Neurology - Dyskinesias Flashcards

1
Q

Dyskinesia

A

= Abnormal involuntary movement

Caused by extrapyramidal or cerebellar lesions

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2
Q

Tremor

A

= Regular, rhythmic oscillation

NB Asterexis is not the same, it is rhythmic myoclonus

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3
Q

Types of tremor

A

“RAPID”

  • Resting (e.g. PD)
  • Action/ Postural
  • Intention (e.g. cerebellar)
  • Dystonic
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4
Q

What are the features of resting tremor?

A

4-6Hz pill rolling
Abolished on voluntary movement/ action
Increases with distraction - e.g. counting backwards

Cause - PD (basal ganglia abnormalities manifest at rest)

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5
Q

Features of an action/ postural tremor

A

6-12Hz
Absent at rest
Worse with outstretched hands or movement
Equally bad at all stages of movement

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6
Q

What are the causes of an action/ postural tremor?

A

“BEATS”

  • Benign essential tremor
  • Endocrine - thyrotoxicosis, decr. glucose, phaeo
  • Alcohol withdrawel
  • Toxins - beta agonists, theophyline, valproate
  • Sympathetic - physiological tremor may be enhanced e.g. anxiety
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7
Q

Features of an intention tremor

A

Irregular, large amplitude
Worse at the end of movement - e.g. past pointing

Cause - cerebellar damage

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8
Q

Important features of benign essential tremor

A
Autosomal dominant 
Occurs with action and worse with anxiety, emotion etc
Arms, neck and voice
Doesn't occur during sleep 
Better with alcohol
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9
Q

What is myoclonus?

A

= Sudden involuntary jerks

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10
Q

Causes of myoclonus

A

Epilepsy (i.e. juvenile myoclonic epilepsy, infantile spasms)
Neurodegenerative diseases
CJD - important one to remember!

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11
Q

Chorea

A

= Irregular random movements that have a flowing/ purposeful quality
Any part of the body can be affected

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12
Q

Causes of chorea

A
Hereditary (e.g. Huntington's) 
Post infectious (Sydenhams chorea is associated with rheumatic fever)
Polycythaemia rubra vera
SLE
Wilson's disease
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13
Q

What are tics?

A

Type of dyskinesia
= Rapid, compulsive, repetitive stereotyped movement s
Also referred to as “habit spasms”
Associated with involuntary utterances in Tourette’s syndrome

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14
Q

Hemiballismus

A

= Violent, jerky movements typically restricted to one side of the body
Caused by damage to the contralateral sub thalamic nucleus (often elderly diabetics)
Resolves spontaneously

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15
Q

Athetosis

A

= Slow, within movements often seen with congenital brain damage in cerebral palsy

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16
Q

What is the difference between physiological and pathological tremor?

A

Physiological tremor - appears when maintaining a fixed posture, fine character

Pathological tremor - occurs at rest or movement (action), slow rate and course character

17
Q

How should a tremor be examined?

A

Observe the tremor at rest (e.g. PD)
Observe on maintaining posture (e.g. physiological or postural)
Observe on movement and at the end of movement - finger nose pointing (e.g. cerebellar)

18
Q

What is dystonia?

A

= Prolonged muscle contracture causing unusual joint posture or repetitive movements

19
Q

How are dystonia’s classified?

A

By distribution:

  • Generalised
  • Focal - limited to one area of the body

By aetiology:

  • Primary - often genetic involvement
  • Secondary - e.g. drugs, metabolic disorders etc
20
Q

Idiopathic generalised dystonia

A

Autosomal dominant
Childhood onset
Begins in one leg then spreads to that side of the body over 5-10 years

21
Q

Idiopathic focal dystonia

A
Most common form of dystonia 
Affects one part of the body
Worsened by stress
Types:
- Spasmodic torticolis (cervical dystonia)
- Writers cramp
- Oromandibular dystonia
22
Q

Negative features of extrapyramidal disease

A

Hypokinetic features of extrapyramidal disease

  1. ) Bradykinesia - loss or slowness of voluntary movement
  2. ) Postural disturbance - flexion of limbs and trunk is associated with a failure to make quick postural “righting” adjustments to correct balance
23
Q

Positive features of extrapyramidal disease

A

Hyperkinetic features of extrapyramidal disease

  1. ) Abnormal involuntary movements - “Dyskinesia”
  2. ) Rigidity
24
Q

What is the definition of rigidity?

A

Velocity independent increase in tone
Affects flexor and extensor muscle groups equally
Also called “Lead pipe rigidity” - indicates extrapyramidal disease

25
Cog wheel rigidity
Tremor superimposed on rigidity
26
Tardive dyskinesia
= Delayed onset ("tardive") following chronic exposure to Da agonists (e.g. antipsychotics, anti-emetics, L-DOPA) Da receptors become super-sensitive
27
How is tardive dyskinesia treated?
1) Using new atypical antipsychotics | 2) Early recognition and drug withdrawal
28
Wilson's disease
(Hepatolenticular degeneration) | Autosomal recessive disorder - build up of copper in the lentiform nucleus (= globus pallidus + putamen)
29
What is the underlying biochemical defect in Wilson's disease?
Deficiency of alpha 2 globulin - Ceruloplasmin - normally binds 98% of plasma copper and transfers to a copper enzyme Increase in loosely bound copper/ albumin and organ deposition (esp liver and brain)
30
Acute form of Wilson's disease
``` There are 2 clinical forms of Wilson's disease Acute: 1) Bradykinesia 2) Behavioural change 3) Involuntary movements 4) Liver involvement common ``` HIGH mortality rate if untreated (death in approx 2 years)
31
Chronic form of Wilson's disease
Proximal "wing beating" tremor Dysarthria, dystonia, and rigidity Psychosis and behavioural disorders Liver involvement is less severe
32
How are neurotransmitter substances altered in extrapyramidal disease?
Dopamine - inhibitory ACh - excitatory Extrapyramidal disease involves imbalance of these 2 neurotransmitters: - Dopamine deficiency or ACh excess - akinetic rigid syndrome - Dopamine excess or ACh deficiency - involuntary movements (i.e. dyskinesia)