Parkinson's Disease and Basal Ganglia Disorders

Question 1

What is the parkinsonian syndrome?

Answer 1

Rigidity, akinesia/bradykinesia and resting tremor

Question 2

What is dystonia?

Answer 2

Prolonged muscle spasms and abnormal postures

Question 3

What is chorea and ballismus?

Answer 3

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

Question 4

Motor symptom presentation of PD (5)

Clinical syndrome of Parkinsonism

Answer 4

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

Question 5

What changes in the brain cause PD

Answer 5

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

Question 6

Neurohistological hallmark of PD

Answer 6

Lewy bodies

Question 7

Risk factors for PD

Answer 7

Old age
Family history
Possibly pesticide exposure

Question 8

What gene is commonly associated with PD

Answer 8

PARK gene

Question 9

Is smoking a PD risk factor?

Answer 9

No - actually shows protection against PD

Question 10

Bradykinesia in PD

Answer 10

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.

Question 11

Signs of bradykinesia

Answer 11

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

Question 12

Rest tremor in PD

Answer 12

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

Question 13

Most distinguishing type or resting tremors in PD

Answer 13

Pill rolling tremor

or finger flexion/extension or abduction/adduction

Question 14

When is it best to observe the tremor?

Answer 14

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

Question 15

Is head tremor typical for PD?

Answer 15

no

Question 16

Describe rigidity and when it is felt

Answer 16

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

Question 17

What is cog wheel rigidity

Answer 17

Rigidity + resting tremor

Especially felt at wrist

Question 18

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

Answer 18

Spasticity increases with higher mobilizing speed

Question 19

Describe a positive Froment’s manoeuvre

Answer 19

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

Question 20

Describe a parkinsonian gait

Answer 20

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

Question 21

Describe posture of a typical patient with PD

Answer 21

Stooped owing to impaired postural reflexes

Question 22

Describe early nonmotor symptoms of PD (4)

Answer 22

Hyposmia
REM
Constipation
Depression

Question 23

Describe late non motor symptoms of PD (2)

Answer 23

Dementia (over 80% pts after 20yrs)

Hallucinations

Question 24

Investigations of PD

Answer 24

– 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)

Question 25

Common causes of Parkinsonism (7)

Answer 25

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

Question 26

Drugs which can induce Parkinsonism (6)

Answer 26

``` Drugs which block the action of dopamine Metoclopramide Antipsychotics Some CCBs Amiodarone Chloroquinine Prochlorperazine ```

Question 27

Uncommon causes of parkinsonism (6)

Answer 27

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

Question 28

Describe vascular parkinsonism

Answer 28

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

Question 29

Describe drug induced Parkinsonism (5)

Answer 29

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

Question 30

How is Essential Tremor distinct from PD tremor

Answer 30

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

Question 31

Describe dementia with lewy body presentation (5)

Answer 31

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

Question 32

What is the core triad in multi system atrophy

Answer 32

Dysautonomia Cerebellar features Parkinsonism

Question 33

Presentation of multi system atrophy

Answer 33

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

Question 34

Progressive supranuclear palsy. Similarities and differences to PD

Answer 34

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

Question 35

What is FXTAS?

Answer 35

Fragile X- tremor ataxia syndrome | Late onset neurodegenerative disorder.

Question 36

Non motor symptom treatments in PD

Answer 36

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

Question 37

Treat motor symptoms in PD

Answer 37

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

Question 38

Side effect of levodopa

Answer 38

Peak dose dyskinesias wearing off and sudden off states

Question 39

Side effects of dopamine agonists

Answer 39

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

Question 40

Initial management of PD

Answer 40

Levodopa start with 50mg TDS and not exceed 600mg/day | All other motor symtpom treatment can be combined with levodopa