PARKINSONISM Flashcards

1
Q

Role of BASAL GANGLIA

A
  • initiation of movements
  • modulation of movement
    (relays info recieved from the cerebral cortex BACK to the cerebral cortex)
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2
Q

What occurs with basal ganglial disease?

A
  • HYPOKINETIC or HYPERkinetic movement disorders
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3
Q

DIsease of the corticospinal/pyradmidal tract results in __________ and ______

A
  • SPASTICITY
  • PYRAMIDAL weakness

(UMN fts)

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4
Q

Disease of the cerebellum results in ________

A

ATAXIA

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5
Q

Disease of Basal Ganglia

A
  1. Hyperkinetic MD (dystonia/ Tics/ myoclonus/ chorea/ tremor)
  2. Hypokinetic MD
    - parkinsonism
    - parkinson’s disease
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6
Q

Pathological hallmark of parkinson’s?

A
  • loss of dark pigment in the substantia nigra and locus ceruleus
  • pigment loss correlates with dopaminergic cell loss
  • LEWY bodies on histology
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7
Q

Loss of how many percent of dopaminergic neurones is requires for symptoms to become clinically apparent.

A

50-60% of neurones from the PARS COMPACTA of the SUBSTANTIA nigra

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8
Q

What are lewy bodies

A
  • misfolded a-synuclein : insoluble and aggregated will form intracellular inclusions
  • —LEWY bodies may also be seen in spinal cord and the peripheral nervous system
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9
Q

What are the motor symptoms of parkinson’s?

A

Tremor
Rigidity
Bradykinesia

postural instability

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10
Q

Non-motor symptoms of Parkinson’s?

A
  • sleep d.o
  • hallucinations
  • GI dysfxn
  • depression
  • cognitive impairment/ dementia
  • anosmia
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11
Q

According to BRAAK staging of LEWY pathology what are the first abnormalities seen?

A
  • olfactory bulb

- enteric nervous system

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12
Q

What are the 2 subtypes of PD?

Which subtype has a small rate of progression?

A
  1. Tremor Dominant (relative absence of the other motor symptoms) —SLOWER progression (less functional dysability)
  2. Non-tremor dominant PD (akinetic-rigid syndrome and postural instability gait d.o)
  3. MIXED
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13
Q

Name a few prodromal symptoms of PD?

A
  • constipation
  • REM sleep Behavious d.o
  • EDS/ HYPOSMIA/ DEPRESSIOn
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14
Q

What are additional fts which help in dx of PD?

A
  • Bradykinesia AND one or MORE of the following: RESTING tremor, rigidity, postural instability
  • ADDITIONAL motor fts (STOOPED, fixed posture, DYSTONIC postures, hypomimia, shuffling, short-stepped gait
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15
Q

To confirm dx of parkinson’s, what should the pts NOT present with?

A
  • esrly onset bulbar problems, hallucinations, dementia, preferential involv. of lower limbs
  • prominent eye movement
  • intrusive early autonomic problems
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16
Q

If dx tests are needed, what invx are available?

A
  • structural brain imaging

- SPECT (DaTSCAN)

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17
Q

What is seen on DaTSCAN to confirm PD dx?

A
  • period-shaped
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18
Q

What may be ddx for parkisons?

A
  • MPTP exposure
  • NEGATIVE response to large doses of levodopa (malabsorpt. excluded)
  • babinski sign
  • cerebellar signs
  • supranuclear gaze palsy
  • cerebral tumor/ communicating hydrocephalus on MRI/CT
  • early autonomic involvement
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19
Q

What must be ruled out when considering postural instability as a PD sign?

A
  • should not be caused by primary visual, cerebellar, vestibular, proprioceptive dysfxn
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20
Q

Who is most likely to get PD?

A
  • men
  • of advancing age
  • w. FAMILY HX
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21
Q

What suggests genetic cause of PD?

A
  • early onset (< 40 y.o)
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22
Q

What gives rise to idiopathic PD?

A
  • susceptible genes+ env. triggers+ AGE
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23
Q

Name a few env. triggers.

A
  • pesticide and manganese exposure
  • prior head injury
  • rural living
  • beta-blocker use
  • well water drinking & agricultural job
  • mining, welding jobs
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24
Q

What reduces the risk?

A
  • tobacco smoking
  • coffee drinking
  • alcohol
  • calcium channel blocker
  • NSAID use
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25
How many genes are involved in PD?
- mutations in 11 genes
26
Name 2 important monogentic forms of PD?
- LRRK2 | - PARKIN
27
How to treat bradykinesia and rigidity seen in severe PD?
- symptomatic rx - dopaminergic rx alleviates motor symptoms - ----levodopa - ----MAO B-inhibitors
28
What is given to treat tremor?
- anticholinergic drugs (clozapine/ trihexyphenidyl)
29
What are the s.e one gets from use of dopamine agonists?
- nausea - daytime somnolence - edema - impulse control d.o (gambling/hypersexuality/bingeating ) - HALLUCINATIONS
30
What is seen with long term use of levodopa?
- a.w motor complications | - (dyskinesia and motor fluctuations)
31
Summary of long-term complications of levodopa?
- Motor Fluctuations - Non-motor fluctuations - Dyskinesia - drug-induced PSYCHOSIS
32
What is seen with drug-induced psychosis (levodopa) ]?
- visual (more commone) - less common= non-visual (tactile/auditory/olfactory) - illusions - delusions (paranois)
33
What is dyskinesia?
- involuntary choreiform/ dystonic movements | - occurs when levodopa is at peak dose
34
What can be given to treat REM sleep behav. d.o?
- benzodiazepine | CLONAZEPAM
35
What is given to treat dementia ?
- ACh-E inhibitor | RIVASTIGMINE
36
What is given to treat psychosis?
atypical antipsychotics= quetiapine and CLOZAPINE
37
How to manage PD?
- have a good care-taker system involved - MDT - non-oral therapies can be considered (DUODOPA- direct delivery through gastrostomy tube/ deep brain stimulation/ continuous apomorphine infusion)
38
What is the use of apomorphine injection?
- reduces the "off" time
39
How is bradykinesia tested?
- ask pt to perform repetitive movement ----it will shows the slowness of movement with progressive LOSS of amplitude
40
What are other form of bradykinesia apart from the usual movement d.o?
- hypomimia - hypophonia - micrographia
41
Does rest tremor of PD vanish with active movement?
YES | - reappears when hands are held outstretched
42
Most common resting tremor?
- pinr-rolling | - finger flexion-extension/ adbuction-adduction
43
How to distinguish rigidity from spasticity (seen in UMN) ?
- resistance is felt through out full range of passive movement - NO increase in rigidity with higher speed (in PD rigidity) = COGWHEEL rigidity
44
Festination is often seen in PD. What is it?
- very fast succession of steps and difficulties stopping
45
Describe the PD gait.
- slow - narrow base - short, shuffling steps - decr. arm swinging
46
Parkinsonism affecting the lower limbs predominantly, is called______. It presents with other _______
- VASCULAR Parkinsonism | - shows other brain vascular lesions like (spasticity/ hemiparesis/ pseudobulbar palsy)
47
What sets vascular parkinsonism apart from PD?
- resting tremor is UNCOMMON - poor levodopa response - structural brain scanning helps
48
What are the fts like in drug-induced parkinsonism?
- symmetrical - coarse postural tremor - presence of other drug induced d.o (orolingual dyskinesias- face and mouth involvement, tardive dystonia, akathisia- restlessness ) - note series of events (sx after drug exposure?/ improvment with drug halt)
49
How is essential tremor distinct from PD tremor?
- NOT seen at rest - SYMMETRIC, POSTURAL or kinetic tremor (with high freq.) - Autosomal dominant inheritance with mean onset of 15 years - ---alcohol responsiveness - HEAD tremor (mild)
50
What is a common cause of degen. parkinsonism? When is it seen?
- MULTI-SYSTEM ATROPHY | - 60-70 Y.O
51
What is the triad of Multi-system atrophy>
1. Dystautonomia 2. Cerebellar fts 3. Parkinsonism
52
What is seen on MRI of Multisystem Atrophy?
- cerebellar and pontine atrophy (HOT CROSS BUN) | - ----hypertense rim around PUTAMEN in T2 seq.
53
Which gaze palsy may also be a.w Parkinsonism?
- Progressive Supranuclear Palsy (vertical gaze palsy+ STARING+gait problems+ RETROCOLLIS- head tilted back+ Frontal-subcortical cognitive decline+ pseudobulbar sx+ parkinsonism )
54
What genetic manifestation results in FXTAS? And its course?
- those with abnormal no. of CGG repeats in the FMR1 gene | - LATE-onset and SLOW Progression
55
What are the CORE sx of Fragile X-tremor Ataxia Syndrome?
cerebellar GAIT ataxia+ postural/intention tremor+dysautonomia+ cog. decline of FRONTAL type and PERIPHERAL neuropathy
56
In women FXTAS is a.w with what other conditions?
- Premature Ovarian FAILURE | - MENOPAUSE
57
What is seen on MRI of FXTAS?
- MRI T2 hyperintensities in the MIDDLE cerebellar peduncles (MCP)
58
Which parkinson associated conditions have POOR/NO response to Levodopa?
- No response: Progressive Supranuclear Palsy and Vascular Parkinsonism - short-lived resp. to Levodopa in 1/3 of pts. in Multisystem atrophy
59
What investigations can be done for PD?
- r.o treatable probs of Asthenia (Hypothyroidism and anemia) - PET with fluoro-dopa ($$) - DATSPECT - POSITIVE levodopa challenge (s.c apomoprhine _ genetic testing
60
How useful is dopamine functional imaging to distinguish PD from other parkinsonism?
- UNABLE to distinguish PD from other degen. parkinsonism ---imaging should be NORMAL in ESSENTIAL tremor, dystonic tremor and psychogenic parkinsonism
61
What signs should not be seen in a pt to diagnose PD?
- ABSENT sensory, pyramidal and cerebellar signs - NO OTHER movement d.os - gaze palsies should NOT be present
62
What are the non -motor sx of PD? Early and late?
EARLY: - hyposmia - REM sleep d.o - constipation - depression LATE: dementia and hallucinations
63
When does dementia set in?
>80% of pts, sets in after 20 years
64
How is depression a.w PD managed?
TCA (desipramine/ nortriptyline) SSRIs (citalopram/ paroxetine/ sertraline) -----EXTENDED release formulation of VENLAFAXINE
65
What surgcial rx are available for PD ?
DEEP BRAIN stimulation targeting SUBTHALAMIC nucleus or GLOBUS pallidus internus - for rx of motor symptoms
66
What are the other signs of Multisystem Atrophy?
- Antecollis - dysarthria/ dysphonia - inspiratory sighing - OROFACIAL dystonia - babinski sign - generalised HYPERREFLEXIA