ALS Flashcards
Huntington's and ALS_2025
Is ALS common with a family history?
Nope 90% of ALS cases occur without a family history
what is the usual age of onset?
40-70 years ago
what is the life expectancy for ALS?
Life expectancy is 2 to 4 years (always fatal)
describe little about the prevalence of ALS?
By 2040 ALS incidence is predicted to increase worldwide by 70%
Veterans have 2X the incidence of ALS compared to others
disproportionately large % of men affected by ALS have a history of military service: toxin exposure, extreme exhaustion, specific wars
The Midwest has the highest prevalence of ALS in the US
Rates in Michigan almost doubled over 10 years!
what is the etiology of ALS? what are the risk factors for environmental ALS?
unknown
Can be sporadic or familial
environmental:
exposure to heavy metals (lead, iron, cadmium, selenium, mercury)
exposure to pesticides
exposure to electromagnetic fields (EMF)
BMI and nutritional states
what is the pathophysiology of ALS?
Progressive, degenerative disease
Affects descending corticobulbar & lateral corticospinal tracts (UMN) and anterior horn cells (LMN)
loss of motor neurons
astrogliosis in the brain and brain stem
neuronal inclusions (e.g. Bunina bodies)
what does the corticospinal tract have control over?
toes
ankles
knee
hips
trunk
shoulder
elbow
wrist
fingers
thumb
what does the corticobullbar tract have control over?
neck
brow
eyelid
nares
lips
tongue
larynx
what is needed for a medical diagnosis of ALS?
The diagnosis of ALS requires:
Evidence of LMN degeneration by clinical, electrophysiological or neuropathological examination
Evidence of UMN degeneration by clinical examination, and
Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination,
Together with the absence of:
Electrophysiological and pathological evidence of other disease that might explain the signs of LMN and/or UMN degeneration, and
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs
what is the general presentation for ALS patients?
distal to proximal
UMN: spasitisty and hyperreflexia
LMN: muscle atrophy, cramping, and muscle fascinations
what are the LMN signs seen with bulbar areas of the body in als?
facial weakness (lower half)
facial fasciculations
tongue atrophy
tongue fasciculation’s
flaccid dysarthria
dysphagia
what are the cervical/upper limb LMN signs seen in a person with ALS?
muscle weakness
hypotrophy/atrophy
fasciculations
decreased or absent DTR’s
what are the thoracic/ trunk and diaphragm LMN signs seen in ALS?
muscle weakness
axial instability
blended posture
fasciculations
Dyspnoea, orthopnoea
what are the LMN signs seen in the Lumbar and lower limbs for ALS?
muscle weakness
hypotrophy/atrophy
fasciculations
decreased or absent DTR’s
what are the UMN signs for the bulbar face, pharynx, and tongue seen in ALS?
increase or spastic muscle tone
decreased tongue motility
spastic tongue protrusion
increased jaw and palmomental reflexes
spastic dysarthria
pseudobulbar affect
what is the umn sign in the cervical and upper limbs in ALS?
increased or spastic muscle tone
increased or cloniform DTRs
preserved DTRs in atrophic muscles
Hofmanns, Wartenbergs, jacaobsohn’s sign
what is the umn signs in the thoracic spine, trunk and diaghram in ALS?
absent superficial abnormal reflex
increased deep abdominal reflex
what are the UMN signs for the lumbar and Lower legs seen in patients with ALS?
increased or spastic muscle tone
increased cloniform DTRs
preserved DTRs in atrophic muscles
pyramidal signs
when talking about system preservation with a patient that has ALS what systems are you targeting?
sphincter control
sensory function
extra ocular muscles
intellectual abilities
skin integrity
what are the early symptoms of ALS?
Muscle twitches in the arm, leg, shoulder, or tongue
Muscle cramps
Tight and stiff muscles (spasticity)
Muscle weakness affecting an arm, a leg, or the neck
Slurred and nasal speech
Difficulty chewing or swallowing
when ALS progresses what do patients see increased difficulty with?
Dysphagia
Dysarthria
Dyspnea
Dysphonia
Sialorrhea (salivary control)
Unintended crying, laughing, or other emotional displays (pseudobulbar effects)
Constipation
Maintaining weight, getting enough nutrients
A patient comes into your clinic complaining of Early disease, mild focal weakness, asymmetrical distribution, hand cramping, fasciculations what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 1
b) Education, energy conservation, home modifications, referrals; AROM/ stretching, resistive strengthening to unaffected muscles, aerobic activities
A patient comes into your clinic complaining of Moderate weakness in groups of muscles, some muscle wasting, modified independence with AD what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 2
b) Equipment recs, AAROM/PROM, stretching, strengthening of unaffected muscles, aerobic exercise
A patient comes into your clinic complaining of Severe weakness of specific muscles, increased fatigue. Mild to moderate functional limitations, ambulatory what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 3
b) Keep independent, adaptive equipment
A patient comes into your clinic complaining of Severe weakness and wasting of LEs, mild weakness of UEs, moderate assistance and AD required, wheelchair user what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 4
b) AAROM, PROM, strengthening on unaffected muscles, positioning, skin integrity
A patient comes into your clinic complaining of Progressive weakness with deterioration of mobility and endurance, increased fatigue, moderate to severe weakness of whole limbs and trunk, spasticity, hyperreflexia, loss of head control, max assist what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 5
b) Equipment, positioning
A patient comes into your clinic complaining of Bedridden, dependent ADLs and functional movement, progressive respiratory distress what stage of ALS is this individual in?
b) what are you including in your treatment plan?
stage 6
b) Airway clearance, deep breathing, assisted coughing
what should be included in you examination for a patient with ALS?
Vital signs
Respiratory function
Motor function: examine for atrophy, widespread weakness, muscle cramping, muscle twitching, spasticity, coordination
Sensory function
Reflexes: are pathologic reflexes present? (Babinski)
Functional status: monitor for fatigue, persistent weakness following activity
ALS Functional Rating Scale (ALSFRS): assess disease progression and function; 0-40 scale with lower score linked to poorer function
what is an exercise precaution to remember when working with patients with ALS?
monitor fatigue levels closely;
avoid overwork injury
what should the generalized treatment of a patient with ALS encompass?
Exercise program: focus on preventing further deconditioning and disuse atrophy while avoiding overwork damage, focus on functional activity as weakness progresses
mild aerobic exercise at submaximal levels
must monitor for fatigue
Energy conservation techniques
Education for increasing cough force
Maintain functional independence (AD, orthotics, DME, environmental modifications)
Family education
