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Yr4: Neurology & Special Senses > Neurology: Movement Disorders > Flashcards

Neurology: Movement Disorders Flashcards

1
Q

What is Huntington’s chorea/Huntington’s disease?

A

Inherited, progressive neurodegenerative condition characterised by chorea, incoordination, cognitive decline, personality changes and psychiatric symptoms.

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2
Q

For Huntington’s chorea, discuss:

  • Inheritance pattern
  • Type of mutation
  • Specific mutation
A
  • Autosomal dominant
  • Trinucleotide repeat
  • Mutation in the HTT (Huntington) gene on chromosome 4 (causes CAG repeats)
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3
Q

Huntington’s chorea shows anticipation; explain what is meant by anticipation

A
  • Anticipation is a feature of trinucleotide repeat disorders
  • Successive generations have more repeats in the gene resulting in:
    • Earlier age of onset
    • Increased severity of disease
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4
Q

Describe the pathophysiology of Huntington’s Disease

A
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5
Q

At what age does Huntington’s chorea/disease typically present?

A
  • HD typically begins between ages 30 and 50
  • An earlier onset form called juvenile HD occurs under age 20
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6
Q

Describe typical presentation of Huntington’s chorea

A

Symptoms gradually progress/worsen over time:

  • Often starts with:
    • Cognitive impairment
    • Psychiatric
    • Mood problems/personality change (irritability, impulsivity, apathy, depression)
  • Then get development of movement disorders:
    • Chorea
    • Impaired coordination
    • Eye movement disorders (slow saccades with pt often turning head to compensate)
    • Dysarthria
    • Dysphagia
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7
Q

What is chorea?

A

Chorea is characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.

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8
Q

How is Huntington’s chorea diagnosed?

A

Testing for defect in Huntington gene on chromosome 4 (including pre- and post-test counselling)

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9
Q

Discuss the management of Huntington’s chorea

A

No treatments to slow or stop progression. Management is via MDT and centred around managing symptoms & supporting person and their family.

Supporting Pt and Family

  • Involvement of physio, OT, SALT to help improve quality of life/allow them to adapt to life as best as possible
  • Genetic counselling
  • Advanced care planning (including EOL care planning)

Medical treatment

  • Suppression of disordered movement:
    • Antipsychotics
    • Benzodiazepines
    • Dopamine depleting agents
  • Depression:
    • Antidepressants
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10
Q

Discuss the prognosis of Huntington’s chorea

A
  • Progressive
  • Life expectancy of 15-20yrs after onset of symptoms
  • Death often due to respiratory disease (e.g. pneumonia); as disease progresses pts more susceptible to illness/infection
  • Suicide is more common in comparison to general population
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11
Q

What is Parkinson’s disease?

A

Progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra resulting in reduced dopamine in the basal ganglia. This results in a classic triad of features: bradykinesia, tremor and rigidity. The symptoms of Parkinson’s disease are characteristically asymmetrical.

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12
Q

Describe pathophysiology of Parkinson’s disease

A
  • Degeneration of dopaminergic neurones in substantia nigra pars compacta
  • Leads to gradual, progressive decrease in dopamine production
  • Basal ganglia require dopamine to function correctly
  • Hence, reduced dopamine results in reduced functioning of basal ganglia
  • Basal ganglia responsible for coordinating habitual movements (e.g. walking), controlling voluntary movements and learning specific movement patterns

Direct pathway excitatory on thalamus, indirect pathway inhibitory on thalamus. Direct pathway facilitates appropriate movements (it is excitatory and indirect pathway inhibits inappropriate movements. Dopamine is excitatory on direct pathway (so stimulates appropriate movements) and is inhibitory on indirect pathway (but two negatives make a positive). Hence, overall effect is excitation of cortex.

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13
Q

State some risk factors for Parkinson’s disease

A
  • Male (Male: female is 2:1)
  • Increasing age
  • FH
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14
Q

What is the classic triad of features in Parkinson’s disease?

A
  • Bradykinesia
  • Rigidity
  • Resting tremor
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15
Q

Describe typical presentation of Parkinson’s disease (including symptoms & signs)

A

Symptoms/signs are often asymmetrical (one side worse than other):

  • Resting tremor
  • Lead pipe rigidity & cogwheel rigidity (due to superimposed tremor)
  • Bradykinesia which manifests as:
    • Typical parkinsonian gait (flexed, slow to start, shuffling, reduced arm swing, turn on block)
    • Hypomimia
    • Micrographia
    • Hypophonia
  • Other features:
    • Sleep disturbance (insomnia)
    • Depression
    • Cognitive impairment & memory problems
    • Impaired sense of smell/anosmia
    • Fatigue
    • Postural hypotension
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16
Q

State some characteristics of Parkinson’s gait

State some characteristics of Parkinson’s tremor

A

Parkinson’s gait

  • Slow to start
  • Stooped
  • Reduced arm swing
  • Shuffling
  • Turn on block

Parkinson’s tremor

  • Resting (worse on rest, better with activity. Can exaggerate tremor by getting them to do task with other hand e.g. mimic painting a fence)
  • Pill rolling
  • 4-6Hz
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17
Q

People with suspected Parkinson’s should be referred urgently to specialist for diagnosis and treatment; true or false?

A

True; NICE recommends that they are seen URGENTLY to reduce the chances of complications, and initiate management that will reduce the impact on the individuals life.

18
Q

Compare Parkinson’s tremor with benign essential tremor

A
19
Q

For benign essential tremor, discuss:

  • How common it is
  • Whether it is genetic
  • Presentation
  • Specific features of tremor
  • Management
A
  • Most common type of tremor. Is associated with older age.
  • Genetic- many cases thought to be autosomal dominant
  • Fine tremor affecting voluntary muscles- usually in upper limbs (70%) but may also involve head and speech
  • Features:
    • Fine tremor
    • Symmetrical
    • More prominent on voluntary movement
    • Worse if arms outstretched, tired, stressed or caffeine
    • Improved by alcohol, rest & benzodiazepines
  • Management:
    • First line if affecting life (functionally, psychologically etc…)= propranolol
    • Alternative= primidone
20
Q

Other than Parkinson’s disease, state some other causes of Parkinsonism

A
  • Drug induced **CAUSES SYMMETRICAL SYMPTOMS UNLIKE IDIOPATHIC
    • Antipsychotics
    • Metoclopramide
    • Prochlorperazine
  • Vascular parkinsonism
  • Creutzfeldt-Jakob disease
  • Wilson disease
  • Parkinson plus syndromes:
    • Multisystem atrophy (parkinsonism + cerebellar signs + autonomic dysfunction)
    • Lewy body dementia (parkinsonism + deteriorating cognitive function + hallucinations + fluctuating course)
    • Progressive supranuclear palsy (parkinsonism + speech disturbance + personality change + vertical gaze palsy)
    • Corticobasilar degeneration (parkinsonism + alien limb syndrome + apraxia + aphasia)
  • ***Don’t need to know details of Parkinson plus syndromes just know they exist.*
  • ***Improvement with treatment will distinguish IPD from other causes of Parkinsonism*
21
Q

How is Parkinson’s disease diagnosed?

A
  • Clinical diagnosis
  • NICE recommend using the UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria

*SPECT or DaTSCAN can be used if unsure of diagnosis

22
Q

What do patients & clinicians mean when they describe patients with Parkinson’s disease as ‘on’ and ‘off’

A
  • On= moving freely (and so medications working)
  • Off= not moving freely (so medications not working or wearing off before next dose)
23
Q

State 2 staging systems for IPD

A
  • Heohn and Yahr Scale (see image)
  • UPDRS (unified Parkinson’s disease rating scale)
    • Four parts each with multiple parts/points that are individually scored 0-4 (4 being worst). Can score 0-199 (199 being worst). Parts are:
      • 1= intellectual function, mood & behaviour
      • 2= ADLs
      • 3= motor examination
      • 4= motor complications
24
Q

We recognise 4/5 different stages of IPD; describe each of these

A

Features of advanced IPD:

  • Unable to complete ADL’s
  • Psychosis
  • Bedridden or need wheelchair
  • Risk of falls/falls
  • Cognitive problems
25
Q

Treatment for Parkinson’s is guided & initiated by specialists; it is tailored to each individual with aim of controlling symptoms and minimising side effects. State some example medications used in Parkinson’s disease (think about which are used first line, second line & third line)

What can be used if medications do not work?

A

First line drugs

  • If motor symptoms having impact on pts life:
    • Levodopa (+ peripheral decarboxylase inhibitor)
  • If motor symptoms not affecting pts QoL:
    • Dopamine agonist
    • MOA-B inhibitor
    • Levodopa (+ peripheral decarboxylase inhibitor)

*Levodopa has reduced effectiveness with time which is why prefer to start it later if we can.

Adjuvant therapy (if continued to have symptoms on L-dopa or developed dyskinesia)

  • Add COMT inhibitor (must always be given with Levodopa not on it’s own)
  • Add MAO-B inhibitor
  • Add dopamine agonist

Third line

  • Amantadine
  • Apomorphine SC (intermittent or continuous)

If medications don’t work:

  • Deep brain stimulation
26
Q

Compare levodopa, dopamine agonists and MAO-B inhibitors in terms of:

  • Effect on motor symptoms
  • Effect on ADLs
  • Motor complications
  • Adverse events
A
27
Q

Compare dopamine agonists, MAO-B inhibitors and COMT inhibitors in terms of:

  • Effect on motor symptoms
  • Effect on ADLs
  • Off time
  • Adverse events
  • Hallucinations
A
28
Q

Explain the mechanism of action of levodopa in Parkinson’s disease

A
  • Levodopa taken up by dopaminergic cells in substantia nigra pars compacta and converted to dopamine (increase dopamine in basal ganglia)
  • Must co-prescribe peripheral DOPA decarboxylase inhibitor to prevent levodopa being converted into dopamine in peripheral tissues
29
Q

Explain the mechanism of action of dopamine agonists in Parkinson’s disease

A

Bind to dopamine receptors and stimulate them

30
Q

Explain the mechanism of action of MAO-B inhibitors in Parkinson’s disease

A
  • Monoamine oxidase B metabolises dopamine
  • Predominantly found in dopamine containing regions in brain
  • Hence, inhibitors of MAO-B can increase dopamine
31
Q

Explain the mechanism of action of COMT inhibitors in Parkinson’s disease

A
  • Catechol-O-methyl transferase breaks down levodopa. COMT acts mainly in periphery. COMT inhibitors inhibit COMT and hence inhibit breakdown of levodopa to increase levodopa
  • In addition, breakdown product formed when COMT breaks down L-dopa competitivley inhibits L-dopa active transport into brain; decreasing the formation of this breakdown product increases L-dopa transport into CNS
32
Q

State some adverse effects of levodopa

A
  • Dystonia: This is where excessive muscle contraction leads to abnormal postures or exaggerated movements.
  • Chorea: These are abnormal involuntary movements that can be jerking and random.
  • Athetosis: These are involuntary twisting or writhing movements usually in the fingers, hands or feet
  • Impulse control disorder
  • Dry mouth
  • Postural hypotension
33
Q

State examples of:

  • Levodopa and peripheral dopa decarboxylase combinations
  • Dopamine agonists
  • MOA-B inhibitors
  • COMT inhibitor
A
  • Levodopa and peripheral dopa decarboxylase combinations:
    • Co-careldopa (levodopa + carbidopa)
    • Co-benyldopa (levodopa + benserazide)
  • Dopamine agonists
    • Bromocriptine
    • Ropinirole
    • Cabergoline
    • Apomorphine
  • MAO-B inhibitors
    • Selegiline
    • Rasagiline
  • COMT inhibitor
    • Entacapone
34
Q

State some adverse effects of dopamine agonists

A
  • Impulse control disorders
  • Drowsiness/sedation
  • N&V
  • Hypotension
  • Psychiatric symptoms e.g. hallucinations

*Less motor ADRs but more other ADRs

35
Q

State some adverse effects of MAO-B inhibitors

A
  • Impulse control disorders
  • N&V
  • Dry mouth
  • Hypotension

ASK ABOUT tyrosine rich foods as MAO usually breaks down tyramine

36
Q

State some adverse effects of COMT inhibitors

A
  • Impulse control disorders
  • Bright red/orange urine
  • Sedation
  • N&V
37
Q

What should you ask patients when starting them on Parkinson’s disease medications?

*HINT: thinking about impulse control disorders

A
  • Hx of previous impulsive behaviours e.g. gambling, collecting etc..
  • Hx of alcohol or smoking
38
Q

What could you prescribe for a patient with Parkinson’s disease suffering with excess secretions?

A

Glycopyrronium bromide

39
Q

Discuss which antiemetics you would and wouldn’t prescribe for a pt with Parkinson’s disease

A

Don’t prescribe:

  • Haloperidol (D2 antagonist)
  • Metoclopramide (D2 antagonist & 5HT3 agonist)
  • Prochlorperazine (can cause tardive dyskinesia, tremor & Parkinson’s symptoms)

Prescribe:

  • Domperidone= antiemetic of choice
  • Other options if can’t give the above:
    • Cyclizine
    • Ondansetron
40
Q

State some potential causes of tremor

A

Yr4: Neurology & Special Senses (28 decks)

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