Dystonia Flashcards by s y

What is dystonia?

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

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How do dystonic movements present?

Patterned
twisting
tremulous
sustained

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What is dystonia initiated or made worse by?

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

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What was the former classification system?

Age of onset
Distribution
Etiology

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

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

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How are the etiologies classified for dystonia?

Nervous system pathology
Inherited vs. acquired
Idiopathic

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What is focal dystonia?

Single body part

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What is Segmental dystonia?

Contiguous body parts

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What is Multifocal Dystonia?

Non-contiguous body parts

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What is generalized dystonia?

entire body

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What is hemi-dystonia?

Dystonia on half the body

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When is the peak of generalized dystonia?

~10 yrs.

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What is the age distribution of Focal dystonia?

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

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What is the distribution of segmental dystonia?

Bimodal with peaks at 9/10 and 40

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Prevalence of early onset (<28) primary dystonia?

2-50 cases per million

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Prevalence of late onset (>28) primary dystonia?

30-7320 cases per million

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Specific types of focal dystonia?

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

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

No changes on standard imaging and no underlying injury or disease

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Damage to which areas can cause an acquired dystonia?

Basal ganglia
thalamus
Brainstem
cerebellum
Cortex

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What type of injuries can cause an acquired dystonia?

Injury to nervous system

stroke
TBI
limb injury

What type of degenerative disorder can cause an acquired dystonia?

Parkinson’s disease

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

Volumetric enlargement of the basal ganglia

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

Increased grey matter density on primary sensory cortex

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

Increased metabolic activity in sensory and motor cortex during motor tasks

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

Altered D2 dopamine receptor level

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

It is impaired

What happens to surround inhibition with dystonia?

Surround inhibition is reduced

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

You need an environmental trigger

What is the basal ganglia consisted of?

Interconnected subcortical nuclei

What is the "general" job of the Basal ganglia?

Fundamental processing units of the brain

What are some hypokkinetic movement disorders?

Parkinson's disease | Stiffness syndromes

What are some hyperkinetic movement disorders?

``` Dystonia Huntington's Disease Chorea, ballism, athetosis Tics, tourettes syndrome Myoclonus ```

What is surround inhibition?

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

What are symptoms of basal ganglia disorders?

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

What link is abnormal in dystonia?

Abnormal links between sensory inputs and motor outputs

What type of movements can dystonia be associated with?

Highly stereotyped, over-learned movements

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

Posture and Movement

What is the definition of postural control?

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

What is the definition of Orientation?

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

What is the definition of stability?

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

What does the tonic postural program do?

Maintenance of baseline muscle tone and body shape

What does the phasic postural control program do?

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

What is neuroplasticity?

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

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

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

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

Excessive lateral trunk flexion Excessive cervical flexion Excessive trunk flexion

How does post traumatic dystoina present?

Presents after injury | is often fixed and less responsive to treatment

What is paraneoplastic syndrome?

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

What type of medications are used to treat

``` Denzodiazepines Sinemet (dopa responsive) Trihexyphenidyl Tetrabenazene Botox injections ```

Where are DBS used for dystonia?

Globus pallidus internus