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
Q

Parkinsonism causes

A

Neurodegenerative

  • Idiopathic [PD], over 80%
  • Diffuse Lewy body disease
  • Atypical parkinsonism

Drugs
CVD
Hydropcephalus
Toxicity

Genetic

  • Wilson’s disease [copper deposits]
  • Familial causes
26
Q

MAO inhibitors

A

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
Q

L dopa

A

Precursor of dopamine

Sold as: Madopar, sinemet

Combine with dopa decarboxylase inhibitor to prevent peripheral conversion to dopamine

28
Q

Amantadine

A

Early PD drug treatment

- Glutamate agonist [initially anti-flu agent]

29
Q

Anti-cholinergics and PD

  • Side effect
  • Use
  • Examples
A

Originally used to treat and help with tremors

Limited by side effects

  • Confusion
  • Urinary retention
  • Dry mouth

Examples:

  • Procyclidine
  • Benzhexol
30
Q

Entacapone/ Tolcapone

A

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
Q

Duodenal L-dopa infusion [Duodopa]

A

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
Q

DA agonists

  • Ergot
  • Non-ergot
  • Cons
A

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
Q

Deep brain stimulation

A

High frequency stimulation targetted at

  • STN [PD]
  • Pallidum [Dystonia]
  • Thalamus [tremor]

Does not prevent progression or have an effect on non-motor symptoms