Amyotrophic Lateral Sclerosis (ALS) Flashcards
what is amyotrophic lateral sclerosis?
- disorder that affects the nerve cells responsible for controlling voluntary muscle movement
- known as motor neurons
what type of disease is ALS? what does it mean?
- progressive and neurodegenerative disease, which means it gradually degenerates and kills these motor neurons
what does ALS lead to? (2)
- leads to muscle weakness and atrophy
is the cause of ALS known?
- unknown in most cases (sporadic)
- but 5-10% of cases are familial (genetic)
what degeneration occurs in ALS?
degeneration of both upper motor neurons (in the brain) and lower motor neurons (in the spinal cord & brainstem)
what does degeneration lead to?
- leads to disruption of muscle signals
- causing progressive weakness
what does ALS typically present with?
- muscle weakness
- often starts in one region of the body (such as hand or arm) and gradually spreading
what are other early symptoms of ALS? (3)
- muscle cramps
- twitching
- stiffness
what symptoms do individuals experience as the disease progresses?
- as the disease progresses, individuals experience increasing difficulty with movement, speech, swallowing and breathing
is there a test for ALS? how do you diagnose ALS?
- no single test for ALS so diagnosis involves ruling out other conditions through clinical evaluation, electromyography, nerve conduction studies and sometimes genetic testing
what criteria is often used to aid in diagnosis?
- EI Escorial criteria are often used to aid in diagnosis
is there a cure for ALS?
- no cure for ALS
what does treatment of ALS focus on?
- focuses on managing symptoms and maintaining quality of life
what medications can slow disease progression of ALS?
- riluzole
- edaravone
what supportive care would be given for individuals with ALS? (5)
- physical therapy
- occupational therapy
- speech therapy
- nutritional support
- respiratory care
what type of disease is ALS?
- ALS is a progressive disease
how quickly do people with ALS die?
- die from respiratory failure within 3 to 5 years from the onset of symptom
what does the progressive nature of ALS lead to?
- leads to a range of complications, primarily due to the gradual loss of muscle function
what are the range of complications that ALS can cause? (8)
- muscle weakness and atrophy
- speech and swallowing difficulties
- respiratory problems
- cognitive and behavioural changes
- nutritional changes
- emotional and psychological impact
- pain and discomfort
- care and support
what does addressing these problems require?
- require a multidisciplinary approach involving neurologists, physiotherapists, occupational therapists, speech and language therapists, respiratory therapists, dietitians and mental health professionals
how does comprehensive care help individuals with ALS?
- helps with the symptoms of ALS
- maintains quality of life
- supports the individual and their family
what is crucial for people with ALS ?
- maintaining ROM is crucial
what do range of motion exercise improve? (3)
- mobility
- posture
- self care
what do range of motion exercises reduce?
- pain
- spasticity
- wound risk
what do range of motion exercises counter act?
- counter act the anticipated loss of strength and function
what are common indications of respiratory dysfunctions resulting from neuromuscular weakness in ALS?
- dyspnoea
- orthopnoea
- hypoventilation
- poor airway clearance
what respiratory exercises may be included?
- lung volume recruitment (breath stacking, glosspharyngeal breathing), muscle strengthening and airway clearance techniques
what are the main airway clearance techniques?
- manually assisted cough (MAC)
- huff
- squeeze coughing
how do you maintain independence?
- mobility aids
- wheeled zimmer frame, walker stick, delta frame
what are examples of orthotic devices used for ALS patients?
- splints
- AFO
- ankle brace
- foot up
- hand and wrist splint
- neck braces
- thorax and lumbar support/ braces
what does spending time out of bed involve?
- transfer aids
- transfer techniques
- self- positioning
- positions of ease/ confront
- skin integrity/ risk of break
what are the 4 interventions to reduce dependency?
- transfers education
- balance and mobility
- physical activity
- exercise prescription
how is quality of life maintained in ALS patients?
- pain management
- caregiver/ family support
- home assessment
what does essential mobility include?
- mobility and walking aids
- wheelchair support
- referral/ assessment
- adaptive equipment
- supportive equipment
- skin pressure relief
what is fall risk associated with?
- associated with reduced muscle power (lower extremities)
what is important for reducing fall risk? (2)
- balance (sitting, static and dynamic)
- strength
how is spasticity managed?
- botulinum toxin and neurolysis with phenol/ alcohol creates a temporary plastic state in the neuromuscular system for motor re-learning and recovery
what do new methods for spasticity management facilitate?
- facilitates neural plasticity via rehab, brain stimulation and drugs
what other methods are used in spasticity management?
- non- invasive electrical stimulation (FES)
- use of orthoses and assistive devices
