5.1multiple Sclerosis

8/2/2019 5.1multiple Sclerosis

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Multiple

Sclerosis


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DEFINITION


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What is multiple sclerosis?

(MS) is a chronic autoimmune

inflammatory demyelinating disease of the

brain and spinal cord.


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DESCRIPTION


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What is myelin?

Myelin =

the fatty and protein material that

surrounds certain nerve fibers in the

brain and spinal cord,


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What is a demyelinating disease?

is any condition that results in damage to the protectivecovering (myelin sheath) that surrounds nerve fibers in yourbrain and spinal cord.

When the myelin sheath is damaged, nerveimpulses slow or even stop, resulting inimpaired transmission of nerve impulses

causing neurological problems.


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What is Multiple Sclerosis?

It is an Auto Immune Disease which

is when the body starts to destroy itself.

It is a life-long disease with no cure.


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What is Multiple Sclerosis?

Currently in the US, 250,000-300,000

people have been diagnosed withMS and there are 200 new cases

diagnosed every week.


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HISTORY


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History of Multiple Sclerosis

Multiple Sclerosis, akaMS, was given its name, multiplebecause of the numerous sites of demyelinationand sclerosis which meansscarring.

first illustrations and clear clinical description of the disease

appeared in 1838


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History of Multiple Sclerosis

It was in Holland on August 4, 1421, that the earliestdescriptions were seen. Even though the previousdescription, the first actual case was first diagnosed in

1849. It wasJean-Martin Charcot who is credited withgiving us the first signs and symptoms of Multiple Sclerosis.


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PREVALENCE


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How Common is MS and Who Gets It?

8,000 10,000 new cases are diagnosed annually

Affects nearly 500,000 individuals in the U.S.

Occurs most frequently between ages 25 35

Female: male ratio = 2:1

Whites: non-whites=2:1

More frequent in populations native to areasfarther away from the equator


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Risk of Developing MS and Region of Origin


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CAUSES/ predisposing factors


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What Causes MS?

Genetics

Environmental factors Sex

Latitude

Racial GroupSES

Family historyMigration

Infections- molecular mimicry

Roseola (HHV6A)


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Genetic and the Immune System

XX9

XX1

XX13

XX7

XX10

XX

8

XX11

XX2

XX12

XX4

XX3

XX5

XX6

XX9

XX1

XX13

XX7

XX10

XX

8

XX11

XX2

XX12

XX4

XX3

XX5

XX6

X

X

ImmuneResponseGenes

XX9

XX1

XX13

XX7

XX10

XX

8

XX11

XX2

XX12

XX4

XX3

XX5

XX6

XX9

XX1

XX13

XX7

XX10

XX

8

XX11

XX2

XX12

XX4

XX3

XX5

XX6

X

X

ImmuneResponseGenes


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Genetic Factors

Sex: Women are more likely to have MS than men by a2:1 ratio.They also think that this is true because women are in general

more likely to have an Auto immune Disease.

Racial Group: Whites are more than twiceas likelyas other races to develop MS ( Hope 2).


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Groups for Which MS Genetic Traits Have Been Identified:

Japanese African American Mexican

Arabian Sardinian (Italy) Swedish Norwegian French Canadian Multi-ethnic Caucasian

Finnish

G i F


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Genetic Factors

Family History:

In a normal population the chance of someone to exhibit th

symptoms of MS is only 0.1%.

Now if someone in your family has MS, the riskincreases.

G i F


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Genetic Factors

Family History:

your first-degree relatives =3%.

second-degree relative= 1% chance of having MS.

both parents = 20%.

G ti F t


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Genetic Factors

Family History:

half sister/brother= 1.5%

identical twin= 30%

fraternal twin = 3-4%

***Remember that women have a slightly higher risk and

that if one ofthe identical twins has MS it is not 100%

positive that the other twin will have MS due to theenvironmental factors.


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Family Studies

Monozygotic (identical) twins

Dizygotic (non-identical) twins

Child of parent with MS

Sibling of person with MS

25 30%

3 4.5%

1.9%

0.9%

Up to 19% of patients havean affected relative


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Not Everyone with a Genetic Risk WillDevelop MS Why?

Risk is modified by Environmental

factors -Sunlight -Diet (e.g., vitamin D) -Other lifetime experiences (infections?)


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Environmental FactorsLatitude:

As you increase latitude, mainly above and below

40 latitude, MS is more common.

These are temperate and cooler climates.

It is 5x more likely

in these regions.


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Environmental FactorsLatitude

(1) MS frequency increases with increasing latitude, which is strongly

inversely correlated with duration and intensity of UVBfrom sunlight and vitamin D concentrations;

(2) prevalence of MS is lower than expected at high latitudes in

populations with high consumption ofvitamin-D-rich fatty fish;and

(3) MS risk seems to decrease with migration from high to lowlatitudes.


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Environmental FactorsSES: Your socioeconomic status can also

affect the occurrence of MS. It is

least common in thelower class and in

rural residence.


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Environmental FactorsMigration: The age at which you may move

may also be an important factor. If you move

before the age of 15, your risk is that of the

people in the country you move to. If you moveafter the age of 15, your risk stays fixed at that

of the country you grew up in (OConnor 15)..

Environmental Factors


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Infection: MS is a delayed reaction to a viral infection contracteduring childhood by a genetically susceptible person

1.shingles,

2. chicken pox,

3. measles, or4. certain herpes.

AGE at which you get the infection:.

The older you are the higher the risk for MS.


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Infection: molecular mimicry Roseola

(HHV6)


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Conditions that precede onset or exacerbation

1. emotional stress

2. fatigue

3. pregnancy 4. acute respiratory

infections


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Anatomy and physiology

The Brain


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The Brain

Your brain controls all aspects of your body, suchas sensory input, emotions and involuntaryfunctions.

It has three main parts: the cerebrum, thecerebellum and the medulla..

The Brain


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The Brain

The cerebrum controls higher functions--sensory input,thought and emotion.

The cerebellum works with your sensory

organs and muscles to coordinate movement.

The medulla controls your basic life functions--heartbeat, breathing, digestion, etc.

The Brain


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The Brain

When your brain receives a stimulus, it

processes it in the appropriate portion and tellsthe body what to do in response to the stimulus.


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cerebrum

1. motor control.

2. attention and

3. language,

4. in regulating fear and pleasureresponses

5. movement-related functions
http://en.wikipedia.org/wiki/Motor_controlhttp://en.wikipedia.org/wiki/Attentionhttp://en.wikipedia.org/wiki/Languagehttp://en.wikipedia.org/wiki/Fearhttp://en.wikipedia.org/wiki/Pleasurehttp://en.wikipedia.org/wiki/Pleasurehttp://en.wikipedia.org/wiki/Fearhttp://en.wikipedia.org/wiki/Languagehttp://en.wikipedia.org/wiki/Attentionhttp://en.wikipedia.org/wiki/Motor_control


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Neurons

neurons work by transmitting sodium andpotassium ions through special channels,which act on muscles and nerves.

Your nervous system contains twotypes of neurons: afferent and efferent.


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Neurons

Afferent neurons, also called sensory neurons,attach to the sensory organs: eyes, ears,nose, tongue and skin.

They carry sensory signals to yourbrain, which processes them anddetermines the appropriate message tosend along your efferent neurons.


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Neurons

Efferent neuron

-motor neurons--connect to muscles and glands.

.


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Neurons---Not all neurons lead from the brain; some lead from thespinal column

Afferent-efferent neuron--pairs that connect to the spinal column

without any connection to the brain controlreflex actions.

Pain receptors are an example of this

--you don't have to think about pulling your handaway from something hot, it just happens.

The Spinal Cord


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The Spinal Cord

a. The other nerves of the body branch out from thespinal cord to form the peripheral nervoussystem.


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pathophysiology


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The Human Nervous System

Areas affected byMS

Brain

Spinal cord Optic nerves

tp://web.lemoyne.edu/~hevern/psy340/lectures/psy340.04.2.ns.structure.html)


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MS is an Immune-Mediated Disease

BBB=blood-brain barrier; APC=antigen-presenting cell.Adapted from Miller et al. Continuum: Multiple Sclerosis (Part A). 1999;5:7.

MS is a Demyelinating Disease
http://pathopshysiology.docx/http://ms%20pathopshysiology.docx/http://ms%20pathopshysiology.docx/


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MS is a Demyelinating Disease

Myelin provides

insulation to nerve

processes (axons)

Blood vessel

Blood vessel

Blood vessel

Inflammation

Inflammation

Inflammation

Myelin provides

insulation to nerve

processes (axons)

Blood vessel

Blood vessel

Blood vessel

Blood vessel

Blood vessel

Blood vessel

Inflammation

Inflammation

Inflammation

Inflammation

Inflammation

Inflammation
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Lets check

the video


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Signs and symptoms

Signs and s mptoms


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Signs and symptoms

Individualistic disease process with

symptoms, progression, and severityvarying person to person.

Initial Presentation of MS


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Initial Presentation of MS

Incidence (%)

Optic nerve inflammation 1429

Poor balance (ataxia) 218

Dizziness (vertigo) 29

Weakness 1040

Double visions (diplopia) 818

Bladder, bowel dysfunction 014

Pain 2140

Sensory loss 1339

P h h i l i h


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Pathophysiologic changes

1. Sensory impairment/Dysesthesias

a. Burning,Pins, Needles, Electrical sensation

(Lhermittes sign-electrical sensation down thespine on neck flexion) during ordinary activities

b. fatigue

P th h i l i h


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2. ---Weakness,

-paralysis ranging from

monoplegia to

quadriplegia,

-spasticity,-hyperrefllexia,

-intention tremor and

- gait ataxia from impaired

motor reflex

-Sexual dysfunctionerectile dysfunctionis te mostcommon

P th h i l i h


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3. incontinence,-urinary frequency and

urgency,

- frequent infections fromimpaired transmission

involving sphincter

innervation

P th h i l i h


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4. Involuntary evacuation or constipation fromaltered impulse transmission to internal sphincter

P th h i l i h


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5. Poorly articulated or measured and slowspeech and dysphagia from impairedtransmission to the cranial nerves and sensorycortex

P th h i l i h


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6. Cognitive symptoms , including depression,euphoria, inattentiveness, apathy, forgetfulnessand memory loss due to primary damage in thecerebrum

P th h i l i h


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7. Chronic Pain-appears after a lesion to the ascending ordescending tracts that control the transmission of

painful stimulus.

-Acute temporary pain is common as well as the

result of the disease process.


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Courses of MS

f


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Courses of MSListed below are the different paths that MS can take.

Relapse-remitting MS (RRMS): Here you have an attack, go intocomplete or partial remission, then have the symptoms return.

Primary-progressive MS (PPMS): Here you continually decline

and have no remissions. There may be a temporary relief insymptoms.

A few patients have malignant MS which is where they have aquick decline which leaves them severely disabled or even lead todeath.

C f MS


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4 Courses of MSListed below are the different paths that MS can take.

1. Relapse-remitting MS (RRMS)

2. Primary-progressive MS (PPMS)

3. Secondary-progressive MS (SPMS)

4. Progressive-relapsing MS (PRMS)

C f MS


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Courses of MS

Relapsing-remitting:

Periods of neurological

dysfunction followed by partial orfull recovery

C f MS


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Courses of MS

Relapsing-remitting:

Here you have an attack,

go into complete or partialremission,

then have the symptoms return.


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Multiple Sclerosis Clinical Subtypes

Lublin FD et al. Neurology. 1996;46:907-911.

Relapsing-remitting

Primary-progressive

Disability

Time

Time

Disability

Courses of MS


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Courses of MS

Primary-progressive:

Steady decline with periods ofminimal recovery (fairlyuncommon).

Courses of MS


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Courses of MS

Primary-progressive:

Here you:

a. continually decline

b. have no remissions.

c. a temporary relief in symptoms may be experienced.

A few patients have malignant MS which is

where they have a quick decline whichleaves them severely disabled or even lead todeath.


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Relapsing-remitting

Primary-progressive

Disability

Time

Time

Disability

C f MS


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Courses of MS

Secondary-progressive:

Initial pattern of relapse and recovery,

which becomes steadily progressiveover time.

C f MS


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Courses of MS


a. starts with RRMS symptoms

b. continues on to show signs of PPMS.



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Secondary progressive:

1. Relapse-remitting MS (RRMS)

2. Primary-progressive MS (PPMS)


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Secondary-progressive

Progressive-relapsing

Time

Time

Disability

Disability

Courses of MS


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Courses of MS

Progressive-relapsing:

Progressive from onset with clear exacerbations (rare).

a. periods of exacerbations

b. Periods of remissionsc. progressive inapproximately 60% of patients.

Individual prognosis is variable and unpredictable,presenting complex physical, psychosocial, andrehabilitative issues.

Courses of MS


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Courses of MS

Progressive-relapsing:

This is a rare form

takes a progressive route made worse by acuteattacks.

20% of the people with MS have a benignform.

Here they show little progression after the firstattack.

M l i l S l i Cli i l S b


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Secondary-progressive

Progressive-relapsing

Time

Time

Disability

Disability


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DIAGNOSIS

How Is MS Diagnosed?


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ow s S ag osed?

At least two episodes of symptomsOccur at different points in time

Result from involvement of different areas

of the central nervous system

Absence of other treatable causes for

the symptoms

Results of neurological testing

Examples of MS Onset


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Case 1: 26 year old woman

Decreased vision in the right eye in 9/05

Left leg numbness in 1/06

Right face numbness, right arm and leg weakness in 4/06

Left leg weakness in 8/06

Case 2: 45 year old man

Left arm weakness in 2/93

Numbness below the waist in 4/07

Other Potential Causes ofMS lik S


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MS-like Symptoms

Lyme disease

Lupus

Migraine

Non-recurrent inflammatory process

Encephalitis

Stroke Tumor of the brain or spinal cord

assessment and diagnostic findings


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g g

1.Historyof a viral illness

assessment


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Visual disturbances

blurring of vision diplopia scotoma (blind spot)

Impaired sensation to touch, pain, pressure,heat and cold Tingling sensation paresthesias

numbness Lhermittes sign (electrical sensation

down the spine on neck flexion).

assessment


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Mood swings euphoria sense of well-being

Impaired motor activity weakness spasticity paralysis

assessment


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Impaired cerebellar function Charcots triad: ataxia (unsteady gait), nystagmus,

intentional tremors

Scanning speech

Ex. "Walk (pause) ing is good ex (pause) er

(pause) cise"

Urinary retention or incontinence

Constipation

Decrease in sexual capacity


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DIAGNOSTIC STUDIES



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Neurological examination

Magnetic resonance imaging (MRI) Scan

EEG

Blood tests

Lumbar Puncture (spinal tap):

occasionally

Evoked potential studies



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=multifocal white matter lesion

Magnetic Resonance Imaging in MS


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Magnetic Resonance Imaging in MS

Spinal cord

Optic nerve

Brain

Spinal cordSpinal cord

Optic nerveOptic nerve

BrainBrain

Assessment of the Appearance ofMS Lesions Over time


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MS Lesions Over time

Time lapse = 1 year

Brain Atrophy (Shrinkage)


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p y ( g )

in Untreated MS

Images acquired over the course of 7 years from a single personwith untreated MS



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in Untreated MS



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Lumbar Puncture (spinal tap): occasionallyperformed.

CSF evaluation:a. Normal total CSF protein

b. Elevated IgG= hyperactivity of the immune system

c. Possible elevated CSF WBCcount



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Electroencephalography

Demonstrates abnormalities in brain

waves



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Neurological examination


EEG

Blood tests

Lumbar Puncture (spinal tap):

occasionally

Evoked potential studies


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PHARMACOTHERAPY


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Medications used for MS

Spasticity- Baclofen, Tizanidine, Diazepam, Dantrolene

Optic Neuritis-Methlyprednisolone, Oral steroids

Fatigue-Antidepressant, Amantadine

Pain-Codeine, Aspirin

Sexual Dysfunction-Viagra, Pravatine

Tremor-Isoniazid, Primidone, Propranolol

Disease-Modifying Drugs- Interferon beta 1a and 1b, andGlatiramer acetate

Disease-Modifying Drugs


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Disease Modifying Drugs

Interferon Beta 1a (Avonex and Rebif):

is a protein that is a replica of human interferon.

suppress the immune system and helps to maintain the blood-brain barrier.

inject Avonex into the muscle once a week and Rebif isinjected under the skin three times a week.

This drug is useful to people who have definite progressiveMS.

S/Eof the drug is a flu like symptom.

Disease-Modifying Drugs


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Interferon Beta 1b (Betaseron):

is slightly different from our own interferon.

This medication does the same thing as beta 1a, but isinjected just under the skin every two days.

S/E include irritation, bruising, and redness at the site ofinjection and the flu like symptoms.

This is also given to people who have definite

progressive MS.


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Disease-Modifying Drugs (cont)


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Disease Modifying Drugs (con t)

.

all three of these drugs

a.decrease relapses by 33%,

b.have manageable side effect,c.are injected,

d.stabilize the disease,

e. tend to be costly.


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Nursing diagnoses and their respective management

Nursing Diagnosis


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1. Impaired bed and physical mobility related to

weakness, muscle paresis, spasticity

Promoting Physical mobility


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Instruction in the use of assistive devices may be needed toensure their safe and correct use.

Minimizing spasticity and contractures by using warm packsbut hot baths should be avoided because of risk for burn injury

secondary to sensory loss and increasing symptoms that may

occur with elevation of the body temperature.



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Mild spasticity may be managed by stretching and exerciseprograms such as:

a. water therapy,

b. ROM exercises

c. yoga, and

d. physical therapy.

e. Medication is indicated when stiffness, spasms, or clonus

interferes with function or sleep



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Minimizing effects of immobility and measures to prevent

such complications include assessing and maintaining skin

integrity and having the patient perform coughing and deep

breathing exercises.

Nursing Diagnosis


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2. Risk for injury related to sensory and

visual impairment

Preventing Injury


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Teach the patient about the disease and how it affects hisADLs.

Teach the patient about the use of call bell/ call light.

Instruct to ask for assistance upon ambulation or whennecessary.

Instruct patient not to ambulate in darkened areas othe room.

Instruct significant others to remove rugs from floorsto prevent slips and falls.

Encourage family to place patient on a room on theground floor of the house.

Nursing Diagnosis


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g g

3.Impaired urinary and bowelelimination (urgency, frequency,incontinence, constipation) related tonervous system dysfunction

Enhancing Bladder and Bowel control


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a. Increase OFI, high fiber diet (forconstipation),

b. acid-ash in diet to acidify urine to prevent

bacterial

Multiplication

-cranberry juice,

-prunes,-grape juice,

- vitamin c, orange and

- pineapple juice.)

Enhancing Bladder and Bowel control


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c. Insertion of an indwelling catheter and suprapubic tubes as ordered

d. Use bowel-training strategies

Nursing Diagnosis


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g g

4. Impaired verbal communicationand risk for aspiration related to

cranial nerve involvement

Enhancing communication and managing swallowing difficulties


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Speech therapy for dysarthria

Nursing Diagnosis


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g g

5. Disturbed thought process (loss ofmemory, dementia, euphoria) related tocerebral dysfunction

Improving sensory and cognitive function


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Vision:

----An eye patch or a covered eyeglass lens may be used to blockthe visual impulses of one eye if the patient has diplopia (doublevision)

----vision therapy or adaptive lenses to manage visual problems



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Cognition and Emotional responses:

a. The patient is assisted to set meaningful and realistic goals.

b. The family should be made aware of the nature and degree of

cognitive impairment.



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Strengthening Coping Mechanisms:

a. alleviating stress,

b. making appropriate referrals for counselingand support to minimize the adverse effects ofdealing with chronic illness.

Nursing Diagnosis


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6. Impaired home maintenance managementrelated to physical, psychological, and social limitimposed by MS

Improving Home management


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Counseling should be given to patients especially if the one

affected is the head of the family Allow the patient to perform roles according to his own

strength and limitation

Promote emotional stability by helping the patient establish a

daily routine to maintain optimal functioning Inform the patient that exacerbations are unpredictable ,

necessitating physical and emotional adjustments in lifestyle

Nursing Diagnosis


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7. Potential for sexual dysfunctionrelated to lesions or psychologicalreaction

Promote sexual functioning


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careful sexual history may reveal problems such as:

a. feelings of sexual inadequacy,

b. impaired libido,

c. or direct sexual dysfunction resulting from erectiledysfunction,

d. impaired lubrication,

e. spasticity,

f. or heat-related sensory dysesthesias

Promote sexual functioning


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careful sexual history may reveal problems such as:

a. feelings of sexual inadequacy,

b. impaired libido,

c. or direct sexual dysfunction resulting from erectiledysfunction,

d. impaired lubrication,

e. spasticity,

f. or heat-related sensory dysesthesias

Fatigue related to conduction deficits and impairedi l i i l h fib


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impulse transmission along the nerve fiber

a. Promote the following:

Frequent rest periods

Aerobic exercise Cooling techniques

-air conditioning

-breezes-water sprays

b. Administer antidepressants as ordered


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Alternative treatment

Alternative Treatments Used worldwide


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Home therapy

Injection of Venom such as snake and

bee

Massage

Meditation

Reflexology

Tai Chi

Yoga

Acupuncture

Alternative Treatments Used worldwide


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Aromatherapy

Cannabis (Marijuana)

Chiropractic

Cold Immersion

Dietary Supplements

Herbal Medication


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New technological possibilities being studied

MS Therapies: What LiesAhead?


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Ahead?

Neural protection

Regenerative therapies Cell replacement (stem cells)

Dietary approaches (vitamin D)

Multiple Sclerosis Research at the University ofMaryland


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Drug therapies Injectable drugs with increased efficacy New oral agents

Cell replacement therapies

Stem cell research MS vaccine Novel rehabilitation techniques Robotics

Dietary approaches Studies of the role of vitamin D in MS

Summary MS is a common inflammatory disease of the CNS that

ff f l f l h l


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affects females more frequently than males.

The cause of MS appears to be a combination of genetic andenvironmental factors.

The symptoms of MS can be quite variable.

MRI is a sensitive test for making the diagnosis of MS.

Treatments are available for reducing the number of MSattacks and for slowing MS disease progression.

5.1multiple Sclerosis

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