Dystonia

Degenerative

Parkinson's and Parkinson's Plus Syndromes Excessive trunk and cervical trunk flexion, as well as flexion of cervical region

Types of Hypokinetic disease

Parkinson's and Stiffness Syndromes

Dystonia used to be thought to be what type of disease in the past?

Psychological

Parts of Sensation Exam

i. 2 pot discrimination ii. Grapheshesia iii. Stereognosis iv. Kinesthesia v. Touch localization

Sensori-Motor Learning Hypothesis

i. Nervous system has finite capacity for adaptation ii. As movements become automatic, timing of inputs become nearly simultaneous, losing individual differentiation iii. Stereotypic repetition lead to degradation in somatotropic representations iv. De-differentiate cortical sensory representation through altering neuroplasticity

Part of Neurological Exam

i. Oculomotor ii. Reflexes iii. Postural

Dystonia Tx for lack of fractionated digits

i. Pennies in macaroni ii. Texture board iii. Dot cards

Non-targeted Tx activities for Dystonia

i. Sensory discrimination / fractionation ii. Holding non - targeted implements (spoon, ruler etc) in writing posture

Parts of Musculoskeletal Exam

i. Strength ii. ROM iii. Joint Mobility

3 Characteristics of Dystonia

i. Typically patterned, twisting, and may be tremulous ii. Often initiated or worsened by voluntary action iii. Associated with overflow muscle activation

Targeted Activities for Dystonia

i. Viewing pen resting on table for increasing periods of time while maintaining relaxation of hand and forearm ii. Holding pen in left hand without increased tension in right. iii. Viewing targeted object, hands in lap iv. Viewing targeted object hands on table v. Holding targeted object in left hand vi. Writing with left hand vii. Holding targeted object in right hand in a non-writing posture viii. Pen to paper without pressure ix. Position to write

Movement Disorders Associated with Segmental LE Dystonia

i.Dystonic posturing involving abdomen, hips, and left LE ii.Forward flexion of trunk/PPT at toe off iii.Reduced isolation of hip and lower trunk on left through swing and increased knee flexion iv.Initial contact occurs in PF due to fx shortening of extremity

How to alleviating maneuvers or sensory tricks work

if the BG are responsible for associating sensory stimuli with a particular motor output, derangement of this link may result in a deranged input - output connection. Altering the sensory input from the effected body part results in an alteration in this input-output link and we see a change in the motor output.

Tricks to alleviate cervical dystonia

ii. Eliminated tension by touching chin, placing left hand at lateral aspect of left neck, resting head against wall iii. Known as sensory tricks

Why conduct PT Exam?

**Purpose assess response to alternate postures, evaluate sensory tricks, assess spread**

What is known about cervical Dystonia Tx?

Further research is need to establish effectiveness of PT for improving long-term management

Interventions for Dystonia

1. Alter neuroplasticity in positive way 2. Alter sensory inputs to achieve a different motor input 3. Educate regarding use of alleviating maneuvers/postures 4. Flexibility exercises in non-provoking positions 5. Strengthening exercises in non-provoking postures 6. Sensorimotor reintegration as appropriate 7. Establish an aerobic exercise regimen if possible

3 Types of Limb Injury

1. Complex Regional Pain Syndrome 2. Post-Traumatic Dystonia 3. Paraneoplastic Syndrome (around cancerous tumor)

3 Way of having acquired dystonia

1. Damage to multiple brain regions 2. Injury to nervous system 3. Degenerative Diseases

Hyperkinetic Disease

1. Dystonia 2. Huntington's Disease 3. Chorea, Ballism, Athetosis 4 Tics, Tourettes

What breaks down in Dystonia?

1. Link between Posture and movement 2. Link between Sensory inputs and motor outputs 3. Movements associated in postural patterns 4. Neuroplasticity 5. Ability to perform highly trained movements and postures

Characteristics of idiopathic dystonia

1. No changes seen on standard imaging 2. No underlying injury or disease 3. Genetic (associated with mutations in more than 30 different genes

Phasic postural programs

1. Orientation in Space (anti-gravity alignment) 2. Maintains balance during static states and during movements APAs 3. Involved in orienting body/parts during movement (stabilizing forces)

What are other treatment options

1. Other medications i. Benzodiazepines ii. Trihexyphenidyl iii. Tetrabenazene iv. Sinamet (Dopa Responsive) 2, Deep Brain Stimulation of Globus Pallidus INternus

How does chain of events result in dystonia?

1. Predisposition + trigger 2. Sensori=Motor Mismatch 3. Altered Posture/Movement 4. Negative Neuroplasticity 5. Negative neuroplasticity feeds back into sensori-motor mistmatch

3 Types of Injury to the nervous system

1. Stroke 2. TBI 3. Limb Injury

What are some considerations with the brace

A. Assess available range in alternate postures and/or during sleep B. Use only if patient perceives benefit C. Use with caution

What orthotics can be used?

AFO, wrist splints, cervical thoracic support (minerva)

Gait/Locomotion training for Dystonia

Ambulate on level surfaces without device with gait patterns as described in above in movement disordoders

Types of inherited dystonia

Autosomal dominant, recessive, x-linked recessive and mitochondrial

Locomotion Execution

BG project to Mesencephalic Locomotor Region MLR drives CPG in spinal cord for walking Motor output is tied to sensory input Changing sensory input can change motor output

What is the front line of treatment for dystonia, especially cervical dystonia

BOTOX

Generalized Dystonia is typically diagnosed when?

Childhood

Characteristics of acquired dystonia

Degeneration and structural lesions

What is altered when you change sensory input?

Dimension of task and associated stabilization requirements

What is needed in order for dystonia to appear?

Environmental trigger or rirritant

Impaired intracortical inhibition in sensory cortex indicates what?

Impaired surround inhibition due to problem with sensory gating

What does movement requires?

Interaction between tonic and phasic postural programs across a continuum.

Tonic phase of postural programs

Maintenance of baseline muscle tone and body shape

What is surround inhibition?

Modulation of motor control by basal ganglia by balancing excitation and inhibition of competing motor programs. Also enhances signals for desired movement and inhibits competing movements.

What is Dystonia?

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

If you have posturing during ambulation, will you have posturing during backwards walking or stair climbing?

NO, it's a different motor plan.

Can you treat dystonia the same way you'd treat spasticity?

No

Is dystonia well understood and have a standardized tx intervention?

No

Does dystonia have single locus of pathology?

No Is likely associated with the fact that motor control involves multiple brain regions or loops

Characteristics of Dystonia

Non-velocity dependent

What two things result in abnormal movements?

Normal inputs + altered processing

Focal Dystonia is typically diagnosed when?

Over 40

AcquiredDystonia may be seen in what pathologies?

PD, Stroke also: Perinatal TBI, infection, drug, toxi, vascular , cancer, pyschogeneic

What is spasticity?

Reduced cortico-spinal drive onto spinal motor neurons results in excessive activation of those motor neurons by reflex pathways (hyperreflexia)

What is dystonia?

Reduced inhibition from GP results in excess activation or excessive movement from motor cortex Altered function in Basal Ganglia results in over activation / fluctuating activation at the level of the thalamus and resultant fluctuation in drive from the motor cortex onto the spinal motor neurons.

Dystonia Tx for tension in hand

Relaxation with emphasis on becoming aware of tension

Response to altered sensory input

Shorten step length or increase emphasis on heel strike to combat knee flexion

Response to altered sensory input

Shorten step length or increase emphasis on heel strike to combat knee flexion Use PLS

Secondary Dystonia is a result of what?

Something irritating the nervous system

Postures that don't provoke dystonia

Supine, prone, quadruped

Stroke

Tone/posturing of joint or body part that is not consistent with overall motor recovery of body part

Many people carry gene for dystonia, but do not show any signs. T?F

True

Characteristics of Spasticity

Velocity Dependent and characterized by hyperreflexia

Does dystonia have robust association between posture and movement?

Yes

Will you see reduction in dystonic posture in different positions and if so, why?

Yes Task that dystonic movement is associated with has associated postural or positioning requirements as well.

Characteristics of Writer's Cramp Dystonia

a. Activated only when thinking or physically holding pen to write b. Can occur at rest if patient thinking about task c. Muscles can be activated when performing task with other hand called spread or overflow d. Very specific to holding specific object (write with pencil vs. spoon)

Types of Genetic Dystonia

a. Cervical Dystonia b. Blepharospasm c. Oralmandibular Dystonia d. Writer's Cramp Dystonia e. Limb Dystonia (LE) f. DYT1 Idiopathic torsion dystonia

Post-Traumatic Dystonia (limb)

a. Develops following injury, often fixed b. Less responsive to treatment

Intervention considerations for dystonia

a. Do not perform activities, passive or active, that engage or increase dystonic symptoms! b. Maximize integration in physical and social activities c. Remember - Predisposition or sensitivity to functional cortical reorganization...don't want to reinforce abberent plasticity

Principles for Intervention

a. Dystonia is a central phenomenon b. Dystonic symptoms/postures cannot be modified at the musculoskeletal level. c. Interventions typically involve altering sensory input or re-organizing central processing using principles of neuroplasticity. d. Sensorimotor learning

What is required for motor contol

a. Intact sensory inputs b. Postural control and stabilization c. Brain regions organized somatotopically

Complex Regional Pain Syndrom (limb injury)

a. Marked DF with inversion, knee flexion, hip flexion, and external rotation b. Extremely disabling

Factors implicated in pathogenesis of Dystonia

a. Motor Circuitry Dysfunction b. Aberrant/Increased Plasticity c. Gene Mutations d. Reduced Surround Inhibition e. Ion Channel, Intracellular Signaling Dysfx f. Neurontransmitter Dysfx (DA, GABA, Ach) g. Reduced Spatial Temporal Discrimination

Paraneoplastic Syndrome (limb)

a. Rapid onset focal dystonia b. Occurs in non-contiguous body part c. Treat cancer

Guidelines for segmental dystonia

a. Take away postures that cause dystonia (typically gravity) b. Allow pt's to assume whatever postures are most comfortable

Types of Sensorimotor Retraining

a. Task with EC to promote attention to sensory detail b. Braille c. Find pennies in macaroni d. Textures e. Tasks with other implements f. Imagery

Alleviating Maneuvers or Sensory Tricks

a. Touch near affected body part alters dystonic posture or movement b. Change in position of extremity may change dimensions of task

Associated abnormalities in Brain Sensorimotor Circuitry

a. Volumetric enlargement of basal ganglia b. Increased grey matter density in primary sensory cortex c. Increased metabolic activity in sensory and motor cortex during motor tasks d. Altered D2 dopamine receptor levels e. Impaired intracortical inhibition in sensory cortex

Targeted interventions for writing

a. Writing ND hand, interference object D hand b. Writing ND hand, no tension c. Writing D hand on wall, cc-wise→c-wise d. Writing D hand backwards, block letter e. Writing D reversed, block letter