OPTA 222 (ALS and MS)
ALS: Epi
1-2/100000
age of onset 57years
men more affected
What causes ALS?
possibly by
-oxidative stress
-abnomalities in immmunal system
-Glutamate toxicity
inherited:5-10%
randomly:90-95%
Patho of ALA
- upper and lower motor neuron disorder
- demmylination and gliosis of the corticospinal and corticobulbar tracts
-death within 3 years
ALS Upper motor Neuron diagnosis
Lesions are diagnosed by flex testing
- Positive Hoffman's Sign(Flexion or adduction of thumb)
- positive Babinski Reflex(Fexion of big toe)
- spastic paralysis( tight hard muscle, spams or twitch)
ALS Lower Motor Diagnosis
Lesions are diagnosed by
loss of muscle
Flaccidity( foot drop, weak muscles)
Atrophy
Weak or asent deep tendon flexion
ALS Clinical Picture
- Fatigue or loss of exercise tolerance is the most common complaint of patients presenting for diagnosis (80% motor neurons lost at this point)
- Shows upper and lower motor neuron signs in 2 spinal regions and with bulbar signs
- Progression of signs and symptoms
- Exclusionary criteria:presence of sensory and autonomic nervous system dysfunction, sphincter control issues, oculomotor nerve pathway abnormalities,
cognitive deterioration
- Onset symptoms are varied (ie. lower, upper, bulbar)
- Sensory neurons NOT affected
- Cranial nerves III, IV and VI are not affected
-Cognition not affected but can have increased risk of dementia
Diagnosis and Prognosis of ALS
- Diagnosis: EMG, gene testing, nerve conduction studies: Difference between EMG and nerve conduction studies**
- Prognosis: eventual death from respiratory failure
- Death usually within 3 years on onset
ALS Early Stage
- Muscles can be weak and soft or strong and spastic
- Loss of muscle bulk occurs
- Can get fasciculations (characterisitcs of lower motor meuron legion)
- Often effects only a specific area
- Fatigue
- Balance issues
- Tripping
- Begins in upper or lower extremities
- Footdrop
- Hand weakness or clumsiness
- Slurred speech
- Dysphagia
ALS Middle Stage
- Symptoms affect more areas of body
- Swallowing muscles can be affected causing choking
- Some muscles paralyzed can develop contractures
- Breathing muscles can become weakened (worse in lying)
- Emotional symptoms begin:
- Pseudobulbar affect (50% of ALS cases): uncontrollable laughing or crying - Driving privileges taken away
ALS End Stage
- Extremely limited mobility
- May not be able to eat by mouth as swallowing muscles paralyzed - Breathing muscles limited:
- Can cause lack of oxygen
- Susceptible to pneumonia - Death
- Mostly caused by respiratory failure
- Some caused by swallowing issues, malnutrition
ALS Theuraputic Goals Physiscal
mobility aids, positioning, maintaining function, breathing exercises, seating, PROM/pain management
ALS Theraputic Goals: Mental
coping skills, meditation, support groups,
ALS theraputic Goals: Affective
knowing triggers for fluctuations, empathy, support groups, educating family members, coping skills, improving the environment, comfort, provide dignity
ALS Theraputic Goals: Social
improving environment, achieving goals (voice banking), communication strategies, memory preservation
ALS Psychosocial Issues
- 4 Major Fears when given terminal diagnosis (isolation, dependence, pain, death)
- Family given all information: Diagnosis,Prognosis, Possible patterns of progression
- Pseudobulbar affect seen in 50% of ALS patients can complicate issue of depression
- Respect patient’s point of view, allow them to talk or not to talk
MS Epi
-Different for every one symtoms vary day to day even hour tohour
-100,000 Canadian Affected
-life expectancy doesn't change, QOL might
MS Patho
-autoimmunal disorder affects the nervous system or even optic nerves
-Lower Motor Neuron Disease
- slow stipping of myelin called demyelination
-myelin becomes inflamed replace by scare tissue
-interfers with brain's ability to control functions:seeing walking,talking. and smooth flow of messages
MS Course
- Relapses can occur, also called an attack, exacerbation, or flare-up
- during a relapse new symptoms appear or existing ones get worse
- lasting at least 24-48 hours in absence of other illness or infection
- can last days, weeks, or several months
- average relapse lasts 3 to 12 weeks
- Each person will have attacks that vary in symptoms and duration.
Causes of MS
- Genetic predisposition
- Viruses (Epstein Barr virus)
- Environmental factors – smoking, pollution, diet, vitamin D deficiency
MS Prevalence
- Age – Onset at 15-40
- Gender – women 4X more likely than men (ALS more common in men)
- Geography – 100, 000 Canadians
MS Type: Clinically Isolated Syndrome
First incident of signs of MS
MS Type: Relapse Remitting
- onset of new symtome or old symtoms relapse
- can convert to Secondary Progressive
MS Type: Primary Progressive
-The most severe gradual growing Symtoms
MS Type: Secondary Progressive
-like similar to RR but has less dramatic relapses
Treatment for MS
- Relapses: steroids, IV immunoglobulin (decreases symptoms – inflammation of myelin attacks)
- Symptom management/ improve function
- Disease modifying medications
- Complementary and alternative therapies – massage, acupuncture
- Rehabilitation: for degenerative neurological conditions; less intense, positive patient monitoring, don’t want to overdo it
- Psychosocial support
- Conserve energy; rest and use aids
- Manage stress levels
- Keep cool and exercise regularly
MS Invisable Symtoms
- fatigue
- visual problems
- sensory dysfunction
- numbness, tingling, ‘pins & needles’, NOT IN ALS
- cognitive impairment, NOT IN ALS
- bladder and bowel
- Pain, dizziness
- depression and mood alterations
- sexual dysfunction
MS Visible Symtoms
- motor functions:Walking, muscle weakness, spasticty,tremors,drop foot
- speech problems
- lack of balance and coordination
- Blurred vision
MS Fatigue
- Debilitating fatigue. Is generally more severe than normal fatigue - Generally occurs on a daily basis
- May occur early in the morning, even after a restful night's sleep - Tends to worsen as the day progresses
- Can be aggravated by heat and humidity
- Lasts for a few hours compared with more persistent fatigue associated with
depression
MS Heat Intolorance
Uhthoff’s phenomema
- demyelinated axons are heat sensitive
- can temporarily exacerbate other MS symptoms – fatigue, weakness, cognitive
functions
MS affect on Cognition
- cognition affects 50% of MS population
- Intelligence is usually intact
- No correlation between physical & cognitive disability
- Cognitive impairment can worsen during exacerbations and improve during
periods of remission
- Short-term memory, information processing, attention, concentration and
judgment may be impaired
- Difficulty learning new material or needing to spend longer to make it stick
- Forgetting recent conversations or missing the point in conversations
- Difficulty following complex explanations
- Slowness in understanding
- Being too literal or concrete
MS Affect on Speech and Balence
- Common problems; volume, slurring, pitch, breathiness
- Balance, and vertigo, or dizziness problems are common in MS: higher risk for falls and potential for added disability related to injury, fatigue and weakness may make balance and coordination problems seem
worse
Things to remeber about MS
- Don’t hesitate to ask for advice.
- Create an environment that is welcoming, accessible and friendly by taking into considerations the symptoms of MS.
- Listen to the person with MS.
- Understanding multiple sclerosis and providing exercise program can be difficult at times, there are experts in the field that can help in this regard.
- They know their body the best (i.e., MS symptoms, what they can and cannot do, the impact of fatigue).