L6 ALS Flashcards
ALS definition
progressive neurodegenerative disease that affects upper and lower motor neurons
eventually leads to paralysis and death
no cure for ALS, can be medically managed to reduce S/S and prolong life
Mean survival time
2-5 years on average
Epidemiology ALS
90-95% of cases are sporadic
prevalence of disease is 5.2 per 100,000
most people are between 40 to 70, average of 55
more common in men than women, caucasian
Pathophysiology of ALS
onset of this disease is sporadic and cause is unknown
motor neurons are uniquely vulnerable to the disease process
Pattern of loss for motor neurons
loss of UMNs in CST and motor cortex
loss of LMNs in brainstem and anterior horn of SC
Pathophysiology of ALS
factors that contribute to motor neuron cell degeneration and/or death
-oxidative stress
-defective glutamate metabolism
-genetic variations
-cytoskeletal protein defects preventing normal cell
-autoimmune dysfunction
-inflammatory responses
Limb-onset ALS
extremity and bulbar tract signs
most common, 2/3rds of patients
often starts in the limbs and then progresses to proximal tracts controlling swallowing and respiration
Bulbar ALS
1/3 of patients
starts with difficulty with speech and trouble swallowing that can be followed by limb involvement in later stages
progresses more quickly than limb onset variant, shortening survival time
ALS-plus syndrome
extremity and bulbar symptoms and dementia
Medical Diagnosis of ALS
diagnosis of exclusion, delayed diagnosis and treatment
average time to diagnosis to 10-15 months
Arlie House Criteria
presence of LMN and UMN symptoms in the bulbar region and at least 2 spinal regions
presence of LMN and UMN symptoms in 3 spinal regions
Other characteristics suggestive of ALS
absence of sensory symptoms
progressive spread of symptoms or signs
no evidence of other disease processes
Diagnoses that are against ALS
presence of isolated radicular pain
symmetric proximal OR distal limb weaknesses
cog wheel rigidity
prominent sensory loss
isolated fasciculations or cramps without weakness
rapid onset with no progression
UMN Lesion Characteristics
Weakness
Increased Reflexes
Increased Muscle Tone
LMN Lesion characteristics
Weakness
atrophy
fasciculations
decreased reflexes
decreased muscle tone
Features of ALS associated with poor prognosis
-elderly onset
-elderly females presenting with bulbar-onset
-pts whose ALS diagnosis has been given less than 8 mo after symptom onset
-patients losing more than 1.4 points/mo
Medical Treatment ALS
predominately symptom management
slow down the progression of disease
Predominately symptom management
-respiratory
-dysphagia
-sialorrhea = excesive salivation
-pseudobulbar affect = episodes of uncontrollable laughter or crying
-communication
-muscle spasms and pain
-weakness and loss of functional mobility
-depression
Slowing the progression of the disease
riluzole
thought to reduce glutamate-induced excitotoxicity
improved survival
Patient and family education
-disease progression, will lead to death
-no cure
-medications can lessen symptoms
-therapy and AT can help improve QOL
-discussion of palliative progressing
Speech Language Pathology
early in the disease process
see SLP to establish a baseline of speech rate
begin message and voice banking so that your own voice can be used in a speech generating device
Alternative tech
thick barreled pen to improve grip strength
increasing cursor speed of a mouse so less movement is required
switch to single click on tech rather than double click
head, foot, eye movement controls
ALS Functional Rating Scale Revised
measure of physical function for patients with ALS created in 1991
12 question scale with likert scale responses
total score range 0-48, with lower score is more impaired
stronger predictor of survival than muscle strength or respiratory function
Areas that are measured in ALS functional rating scale
Bulbar = speech, salivation, swallowing
fine motor = handwriting, cutting food, dressing and hygiene
gross motor = turning in bed, walking, climbing stairs
respiratory = dyspnea, orthopnea, respiratory insufficiency
Palliative Care
meant to improve the quality of life of patients who have a serious or life-threatening disease. approach to care that addresses the person as a whole, not just their disease. Goal is to prevent or treat as early as possible, the symptoms and side effects of the disease and its treatment in addition to any related psychosocial and spiritual problems. Anyone can receive palliative care regardless of their age or stage of disease
Overall goal of care for ALS
slow the decline of loss of function and provide more support as they decline
Short term effects of exercise in ALS
Improved ALSFRS-R = possibly hypertrophy, more likely due to increased recruitment by collateral innervation
No improvement in ALSFRS-R = early in the disease it is difficult to maintain high score levels of b/c of natural history, possible that these patients were under-dosed
Clinical Bottom Line of short term effects of exercise
you need to use outcome measures to monitor your patient’s response to a new exercise program and adjust plan of care accordingly
Medium Term Effects of Exercise in ALS
4/5 studies showed less loss of function for those that did aerobic exercise
one study showed an exception; they did both strength and aerobic exercise
Clinical Bottom Line of Medium Term Effects of Exercise in ALS
Training must be balanced between muscle overwork and underwork. overwork may increase fatigue and weakness
five times/week would not be recommended for ALS patients
not enough research for intensity/frequency
Long-Term Effects of Exercise in ALS
5/6 studies showed less loss of function in exercise group
there is a consistent documented benefit of exercise on function in patients with ALS
even small changes can improve QOL
Overall Clinic Bottom Line for exercise
combining strength training and aerobic exercise at moderate intensity, 2/week
optimize functional benefit without increasing fatigue
must monitor for overwork fatigue
Rehab in Reverse
as the disease progresses so will your interventions and goals
go from least restrictive to more supportive equipment
education from how to be independent to caregiver support
exercise to increase strength to then maintain strength for as long as possible
Late stage ALS
most individuals wished they had begun sooner with wheelchair
