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Neurology > Parkinson's and Movement Disorders > Flashcards

Parkinson's and Movement Disorders Flashcards

1
Q

What are basal ganglia?

A

Deep set nuclear structures = involved in initiation of movement and modulation of movement

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2
Q

How do basal ganglia interact with the cerebral cortex?

A

Receive input from cerebral cortex, process it and relay back to the cerebral cortex via the thalamus

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3
Q

What can basal ganglia disease lead to?

A

Hypokinetic or hyperkinetic movement disorders

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4
Q

What are features of movement disorders affecting the corticospinal/pyramidal tracts?

A

UMN features = pyramidal weakness, spasitcity

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5
Q

What are some features of movement disorders affecting the basal ganglia?

A 
Hypokinetic = rigidity, bradykinesia, Parkinsonism
Hyperkinetic = ticks, dystonia, myoclonus, weakness, spasticity
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6
Q

What do movement disorders affecting the cerebellum present with?

A

Ataxia

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7
Q

What is the inheritance of Parkinson’s disease?

A

95% idiopathic and 5% familial

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8
Q

What are the pathological hallmarks of Parkinson’s disease?

A

Sections through brainstem reveal loss of normally dark black pigment in the substantia nigra and locus coeruleus

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9
Q

What does pigment loss correspond to?

A

Dopaminergic neuron loss = need reduction of 50-60% for symptoms to become clinically apparent

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10
Q

What is the relevance of Lewy bodies in Parkinson’s disease?

A

Non-specific hallmark = marker of poor neuron health, not present in some genetic PD

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11
Q

What are the motor symptoms of Parkinson’s disease?

A

Tremor, bradykinesia, rigidity, postural instability

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12
Q

What are the non-motor symptoms of Parkinson’s disease?

A

Sleep disorders, hallucinations, GI dysfunction, depression, cognitive impairment/dementia, anosmia

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13
Q

Where are dopaminergic neurons lost from?

A

The pars compacta region of the substantia nigra

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14
Q

What is Braak’s staging used for?

A

Helps explain the non-motor symptoms of Parkinson’s disease = first abnormalities are in olfactory bulb

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15
Q

What are the subtypes of motor features in Parkinson’s disease?

A

Features are heterogenous = tremor dominant, non-tremor dominant, mixed/indeterminate phenotype

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16
Q

What are some features of tremor dominant Parkinson’s disease?

A

Associated with slower rate of progression and less functional disability = relative absence of other motor symptoms

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17
Q

What are some examples of non-tremor dominant Parkinson’s disease?

A

Akinetic-rigid syndrome and postural instability gait disorder

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18
Q

What are some features that support a diagnosis of Parkinson’s disease?

A

Unilateral onset of bradykinesia and at least one of resting tremor, rigidity or postural instability

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19
Q

What are some additional motor features that may support diagnosis of Parkinson’s disease?

A

Stooped fixed posture, dystonic posture, hypomimia, shuffling short stepped gait

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20
Q

What are some additional non-motor features that may aid a diagnosis of Parkinson’s disease?

A

Late onset hyposmia, depression and anxiety, constipation, bladder problems, pain, subtle mental impairment

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21
Q

What kind of rigidity occurs in Parkinson’s disease?

A

Lead pipe = constant resistance throughout passive movement

Cog wheel = superimposed clicking resistance which is attributed to underlying tremor

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22
Q

What symptoms would patients with Parkinson’s disease be unlikely to present with?

A

Early onset bulbar problems, dementia, prominent eye movement disorder, intrusive early autonomic problems

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23
Q

What is the epidemiology of Parkinson’s disease?

A

More common with advancing age and in men

Risk factors = older age, positive family history, male

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24
Q

What would indicate a genetic cause of Parkinson’s disease?

A

Onset age <40

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25
Q

What mutations are implicated in Parkinson’s disease?

A 
LRRK2 = most relevant autosomal dominant mutation
PARKIN = most relevant autosomal recessive mutation
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26
Q

What treatment do bradykinesia and rigidity respond to?

A

Dopaminergic treatment

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27
Q

What is used to treat severe Parkinson’s disease symptoms?

A

Levodopa and dopamine agonists

28
Q

What drug class is used to treat tremors?

A

Anticholinergics = trihexyphenidyl or clozapine

29
Q

What are some features of dopamine agonists?

A

Associated with impulse control disorders
Avoid in history of addiction, OCD or impulsive personality
Don’t prescribe in elderly (especially with cognitive impairment) = cause hallucinations

30
Q

What side effects are dopamine and levodopa associated with?

A

Nausea, daytime somnolence and oedema

31
Q

What is longterm levodopa use associated with?

A

Dyskinesia and motor fluctuations

32
Q

What are the longterm complications of dopaminergic agonists?

A

Motor and non-motor fluctuations, dyskinesia, drug induced psychosis

33
Q

What are the first line agents for treating Parkinson’s disease?

A

Dopamine agonists, levodopa, MAO inhibitor

34
Q

What are add on medications for treating Parkinson’s disease?

A

Catechol-O-methyltransferase inhibitor, MAO inhibitor, dopamine agonists, apomorphine

35
Q

What does the Parkinson’s MDT consist of?

A

PD specialist nurses, physiotherapists, pharmacists, occupational therapists, movement disorder specialists, GPs

36
Q

What are some non-oral therapies for treating Parkinson’s disease?

A

Deep brain stimulation, DNODOPA, continuous apomorphine infusion

37
Q

What is bradykinesia?

A

Slowness of movement with progressive loss of amplitude/speed during attempted rapid alternating movement of body segments

38
Q

How do you assess bradykinesia?

A

Ask patient to perform some repetitive movements as quickly and widely as possible or getting patient to stand

39
Q

What are some associated features of bradykinesia?

A

Hypomimia, hypophonia and micrographia

40
Q

What is a Parkinsonian tremor?

A

Rhythmic involuntary oscillatory movement of affected body part = usually pill-rolling type tremor

41
Q

What are some features of a Parkinsonian tremor?

A

Low-mid range frequency (3-6Hz) with variable amplitude
Vanishes with active movement
Reappears when arms held outstretched

42
Q

When is a Parkinsonian tremor best observed?

A

When patient is focused on a particular task

43
Q

What is rigidity?

A

Increased muscle tone felt during examination by passive movement

44
Q

What are some features of rigidity?

A

Resistance felt throughout full ROM = no increase with higher mobilising speed

45
Q

What manoeuvre confirms the presence of rigidity?

A

Froment’s manoeuvre = rigidity increases in examined body segment by voluntary movement of other body parts

46
Q

What is the typical posture of a patient with Parkinson’s disease?

A

Stooped posture = due to impaired postural reflexes, may have extreme anterior truncal flexion (camptocornia)

47
Q

What kind of gait do patients with Parkinson’s disease have?

A

Slow, narrow base with short shuffling steps, decreased arm movement

48
Q

What is festination?

A

Very fast succession of steps and difficulty stopping

49
Q

What investigations can be done for Parkinson’s disease?

A

Structural brain imaging and genetic testing
Dopamine functional imaging
Positive levodopa challenge

50
Q

What are some examples of dopamine functional imaging techniques?

A

PET with fluoro-dopa = less common

Dopamine transporter imaging with single photon emission CT (DAT-SPECT)

51
Q

What are some red flags for Parkinson’s disease?

A

Symmetrical symptoms and fast disease progression
Frequent falls and marked autonomic dysfunction
Severe axial/lower limb involvement and apraxia
Eye movement disorder and bulbar features
Other unexplained movement disorder
Pyramidal/cerebellar dysfunction
Parietal associative sensory disturbances
Severe cognitive deterioration or psychosis
Negative levodopa challenge

52
Q

What are the differentials of Parkinson’s disease?

A 
Vascular and drug induced Parkinsonism
Essential tremor
Multisystem atrophy 
Progressive supranuclear palsy
Fragile X-tremor ataxia syndrome (FXTAS)
53
Q

What are some features of vascular Parkinsonism?

A

Predominantly lower limbs = spasticity, hemiparesis, pseuobulbar palsy
Rest tremor uncommon and poor levodopa response
Structural brain imaging guides diagnosis

54
Q

What are some features of drug induced Parkinsonism?

A

Symmetrical = coarse postural tremor, tardive dystonia, orolingual dyskinesia, akathsia
Symptoms emerge after drug introduction and resolve after its withdrawl

55
Q

What are some features of an essential tremor?

A

Symmetric postural or kinetic tremor with higher frequency = infrequently observed at rest
Autosomal dominant and age of onset about 15
Mild head tremor and shows alcohol responsiveness

56
Q

What is multisystem atrophy?

A

Common cause of degenerative Parkinsonism = age of onset 60-70
Core triad of dysautonomia, cerebellar features and Parkinsonism

57
Q

What are some features of multisystem atrophy?

A

Jerky postural tremor, generalised hyperreflexia and extensor plantar responses, severe dysarthia/dysphonia, marked antecollis, inspiratory sighing, orofacial dystonia

58
Q

What does an MRI of a patient with multisystem atrophy show?

A

Cerebellar and pontine atrophy = hot cross bun sign

59
Q

What is progressive supranuclear palsy?

A

Symmetric akinetic-rigid syndrome with predominantly axial involvement = gait/balance impairment, retrocollis, pseudobulbar symptoms

60
Q

What are some features of progressive supranuclear palsy?

A

Continuous activity of frontalis muscle with eyes wide open and vertical gaze supranuclear palsy
Frontal-subcortical cognitive deficits
No response to levodopa
Potentially Parkinsonism

61
Q

What is fragile x-tremor ataxia syndrome?

A

Late onset (age >50) disorder in patients with an abnormal number of CGG repeats in FMR1 gene in premutation range (55-200)

62
Q

What are some symptoms of fragile x-tremor ataxia syndrome?

A

Slow disease progression = cerebellar gait ataxia, postural or intention tremor, Parkinsonisms, dysautonomia, cognitive decline, peripheral neuropathy

63
Q

What are are the symptoms of fragile x-tremor ataxia syndrome in females with premutation?

A

X-linked inheritance so milder symptoms = premature ovarian failure and menopause

64
Q

How is fragile x-tremor ataxia syndrome diagnosed?

A

MRI = T2 hyperintensities in middle cerebellar peduncles

Molecular testing

65
Q

What may condition may children of fragile x-tremor ataxia syndrome develop?

A

Classical fragile x syndrome

Neurology (48 decks)

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