Parkinson's Disease and Parkinsonism

Question 1

what are features suggestive of a pyramidal/UMN lesion

Answer 1

muscle rigidity/increased tone
spasticity
hyperreflexia

Question 2

what are features suggestive of a hyperkinetic (too much) extrapyramidal lesion

Answer 2

Dystonia,
Tics,
Myoclonus
Chorea
(Tremor)

Question 3

what are features suggestive of a hypokinetic (too little) extrapyramidal lesion

Answer 3

Parkinsonism

Parkinson’s disease

Question 4

what are features of a problem in the cerebellum

Answer 4

ataxia

Question 5

what is the features of the Parkinsonian syndrome

Answer 5

rigidity
akinesia / bradykinesia
resting tremor

Question 6

what is dystonia

Answer 6

prolonged muscle spasms and abnormal postures

Question 7

what is chorea

Answer 7

Fragments of movements flow irregularly from one body segment to another causing a dance-­‐like appearance.

Question 8

what is chorea-ballismus

Answer 8

if amplitude of these irregular movements is large

Question 9

what does PD present with

Answer 9

tremor
muscle rigidity
akinesia = inability to produce movement
rest tremor
gait
postural impairment

Question 10

what are the subtypes of PD

Answer 10

tremor dominant

non-tremor dominant

Question 11

which type is associated with a slower rate of progression and less functional disability

Answer 11

tremor dominant

Question 12

what are non motor features of PD

Answer 12

• olfactory dysfunction
• cognitive impairment e.g. dementia
• psychiatric symptoms e.g. visual hallucinations, depression
• sleep disorders e.g. REM sleep behaviour disorder
• pain/aching limbs
• fatigue
• micrographia

Question 13

why are non motor features important to recognise

Answer 13

often present before onset of classical motor features

Question 14

what is the ‘honeymoon phase’ in PD

Answer 14

motor features of PD initially respond well to symptomatic therapies before deteriorating

Question 15

what is rapid eye movement sleep behaviour disorder

Answer 15

abnormal or disruptive behaviours which occur during rapid eye movement sleep, often related to dream enactment

Question 16

Ix for rapid eye movement sleep behaviour disorder

Answer 16

over night polysomnography

Question 17

Tx of rapid eye movement sleep behaviour disorder

Answer 17

clonazepam or melatonin at bedtime

Question 18

how is a diagnosis of PD made

Answer 18

Clinically

there is no diagnostic test

Question 19

what are the 3 steps needed for a diagnosis of PD

Answer 19

1 - diagnosis of parkinsonian syndrome
2 - exclusion criteria for PD
3 - supportive prospective positive criteria for PD

Question 20

what are the pathological features of PD

Answer 20

degeneration of dopaminergic neurones in the substantia nigra in the basal ganglia

Question 21

what is a neurohistological hallmark of PD

Answer 21

lewy bodies

Question 22

mutations in what genes are associated with PD

Answer 22

SNCA
LRRK2
Parkin
GBA

Question 23

what does SNCA do

Answer 23

encode the protein alpha-synuclein

Question 24

what are mutations in LRRK2 and parkin most commonly responsible for

Answer 24

LRRK2 - dominant PD

Parkin - recessive PD

Question 25

what mutation carrier the greatest genetic risk factor for PD

Answer 25

GBA

Question 26

when should treatment be started in PD

Answer 26

when symptoms cause disability or discomfort | aiming to improve QOL and function

Question 27

what are SE of dopamine agonists

Answer 27

``` nausea daytime somnolence oedema pathological gambling hypersexuality binge eating compulsive spending hallucinations drug induced psychosis ```

Question 28

what are SE of levodopa

Answer 28

nausea daytime somnolence oedema long term use can cause motor complications - dyskinesia - motor fluctuations

Question 29

in who should dopamine agonists be avoided in

Answer 29

Hx of addiction OCD Cognitive impairment

Question 30

what drugs can be added to reduce dopamine fluctuations

Answer 30

- dopamine agonists - MAOB inhibitor - COMT inhibitor

Question 31

how is psychosis in PD managed

Answer 31

clozapine or quetiapine

Question 32

what is the major SE of clozapine

Answer 32

agranulocytosis

Question 33

what Tx is used for psychosis in PD in patients with pre-existing dementia

Answer 33

Cholinesterase inhibitors e.g. rivastigmine

Question 34

what is bradykinesia

Answer 34

slowness of movement with progressive loss of amplitude or speed during attempted rapid alternating movements of body segments

Question 35

how can bradykinesia be assessed

Answer 35

asking the patient to perform repetitive movements as quickly and widely as possibly (opening and closing the hand, foot tapping) asking them to stand up

Question 36

what is hypomimia, hypophonia and micrographia

Answer 36

hypomimia - decreased facial expression and eye blinking hypophonia - soft speech micrographia - progressively smaller handwriting

Question 37

what is a resting tremor

Answer 37

Rhythmic involuntary movement [of affected body part] at rest that vanishes with active movement

Question 38

what is the phenomenon 'reemerging tremor'

Answer 38

tremor reappears after few seconds when arms held outstretched

Question 39

what is the most distinguishing resting tremor

Answer 39

"pill-rolling"

Question 40

what odd places can a tremor also affect

Answer 40

lower limbs jaw tongue

Question 41

what is rigidity

Answer 41

increased muscle tone felt during examination by passive movements resistance is felt throughout full range of movement no increase with higher mobilising speed

Question 42

what is a positive Froment's manoeuvre

Answer 42

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

Question 43

what are features of the Parkinsonian gait

Answer 43

- slow, short, shuffling steps - decreased arm swing - slow turning with multiple small steps - freezing - festination

Question 44

what is meant by 'a positive levodopa' challenge

Answer 44

when the patient was given Levodopa their symptoms got better = likely PD

Question 45

what is vascular parkinsonism

Answer 45

Parkinsonism affects predominantly lower limbs

Question 46

what are features of vascular parkinsonism

Answer 46

``` rest tremor is uncommon spasticity hemiparesis pseudobulbar palsy poor levodopa response ```

Question 47

what is a common cause of degenerative parkinsonsims

Answer 47

multi system atrophy

Question 48

what are Sx of multi system atrophy

Answer 48

- dysautonomia - cerebellar features - parkisonism - jerky postural tremor - generalised hyperreflexia - extensor plantar response

Question 49

what is seen on MRI in multi system atrophy

Answer 49

cerebellar and pontine atrophy | - "hot cross bun" sign

Question 50

what is Fragile X-­‐tremor ataxia syndrome (FXTAS)

Answer 50

Late-­‐onset (>50 years) neurodegenerative disorder with an abnormal number of CGG repeats in the FMR1 gene

Question 51

what is a reversible cause of dementia

Answer 51

Normal pressure Hydrocephalus

Question 52

what is the Sx of Normal pressure Hydrocephalus

Answer 52

triad of - dementia - urinary incontinence - gait abnormality [DUG]