Dystonia Flashcards

1
Q

What is dystonia?

A

Movement disorder characterized by sustained or intermittent muscle contraction causing abnormal, often repetitive movements, postures or both

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2
Q

How do dystonic movements present?

A

Patterned
twisting
tremulous
sustained

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3
Q

What is dystonia initiated or made worse by?

A

Initiated or made worse by voluntary action, stress, fatigue and associated with overflow muscle activation

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4
Q

What was the former classification system?

A

Age of onset
Distribution
Etiology

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5
Q

What are the 4 clinical characteristics of Dystonia on the classification?

A

Age
body distribution
Temporal Pattern (static/progressive, variability)
Associated Features

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6
Q

How are the etiologies classified for dystonia?

A

Nervous system pathology
Inherited vs. acquired
Idiopathic

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7
Q

What is focal dystonia?

A

Single body part

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8
Q

What is Segmental dystonia?

A

Contiguous body parts

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9
Q

What is Multifocal Dystonia?

A

Non-contiguous body parts

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10
Q

What is generalized dystonia?

A

entire body

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11
Q

What is hemi-dystonia?

A

Dystonia on half the body

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12
Q

When is the peak of generalized dystonia?

A

~10 yrs.

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13
Q

What is the age distribution of Focal dystonia?

A

Goes from 20-70 with peak in middle age around 30-40

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14
Q

What is the distribution of segmental dystonia?

A

Bimodal with peaks at 9/10 and 40

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15
Q

Prevalence of early onset (<28) primary dystonia?

A

2-50 cases per million

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16
Q

Prevalence of late onset (>28) primary dystonia?

A

30-7320 cases per million

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17
Q

Specific types of focal dystonia?

A
Cervical dystonia
Blepharospasm
Oromandibular dystonia
Writers Cramp dystonia
Limb (LE) dystonia
DYT1 idiopathic torsion dystonia
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18
Q

What type of changes on imaging do you see with idiopathic genetic dystonia?

A

No changes on standard imaging and no underlying injury or disease

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19
Q

Damage to which areas can cause an acquired dystonia?

A
Basal ganglia
thalamus
Brainstem
cerebellum
Cortex
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20
Q

What type of injuries can cause an acquired dystonia?

A

Injury to nervous system

  • stroke
  • TBI
  • limb injury
21
Q

What type of degenerative disorder can cause an acquired dystonia?

A

Parkinson’s disease

22
Q

What do you see happen to the basal ganglia on imaging with a dystonia?

A

Volumetric enlargement of the basal ganglia

23
Q

What happens to the grey matter density on imaging to ts with dystonia?

A

Increased grey matter density on primary sensory cortex

24
Q

What happens to the metabolic activity in the brain on imaging with dystonia?

A

Increased metabolic activity in sensory and motor cortex during motor tasks

25
Q

What happens to Dopamine receptors in pts with dystonia on imaging?

A

Altered D2 dopamine receptor level

26
Q

What happens to the intracortical inhibition in the sensory cortex with dystonia on imaging?

A

It is impaired

27
Q

What happens to surround inhibition with dystonia?

A

Surround inhibition is reduced

28
Q

What do you need to get dystonia if you have a genetic predisposition?

A

You need an environmental trigger

29
Q

What is the basal ganglia consisted of?

A

Interconnected subcortical nuclei

30
Q

What is the “general” job of the Basal ganglia?

A

Fundamental processing units of the brain

31
Q

What are some hypokkinetic movement disorders?

A

Parkinson’s disease

Stiffness syndromes

32
Q

What are some hyperkinetic movement disorders?

A
Dystonia
Huntington's Disease
Chorea, ballism, athetosis
Tics, tourettes syndrome
Myoclonus
33
Q

What is surround inhibition?

A

Basal Ganglia inhibits competing motor programs so only desired motor program is generated

34
Q

What are symptoms of basal ganglia disorders?

A

Inability to initiate movement
INability to suppress involuntary movement
Abnormalities in velocity and amplitude of movement
abnormal muscle tone

35
Q

What link is abnormal in dystonia?

A

Abnormal links between sensory inputs and motor outputs

36
Q

What type of movements can dystonia be associated with?

A

Highly stereotyped, over-learned movements

37
Q

What are the two complimentary but opposing forces of two distinct motor systems?

A

Posture and Movement

38
Q

What is the definition of postural control?

A

The ability to control ones position in space for the dual purpose of stability and orientation

39
Q

What is the definition of Orientation?

A

(Posture) The ability to maintain an appropriate relationship between the body segments and between ones body and the enviroment for a task

40
Q

What is the definition of stability?

A

(Balance) The ability to control the COM in relation to the base of support

41
Q

What does the tonic postural program do?

A

Maintenance of baseline muscle tone and body shape

42
Q

What does the phasic postural control program do?

A

Orientation in space
Maintains balance during static states and during movements
Orienting the body/parts during movement

43
Q

What is neuroplasticity?

A

The brain reorganizing by forming new neural connections throughout life
can be positive or negative

44
Q

What do you see with dystonia in Chronic regional pain syndrome (LE)?

A

Marked DF< inversion, knee flexion, hip flexion and ER

45
Q

What type of posture do you see with Parkinson’s plus syndromes?

A

Excessive lateral trunk flexion
Excessive cervical flexion
Excessive trunk flexion

46
Q

How does post traumatic dystoina present?

A

Presents after injury

is often fixed and less responsive to treatment

47
Q

What is paraneoplastic syndrome?

A

Rapid onset focal dystonia occuring in a non-contiguous body part

48
Q

What type of medications are used to treat

A
Denzodiazepines
Sinemet (dopa responsive)
Trihexyphenidyl
Tetrabenazene
Botox injections
49
Q

Where are DBS used for dystonia?

A

Globus pallidus internus