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Y3 - Neuro > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

What does ‘movement disorders’ divide into?

A

Hypokinesia

Hyperkinesia

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2
Q

What is hypokinesia?

A

Slowed movement, increased tone

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3
Q

What is hyperkinesia?

A

Excessive movement

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4
Q

What is idiopathic Parkinson’s Disease?

A

Condition of progressive reduction of dopamine in the basal ganglia leading to hypokinetic movement disorder

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5
Q

What are the risk factors for idiopathic Parkinson’s Disease?

A 
Age >65
Male sex
Pesticide exposure
Occupation: farmer
Family history
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6
Q

What is a protective factor for idiopathic Parkinson’s Disease?

A

Smoking

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7
Q

What is the underlying pathology for idiopathic Parkinson’s Disease?

A

Degeneration of dopamine neurones within the substantial nigra
Accumulation of Lewy bodies, initially within the substantial migration then the rest of the brain

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8
Q

What are the main clinical features of idiopathic Parkinson’s Disease?

A

Tremor
Rigidity
Akinesia (bradykinesia)
Postural changes

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9
Q

Are features of idiopathic Parkinson’s Disease symmetric or asymmetric?

A

Asymmetrical

The initially affected side will always be worse

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10
Q

Describe the tremor seen in idiopathic Parkinson’s Disease?

A

Resting tremor (can re-emerge after few secs when hands held outstretched)
Pill rolling tremor
Made worse by emotion and stress

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11
Q

Describe muscle tone in idiopathic Parkinson’s Disease?

A

Increased tone
Cog wheel rigidity (rating tremor + rigidity)
Positive Froment’s manoeuvre (rigidity increases in examined body segment by voluntary movement of other body parts)

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12
Q

Describe bradykinesia in idiopathic Parkinson’s Disease?

A

Difficulty initiating movement
Progressive reduction in speed amplitude with attempted rapid alternating movement of body segments
Reduced blinking, facial expression, volume of voice
Progressive reduction in size of handwriting

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13
Q

Describe postural changes in idiopathic Parkinson’s Disease?

A

Difficulty standing up
Slow to start walking
Small shuffling steps with reduced arm swing
Stooped posture
Difficulty turning and navigating objects
Falls in late disease

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14
Q

What are the non-motor symptoms in idiopathic Parkinson’s Disease?

A

Drooling
Reduced sense of smell
REM sleep disorders
Autonomic (constipation, urinary urgency, frequency)
Psychiatric (depression, dementia, psychosis, personality change, visual hallucinations)

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15
Q

What is the usual progression of symptoms in idiopathic Parkinson’s Disease?

A

Constipation, hyposmia, depression (prodromal)
Bradycardia, rigidity, tremor, pain, fatigue (first few years)
Fluctuations, dyskinesia
Urinary symptoms, dementia, dysphagia, postural instability, falls(10 years)
Psychosis (20 years)

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16
Q

What is the diagnostic investigation in idiopathic Parkinson’s Disease?

A

Clinical examination

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17
Q

What is assessed in clinical examination of idiopathic Parkinson’s Disease?

A 
Tremor
Gait
Upper and lower limb:  tone, reflexes
Eye movements
Speech
Writing
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18
Q

What investigations other than clinical can be done for idiopathic Parkinson’s Disease?

A

Levodopa challenge test
Genetic testing if very young or very strong family history
Structural brain imaging if doubt

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19
Q

What is the gold standard management for idiopathic Parkinson’s Disease?

A

Levodopa + Carbidopa

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20
Q

How does Levodopa work, and how does Carbidopa improve its effects?

A

Levodopa crosses the BBB and is converted into dopamine in the brain
Carbidopa prevents peripheral breakdown of dopamine

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21
Q

What are the side effects of Levodopa + Carbidopa as treatment for idiopathic Parkinson’s Disease?

A

Nausea, vomiting
Dyskinesia/dystonia
Sudden off states
Psychiatric: psychosis, hallucinations, reduced impulse control

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22
Q

What is used in idiopathic Parkinson’s Disease to manage the nausea that can be caused by medication, and why?

A

Domperidone
Used as it is selective to peripheral dopamine receptors so won’t prevent activity of levodopa in the brain
Avoid Metoclopramide

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23
Q

When is levodopa used in idiopathic Parkinson’s Disease, and why then?

A

Use is delayed until significant disability

It has diminishing effects, with action lasting 5-10 years

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24
Q

What drugs are used in idiopathic Parkinson’s Disease until levodopa used?

A

Dopamine agonists: Ropinirole, Pramipexole, Apomorphine)
COMT inhibitors: Entacapone
MOAB inhibitors: Selegeline, Rasagiline

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25
Q

What are Parkinsonian disorders?

A

Word used to describe a cluster of disorders associated with symptoms of idiopathic Parkinson’s but have a defined underlying cause

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26
Q

How are Parkinsonian disorders differentiated from actual Parkinson’s?

A 
Symmetrical presentation
Absence of tremor
Levodopa unresponsiveness
Fast progression
Sensory disturbance
Pyramidal signs
Early falls
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27
Q

What are examples of Parkinsonian disorders?

A 
Progressive supranuclear palsy
Multi-System Atrophy
Cortico-basal degeneration
Vascular Parkinson's
Dementia with Lewy bodies
Drug-induced Parkinson's
Wilson's disease
Fragile X-tremor ataxia syndrome
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28
Q

Describe progressive supranuclear palsy

A 
Vertical gaze palsy
Lack of tremor
Early postural instability and falls
Speech and swallowing problems
Symmetrical truncal rigidity more prominent than limb rigidity
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29
Q

Describe multi-system atrophy

A

Onset in 6th or 7th decade
Core triad: Early autonomic disturbance (postural hypotension, bladder dysfunction), ataxia, cerebellar signs
Pyramidal signs (spasticity, weakness, slowing of rapid alternating movements, hyperreflexia)
Jerky, postural tremor
MRI: hot cross bun sign due to cerebellar and pontine atrophy

30
Q

Describe cortico-basal degeneration

A 
Rigidity and weakness in one limb
Myoclonus 
Dementia
Apraxia - loss of purposeful movements
Sensory disturbance, with severe cases causing alien limb phenomenon
31
Q

Describe vascular Parkinson’s

A

Caused by multiple small infracts
Predominately affects lower limbs
Associated with prominent gait disturbance
Pyramidal signs and additional brain dysfunction seen due to infarcts
Structural brain imaging for diagnosis

32
Q

Describe dementia with Lewy bodies

A

Cognitive decline with visual hallucinations
Differentiated based on onset of cognitive decline before the symptoms of Parkinson’s and not the other way around if the decline is caused by actual Parkinson’s

33
Q

Describe drug-induced Parkinson’s

A

Responsible drugs: metoclopramide, antipsychotics, amiodarone
Drugs block the action of dopamine
Symmetrical Parkinsonism
Series of events important - symptoms emerging after drug exposure
Improvement/resolution within a few months of complete drug withdrawal

34
Q

Describe Wilson’s disease

A

Hepatic and renal failure
Behavioural changes and cognitive decline
Keyser fisher rings in the eyes
Diagnosed by checking ceruloplasmin level

35
Q

Describe fragile X-tremor ataxia syndrome

A

Late-onset (>50 years)
Cerebellar signs, dyssutonomia, cognitive decline of frontal type, peripheral neuropathy, Parkinsonism
Slow progression
Milder symptoms in females with permutation - premature ovarian failure and menopause
MRI shows hyper intensities in middle cerebellar peduncles (MCP)
Molecular testing

36
Q

What is a tremor?

A

Rhythmic wave-like oscillations of a body part

37
Q

What are the types of tremor?

A

Resting
Postural
Intention

38
Q

What are the features of a resting tremor?

A 
When muscles relaxed
Slow
Asymmetrical
Abolished by movement
Made worse by distraction
Re-emergent
39
Q

What can cause a resting tremor?

A

Parkinson’s

40
Q

What are the features of a postural tremor?

A

Absent at rest, present on maintained posture
May persist during movement but key - doesn’t get worse with movement
Variable characteristics

41
Q

What are causes of a postural tremor?

A 
Anxiety
Alcohol withdrawal
Benign essential tremor
Thyrotoxicosis
Drugs (beta agonists - salbutamol, caffeine, lithium, valproate, tricyclics)
42
Q

What is a benign essential tremor?

A

Autosomal dominant
Bilateral, fast, low amplitude tremor
Symptoms improve with alcohol consumption

43
Q

What is the character of a thyrotoxicosis tremor?

A

Postural

Bilateral, symmetrical, fast

44
Q

What are the features of an intention tremor?

A

Task orientated tremor

Made worse throughout range of movement

45
Q

How does a cerebellar intention tremor present, and how is it assessed?

A

Gets worse as hand moves towards target

Assessed by finger nose test

46
Q

What are causes of an intention tremor?

A 
Cerebellar disease (MS, stroke, haemorrhage)
Wilson's disease
47
Q

What is dystonia?

A

Involuntary, sustained muscle spasms that result in abnormal posturing and repetitive movements in the context of an associated tremor
Movements are typically patterned, twisting, tremulous
Often worsened by voluntary action

48
Q

What is the difference between focal and generalised dystonia?

A

Focal - limited to single muscle groups

Generalised - occur all over the body but typically start in one limb and then spread

49
Q

What are examples of focal dystonias?

A 
Cervical neck: torticollis
Eyes: blepharospasm (rapid blinking) and forced eye closure
Jaw: clenching of the jaw
Vocal cords: coarse, strained voice
Upper and lower limbs
50
Q

What are causes of focal dystonias?

A 
Cervical: muscle strain, OA
Eyes: myasthenia, dry eyes
Jaw: myasthenia, TMJ dysfunction
Vocal cords: laryngitis, polyps
Limbs: overuse, nerve entrapment
51
Q

What are causes of generalised dystonias?

A

Genetic: dopa responsive dystonia, primary torsion dystonia

Drug induced: levodopa, anti psychotics, metoclopramide/Cyclizine

52
Q

What is the presentation of drug-induced dystonia?

A

Torticollis
Trismus (jaw clenching)
Oculogyric crisis (eyes drawn up)

53
Q

What is the management of dystonia?

A

Anti-spasmodics: Bacolfen
Botox (focal dystonia)
Anti cholinergic: Procyclidine (acute dystonia)

54
Q

What is chorea?

A

Non-rhythmical irregular purposeless movements that flit and flow from one body part to another

55
Q

What are causes of chorea?

A

Infection: HIV
Neurological: Parkinson’s
Genetic: Wilson’s, Huntington’s
Autoimmune: SLE, anti-phospholipid, Sydenham’s
Metabolic: thyrotoxicosis, sodium or magnesium disturbance
Drugs: anti psychotics, anti convulsants, levodopa, OCP

56
Q

What is Huntington’s Disease?

A

Autosomal dominant neurodegenerative disease associated with CAG expansion in chromosome 4, which results in the production of a neurotoxin

57
Q

What is the penetrance for Huntington’s Disease?

A

100%

58
Q

What is meant by Huntington’s Disease showing ‘acceleration’?

A

Gets worse as it is passed down though the generations

59
Q

Who presents with Huntington’s Disease?

A

Usually 4th decade (although age of onset variable)

M=F

60
Q

What is the presentation of Huntington’s Disease?

A

Triad: emotional, cognitive, motor disturbance
Early: clumsiness, chorea, irritability, agitation, depression
Late: myoclonus, rigidity, fits, cognitive decline, dementia, loss of ability to speak or swallow

61
Q

How is Huntington’s Disease diagnosed?

A

Genetic testing

62
Q

What is the management of Huntington’s Disease?

A

Supportive only

63
Q

What is the prognosis of Huntington’s Disease?

A

Average lifespan after diagnosis is 15 years

64
Q

What is Sydenham’s chorea?

A

Self limiting chorea that is usually seen in children

Develops after a strep throat infection

65
Q

What is ballism?

A

Extreme variant of chorea
Particularly involves proximal joints resulting in large amplitude flinging movements
Most often in a hemi-body distribution (‘Hemibalism/Hemichorea’)
Strongly suggestive of a contralateral lesion typically involving the striatum of subthalamic nucleus

66
Q

What is myoclonus?

A

Sudden, involuntary focal or generalized muscle jerks

67
Q

What is defined as abnormal myoclonus?

A

Over 5 muscle jerk movements

68
Q

What are causes of myoclonus?

A

Genetic: benign essential myoclonus
Neurological: stroke, head injury, SOL
Infection: CJD (dementia, ataxia, myoclonus)
Drugs: levodopa, anti convulsants, alcohol withdrawal
Metabolic dysfunctions: liver failure, renal failure, hyponatremia

69
Q

What are tics?

A

Involuntary stereotyped movements or vocalizations

Unable to be suppressed and attempts to suppress cause anxiety and discomfort, which is relieved upon completing the tic

70
Q

What is the main primary tic disorder?

A

Tourette’s

71
Q

What are causes of secondary tic disorders?

A

Autism
Huntington’s
Wilson’s
Haemochromatosis

Y3 - Neuro (14 decks)

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