Neuromuscular Flashcards
Autoimmune Neurological Disorders
- Multiple Sclerosis
- Myasthenia gravis
- Guillain-Barre syndrome
Multiple Sclerosis
A chronic, progressive immune-related disease involving demyelination of CNS
- Typically relapsing and remitting
- Peak age is 25-35; 2X more women
S/Sx of Multiple Sclerosis
- Fatigue
- Depression
- Weakness
- Numbness
- Difficulty in coordination
- Loss of balance
- Pain
MS: Visual Disturbances
- Blurred vision
- Diplopia
- Patchy blindness (scotoma)
- Total blindness
MS: Bladder Dysfunction
- Urgency
- Frequency
- Incontinence
- Retention
MS: Motor Dysfunction
- Weakness in limbs (esp. lower extremities)
- Ataxia
- Poor coordination/loss of balance
MS: Abnormal Reflexes
- Hyperreflexia or absent
- Muscle spasticity (especially in lower legs)
- Tremor possible
MS: Bowel Dysfunction
- Bowel incontinence (rare)
2. Constipation (common)
MS: Sensory Dysfunction
- Decreased perception to pain, temp, and touch
- Numbness
- Pain
- Paresthesia
MS: Emotional/Mental Changes
- Depression
- Labile emotion
- Confusion
- Apathy
- Loss of memory
- Poor concentration
How to treat the emotional/mental changes from MS
SSRI’s and/or other antidepressants
Drugs to treat the fatigue of MS
- Amantadine HCl
- Modafinil
* * Heat and exercise makes fatigue worse
Medications for Relapsing-Remitting MS
Glatiramer acetate SQ daily (is not an interferon, has minimal side effects, but takes longer to show effectiveness)
Medications for Secondary Progressive MS
Mitoxantrone is good for this type (monitor closely for cardiac toxicity)
Types of MS
- Relapsing-Remitting
- Primary Progressive
- Secondary Progressive
Primary Progressive Course of MS can result in what?
- Quadriparesis
- Cognitive dysfunction
- Visual loss
- Brain stem syndromes
Factors that trigger MS relapses
- Viruses and infections
- Cold climate
- Physical injury
- Emotional stress
- Pregnancy
- Fatigue
- Overexertion
- Temperature extremes
- Hot shower/bath
MS: Medical Management (maintenance)
- Interferon Beta-1a IM weekly
- Interferon Beta-1b SQ every other day
- Refib - SQ every other day
* * Should be started as soon as possible
* * Can cause them to have flu-like symptoms
MS: Medical Management (relapse, inflammation)
Corticosteroids (reduce inflammation in acute exacerbation)
- Methylprednisone (IV)
- Prednisone (PO)
- Dexamethasone
MS: Side Effects of Corticosteroids
- Mood swings
- Weight gain
- Watch electrolytes
* * Avoid long-term use
Medical Management of MS muscle spasms
Muscle Relaxants
- Baclofen (** avoid stopping abruptly due to withdrawal symptoms)
- Diazepam
- Tizanidine
- Dantrolene
- Nerve blocks/surgery
Medical Management of MS fatigue/impairment of ADLs
- Amantidine
- Pemoline
- Modafinil
Medical Management of MS ataxia
- Beta blockers (propanolol)
2. Antiseizure meds (gabapentin)
Medical Management of MS bowel/bladder problems
- Oxybutynin helps with bladder spasms
- Nitrofurantoin
- Vitamin C acidifies urine, decrease bacterial growth
- Docusate sodium - stool softener used for constipation
Myasthenia Gravis
Autoimmune disorder affecting the transmission of impulses from nerves to involuntary muscles
- Acetylcholine receptor antibodies interfere with neuromuscular impulse transmission, producing severe muscle weakness
- Acetylcholine is essential for proper neuromuscular function
- Onset gradual
Manifestations of Myasthenia Gravis
- Initially, involves ocular muscles (diplopia, ptosis)
- Bulbar weakness: weakness in the face and throat; impaired swallowing, issues with eyes
- Laryngeal s/sx (dysphonia, choking, aspiration)
- Generalized weakness (extremities and intercostal muscles; strength improves after rest)
Myasthenia Gravis Diagnostics
- Acetylcholinesterase inhibitor test
- MRI (may see enlarged thymus gland)
- EMG (may confirm defect in neuromuscular transmission)
- Serum and AchR (acetylcholine receptor antibodies)
Acetylcholinesterase Inhibitor Test
Edrophonium chloride
SE: bradycardia, sweating, cramping
** Have atropine on hand!!!!
** Edrophonium is given IV. 30 seconds after injected, the ptosis will disappear for at least 5 minutes
Pharmacologic Therapy for Myasthenia Gravis Includes what?
- Anticholinesterase medication
- Immunosuppressive therapy
- IV immune globulin (IVIG)
- Plasmapheresis
Surgical Plan for Myasthenia Gravis
Thymectomy
- Partial or complete remission
- ICU/temporary ventilator (post Thymectomy, monitor resp status closely, observe for signs of pneumo- or hemothorax)
- Benefit may take up to 3 years (circulating T cells have long life span)
Anticholinesterase Medication
For Myasthenia Gravis
- pyrostigmine bromide (1st line of therapy)
- Must give on schedule
Immunosuppressive Therapy
For Myasthenia Gravis
- If pyrostigmine bromide is ineffective
- Corticosteroids (decreases immune response and decreases antibody production)
Nursing Considerations for Myasthenia Gravis
- MUST avoid procaine (novocaine) at dentist
- Careful with abx, cardiac, anti-seizure meds, OTC meds
- MUST talk to HCP about medications to be taken
IV Immune Globulin (IVIG) treatments
For Myasthenia Gravis
- Treats exacerbations, east to administer, involves the administration of pooled human gamma-globulin
Plasmapheresis
For Myasthenia Gravis
- Treats exacerbations
- Temperature decreases circulating antibodies (75% improve, but lasts only a few weeks)
Myasthenic Crisis
- Result of disease exacerbation or a precipitating event
- Severe generalized muscle weakness with respiratory bulbar weakness = mechanical ventilation
- Patient may develop respiratory compromise and failure
- Hypertension
Assessment/Supportive Measures for Myasthenic Crisis
- Ensure airway/respiratory support (chest PT, postural drainage, suction, mechanical ventilation)
- ABGs, serum electrolytes, I/O, and daily weight
- If dysphagia, may have nasogastric feeding
- Avoid meds that cause CNS depression
- Maintain muscle function
- Establish system for communication
Guillain-Barre Syndrome
- Autoimmune Syndrome
- Acute attack on peripheral nerve myeline and cranial nerves secondary to antibodies produced
- Rapid Demyelination
- Possible respiratory failure
- Autonomic nervous system dysfunction
- CV instability
Guillain-Barre Syndrome: Manifestations
- Weakness and diminished reflexes of the lower extremities (usually ascending)
- May involve bulbar weakness, cranial nerve symptoms (swallowing, talking), tachy or brady, hyper or orthostatic hypotension, respiratory difficulty, etc.
- NO alteration in cognition of LOC
Guillain-Barre Syndrome: Timeline
- Initial period: 1 to 3 weeks onset of symptoms until neurological deterioration stops
- Peak severity about 2 weeks after symptoms
- Client may report weakness with no history of injury, virus with 1 to 3 weeks prior
- Decreased/absent deep tendon reflexes
Guillain-Barre Syndrome: Diagnosis
- Recent viral infection
- S/Sx (unique presentation: symmetric weakness, diminished reflexes, upward progression of motor weakness)
- CSF: high protein (otherwise normal cells)
- Vital capacity and negative inspiratory force is ASSESSED!!!
Guillain-Barre Syndrome: Medical Management
Medical Emergency due to rapid progression
- ICU with continuous monitoring/respiratory support
- Prevent complications (possible elective intubation)
- Plasmapheresis/IVIG (with rapidly progressing paralysis, IVIG has fewer side effects)
Guillain-Barre Syndrome: Nursing Management
- Maintain respiratory function (spirometer, HOB up, TCDB, chest PT, ventilation, suction, etc.)
- Monitor for autonomic dysfunction
- Maintain maximum mobility (support extremities, ROM, turn Q2h, padding/pressure relief, TEDs, anticoagulants)
- Adequate nutrition/fluid
- Means of communication; decrease fear/anxiety
Guillain-Barre Syndrome: Potential Complications
- Respiratory failure
- Cardiac dysrrhythmias
- DVT
- PE
Amyotrophic Lateral Sclerosis (ALS)
- Degeneration/loss of motor neurons - these are the nerve cells that control muscles
- As neurons die the muscle atrophy
- Progressive, incapacitating and fatal; life expectancy 3-5 years
- Death usually due to respiratory failure or aspiration
Amyotrophic Lateral Sclerosis: Chief Symptoms
- Muscle fatigue
- Progressive muscle weakness
- Cramps
- Twitching
- Incoordination
* * Weakness starts in the muscles supplied by the cranial nerves in 25% of patients
Amyotrophic Lateral Sclerosis: Diagnosis
- Muscle biopsy
2. EMG with nerve conduction studies
Amyotrophic Lateral Sclerosis: Nursing Management
- Maintain/improve function well-being, quality of life
- Treat s/sx, prevent complications (immobility, aspiration, respiratory problems [cough assist machines])
- Monitor nutrition
- Emotional support
Amyotrophic Lateral Sclerosis: Medications
- Riluzole - glutamate antagonist (only FDA approved drug for ALS)
- Baclofen (for muscle spasms)
Amyotrophic Lateral Sclerosis: End of Life Issues
- Pain
- Dyspnea
- Delirium
- PEG tube
* * Discuss advanced directives and living wills
Scleroderma
- Is a complication of an autoimmune disorder affecting the skin, blood vessel walls, and internal organs
- No specific test to diagnose
- Two types: localized and systemic
- Cause is unknown but thought to have an immunologic basis
CREST Syndrome
Associated with Scleroderma
- C: Calcinosis (abnormal deposition of calcium salts in tissues)
- R: Raynaud’s (arteriolar vasoconstriction, results in coldness, pain, and pallor of the fingertips and toes)
- E: Esophageal hardening (swallowing)
- S: Sclerodactyly (induration of the skin of the fingers and toes)
- T: Telangiectasia (vascular lesion formed by dilation of a group of small blood vessels)
Scleroderma: Teaching Client Self-Care
- Explain disease and disease management (monitoring/prevention of complications)
- Medication teaching
- Pain management
- Joint protection - light exercise
- Self-care with assistive devices
- Exercise and relaxation
- Room temp
