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Flashcards in Dystonia Deck (32):
1

What is the most common movement disorder

essential tremor

2

What is the second most common movement distorder

parkinson's

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What is the 3rd most common movement disorder

Dystonia

4

Dystonia: definition

sustained muscle contractions causing repetitive twisting movements or abnormal postures during functional movements
*misrepresentation of the body in the brain

5

3 axes of classification

1. etiology/cause (primary, heterotodegenerative, secondary)
2. Age of onset (early, late)
3. Distribution (focal, segmental, multifocal, generalized, hemi-dystonia)

6

3 types of primary dystonias

1. Primary "pure"
2. Primary plus
3. Primary Paroxysmal

7

Primary Pure dystonia

Torsion dystonia is the only clinical sign
No identifiable exogenous cause or other inherited or degenerative disease

8

Primary plus dystonia

Torsion dystonia is a prominent sign, but is associated with another movement disorder (myoclonus, parkinsonism)
No evidence of neurodegeneration

9

Primary Paroxysmal Dystonia

torsion dystonia occurs in brief episodes with normalcy in between
idiopathic

10

3 forms of primary paroxysmal dystonia

1. Paroxysmal kinesigenic dyskinesia (PKD)- attacks induced by sudden movement
2. paroxysmal exercise-induced dystonia (PED)- attacks induced by exercise
3. Non-kinesigenic form (PNKD)- attacks induced by alcohol, coffee, tea, etc

11

Heredodegenerative Dystonias

dystonia is a feature (amongst other neurodegenerative signs)
example = wilson's disease
Both hereditary and degenerative

12

Secondary Dystonias

Dystonia is a symptom of identified neurological condition (brain tumor, focal brain lesion, exposure to drugs and chemicals)

13

Early-Onset dystonia

onset at 20-30yrs (before 30)
starts in single leg or arm, progresses to involve other limbs and the trunk

14

Late onset dystonia

starts in the neck, cranial muscles, or one arm
remains localized with restricted progression to adjacent muscles

15

Focal dystonia

single body segment (writers cramp)

16

Segmental dystonia

Contiguous body regions (cranial and cervical, cervical and upper limb- Cervical dystonia is common)

17

Multifocal dystonia

non-contiguous body regions (upper limb and lower limb, cranial and upper limb)

18

Generalized dystonia

both legs and at least one other body region (usually one or both arms)

19

Hemidystonia

half of the body (usually secondary to a structural lesion in the CL basal ganglia)
Cerebellum --> control of IL side affected
Basal Ganglia --> Control of CL side affected

20

Risk factors of dystonia

1. repetition/excessive practice
2. Poor ergonomics
3. stress
4. trauma/anatomic defects
5. compulsive personality, perfectionists, impatient
6. environment
7. aberrant physiology
8. genetics (susceptibility gene)

21

physiological circuits involved in dystonia

1. Basal ganglia
2. cerebellar
3. SMA (primary motor cortex)
4. Sensory motor cortex

22

Pathogenesis of excessive practice and dystonia

excessive practice --> degradation of cortical and subcortical representation --> loss of sensitivity in distinguishing between sensory stimuli
*loss of sensitivity: excessive/persistent firing of neurons in both sensory and motor cortex independent of the stimuli

23

Can excessive practice lead to dystonia on its own?

No! must be in combination with genetic mutations, environmental causes, and susceptibility genes

24

Graphesthesia

ability to identify objects drawn on your hand with another finger without visual cues

25

Stereognosis

being able to identify objects by using only tactile perceptors and fingers (no visual cues)

26

Components of the neuro exam for dystonia

1. motor exam
2. sensory exam (graphesthesia, stereognosis)
3. evaluation of task performance

27

Fahn-Marsden Movement Scale

one of the most common scales used to evaluate presence of dystonia
looks at provoking and severity factors to grade dystonia

28

Fahn-Marsden Movement Scale: Provoking Factors

0 = none
1 = only with particular action (writer's dystonia)
2 = with many actions
3 = on action of distant part of body or intermittently at rest
4 = at rest

29

Fahn-Marsden Movement Scale: Severity Factor

0 = none present
1 = slight dystonia, clinical insignificance
2 = mild, obvious dystonia, but not disabling
3 = moderate, able to grasp with some manual function
4 = most severe, no functional grasp

30

Factors of successful intervention in dystonia

1. time commitment (~30-60mins, must stop the abnormal movement)
2. Expectation: the pt must expect to get better and recognize it may take up to a year
3. supervision: for watching ergonomics and techniques

31

Practice principles of dystonia

1. activities must be learned based, requiring focused attention
2. Address reacquisition of normal movement, then maitenance of the normal movement (break the task into subtasks)
3. Begin practice with a similar task, but only include the normal movements
4. May begin with mental rehearsal (firing 30% of the neurons)
5. start practice in an unusual nontarget position (but ultimately in the normal position)
6. consider compensatory strategy
7. short practice intervals, spaced over time
8. globally repetitive, but progressed with small increments
9. include sensory modificiation strategies
10. mirror imagery

32

Sensory Modification Techniques

1. enhancement of sensory inputs- splint/tape, modifying interface
2. reinforce task performance with other senses (watching video tape, listening to the instrument)