extreme sensitivity to neuroleptic medication
Dysautonomia

32
Q

What is the core triad in multi system atrophy

A

Dysautonomia
Cerebellar features
Parkinsonism

33
Q

Presentation of multi system atrophy

A

late onset into 6th or 7th decades
Jerky tremor and pyramidal signs such as generalised hyperreflexia and extensor plantar responses.
Sub optimal and short live levodopa response in 1/4 pts
Other features - severe dysarthria or dysphonia, marked antecollis inspiratory sighing, orofacial dystonia

34
Q

Progressive supranuclear palsy. Similarities and differences to PD

A

Similar - Some pts present with Parkinsonism, Falls
Differences -
Tremor is unusual
No levodopa response
Continuous staring from frontalis muscle activity
Pseudobulbar symptoms
Retrocollis

35
Q

What is FXTAS?

A

Fragile X- tremor ataxia syndrome

Late onset neurodegenerative disorder.

36
Q

Non motor symptom treatments in PD

A

ED - Sidenafil
Constipation - lactulose
Dementia - cholinesterase inhibitors or memantine

37
Q

Treat motor symptoms in PD

A
  • Levodopa in combo with a peripheral dopa decarboxylase inhibitor
  • Dopamine agonists eg bromocriptine
  • Catechol-O-methyltransferase inhibitor eg entacapone
  • Amanadine
38
Q

Side effect of levodopa

A

Peak dose dyskinesias wearing off and sudden off states

39
Q

Side effects of dopamine agonists

A

Peripheral oedema, Fibrotic reactions - excessive daytime somnolence, impulsive control problems

40
Q

Initial management of PD

A

Levodopa start with 50mg TDS and not exceed 600mg/day

All other motor symtpom treatment can be combined with levodopa