Spasticity in Cerebral PalsyPathophysiology to practice

By: Hamidah Lalani, BSN, RN.

Graduate Student

Alverno College

Objectives

The learner will be able to:

Understand the functions of upper motor and lower motor neurons

Learn definition, epidemiology and causes of Cerebral Palsy (CP).

Understand the pathophysiology of spasticity as it relates to Cerebral Palsy.

Objectives

Understand the role of inflammatory immune response in spasticity.

Understand the role of stress response in spasticity

Identify patients needs and nursing outcomes in caring for the patient with spasticity.

Instructions for tutorial

Read the information carefully followed by the question and possible answers. Click on the answer you think is correct. If you want to go back to the previous slide click on the button. If you want to go to next question click on the bottom left corner of the slide. If you want to start over click on the button.

Cerebral Palsy

It is the disorder of movement and posture that result from a non-progressive lesion or injury of the immature brain.

Leading cause of childhood disability

Cerebral Palsy

Occurs in 2 to 3 per 1000 live births.

Causes: prenatal, perinatal, and postnatal.

765,000 Americans have CP

9000 children are diagnosed each year

1 in 3 with very low birth weight will be diagnosed with CP

http://www.ucp.org/ucp_generalsub.cfm/1/9/1217

Upper and Lower motorneuron

Upper motorneuron Injury to UMN leads to hypertonia.Elicit deep tendon reflexDorsal horn cell in spinal column carry information to the brain and are also called afferent nerve fibers or input association (IA).

Lower motorneuron Injury or lesion to LMN results in hypotonia.Have negative reflexes.Ventral horn cells in spinal column bring information to the muscle fibers and are also called efferent nerve fibers or output association (OA).

The information sent to the brain as input association through the spinal cell column from the muscles goes through:

Dorsal Horn

Ventral Horn

Right!

The dorsal horn is the input association that brings information from the spinal column to the brain.

Really?

The ventral horn brings information to the muscle fiber.

Cerebral palsy is associated with spasticity

What is Spasticity?

Velocity-dependent increase in muscle tone with exaggerated tendon reflexes, due to hyper excitability of stretch reflex.

Causes

Spasticity can be caused by any insult to the brain related to:TraumaAbuseDuring birthBirth defectGenetically acquired Secondary to other disease, e.g. encephalitis, hydrocephalus, MS, spinal dysreflexia, stroke.

Pathophysiology

With any brain lesion, communication from the brain is disrupted and the brain is unable to inhibit the stretch reflex.In case of injury to the cortex the inhibitory signals are lost and the person experiences hyperactivity or spascity.

http://128.104.8.50/courses/neuro/SClinic/Weakness/lmn98.JPG

Spasticity

A lag time may exist between injury and spasticity onset

Severity may wax and wane over time and vary by diagnosis.

Spasticity may be static (always present) or dynamic (increase with intentional movement) in nature.

Stress in Spasticity

Increased activity of the reticular activating system (RAS) and its influence on reflex circuits that controls the muscle tone causes increased tension in the muscle that adds to already tight muscles.

Factors effecting stress in spasticity

Genetic predisposition

Age

Sex

Exposure to environmental stimuli

Life experiences

Diet

Social support

Stress and Immunity

Immune response is triggered by stress.

Immunity is also compromised in stress due to increased levels of cortisol.

Inflammatory immune response

In the event of an inflammatory immune response, the brain cells including neurons produce broad spectrum inflammatory mediators like CRP and cytokines IL-1B and IL6 that can cause tangles and plaques which could in turn cause neuronal loss and ultimately loss of movement.

In inflammatory immune response, tangles and plaques are formed due to the mediators like:

CPK IL- IB, IL- 6

CPK IL- 6

CPK IL- IB

Right!

CPK, IL IB and IL 6 are the inflammatory immune mediators.

Wrong

IL – 6 is also involved in the inflammatory response.

Wrong

IL – IB is also involved in the inflammatory immune response.

In the event of stress, muscle tension is increased due to the increased activity of:

Reticular activating system

Cortical releasing factor

Right!

RAS increases muscle tension in stress.

Wrong!

Cortical releasing factor (CRF) works synergistically with cortisol to inhibit the function of immune system.

The synapses that send nerve conduction to upper extremities are from C5 (cervical) to C8.

The L2 (lumbar) to S1(sacral) segments are responsible for nerve conduction to lower extremities.

Case Study

A three year old girl with a history of shaken baby syndrome came to clinic with complaints of not meeting her developmental stages. A MRI of the spine revealed injury at L3 level of the vertebrae. The injury has affected her:

Arms

Legs

Right!

Legs are affected if the injury is between L2 and S1.

wrong

Injury between C5 and C8 affects arms.

It was determined during the physical examination and history from her guardian that she cannot walk. The tone in her legs was increased and she had spasticity. The injury therefore is in:

Upper motorneuron

Lower motorneuron

Right!

Upper motorneuron causes the hypertonia or spasticity.

Wrong!

Injury to lower motorneuron causes weakness or hypotonia.

Neuromuscular Junction

http://en.wikipedia.org/wiki/Neuromuscular_Junction

Acetylcholine a neurotransmitter,released at the synaptic junction binds itself to the cholinergic receptors in the post synaptic terminal and provide information to the skeletal muscle.

Cholinergic receptors are of two types: nicotinic and muscarinic. Nicotinic are found in the skeletal muscles and helps with receiving acetylcholine.

Acetylecholine binds with cholenergic receptors in the post synaptic junction to provide information for contraction to the skeletal muscle. Acetylecholine is a:

Neurotransmitter

Receptor

Synapse

Right!

Acetylecholine is the neurotransmitter participates in the contraction of the skeletal muscle.

Really?

A synapse helps with action potential in neurons and muscles.

Wrong!

A receptor like cholinergic receptor attaches to the (acetylecholine) neurotransmitter to initiate the muscle contraction.

What is Muscle tone?

It is the tension in a muscle caused by the passive movement of the joint and it is very important for the muscle movement.

Intrafusal muscle fibers lengthens the the muscle.

Extrafusal muscle fibers contracts the muscle.

Muscle Spindle

http://en.wikipedia.org/wiki/File:Skeletal_muscle.jpg

Tonic reflexes are polysynaptic and help with movement and tone of the muscle through the descending excitatory signals from brain.

Phasic reflexes are monosynaptic and exhibit reflexes like deep tendon reflex.

When the neurotransmitter reaches the post synaptic terminal Intrafusal muscle fibers get the information to:

Stretch the muscle

Contract the muscle

Right!

The intrafusal fiber is responsible for lenghtening the muscle fiber .

Wrong!

The extrafusal muscle is responsible for muscle contraction.

Case study

A fifteen year old girl with a history of premature twin birth and diagnosed with cerebral palsy came to clinic. On physical examination, the doctor was unable to elicit knee jerk reflex. Which pathway is interrupted?

Tonic excitatory

Phasic excitatory

Right!

Phasic excitatory pathway effects all reflexes.

Wrong!

Tonic excitation effects the movement and contraction of the muscle like extention and flexion of the arm.

Both her arms were stretched out and the doctor was unable to flex them. Which of the following pathways was interrupted?

Descending excitatory

Descending inhibitory

Right!

Descending inhibitory pathway modulates with the excitatory pathway and helps stop the contraction and allows the muscle to relax.

Wrong!

Descending excitatory pathways help contract the muscle.

Nursing Outcome

The most important nursing intervention in the care of patient with spasticity is the prevention of skin breakdown.Keep the skin clean, and dry through good hygiene, position changes, support in pressure areas.

Mobility

Provide resources for better mobility depending on patients’ ambulatory status. Example, wheel chair (manual, electric), braces for legs, therapy.

Pain

Pain is caused by constantly contracting muscles.

Relaxing the muscles through therapy, exercises etc

Nutrition

Good nutrition should be provided to prevent skin breakdown

Among all the nursing intervention the following is the most important problem that requires nursing intervention.

Mobility

Pain

Skin integrity

Correct!

Skin breakdown is caused by immobility and should be prevented to prevent further complications.

Pain is controlled with medications.

Mobility is provided with the use of wheel chair or walker.

Treatment

Medication management:Baclofen

Dantrolene

Clonidine

Tizanidine

Injections

Botox (Botullinum toxin A)

Phenol

Myobloc (Botullinum toxin B)

Surgical Intervention

Intrathecal baclofen pump

• Patient teaching

•Baclofen trial

•Pump implant

•Follow-up

•Alarm

•Refillhttp://www.medtronic.com/statements/terms/index.htm#copyrights-trademarks

Resources

Orthotics – AFO, SMO, body brace

Therapy – Physical, occupational, speech, aqua therapy, hippo therapy.

Self accommodating equipment – Wheel chair (electronic vs. manual), walker

Augmentative communication

Goals

Functional - hygieneMobilityComfort – free of painSkin integrityCognitionCommunicationPsychosocial coping – family integrityNutritional status – oral vs.G.TSleep disturbances – related to medicationBehavior - medication

References

Alexander, T., Hiduke, R.J., Stevens, K.A., (1999). Rehabilitation Nursing; Procedures Manual. Chicago, Il: McGraw-Hill companies.

Chin, P.A., Finocchiaro, D., Rosebrough, A., (1998). Rehabilitation Nursing Practice. Azusa, CA: McGraw-Hill companies.

Fishman, M.A. (October 1st, 2008). Neurological examination in children. UpToDate, 16.3, Retrieved 2/6/2009, from http://www.uptodate.com/online/content/topic.do?topickey=ped_neur/2836&view=print

Kirshblum, MD. S., Campagnolo, MD. D.I., Delisa, MD., J.A., (2002). Spinal Cord Medicine. Philadelphia, PA.: Lippincott Williams & Wilkins.

References continued..

Lynch, MD, PHD, D., Waldman, MD, A., (10/1/2008). Pelizaeus-Merzbacher Disease. UpToDate, 16, Retrieved 2/17/2009, from http://www.uptodate.com/online/content/topic.do?topickey=demyelin/6613&view

Moorhead, S., Johnson, M., Maas, M., (2004). Nursing Outcomes Classification (NOC) 3rd ed.

Porth, C. M. (2005). Pathophysiology Concepts of Altered Health States. Philadelphia, PA: Lippincott Williams & Wilkins.

Simon, R.P., Aminoff, M.J., Greenberg, D.A., (1996). Clinical Neurology. USA: Lange Medical Books/Mcgraw-Hill.

Wu, MD, MPH, Y. (10/1/2008). Etiology and pathogenesis of neonatal encephalopathy. UpToDate, 16, Retrieved 2/6/2009, from http://www.uptodate.com/online/content/topic.do?topickey=ped_neur/2836&view=print