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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
What are Parkinsonian disorders?
Word used to describe a cluster of disorders associated with symptoms of idiopathic Parkinson’s but have a defined underlying cause
26
How are Parkinsonian disorders differentiated from actual Parkinson's?
``` Symmetrical presentation Absence of tremor Levodopa unresponsiveness Fast progression Sensory disturbance Pyramidal signs Early falls ```
27
What are examples of Parkinsonian disorders?
``` 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 ```
28
Describe progressive supranuclear palsy
``` Vertical gaze palsy Lack of tremor Early postural instability and falls Speech and swallowing problems Symmetrical truncal rigidity more prominent than limb rigidity ```
29
Describe multi-system atrophy
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
Describe cortico-basal degeneration
``` Rigidity and weakness in one limb Myoclonus Dementia Apraxia - loss of purposeful movements Sensory disturbance, with severe cases causing alien limb phenomenon ```
31
Describe vascular Parkinson's
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
Describe dementia with Lewy bodies
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
Describe drug-induced Parkinson's
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
Describe Wilson's disease
Hepatic and renal failure Behavioural changes and cognitive decline Keyser fisher rings in the eyes Diagnosed by checking ceruloplasmin level
35
Describe fragile X-tremor ataxia syndrome
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
What is a tremor?
Rhythmic wave-like oscillations of a body part
37
What are the types of tremor?
Resting Postural Intention
38
What are the features of a resting tremor?
``` When muscles relaxed Slow Asymmetrical Abolished by movement Made worse by distraction Re-emergent ```
39
What can cause a resting tremor?
Parkinson's
40
What are the features of a postural tremor?
Absent at rest, present on maintained posture May persist during movement but key - doesn't get worse with movement Variable characteristics
41
What are causes of a postural tremor?
``` Anxiety Alcohol withdrawal Benign essential tremor Thyrotoxicosis Drugs (beta agonists - salbutamol, caffeine, lithium, valproate, tricyclics) ```
42
What is a benign essential tremor?
Autosomal dominant Bilateral, fast, low amplitude tremor Symptoms improve with alcohol consumption
43
What is the character of a thyrotoxicosis tremor?
Postural | Bilateral, symmetrical, fast
44
What are the features of an intention tremor?
Task orientated tremor | Made worse throughout range of movement
45
How does a cerebellar intention tremor present, and how is it assessed?
Gets worse as hand moves towards target | Assessed by finger nose test
46
What are causes of an intention tremor?
``` Cerebellar disease (MS, stroke, haemorrhage) Wilson's disease ```
47
What is dystonia?
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
What is the difference between focal and generalised dystonia?
Focal - limited to single muscle groups | Generalised - occur all over the body but typically start in one limb and then spread
49
What are examples of focal dystonias?
``` 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
What are causes of focal dystonias?
``` Cervical: muscle strain, OA Eyes: myasthenia, dry eyes Jaw: myasthenia, TMJ dysfunction Vocal cords: laryngitis, polyps Limbs: overuse, nerve entrapment ```
51
What are causes of generalised dystonias?
Genetic: dopa responsive dystonia, primary torsion dystonia | Drug induced: levodopa, anti psychotics, metoclopramide/Cyclizine
52
What is the presentation of drug-induced dystonia?
Torticollis Trismus (jaw clenching) Oculogyric crisis (eyes drawn up)
53
What is the management of dystonia?
Anti-spasmodics: Bacolfen Botox (focal dystonia) Anti cholinergic: Procyclidine (acute dystonia)
54
What is chorea?
Non-rhythmical irregular purposeless movements that flit and flow from one body part to another
55
What are causes of chorea?
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
What is Huntington's Disease?
Autosomal dominant neurodegenerative disease associated with CAG expansion in chromosome 4, which results in the production of a neurotoxin
57
What is the penetrance for Huntington's Disease?
100%
58
What is meant by Huntington's Disease showing 'acceleration'?
Gets worse as it is passed down though the generations
59
Who presents with Huntington's Disease?
Usually 4th decade (although age of onset variable) | M=F
60
What is the presentation of Huntington's Disease?
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
How is Huntington's Disease diagnosed?
Genetic testing
62
What is the management of Huntington's Disease?
Supportive only
63
What is the prognosis of Huntington's Disease?
Average lifespan after diagnosis is 15 years
64
What is Sydenham's chorea?
Self limiting chorea that is usually seen in children | Develops after a strep throat infection
65
What is ballism?
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
What is myoclonus?
Sudden, involuntary focal or generalized muscle jerks
67
What is defined as abnormal myoclonus?
Over 5 muscle jerk movements
68
What are causes of myoclonus?
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
What are tics?
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
What is the main primary tic disorder?
Tourette's
71
What are causes of secondary tic disorders?
Autism Huntington's Wilson's Haemochromatosis

Y3 - Neuro (14 decks)

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