Test #4 Flashcards
What does ALS stand for
Amyotrophic lateral sclerosis
What is ALS
- Progressive loss of motor neurons in the cerebral cortex and spinal cord, which causes muscle atrophy
- Causes firmness in tissues = sclerosis
Breakdown the ALS name
Amyotrophic (muscles wasting away) lateral (happens symmetrically) sclerosis (leads to firmness of the tissues)
Is cognition affected in ALS
No - they are still there (think of steven hawking)
What is the most common complication of ALS
Respiratory (lose muscles to swallow and cough, etc)
Is there a genetic form of ALS
Yes, but most people don’t have the genetic form (autosomal dominant pattern of inheritance) (they might have a genetic predisposition though)
What are risk factors for developing ALS 9
- Smoking
- Exposure
- Severe stress
- Head trauma (football players, etc)
- Living in the western pacific islands
- Persistent physical exhaustion
- Viral infections
- MIs
- Malnutrition
Who is more at risk for ALS
Higher incidence in men, but after menopause, both men and women incidence is equal
What is an early symptom of ALS that people usually write off
- *Asymmetrical painless weakness in one limb (not loss of sensation - will eventually turn into symmetrical bilateral weakness, but typically starts in one part of the body first)
- Dominant arm more commonly affected
What are other common early symptoms of ALS
- Tripping/loss of balance
- Weight loss
- Speech problems
- Difficulty chewing/swallowing
- *Drooling
- Tongue tremors
- Breathing difficulties
- Choking
- Crying spells or periods of inappropriate laughter (caused by bulbar palsy)
- *Vesiculations (muscle twitching)
- Secretions in lungs
- Poor/absent gag reflex
What do we use to help monitor a pt’s ALS progression
The ALS Functional Rating Scale (the higher the number the better, a decrease in number indicates further decline)
How do we diagnosis ALS
- Through an electromyography (EMG)
- Muscle biopsy
- Cerebrospinal fluid analysis
- Pulmonary function tests
- Computed tomography scan
- MRI
- Genetic testing
- HIV testing (to rule out)
- Lyme disease (to rule out)
When do we see the typical onset of ALS? What if we see it earlier?
Typically ALS is seen around 65. If earlier, it is most likely the genetic form.
Are there any tx options for ALS?
Not really - tx is limited. There is riluzole, which can be given to help slow progression.
Go over vent management
What are some nursing interventions for ALS
- Keep upright for 30 min after a meal
- Chest physiotherapy
- IS
- Encourage periods of rest prior to eating
What is Guillain-Barre Syndrome (GBS)? What can it lead to?
- Acute autoimmune response that occurs a few days or weeks after a viral or bacterial infection, where demyelinating polyradiculoneuropathy (AIDP) occurs
- Can lead to paralysis and respiratory failure
What are most GBS patients on
Vents
What is happening in GBS
Immune response, usually from IgG immunoglobulins (antibodies formed from bacteria or virus), after a bacterial or viral infection
Is GBS permanent
No, re-myelination can occur, which can help your body regain functions
Symptoms of GBS
- Lower extremity weakness progresses to flaccidity (usually within hours or days) (starts as ascending)
- Motor loss is symmetrical
- Weakness will continue up and threaten respiratory muscles
- Pain, increasing at night
- Orthostatic hypotension or hypertension
- Abnormal vagal responses like bradycardia, or heart block
- Disfunction with bladder and bowel
- Facial flushing
- Diaphoresis
- Weak or absent reflexes
What is the main feature of GBS
Ascending, rapidly progressing, symmetrical weakness of the limbs
What is the most common cause of death for someone with GBS
Respiratory arrest
How do we diagnosis GBS
- Symptoms
- Cerebral spinal fluid (elevated protein, but normal cell count)
- Nerve conduction studies (will show decreased conduction)
How long can GBS last?
- 1-3 weeks for the paralysis to advanced completely up the body, then it plateaus for 2-4 weeks, then functions descends down the body
Is there a cure for GBS
No, we have to let the disease run its course
What are txs for GBS
- Plasmapheresis (giving plasma)
- Intravenous immune globulin (IVIG) (helps boost immune system)
What are some complications of GBS 5
- Atelectasis
- Pneumonia
- Pneumothorax
- Hypertension
- Tachycardia
Even though GBS can be reversible, what kind of care might they need afterwards
Will require PT/OT afterwards to regain sterngth and functions
What are nursing interventions for GBS
- Enteral feedings
- Helping control pain (can be painful just laying there)
Why would someone with GBS be in pain?
Because they have only lost their motor function, they have not lost their sensory function, so they can still feel
Is consciousness affected in GBS
No
When do we typically see pts show signs of Huntington’s
35-45
What are the symptoms of Huntington’s
- Chorea: excessive, involuntary movements that appear to be writhing and twisting movements of the face, limbs and body (these are progressive, and will continue to get worse and worse)
- Due to movement issues, there will be issues with speech, chewing, and swallowing = leads to aspiration and malnutrition
- Cognitive decline can vary and is usually suttle (involves perception, memory, attention, and learning = many pts will develop dementia in the end)
- Compulsive behaviors may develop
Huntington’s Chorea
Non-voluntary and uncontrolled movements
How does Huntington affect the mind
- Progressive, cognitive impairments
- Many will develop dementia towards the end
- Compulsive behaviors
How can we diagnosis Huntington’s
Genetic testing
How do you get Huntington
Through genetics (autosomal dominant trait - where there are at least 40 repeats of this CAG gene) (the more repeats, the earlier Huntington’s is going to show up)
What is the issue with Huntington’s
By the time it shows up, you have usually already had children, which means that you may have passed the gene to your kids
Is there a cure for Huntington’s
No, we just treat the symptoms
Besides being worried about respiratory, what else should we be worried about when someone is diagnosed with Huntington’s
Suicide - because these patients will know that it will progressively get worse and worse
What is myasthenia gravis
Autoimmune motor disorder, characterized by extreme fluctuating weakness and fatigue
What do we want people with myasthenia gravis to avoid
Want them to avoid any respiratory risks, like coming into contact with sick people, large crowds, etc. (developing a cold or respiratory issues can send them into crisis)
What is the basic pathophys going on in myasthenia gravis
Acetylcholine receptors are blocked due to antibodies produced by your immune system
The drug effects of what disease can make symptoms of myasthenia gravis worse
Drug side effects from RA
What does myasthenia gravis have to with the thymus
Over 70% of patients with myasthenia gravis have Hyperplasia thymus or tumors on the thymus - which causes higher level of antibodies, which can make the disease worse
If someone has myasthenia gravis with a hyperplasia thymus, what might be done
A thymectomy to take out your thymus
What are the 3 types of myasthenia gravis clinical manifestations
- Ocular (ptosis (drooping eyelids) or diplopia (double vision))
- Bulbar (face and neck)
- Generalized weakness
When do myasthenia gravis pts begin to experience symptoms
Within 2 years of onset
Why would we have ptosis or diplopia
Because of the muscle weakness, which can be the eye muscles
What is important to know about myasthenia gravis symptoms
Symptoms worsen with sustained activity (like staring at the computer)
What are specific bulbar symptoms
- Trouble speaking/slurring of speech
- Issues with chewing
- Issues with swallowing
What are generalized symptoms of myasthenia gravis
- Feeling fatigued doing just about anything
- Takes you longer to get dressed, etc.
- Voice fades after talking for a while
Do we see any cognitive issues with myasthenia gravis
No - they are fine cognitively, they also don’t have any sensory loss, issues with reflexes or muscle atrophy
How do we diagnosis myasthenia gravis
- Symptoms
- Serological testing (assay of acetylocholine antibodies)
- Repetitive nerve simulation test (conduction will become slower overtime, as fatigue sets in, which indicates a positive test)
- Tensilon test (give 10mg of Tensilon (short acting anticholinesterase), temporarily will improve neuromuscular weakness for 2-5 minutes and then weakness returns, shows a positive test)
- Chest CT to look at thymus
What are side effects of Tensilon
- Bronchospasm
- Bradycardia
- Diarrhea
What do we want to have on standby, if a pt is having a bad response to Tensilon
Atropine
What is the drug of choice to treat the long-term effects of myasthenia gravis
Pyridostigmine, which is an anticholinesterase inhibitor
Why would we want an anticholinesterase inhibitor for myasthenia gravis
Because acetylcholinesterase is is breaking down acetylcholine, which we need to make our muscles work (so we want to stop this action)
Would someone with myasthenia gravis be at risk for respiratory issues, like aspiration
Yes - due to bulbar symptoms (like issues swallowing) and generalized fatigue
What is the issue with myasthenic crisis
It can present like a cholinergic crisis
What is happening in myasthenic crisis? What symptoms might we see?
- Exacerbation of the weakness, which can lead to respiratory failure caused by an infection
- Tachycardia
- Flaccid muscles
- Cool, pale skin
- Lasts about 2 weeks
- Will need to be in the hospital
What causes a cholinergic crisis? What are the symptoms?
- Cholinergic crisis due to excessive intake of anticholinesterase agents
- Bradycardia
- Muscle twitching
- Pallor
- Excessive secretions
- Small pupils
What can help us distinguish between a myasthenic or cholinergic crisis
- Tensolin test (Give an anticholinesterase drug (Tensolin), which will either improve their symptoms of myasthenia gravis, but will not improve the symptoms if in a cholinergic crisis
What is multiple sclerosis
Autoimmune disease that affects the myelin sheath of neurons in the CNS (brain and spinal cord) causes these neurons to become inflamed and scarred - this leads to a decrease in nerve signal transmission
What is a good trick to remember that MS affects the myelin sheath
Remember that Myelin Sheath starts with MS
Why do people with MS have different symptoms
Well we all have a lot of different neurons, so it just depends which ones are affected, which can vary from person to person
What are MS symptoms if the cerebellar is affected
- Tremors
- Dysarthria (trouble articulating speech)
- Ataxia
- Cognition`
What are the 4 different patterns of MS
- Relapsing - remitting (most common - where they will have these unpredictable attacks followed by periods of remission)
- Primary progressive (steadily becomes worse)
- Secondary progressive (have earlier periods of remission, but you stop having periods of remission and it progressively gets worse)
- Progressive relapsing (steady decline, with with attacks but no remissions)
When is the typical onset of MS
20-40 years
Where is MS more prevalent
In temperate climates 45-65 degrees (might be subjected to an environmental agent before puberty)
Do we know the cause of MS
No
What might be some contributing factors to MS
- Pre-disposed genetically (higher risk if you have a first degree relative)
- Infection
- Smoking
- Physical injury
- Emotional stress
- Excessive fatigue
- Pregnancy
- Poor health
Is the onset of MS usually gradual or aggressive
Gradual - symptoms come and go so you don’t really pay much attention to them
What is a unique symptom of MS
Lhemitte’s sign - when you flex your neck, you feel a shock wave go down your spine
What is a unique speech pattern that some may develop with MS
Scanning speech
What are the many potential symptoms of MS
- Muscle weakness
- *Lhermitte’s sign
- Positive Romberg’s sign
- Weakness or paralysis in limbs, trunk or head
- Spastic muscles
- Scanning speech
- Issues with coordination and balance (they feel drunk)
- Issues with walking or standing
- Tremors
- Dizziness
- Hearing loss
- Numbness and tingling
- Pain
- Vertigo
- Tinnitus
- Chronic neuropathic pain
- Nystagmus (involuntary movement of eyes)
- Dysarthria (speech slurring)
- Fatigue aggravated by heat and sun, deconditioning
- Exaggerated response to medications
- Issues with cognition in half of individuals (appears later)
- Incontinence
- Frequency or retention
- Diarrhea
- Constipation
- Spastic bladder (causes urinary frequency)
- Flaccid/hypotonic bladder (retention)
- ED
- Decreased libido
What is uhthoff’s sign and how does it relate to MS
Where heat actually makes the MS worse
How can we help prevent a person from relapsing with their MS (if they have that type)
- Avoid heat
- Avoid infections
- Avoid stress
- Avoid overexertion
What are vision issues with MS
- Nystagmus (involuntary movement of eyes)
- Optic neuritis, lead to:
- Double vision
- Blurry
- Dull/gray vision
- Pain moving eyes
What cognitive skills are usually safe from MS
Long term memory, conversational skills and reading comprehension
What do people with MS usually die from
Infection, like pneumonia (because you’re not moving around)
What bladder issue are we worried about with MS
Developing a UTI
What is interesting about a women during pregnancy who has MS? What about post-partum?
The pregnancy can cause them to go into remission due to the hormones, but then after pregnancy they are at a greater risk for exacerbation
Do we have a definitive diagnostic test for MS
No - basically we look at history, symptoms, and some tests
What kinds of diagnostic tests can we do for MS
- MRI of the brain and spinal cord looking for plaques, inflammation, atrophy, tissues breakdown and destruction
- Cerebral spinal fluid analysis (might see an increase in immunoglobulin G or the presence of oligoclonal banding, which indicates inflammation)
- Evoked potential study: responses are often delayed because of decreased nerve conduction from eye and ear to brain
So what things would we need in order to state that someone has MS
- Evidence of at least two inflammatory demyelinating lesions in at least two different locations within the CNS
- Damage or an attack occurring at different times, usually greater than 1 month apart
- Rule out all other possible diagnoses
What are the two biggest take away regarding drug therapy for MS
We try to treat the symptoms and prevent lymphocytes from reaching the CNS and causing damage through different drugs like immunomodulators (anti-inflammatory), corticosteroids, etc…
Besides drug therapies, what are other interventions to help slow down the progression of MS
- Exercise, especially early on
- Good sleep
- Proper nutrition
- Stay away from large crowds
Besides drug therapies, what are other interventions to help slow down the progression of MS
- Exercise, especially early on
- Good sleep
- Proper nutrition
- Stay away from large crowd
How can we treat the spasticity of MS
- Muscle relaxants
- Surgery (neurectomy, rhizotomy, cordotomy
- Dorsal column electrical stimulation
- Intrathecal baclofen pump
Should a person with MS exercise during a period of exacerbation
No
What are three benefits of exercise for someone with MS
- Decreases spasticity
- Increases coordination
- Retrains unaffected muscles to act for impaired ones
What is one of the best ways to exercise if you have MS
Water exercise
What are some potential triggers that may cause an exacerbation of MS
- Infections (especially respiratory and UTIs)
- trauma
- Immunizations
- Childbirth
- Stress
- Changes in climate
What are the characteristics of Parkinson’s Disease (PD)
- Bradykinesia (slowness in your movements)
- Rigidity
- Tremor at rest
- Gait changes
What are the causes of PD
Exact cause is unknown, but we think there are some genetic risk factors in combination with environmental risk factors.