Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

NUR 202 Week 7 > Progressive Neuromuscular Disorders Pt 1: MS & ALS > Flashcards

Progressive Neuromuscular Disorders Pt 1: MS & ALS Flashcards

1
Q

MS: Pathophysiology

Is a chronic neurological disorder in which the nerves of the CNS (brain & spinal cord) degenerate & derives its name from the buildup of scar tissue (___) or plaques that form during ___ (destruction of myelin sheath)

A

sclerosis; demyelinaton

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

MS: Clinical manifestations

  • Impaired sensation, movement, & thinking
A
  • Numbness or weakness in limbs, partial or complete vision loss, tingling or pain, electric-shock sensations w/head movements, tremor, lack of coordination, or unsteady gait, fatigue, & dizziness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
  • Exact cause of MS is unknown
  • Both immune processes & infectious agents have been implicated in MS pathogenesis
  • Demyelination is suspected to be triggered by a viral infection, probably by EBV
A
  • Variety of precipitating factors can precede the onset or an exacerbation of MS like infection, physical or emotional stress, pregnancy, & fatigue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Etiology, Incidence, & Prevalence

  • Immune-mediated dz
  • 2.3 mil worldwide have MS w/onset between 20-50 yrs
  • Affects women > men
A
  • Immune-mediated dz that affects the myelin sheath & conduction pathway of the CNS

Causes
- Immune
- Environment
- Infectious
- Genetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Immune

  • Scientists know that there’s an abn immune-mediated response that attacks the myelin coating, CNS, & nerve fibers, but are unclear what sets this in motion
A

Environmental

  • Some evidence supporting that those closer to the equator have a stronger immune system & lower chance of development; colder climates see a higher inc rate
  • Smoking inc risk
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Infectious

  • Exposure to viruses, bacteria, & other microbes during childhood; demyelination & inflammation occur & are a triggering factor in MS development
  • Studied but no proof yet → measles, canine distemper, HSV-6, EBV, chlamydia pneumonia
A

Genetic

  • MS is not hereditary but having a 1st deg relative sig inc risk
  • Id’ing >100 gene variants
  • Studying epigenetics aka lifestyle choices that turn off & on genes
  • Some discounted causes - living w/a dog or small pet d/t canine distemper; allergies; heavy metal exposure; physical trauma; aspartame
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

4 Major types of MS

  • Relapsing-remitting
  • Primary progressive
  • Secondary progressive
  • Progressive-relapsing
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

?

Is characterized by frequent relapses w/partial recovery but not a return to baseline

This type is seen only in a small % of pts; progressive, cumulative sx’s & deterioration occur over several yrs

A

Progressive-relapsing (PRMS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

?

Involves a steady & gradual neurologic deterioration w/o remission of sx’s

Pt has progressive disability w/no acute attacks

Pts w/this type of MS tend to be between 40-60 y.o. @ dz onset

A

Primary progressive (PPMS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

?

Begins w/a relapsing-remitting course that later becomes steadily progressive

About 1/2 of all ppl w/RRMS develop ___ within 10 yrs

Current addn of dz-modifying rx’s as part of dz management may dec the development of ___

A

Secondary progressive (SPMS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

?

Is the classic picture that occurs in most cases of MS; course of dz may be mild or moderate

Sx’s develop & resolve in a few wks to mos, & pt returns to baseline

During relapsing phase, pt reports loss of function & the cont’g development of new sx’s

A

Relapsing-remitting

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MS: Management

  • No spec test & can be difficult to diagnose
  • Hx, physical, & neurological exam
  • No cure
A

Treatment

  • Improving the speed of recovery from attacks
  • Reducing the # of attacks
  • Slowing the dz progression
  • Rx’s used to treat clinical manifestations
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Interferon beta-1a (AVONEX or Rebif)

An immunomodulator that modifies the course of dz & also has antiviral effects

A

Interferon beta-1b (Betaseron, Extavia)

  • Another immunomodulator w/antiviral properties
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Glatiramer acetate (Copaxone)

A synthetic protein that is similar to myelin-based protein

A

Mitoxantrone (Novantrone)

An anti-neoplastic anti-inflammatory agent used to resolve relapses but w/risks for leukemia & cardiotoxicity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

?

The 1st Mab approved for MS that binds to WBCs to prevent further damage to the myelin (may cause PML, a viral infection of the brain)

Monitoring near status is key; PML confirmed by MRI & CSF

! Rx also causes damage to the liver; routine LFT’s & self-monitoring req’d

A

Natalizumab (Tysabri)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Fingolimod (Gilenya)
teriflunomide (Aubagio)
dimethyl fumarate (Tecfidera)

Are newer oral immunomodulating drugs

! May cause bradycardia, esp within the 1st 6 hrs after taking it

A

Common s/e’s are facial flushing & GI dist

Routine labs essential as this may cause dec in WBC, predisposing pt to infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q
  • Medical marijuana (cannabis) also used sometimes for sx management to help reduce pain, muscle stiffness, & spasticity
  • Pts need to be counseled on use as it may impair cognitive function, cause dizziness &/or intoxication, depending on form
A
  • Corticosteroids (e.g., methylprednisolone (SoluMedrol), dexamethasone, or prednisone), anti-inflammatory, & immune-suppressing rx’s used short-term for exacerbation or initial onset

! Is just for an acute or initial episode; then pt is maintained on prev mentioned rx’s

18
Q

Medication Symptom Management: Spasticity

▴ muscle relaxers
* baclofen (Lioresal)
* tizanidine (Zanaflex)
* dantrolene sodium (Dantrium)

▴ benzodiazepines
* diazepam (Valium)

A

▴ Also, a potassium channel blocker may be prescribed to help walking speed & ability - dalfampridine (Ampyra)

19
Q

Other Symptoms

▴ Paresthesias - anti-epileptics & anti-depressants; e.g., carbamazepine (Tegretol); amitriptyline (Elavil)

▴ Cerebellar ataxia - may use propanolol & clonazepam

A

▴ Fatigue may utilize amantadine HCl (Symmetrel), methylphenidate (Ritalin), modafinil (Provigil)

▴ Constipation - docusate sodium [stool softeners] (Colace)

▴ Urinary retention - bethanechol (Urecholine)

▴ Urinary incontinence - oxybutynin (Ditropan)

20
Q

MS - Assessment: Recognize Cues

History
* Vision, mobility, sensory perception changes

Physical assessment/s&s
* Muscle weakness & spasticity
* Tremors
* Dysmetria
* Dysphagia

A

Psychosocial assessment
* Anger & frustration @ length of time to obtain dx

Lab assessment
* CSF

Other diagnostic assessment
* MRI brain

21
Q

?

Is a condition in which there is improper measuring of distance in muscular acts

?

Is overreaching (overstepping)

?

Is underreaching (understepping)

A

Dysmetria

Hypermetria

Hypometria

22
Q

Common clinical manifestations

▴ Muscle stiffness or spasms

▴ Paralysis, often in the legs

▴ Problems w/bladder, bowel, or sexual function

A

▴ Mental status changes - memory loss, problems concentrating, visual perception, word finding but general intellect is intact; emotions can be anger, depression, euphoria

23
Q

Complications

  • B/c MS affects nerves throughout body, comp’s can vary
  • May be periods of worsening & have a recovery period w/some improvement
  • Radicular (nerve root) pains may be present part’ly in low thoracic & abd regions
A
  • Lhermitte’s sign is electrical shocks that shoot down the spine when head is flexed forward; commonly seen in MS but also in cervical problems like spondylosis, herniation, etc. (compression of spinal cord)
24
Q

?

Refers to involuntary muscle contractions that’re very painful

1st line of treatment is exercise &/or muscle relaxants like Valium, Baclofen

A

Spasticity

25
Q
  • Bladder problems can be incontinence or urinary retention. Many pts w/MS develop a neurogenic bladder & have to self-cath
  • General intellect, incl long-term memory, conversational skills, & reading comprehension, remains unchanged & intact
A
  • Normal life expectancy if sx’s are well managed. Death usually occurs as a result of infectious complications (e.g., pna), immobility, or b/c an unrelated dz
26
Q

?

Is a disorder of language

A

dysphasia

27
Q

?

Is a disorder of speech

A

dysarthria

28
Q
  • Speech is the process of articulation & pronunciation; it involves the bulbar muscles & the physical ability to form words
  • Language is the process in which thoughts & ideas become spoken; it involves the selection of words to be spoken, called semantics, & the formulation of appropriate sentences or phrases, called syntax
A

Sensory
▴ numbness, tingling, blurred vision
▴ vertigo, tinnitus, dec hearing
▴ chronic neuropathic pain, Lhermitte’s sign

Cerebellar
▴ nystagmus, ataxia, dysphagia
▴ scanning speech - pauses between syllables of words (form of dysarthria)
▴ seizures

Sexual dysfunction
▴ ED, dim libido
▴ difficulty w/orgasmic response, painful intercourse, dec lubrication

29
Q

?

Is an intense burst of pain like an electric shock that runs down the back into the arms and legs when the neck is moved

“barber chair” sign; can be painful, but is not life-threatening

Sometimes r/t severe B12 deficiencies

A

Lhermitte’s sign

30
Q

?

Occurs b/c of damage to the optic nerve

Interferes w/the transmission of signals between the eyes & brain

Sx’s incl blurry or reduced vision & it’s often 1 of the 1st sx’s of MS

A

Uhthoff’s sign (think hot)

31
Q

MS - Analysis: Analyze Cues & Prioritize Hypotheses

  • Impaired immunity d/t the dz & rx therapy for dz management
  • Dec or impaired mobility d/t muscle spasticity, intentional tremors, &/or fatigue
  • Dec visual acuity & cognition d/t dysfunctional brain neurons
A

Diagnostic Testing

  • MRI shows presence of multiple plaques
  • Electrophoresis of CSF
  • Evoked potential studies
  • Neuropsychological test (assess cognitive impairment)
32
Q

90% of ppl w/MS have been found to have elevated amounts of an antibody protein called ___ in their CSF to a greater deg than is present in their blood serum

A

IgG

33
Q

?

Are the electrical signals generated by the nervous system in r/t sensory stimuli

Auditory, visual, & somatosensory stimuli are used commonly for clinical studies (DELAYED in persons w/MS)

Can help define the extent of the dz; are used for clinical dx in pts w/neurologic dz

A

Evoked potentials

34
Q

MS - Planning & Implementation: Generate Solutions & Take Action

  • Managing impaired immunity
  • Improving mobility - exercise will help to preserve function
  • Managing dec visual acuity & cognition
A

MS - Care Coordination & Transition Management

  • Home care management
  • Self-management education
  • MS affects the whole family & is often unpredictable & uncertain in terms of dz course
  • Healthcare resources (e.g., National MS Society)
35
Q

Amyotrophic Lateral Sclerosis (ALS)

  • Lou Gehrig’s dz
  • Sx’s usually develop >age 50 (40-60)
  • Prevalence 3.9 per 100,000 people
  • Death d/t resp failure within 3-5 y.o. sx onset
  • Affects voluntary muscle control & men > women; no treatment course & considered 100% fatal
  • Both upper & lower neurons degenerate & die
A
  • Unable to function, muscles gradually weaken, atrophy, & twitch (fasciculation); progressive degeneration leads to death of cells, resulting in brain losing ability to initiate & control muscle movement
  • Eventually all voluntary muscles are affected & pts are paralyzed but doesn’t impair senses or ability to think
36
Q

ALS: Clinical Manifestations

  • Muscle cramps or stiffness, muscle weakness, slurred speech, & difficulty swallowing
  • Upper motor neuron damage assoc w/spasticity
  • Lower motor neuron damage assoc w/flaccidity
A
37
Q

Not-so-Diagnostic testing

❐ No single test can be used to dx ALS
❐ CT scan &/or MRI of neck & head to r/o other cond that can mimic ALS like spinal cord tumors, herniated discs, spondylosis, MS, etc.
❐ LP for CSF protein/cytology
❐ Ck will be elevated

A

❐ EMG shows fibrillations or fasciculation; US can show these in deep muscles & can lead to early dx’s

❐ Repeated muscle strength testing to look for loss

❐ Swallow evals to r/o risk for aspiration

38
Q

❐ RPR for neurosyphilis
❐ ANA for general autoimmune screen
❐ TSH for hypothyroid
❐ Serum protein electrophoresis for protein disorders

A

❐ Rheumatoid factors
❐ ESR as a general inflammatory screen
❐ Creatine phosphokinase (CPK) for muscle injury
❐ Electrolytes for imbalances
❐ CBC’s for leukemias/plasma cell dyscrasias

39
Q

❐ Anti-MAG & anti-GM1 antibodies for other neuromuscular diseases

❐ Parathormone for deficiency

❐ UA/24 hr urines for heavy metals

A

❐ Serum lead, acid maltase, & hexosaminidase lvls for other metabolic-neuromuscular diseases

40
Q

Management - Symptomatic Treatment

▻ benzothiazole (Riluzole, Rilutek)

▻ Muscle relaxants for antispasmodics - baclofen for muscle spasms

▻ Laxatives (Miralax, MOM) for constipation

A

▻ Analeptics (Provigil or Doxapram) for fatigue

▻ TCAs (e.g., amitriptyline) for excessive salivation, pain, & depression

▻ Antimuscarinics, anticholinergics to help secretions

41
Q

benzothiazole (Riluzole, Rilutek)

▻ Decreases the glutamate lvls, an amino acid that affects the nerves that send msgs from the brain to the muscles

S/e’s
* hepatic injury
* neutropenia
* interstitial lung dz

A
  • Drug trials w/Gilenya (an MS drug) but not appvd for ALS
  • IPLEX - under investigational new drug application; used to treat growth failure in children w/severe primary IGF-1 deficiency or w/GH gene deletion who have developed neutralizing antibodies to GH
42
Q
  • Treatment includes rx’s for symptomatic relief, such as baclofen for muscle cramps, laxatives & stool softeners for constipation, analeptics (CNS stimulants) for fatigue & weakness, & TCA’s for excessive salivation, pain, & depression
A

Nursing Care

  • Maintain patent airway
  • Facilitate effective communication
  • Make appropriate referrals
  • Assess coping & depression
  • Assess swallow reflex
  • Nutritional needs
  • Utilize conservation measure(s)
  • Address possible advance directive

Remember - the psychological effects of intact cognitive ability in the face of progressive paralysis & wasting

NUR 202 Week 7 (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions