ALS
If a client displaying the following symptoms were to be admitted into the hospital, what do these manifestations indicate?
Muscle weakness, Muscle cramps, Stiffness, Spasticity, Slurred speech, Muscle fasciculations, Muscle Atrophy
EARLY SYMPTOMS of ALS
A patient has come in with the following symptoms, what do you suspect the patient has?
Optic nerve: painful eye movement and blurred vision
Cerebellum: tremors and loss of balance and coordination
Brain stem: blurred or double vision
Spinal cord: limb and muscle weakness, spasms, and numbness
MULTIPLE SCLEROSIS
A 35-year-old female patient has come in with trouble thinking/focusing, feeling fatigued, and retro-orbital pain. After exercising in the hot sun, the patient mentioned that she felt very weak afterwards. What do you think the patient is experiencing?
This patient has symptoms indicating MS. After exercising in extreme heat, we can suggest that she is experiencing an attack or flare-up! Complicated by HEAT (UHTOFF's SIGN) and EXERCISE!
What exacerbations can occur in patients who have MYASTHENIA GRAVIS? How can you tell them apart?
MYASTHENIA CRISIS and CHOLINERGIC CRISIS!
MYASTHENIA CRISIS - TOO LITTLE ACh
CHOLINERGIC CRISIS - TOO MUCH ACh
MYASTHENIA CRISIS (DRY SYMPTOMS)
CHOLINERGIC CRISIS (WET S/S)
What nursing considerations should you keep in mind when caring for a patient with MYASTHENIA GRAVIS?
Monitor: neuromuscular status
Medication management
Energy conservation
Safe eating strategies
S/S of myasthenia and cholinergic crisis
How do we treat patients with ANS? Is it curable?
Include Nursing Interventions.
NO CURE FOR ALS.
Medical Management
Disease Modifying:
Supportive care
Riluzole (Rilutek): may help prevent injury to motor neuron, 3-6 months
Symptoms
Symptoms Management
Education
Disease process
Communication
Nutrition
Palliative and Hospice care
A patient has been experiencing:
- FATIGUE
- TROUBLE THINKING/PROBLEM SOLVING
- NYSTAGMUS/RETRO-ORBITAL PAIN
- PARESTHESIA
- LOSS OF BALANCE
What do you think the person has?
MULTIPLE SCLEROSIS
Emotional and Cognitive Symptoms: fatigue, depression, emotional labile, trouble thinking, focusing or problem solving
Speech problems: dysarthria (slowed or slurred speech), dysphagia
Visual System: nystagmus (involuntary, rhythmic eye movements), optic neuritis (diminished vision and color perceptions), diplopia, and retro orbital pain
Sensory System: paresthesia, hyperesthesia, pain, dizziness, vertigo, + Rombergs (a neurological test used to assess balance and coordination) and + Lhermitte’s sign (an electric shock-like sensation that runs down the back and into the limbs when bending the neck forward)
Motor System: tremors, spasticity, spams (painful), ataxia (clumsy), weakness, loss of balance and loss of tendon reflexes
Elimination: bladder dysfunction, constipation, diarrhea or incontinence, sexual dysfunction
What is the following disease?
- AUTOIMMUNE DISEASE WHERE THE BODY ATTACKS RECEPTORS AT THE NEUROMUSCULAR JUNCTION!
- The neuromuscular junction allows VOLUNTARY MUSCLE CONTROL, and ATTACKING these receptors at this junction eventually leads to MUSCLE WEAKNESS!
MYASTHENIA GRAVIS
How do we treat MYASTHENIA GRAVIS?
1. PYRIDOSTIGMINE (ANTICHOLINESTERASE)—1st LINE Tx
Used to increase MUSCLE STRENGTH! by preventing ACh BREAKDOWN!
2. Neostigmine
3. prednisone—IMMUNOSUPPRESSANT
4. IMURAN - only use if steroids are INEFFECTIVE (can cause LEUKOPENIA and HEPATOTOXICITY)
5. IVIG - removes the attacking antibodies
A patient has come in with the following symptoms. What do you suggest they are experiencing?
Pupil constriction, decrease HR and BP, increased tears, salivation and respiratory secretions, V/D, abdominal cramping, muscle fasciculations (twitching), can lead to temporary paralysis
What is ALS and what causes ALS?
What manifestations can we see in patients who have ALS?
How do majority of people die from ALS?
a neurodegenerative disorder affecting nerve cells responsible for controlling VOLUNTARY MUSCLE MOVEMENT!
- progressive muscle weakness starting at the LIMBS
Early symptoms: Muscle weakness, Muscle cramps, Stiffness, Spasticity, Slurred speech, Muscle fasciculations, Muscle Atrophy
Middle stages: Difficulty with mobility (walking), swallowing, breathing
Late stages: Most voluntary muscles are paralyzed, decreased cognition, emotionally labile
End stage: Majority of deaths in ALS result from respiratory failure
A patient with MULTIPLE SCLEROSIS will have two positive test results, which are:
+ ROMBERG'S SIGN (BALANCE)
+ LHERMITTE's SIGN (ELECTRICAL-SHOCK)
In MYASTHENIA GRAVIS, as an autoimmune disease, it causes the body to ATTACK RECEPTORS AT THE NEUROMUSCULAR JUNCTION!
Which NEUROTRANSMITTER IS RELEASED BY THIS JUNCTION, AND WHAT DOES IT DO?
ACETYLCHOLINE (ACh)
- The neurotransmitter that ALLOWS MUSCLE CONTRACTION.
- IN MG, some ACh receptors are BLOCKED, which keeps muscle fibers from accessing ACh, eventually LEADING TO MUSCLE WEAKNESS!
What is the antidote to ACh OVERLOAD (cholinergic crisis or pyridostigmine overdose)?
ATROPINE!
What can result in SEVERE MUSCLE WEAKNESS and RESPIRATORY FAILURE?
CHOLINERGIC CRISIS
What does ALS affect?
Life-Expectancy?
ALS AFFECTS:
- Upper motor neurons, which send signals from the BRAIN to the SPINAL CORD
- Lower motor neurons, which send signals from the SPINAL CORD to the MUSCLES
LIFE-EXPECTANCY: 3-5 years from the time of diagnosis.
ALSO HAS NO CURE AND IS FATAL!
What is MULTIPLE SCLEROSIS, and HOW DO WE TREAT IT?
Chronic Autoimmune Disease
Myelin sheath is damaged → decreased transmission of nerve signals in the central nervous system
Interferon Beta 1 A (Avonex, Rebif)
Major Action
Mechanism of Action
How can we determine if a patient has MYASTHENIA GRAVIS?
THERE ARE 2 MAIN WAYS!
1. TENSILON TEST (NOT USED ANYMORE):
EDROPHONIUM IS INJECTED (prevents ACh from breaking down!)—if the patient becomes stronger after receiving the injection, they have MG!
2. SERUM ANTIBODY TEST - this test is designed to detect the presence of AChR antibodies!
A patient has MYASTHENIA GRAVIS and has been admitted into the hospital with the following symptoms. What medication do you suggest this patient take?
SEIZURES, bronchospasm, excessive secretions, bradycardia, abdominal cramps, diarrhea, excessive salivation, nausea, vomiting, sweating
PYRIDOSTIGMINE (ANTICHOLINESTERASE)
What can result in SEVERE MUSCLE WEAKNESS or RESPIRATORY FAILURE
MYASTHENIA CRISIS
How can we tell someone has ALS?
If a patient is having difficulty with mobility (walking), swallowing, and breathing, what do these manifestations indicate?
NO CAUSE:
DX:
EMG: shows specific changes, loss of nerve supply to muscle - looking for electrical activity in the muscle
END STAGE ALS
How can we tell a patient has MULTIPLE SCLEROSIS?
1. FINDING A DX TAKES TIME.
2. SYMPTOMS COME GRADUALLY. There is no initial symptom that indicates MS
3. MRI - WE ARE LOOKING FOR SCLEROTIC PLAQUES!
4. CSF TEST - OLIGOCLONAL BANDS (INDICATES INFLAMMATION IN THE CNS)
5. EVOKED POTENTIAL TEST - with this test, we are looking for the RATE OF THE PATIENT's NERVE CONDUCTION
What disease does this patient have?
NO PAIN:
W - weakness in the neck, face, arms/legs/hands
E - eye dropping ptosis
A: APPEARANCE: mask-like no expression or look sleepy
K - keeps choking or gagging when eating and will have difficulty swallowing
N - no energy as muscles are used
E - extraocular muscle - double vision
S - slurred speech or hoarse sounding
S - shortness of breath
MYASTHENIA GRAVIS!
What diet considerations should you be educating the patient on?
Timing eating: eat a big breakfast (most energy) and make lunch and dinner small (low energy)
Appropriate consistency: mildly, moderately, and extremely thick
Appropriate texture: soft, minced, moistand smooth/pureed
Temperature
This AUTOIMMUNE DISORDER affects the nerve receptors of voluntary muscles → muscle weakness!
As ACTIVITY INCREASES, THE AMOUNT OF ANTIBODIES PRODUCED ALSO INCREASES!
The thymus functions to produce and mature T-cells, and typically shrinks with age; however, in this disease, the thymus is still large, leading to a thymic tumor
MYASTHENIA GRAVIS