Movement disorders and Parkinson's Flashcards

1
Q

Ballismus

  • Description
  • Pathophysiology
  • Cause
A

High amplitude, flailing limbs.

Hemiballismus- on one side of the body

Pathophysiology

  • Disruption in the subthalamic nucleus
  • Internal globus pallidus is not excited so direct pathway is promoted

Cause
- Stroke

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2
Q

Tics

  • Description
  • Exacerbating and relieving factors
A

Short, repetitive movements with premonitory urge.

Simple movements
- Blinks, coughing

Complex
- Jumping, twirling

Plus (with simple/ complex)
- Motor disorder

Coprolalia [rare]
- Swearing

Exacerbated by:

  • Anxiety
  • Fatigue

Reduced by:

  • Distracting
  • Concentration
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3
Q

Tourette’s syndrome

A

Severe expression of a spectrum of tic disorders

Composed of multiple motor tics and at least one phonic tic

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4
Q

Causes of tics

A

Co-morbidity

  • ADHD [50%]
  • OCD [1/3]
  • Anxiety [up to 50%]

Genetic inheritance
- Involves many genes

Post-infectious immune response

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5
Q

Chorea

- Description

A

Jerky, brief, IRREGULAR contractions

  • Not repetitive
  • Not rhythmic

Flows from one muscle to the next
- Fidgety, restless

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6
Q

Chorea

  • Pathophysiology
  • Causes
A

Disruption in the subthalamic nucleus
- Promotes the direct pathway and inhibits indirect pathway

Cause

  • Huntington’s disease [degenerative]
  • Neuroleptics [antipsychotics]
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7
Q

Huntington’s chorea

- Genetics

A

Autosomal dominance with complete penetrance [ clinical symptoms are present in all individuals affected by the disease]

Trinucleotide repeat

  • Chromosome 4
  • Longer repeat sequence = the earlier the disease presents

Repeat sequence tends to enlarge with each generation

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8
Q

Huntington’s chorea

- Clinical presentation [cognitive, behavioural, physical]

A

Cognitive

  • Inability to make decisions
  • Inability to mutli-task
  • Slow in thought

Behavioural

  • Irritable
  • Depressed
  • Apathic
  • Anxiety
  • Delusions

Physical

  • Chorea
  • Motor persistence [i.e protruding tongue and withdrawing unwilling]
  • Dystonia
  • Eye movements
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9
Q

Myoclonus

  • Description
  • Causes
A

Twitching of a muscle or a group of muscles
- Rapid onset and offset

Postive = muscular contractions
Negative = muscular inhibitions 

Causes:

  • JME
  • Brain hypoxia
  • Prion disease [CJD]
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10
Q

Myoclonus

- Pathophysiology

A

Not fully understood

Suggested
1. Imbalance between excitatory and inhibitory neurotransmitters

  1. Deviation of the motor control system, causing a brief equilibrium
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11
Q

Dystonia

  • Description
  • Causes
A
Abnormal twisting posture
- Axial
- Facial
- Truncal
Jerky tremor

Causes:

  • Stroke
  • Brain injury
  • Encephalitis
  • PD
  • HD
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12
Q

Dystonia

- Pathophysiology

A

Not well understood

  1. Abnormal activity in:
    - Motor cortex + supplementary areas
    - Cerebellum
    - Basal ganglia
  2. Abnormal dopaminergic activity in basal ganglia
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13
Q

Tremor

  • Action
  • Body parts affect
  • Classifications
A
  • Involuntary
  • Rhythmic
  • Alternating movements of part of the body

Parts affected:

  • Limbs
  • Head
  • Chin
  • Soft palate

Can occur at rest, at a particular body position [postural] or when performing an action [kinetic]

Most common
- Essential tremor

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14
Q

Tremor

- Pathophysiology

A

Caused by increased activity in cerebellothalamocortical circuit.

In PD
- Dopamine dysfuntion in the pallidum

In essential tremors
- GABAergic dysfunction in cerebellum

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15
Q

MRI focussed ultrasound therapy

A

Treatment method for resistant essential tremors

- Used for PD

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16
Q

Drug treatments for tics, chorea and ballismus

A

D2 receptor blocking agents

Dopamine depleting agents

Atypical antipsychotics

17
Q

Problems caused by D2 blocking agents

A

Affects the basal ganglia

Acute:

  • Oculogyric crisis
  • Neuroleptic malignant syndrome

Subacute
- Drug induced Parkinsonism

Long term
- Tardive dyskinesias

18
Q

Oculogyric crisis

A

Prolonged, involuntary upward deviation of the eyes

  • Neck + trunk extension
  • Jaw spasm with or without tongue protrusion

Acute side effect of neuroleptics and other D2 blockers

19
Q

Neuroleptic malignant syndrome

A

Acute reaction to D2 blocking drugs

Features:

  • Rigid muscles due to Raised creatine phosphokinase
  • Fever
  • Autonomic instability
  • Confusion
20
Q

Tardive dyskinesia

A

Long term side effect of D2 blocking agents.

  • Choeric oral/facial movements
  • Dystonic trunk

Mechanism
- Dopamine sensitivity of basal ganglia ?

Treatment:

  • Gradually withdrawal drug
  • Substitution with atypcial anti-psychotic
  • Dopamine depleting agent [tetrabenazine]
21
Q

Parkisonism

A

Hypokinetic movement disorders

Symptoms:

  • Slow
  • Stiff
  • Shaky

Signs:

  • Bradykinesia
  • Akinesia
  • Rest tremor
  • Rigidity
22
Q

Non motor Parkinsonism symptoms

A

Mood:
- Depression, anxiety

Dementia

Autonomic:

  • Postural hypotension
  • Hypersalivation

Sleep disturbance

  • Restless legs
  • REM parasomnia

Reduced sense of smell

23
Q

Parkinson’s disease pathophysiology

A

Destruction of cells in substantial nigra

  • Decreased DA input into the striatum
  • Reduced activation of direct pathway
  • Reduced inhibition of indirect pathway
24
Q

Histopathological hallmark of PD

A

Lewy bodies

- Abnormal accumulation of proteins inside nerve cells

25
Parkinsonism causes
Neurodegenerative - Idiopathic [PD], over 80% - Diffuse Lewy body disease - Atypical parkinsonism Drugs CVD Hydropcephalus Toxicity Genetic - Wilson's disease [copper deposits] - Familial causes
26
MAO inhibitors
Type A: Prevents breakdown of - Serotonin - Adrenaline - NADR - DA Type B - Specific to DA Non selective, used for: - Depression - Has problems with metabolising dietary amines/ tryptophans Selective [Type B] used for: - PD
27
L dopa
Precursor of dopamine Sold as: Madopar, sinemet Combine with dopa decarboxylase inhibitor to prevent peripheral conversion to dopamine
28
Amantadine
Early PD drug treatment | - Glutamate agonist [initially anti-flu agent]
29
Anti-cholinergics and PD - Side effect - Use - Examples
Originally used to treat and help with tremors Limited by side effects - Confusion - Urinary retention - Dry mouth Examples: - Procyclidine - Benzhexol
30
Entacapone/ Tolcapone
COMT inhibitor - Reduces peripheral metabolism of L-dopa Pros - Increases L-dopa duration of action and efficacy Cons: - Can make dyskinesia worse - Diarrhoea - Liver disease [tolcapone]
31
Duodenal L-dopa infusion [Duodopa]
Method of administering L-dopa to the duodenum via infusion pump Pros - Can manage motor fluctuations Cons: - Expensive - Does not affect disease progression - Can be affected by gastric problems - Unpredictable bioavailability [hard to hit narrow therapeutic window]
32
DA agonists - Ergot - Non-ergot - Cons
Can bypass nigrostriatal neurones Ergot derived no longer used due to cardiac/ pulmonary fibrosis - Pergolide - Carbergoline Non-ergot: - Pramipexole - Ropinirole - Rotigotine [patch] - Apomorphine [Subcutaneous] Cons: - Dopamine dysregulation syndrome
33
Deep brain stimulation
High frequency stimulation targetted at - STN [PD] - Pallidum [Dystonia] - Thalamus [tremor] Does not prevent progression or have an effect on non-motor symptoms