Parkinson's Disease and Parkinsonism Flashcards
what are features suggestive of a pyramidal/UMN lesion
muscle rigidity/increased tone
spasticity
hyperreflexia
what are features suggestive of a hyperkinetic (too much) extrapyramidal lesion
Dystonia, Tics, Myoclonus Chorea (Tremor)
what are features suggestive of a hypokinetic (too little) extrapyramidal lesion
Parkinsonism
Parkinson’s disease
what are features of a problem in the cerebellum
ataxia
what is the features of the Parkinsonian syndrome
rigidity
akinesia / bradykinesia
resting tremor
what is dystonia
prolonged muscle spasms and abnormal postures
what is chorea
Fragments of movements flow irregularly from one body segment to another causing a dance-‐like appearance.
what is chorea-ballismus
if amplitude of these irregular movements is large
what does PD present with
tremor muscle rigidity akinesia = inability to produce movement rest tremor gait postural impairment
what are the subtypes of PD
tremor dominant
non-tremor dominant
which type is associated with a slower rate of progression and less functional disability
tremor dominant
what are non motor features of PD
- 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
why are non motor features important to recognise
often present before onset of classical motor features
what is the ‘honeymoon phase’ in PD
motor features of PD initially respond well to symptomatic therapies before deteriorating
what is rapid eye movement sleep behaviour disorder
abnormal or disruptive behaviours which occur during rapid eye movement sleep, often related to dream enactment
Ix for rapid eye movement sleep behaviour disorder
over night polysomnography
Tx of rapid eye movement sleep behaviour disorder
clonazepam or melatonin at bedtime
how is a diagnosis of PD made
Clinically
there is no diagnostic test
what are the 3 steps needed for a diagnosis of PD
1 - diagnosis of parkinsonian syndrome
2 - exclusion criteria for PD
3 - supportive prospective positive criteria for PD
what are the pathological features of PD
degeneration of dopaminergic neurones in the substantia nigra in the basal ganglia
what is a neurohistological hallmark of PD
lewy bodies
mutations in what genes are associated with PD
SNCA
LRRK2
Parkin
GBA
what does SNCA do
encode the protein alpha-synuclein
what are mutations in LRRK2 and parkin most commonly responsible for
LRRK2 - dominant PD
Parkin - recessive PD
what mutation carrier the greatest genetic risk factor for PD
GBA
when should treatment be started in PD
when symptoms cause disability or discomfort
aiming to improve QOL and function
what are SE of dopamine agonists
nausea daytime somnolence oedema pathological gambling hypersexuality binge eating compulsive spending hallucinations drug induced psychosis
what are SE of levodopa
nausea
daytime somnolence
oedema
long term use can cause motor complications
- dyskinesia
- motor fluctuations
in who should dopamine agonists be avoided in
Hx of addiction
OCD
Cognitive impairment
what drugs can be added to reduce dopamine fluctuations
- dopamine agonists
- MAOB inhibitor
- COMT inhibitor
how is psychosis in PD managed
clozapine
or
quetiapine
what is the major SE of clozapine
agranulocytosis
what Tx is used for psychosis in PD in patients with pre-existing dementia
Cholinesterase inhibitors e.g. rivastigmine
what is bradykinesia
slowness of movement with progressive loss of amplitude or speed during attempted rapid alternating movements of body segments
how can bradykinesia be assessed
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
what is hypomimia, hypophonia and micrographia
hypomimia - decreased facial expression and eye blinking
hypophonia - soft speech
micrographia - progressively smaller handwriting
what is a resting tremor
Rhythmic involuntary movement [of affected body part] at rest that vanishes with active movement
what is the phenomenon ‘reemerging tremor’
tremor reappears after few seconds when arms held outstretched
what is the most distinguishing resting tremor
“pill-rolling”
what odd places can a tremor also affect
lower limbs
jaw
tongue
what is rigidity
increased muscle tone felt during examination by passive movements
resistance is felt throughout full range of movement
no increase with higher mobilising speed
what is a positive Froment’s manoeuvre
rigidity increases in examined body segment by voluntary movement of other body parts
what are features of the Parkinsonian gait
- slow, short, shuffling steps
- decreased arm swing
- slow turning with multiple small steps
- freezing
- festination
what is meant by ‘a positive levodopa’ challenge
when the patient was given Levodopa their symptoms got better = likely PD
what is vascular parkinsonism
Parkinsonism affects predominantly lower limbs
what are features of vascular parkinsonism
rest tremor is uncommon spasticity hemiparesis pseudobulbar palsy poor levodopa response
what is a common cause of degenerative parkinsonsims
multi system atrophy
what are Sx of multi system atrophy
- dysautonomia
- cerebellar features
- parkisonism
- jerky postural tremor
- generalised hyperreflexia
- extensor plantar response
what is seen on MRI in multi system atrophy
cerebellar and pontine atrophy
- “hot cross bun” sign
what is Fragile X-‐tremor ataxia syndrome (FXTAS)
Late-‐onset (>50 years) neurodegenerative disorder with an abnormal number of CGG repeats in the FMR1 gene
what is a reversible cause of dementia
Normal pressure Hydrocephalus
what is the Sx of Normal pressure Hydrocephalus
triad of
- dementia
- urinary incontinence
- gait abnormality
[DUG]
