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
