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7/28/2019 MND-ALS-AGB

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Neuromuscular Problems Lecture

Rose Bianchi, RN, DNSc.

Copyright 2011, 2009


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Guillain-Barre Syndrome (GBS)

___________________________

Definition:

1. a rare form of polyneuritis

2. acute, rapidly progressing, potentially fatal

form of polyneuritis


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Clinical Manifestations of GBS

___________________________1. Painparesthesias, muscle aches, cramps, &

hyperesthesias

2. Develops 1-3 weeks after an URI or GI infection3. Weakness of L. E. (mostly symmetrical) occurs

over hours to days or weeks, peaks in 14 days,progresses to numbness, tingling then paralysis

4. Absent or diminished deep tendon reflexes, lossof deep sensations

5. Autonomic dysfunction seen in patients withsevere muscle involvement & respiratory muscle

paralysis


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___________________________6. Dangerous autonomic dysfunction may

occur: orthostatic hypotension,

hypertension, & abnormal vagaldysfunctions such as bradycardia, heart

block, & asystole

7. Other ANS dysfunctionsbowel & bladderdysfunction, facial flushing & diaphoresis


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___________________________8. Lower Brainstem progression affects the

following cranial nerves:

Nerve 7FacialNerve 6 - Abducenseye movement

Nerve 3Oculomotoreye movement

Nerve 12Hypoglossaltongue muscleNerve 5Trigeminal;- sensory facial

Nerve 10Vagusmotor larynx, heart & lungs


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Prognosis of GBS

___________________________

Complete recovery months to years if nerve

regeneration occurs


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Diagnostic Tests for GBS

___________________________1. CSF elevated protein level after 7-10 days,

500700 mg / dl

2. EMG (electromyogram) and Nerve ConductionStudies show reduced nerve conduction velocityin affected limbs

3. Some patients not all have:

a. Immunoglobulin M (Ig M) antibodiesb. Anti-GM1

c. Anti-GD1b


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Drug Treatment for GBS

___________________________1. Plasmapheresismust be done within the

first 2 weeks, plasma exchange therapy

2. *IV High Dose Immunoglobulin to protect

body against organisms

3. Corticosteroids have little affect


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Amyotrophic Lateral Sclerosis

(ALS)______________________Definition & Pathophysiology:

1. Also, called Lou Gehrigs Disease

2. A progressive, degenerative disorder of themotor neurons in the spinal cord, brain stem, &motor cortex

3. Lower & upper motor neurons are involved.

4. Degeneration of pyramidal tract & motor cells ofthe anterior gray horns

5. Electrical & chemical messages originating inthe brain do not reach muscles


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Etiology of ALS

___________________________1. 10% of people with ALS have an

autosomal dominant gene on chromosome

21a. 25% of the time this is the defective

superoxide dismutase gene


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Incidence of ALS

___________________________1. Ages of occurrence 40-70 years of age

2. Male to Female ratio 2:1, equalizes after

menopause

3. Peak occurrence at age 50

4. 6-8 cases per 100,000 people


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Clinical Manifestations of ALS

___________________________1. Upper extremityweakness, dysarthria, dysphagia,

muscle wasting, no cognitiveimpairment

2. Progression:1stsinglemuscle group - paresis

2ndasymmetricalinvolvementofcorresponding muscle groups

3rdallstriatedmuscle involvedexceptextraoccular&heart

4thflaccid&spasticparesisparalysis

5th

uncommon

-anal

&

urethral

s hincter weakness


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Clinical Manifestations of ALS

__________________________3. Hypotoniadecreased resistance to passive movement,

hypoactive or absent deep tendon reflexes, absent

abdominal reflexes, cremasteric reflexes, & Babinski sign

4. Atrophysevere muscle wasting; metabolic changesthin

skin & hair, thickened nails, decreased perspiration; &

fasiculationinvoluntary contraction or twitching of

muscle fibers

5. Thick Saliva or Thin Excessive Saliva (Drooling)


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Prognosis of ALS

___________________________1. Average life span 2-6 years after diagnosed

2. Longest life span 15 years

3. Cause of death respiratory infection


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Diagnostic Studies for ALS

___________________________1. EMG & Muscle Biopsyto verify lower motor neuron

degeneration & denervation (muscle bx. is not needed to

confirm diagnosis)

2. Some patients have RNA strands of: echovirus in the

CSF.

3. Patients have elevated levels of myelin basic protein in

CSF


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Drug Therapy for ALS

__________________________1. Rilzole (Rilutek)

a. Antiglutamateprevents neural degeneration from

over excitation

b. Dose 50 mg po q 12 hrs.

c. Side EffectsGI complaints, & worsening symptoms

d. Slows progression by a few months


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Other Treatment for ALS

___________________________1. Symptom Relief

2. Prevent Complications

3. Maintain Maximal Function

4. Optimize Quality of Life (QOL)


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Nursing Diagnoses

___________________________1. Alteration in Respiratory Function R/T Progression of

Disease Process

(Seen in GBS early phase, and ALS in later phase)

a. Expected Outcome

b. Interventions

2. Risk for Aspiration R/T Dysphagia ( Seen in both GBS

& ALS)a. Expected Outcome

b. Interventions


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Nursing Diagnoses

___________________________3. Alteration in Comfort R/T paresthesias, muscle aches and

cramps & hyperesthesias in GBS; and just muscle

weakness in ALS

a. Expected Outcome

b. Interventions

4. Impaired Verbal Communication R/T Intubation or

Paralysis of the Muscles of Speech (Seen in both GBS &

ALS)

a. Expected Outcomes

b. Interventions


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Nursing Diagnoses

___________________________5. Fear or Anticipatory Grieving R/T Uncertain Outcome &

seriousness of the Disease Process

(Seen in Both GBS & ALS)

a. Expected Outcome

b. Interventions

6. Self-Care Deficits R/T Inability to use muscles to

accomplish ADLsa. Expected Outcomes

b. Interventions


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Nursing Diagnoses

___________________________7. Alteration in Urinary Elimination R/T Inability to Void

2ary to Urinary Retention from a Flaccid Bladder (Seen In

GBS)


b. Interventions

8. Alteration in Bowel Elimination R/T Decreased GI

Motility, Immobility, & Diet Changes (Seen in GBS)


b. Interventions


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Nursing Diagnoses

___________________________9. Diversional Activity Deficit R/T

Immobility (Seen GBS & ALS)

a. Expected Outcomesb. Interventions

10. Risk for Impaired Skin Integrity R/T

Immobility (Seen in GBS & ALS)a. Expected Outcomes

b. Interventions

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