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