Degenerative Neurologic Disorders

Multiple Sclerosis

- Multiple sclerosis (MS) is a chronic, progressive, degenerative

disorder of the CNS characterized by disseminated demyelination
of nerve fibers of the brain and spinal cord.
- MS can affect people of any age.
- The onset of MS is usually between 20 and 50 years of age, with
symptoms first appearing at an average of 30 to 35 years of age.
- People diagnosed at 50 years of age or older generally have more
progressive disease.
- MS affects women 2 to 3 times more often than men.
- MS is more prevalent in temperate climates (between 45 and 65
degrees of latitude)

Etiology and Pathophysiology

- Idiopathic, it is unlikely due to a single cause.

- Thought to develop in a genetically susceptible person after an
environmental exposure such as infection.
- The inherited susceptibility to MS likely involves multiple genes.
● A first degree relative with MS increases a person’s risk for
developing the disease.

- Possible precipitating factors include infection, smoking, physical

injury, emotional stress, excessive fatigue, pregnancy, and a poor
state of health.

- MS is marked by 3 processes: chronic inflammation,

demyelination, and gliosis in the CNS.
● The primary condition is an autoimmune process driven by
activated T cells.
○ An unknown trigger in a genetically susceptible person
may start this process.
● The activated T cells in the systemic circulation go to the
CNS and disrupt the bloodbrain barrier.
 ○ This may be the first event in the development of MS.
● Subsequent antigen-antibody reaction within the CNS
activates the inflammatory response and leads to axon
demyelination.

- Attacks on the myelin sheaths of the neurons in the brain

and spinal cord first cause damage to the myelin sheath.
 - The nerve fiber is not affected. Transmission of nerve
impulses still occurs, but it is slowed.
- The patient may have a noticeable impairment of function
(e.g., weakness).
- However, myelin can still regenerate. When it does,
symptoms disappear. At that point, the patient has a
remission.

- As inflammation continues, nearby oligodendrocytes are affected.

- Myelin loses the ability to regenerate. Eventually damage occurs to
the underlying axon.
- Nerve impulse transmission is disrupted, and nerve function is lost
permanently (Fig. 58.2, D).
- Inflammation subsides, glial scar tissue replaces damaged tissue.
This leads to the formation of hard, rigid plaques (Fig. 58.3). These
plaques are found throughout the white matter of the CNS.

- Death usually occurs due to infectious complications of immobility

(e.g., pneumonia) or because of an unrelated disease.
Clinical Manifestations

- The onset of MS is often slow and gradual.

- Vague symptoms occur periodically over months or years.
● Because they do not prompt the patient to seek medical
attention, MS may not be diagnosed until long after the first
symptom.
- For some patients, MS is marked by rapid, progressive
deterioration.
- Others have remissions and exacerbations.
(With repeated exacerbations, the overall trend is.
progressive deterioration in neurologic function.)

- Some patients have severe, long-lasting symptoms early in the

course of the disease.
- Others have only occasional, mild symptoms for several years
after onset.

➔ The first symptom of MS may be blurred or double vision, red

green color distortion, or even blindness in 1 eye.
➔ extremity muscle weakness and problems with coordination and
balance affecting walking or standing.
➔ MS can cause partial or complete paralysis in the worst cases.
Most have numbness and tingling.
➔ Lhermitte’s sign is a temporary sensory symptom described as an
electric shock going down the spine or into the limbs with neck
flexion.
➔ Some patients report pain, especially in the low thoracic and
abdominal regions.
➔ Other frequent problems include speech impairments
◆ hearing loss
◆ tremors
◆ dizziness.
➔ Possible cerebellar signs include
◆ nystagmus
◆ ataxia
◆ dysarthria
◆ dysphagia

➔ Many patients have severe, even disabling fatigue, worsened by

heat, humidity, deconditioning, and medication side effects.
➔ A rigid plaque is in areas of the CNS that control elimination, bowel
and bladder function.
◆ Bowel problems usually involve constipation.
◆ A common problem in patients with MS is a spastic
(uninhibited) bladder. The bladder has a small capacity for
urine, and its contractions are unchecked. The result is
urinary urgency and frequency, often with dribbling or
incontinence.
➔ A flaccid (hypotonic) bladder occurs with a lesion in the reflex arc
controlling bladder function.
◆ The patient generally has urinary retention because there is
no sensation or desire to void, no pressure, and no pain.
➔ A combination of spastic and flaccid bladder can occur.

➔ Physiologic erectile dysfunction may result from spinal cord

involvement in men.
➔ Women may have decreased desire for sexual activity (libido),
difficulty with orgasm, painful intercourse, and decreased vaginal
lubrication.

➔ Problems with cognitive function:

◆ short-term memory
◆ attention
◆ information processing
◆ planning
◆ visual perception
◆ word finding.

➔ General intellect stays unchanged and intact:

◆ long-term memory
◆ conversational skills
◆ reading comprehension.

➔ Symptoms can be mild and thus easily overlooked.

◆ However, about 5% to 10% of patients with MS have such
severe cognitive changes that they significantly impair the
person’s ability to perform activities of daily living (ADLs).
➔ Most of the time, cognitive difficulties occur later in the course of
the disease. However, they can occur early and sometimes are
present at the onset of MS.

➔ People with MS may have emotional changes, such as anger,

depression, or euphoria.
➔ Physical and emotional trauma, fatigue, and infection may worsen
or trigger signs and symptoms.
Diagnostic Studies
There is no definitive diagnostic test for MS, the history, manifestations,
and results of certain diagnostic tests are important. Imaging in MS is
vital.
 ● MRI of the brain and spinal cord
- may show plaques, inflammation, atrophy, and tissue
breakdown and destruction.
● CSF analysis may show an increase in immunoglobulin G and the
presence of oligoclonal banding.
● Evoked potential responses are often delayed because of
decreased nerve conduction from the eye and ear to the brain.

★ To be diagnosed with MS, the patient must have

(1)Evidence of at least 2 inflammatory demyelinating lesions in
at least 2 different locations within the CNS
(2)damage or an attack occurring at different times (usually 1
month or more apart)
(3)all other possible diagnoses ruled out. If evidence exists for
only 1 lesion, or only 1 clinical attack has occurred, the HCP
will monitor the patient for another attack or for an attack at a
different site in the CNS.

Interprofessional Care Drug Therapy

- Interprofessional care is aimed at treating the disease process and
providing symptomatic relief.
- No cases of MS are alike, so we tailor therapy to the disease
pattern and symptoms of each patient.
- Disease-modifying therapy has been found to be more effective
when started early in the course of MS.
- Delays in treatment are linked to poor outcomes.

➔ Treatment of MS begins with use of immunomodulator drugs to

modify disease progression and prevent relapses.
1. Beta interferon
- Decreases the number relapses of symptoms by
decreasing inflammation and the immune systems
response
★ Risk of infection because it decreases WBCs
★ The most common side effects are flu-like symptoms
for 24 to 48 hours after injection.
 - Drug Names: Avonex
- Rotate injection sites with each dose.
2. Baclofen and Diazepam - skeletal muscle relaxants.
3. Oxybutynin - anticholinergics that helps with overactive
bladder by relaxing the bladder muscle to prevent
contractions.
4. Bethanechol - cholinergic that helps with emptying the
bladder by helping with bladder contraction
5. Propranolol - helps with tremors
6. Isoniazid - antibiotic used for infections, especially TB, helps
with certain tremors in MS.
7. Teriflunomide (Aubagio)
- an immunomodulatory agent with antiinflammatory
properties.
- The exact mechanism of action is unknown.
- It may reduce the number of activated lymphocytes in
the CNS.
8. Fingolimod (Gilenya) and siponimod (Mayzent)reduce MS
disease activity by preventing lymphocytes from reaching the
CNS and causing damage.
- These drugs are used to treat relapsing forms of MS.

For more active and aggressive forms of MS, natalizumab (Tysabri),

alemtuzumab (Lemtrada), mitoxantrone, ocrelizumab (Ocrevus), and
dimethyl fumarate (Tecfidera) may be used.

9. Natalizumab
- Given when patients have had an inadequate response
to other drugs.
- An adverse effect of natalizumab is the increased risk
for a potentially fatal viral infection of the brain
(progressive multifocal leukoencephalopathy). Because
of its safety profile, alemtuzumab is reserved for
patients who have an inadequate response to 2 or
more drugs used for the treatment of MS.
 10. Mitoxantrone is an antineoplastic medication with serious
effects.
- These include cardiotoxicity, leukemia, and infertility.

11. Corticosteroids (e.g., methylprednisolone, prednisone)

- Used to treat acute exacerbations of MS.
- They reduce edema and acute inflammation at the site
of demyelination. However, they do not affect the
ultimate outcome or the degree of residual neurologic
impairment from disease exacerbation.
- Therapeutic plasma exchange (plasmapheresis) and IV
immunoglobulin G may be considered for a short time
when treatment with corticosteroids alone does not
achieve symptom improvement.
12. Amantadine ( antiviral and antiparkinsonian)
- It has CNS effects that helps improve fatigue in
patients with MS.
13. Modafinil - CNS stimulant
14. Tricyclic antidepressants and antiseizure drugs are used
for chronic pain syndromes.
15. Dalfampridine (Ampyra)
- may improve walking speed in MS patients. It is a
selective potassium channel blocker that improves
nerve conduction in damaged nerve segments. It
should not be used in patients with a history of seizure
disorder or with moderate to severe kidney disease.

Other Therapies

● Spasticity
- treated mainly with muscle relaxants.
- surgery (e.g., neurectomy, rhizotomy, cordotomy)
● Neurectomy - is a surgical procedure wherein certain
nerves are blocked or severed to relieve severe
chronic pain.
 ● Rhizotomy - a minimally invasive procedure to remove
sensation from a painful nerve by killing nerve fibers
responsible for sending pain signals to the brain.
● Cordotomy - a neurosurgical procedure in which an
image guided needle is used to make a very precise
lesion in the spine. This procedure treats chronic pain
by targeting and disabling specific nerves that are
sending pain signals.
- Dorsal column electrical stimulation, or intrathecal baclofen
(Lioresal)
- A technique where an implanted electrode is used to
manage certain chronic pain syndromes.
- Tremors that become unmanageable with drugs are
sometimes treated by
● Thalamotomy - works by destroying part of the
thalamus to block the abnormal brain activity from
reaching the muscles and cause tremor
● Deep brain stimulation - is a neurological procedure
that uses implanted electrodes and electrical
stimulation to treat movement disorders such as
essential tremor, dystopia and other neurological
conditions.

Neurologic dysfunction sometimes improves with

● physical and speech therapy - sometimes improve neurologic
dysfunction.
● Exercise improves daily functioning for patients not having an
exacerbation.
- Exercise decreases spasticity, increases coordination, and
retrains unaffected muscles to act for impaired ones.
Nursing Management: Multiple Sclerosis

Nursing diagnoses for the patient with MS may include:

● Impaired physical mobility

● Difficulty coping
● Urinary retention
Planning

The overall goals are that the patient with MS will:

(1)Maximize neuromuscular function
(2)Maintain independence in ADLs for as long as possible
(3)Manage fatigue
(4)Optimize psychosocial wellbeing
(5)Adjust to the illness, and
(6)Reduce factors that precipitate exacerbations.

Nursing Implementation

- The patient with MS should be aware of triggers that may cause

worsening of the disease:
● infection (especially upper respiratory and urinary tract
infections [UTIs]
● trauma
● immunization
● childbirth
● stress
● change in climate.

★ Each person responds differently to triggers. Help the patient

identify triggers and develop ways to avoid them or decrease their
effects.

- Help the client deal with anxiety

● During the diagnostic phase, reassure the patient that certain
diagnostic studies must be done to rule out other neurologic
disorders, even if a tentative diagnosis of MS has been
made.
● The patient with recently diagnosed MS may need help with
the grieving process.
 - Prevent complications of immobility to include:
- Respiratory complications
- Urinary tract infections - bladder control is a major problem
for many patients with MS.
- Pressure injuries
- Constipation - high fiber diet.

- Focus patient teaching on general resistance to illness.

● This includes avoiding fatigue, extremes of heat and cold,
and exposure to infection.
● Encourage early treatment of infection when it occurs.
● Teach the patient to seek a good balance of exercise and
rest; minimize caffeine intake; and eat nutritious,
well-balanced meals.
● The patient should know the treatment plan, drug side
effects, how to identify and manage side effects, and drug
interactions with over-the-counter (OTC) drugs. The patient
should consult the HCP before taking any OTC drugs.

Evaluation

The expected outcomes are that the patient with MS will

● Maintain or improve muscle strength and mobility
● Use assistive devices appropriately for ambulation and mobility
● Maintain urinary continence
● Make decisions about health and lifestyle modifications to manage
MS