ALS Flashcards
ALS
amyotrophic lateral sclerosis
degenerative scarring of motor neurons in SC, brainstem, and cerebral cortex
Acquired
UMN symptoms
Types
sporadic 90-95% no hereditary component
familial 5-10% more than one in lineage
epidemiology
incidence 1 to 2 per 100,000
caucasians> AA, asian, hispanic
incidence increases each decade, esp >40 yo, peaks at 74 yo; 50 average age
men>women slightly
Etiology
no known cause
possibilities include:
decrease SOD- enzymes that normally clean up O2 free radicals; unable to and free radicals accumulate in body
increase glutamate- neurotransmitter, excess is toxic
autoimmune
lack of neurotrophic factors- proteins for growth, survival of neurons
Pathophysiology
motor neuron degeneration and death with gliosis (scarring) replacing lost neurons
loss of frontal or temporal cortical neurons (FTD) fronto-temporal dementia
Motor neurons affected with ALS
spinal cord
brainstem (CN 5,7,9,10,12)
UMN in cortex/corticospinal tracts
Usual sparing with ALS
CN 3, 4, 6 (external ocular ms) can still track with eyes
striated ms in pelvic floor
sensory system and spinocerebellar tracts
No sensory problems
ALS Presentation
70-80% limb onset ALS- initial involvement in extremities
20-30% bulbar onset ALS- problems speaking, chewing, swallowing, more common in mid-aged women; worse prognosis
Progression
spread in a contiguous manner (out then up/down)
UMN and LMN signs- problems vary secondary to level of damage
UMN/LMN signs
UMN: weakness with slowness, hypereflexia, spasticity, clonus
LMN: weakness, atrophy, fasiculations, ms cramps, irritability of motor neurons
starts with vague symptoms
signs/symptoms
distal weakness most common first symptom
asymmetrical at first, but eventually symmetrical
may start in the hands or feet, but eventually progresses to proximal ms (extensors>flexors are weakest)
UE weakness
distal- weakness and atrophy in intrinsics of hand
drop things, lack grip, can’t write
proximal weakness and atrophy of SH girdle; SH subluxation or “frozen SH”
Can’t raise arms overhead
LE weakness
distal- weakness and atrophy of intrinsics of feet and DF, leads to foot drop, tripping, falling
proximal- weakness and atrophy of pelvic girdle, lead s to difficulty standing up, climbing stairs
Weakness in neck ms
weak neck extensors; inability to hold head up
cervical orthosis or SOMI to keep head up
bulbar impairments
dysarthria- slow speech, slurred; becomes unintelligable
dysphagia- aspiration risk
sialorrhea- drooling, excess production saliva without ability to swallow; restrict socialization
SLP consult
dysphagia, dehydration, malnutrition (feeding tube), speech/communication problems
long time to eat, choking, coughing, wet voice quality
Respiratory impairments
-decrease respiratory ms strength; decrease VC
usually responsible for death
Cognitive/behavioral impairments
-cognitive- 50% pts, mild to FTD
FTD- change in personality, behavior inappropriate, impulsive, emotional withdrawal; verbal fluency, comprehension, memory, abstract reasoning
behavioral- pseudobulbar affect; tx with nuedexta
depression- 10%
Diagnosis
presence of LMN signs (EMG)
UMN signs by clinical exam
progression of dx with no other evidence of pathology
diagnosis of exclusion
Prognosis for ALS
over 50% die in 3-5 years after diagnosis
typically require WC in full-time in 12-18 mo (if LE involved)
decisions for family- feeding tube, ventilator
valium/baclofen given for spasticity and ms spasms
Younger dx or limb onset typically live longer
Medical management
riluzole- only tx, glutamate inhibitor; modest (2-3 mo) extension of life; weak EBP but pt likes it
meds for pain, depression/anxiety, anti cramping and anti spasticity meds (SH subluxation pain)
agents for sialorrhea
PEG
ventilatory support
NIV
gently assists each breath
flow of air with inhalation, little resistance with exhalation
wearing schedule varies
keeps lungs open
ALS functional rating scale
0-4 scale, monitor progression
addresses: speech, salivation, swallowing, writing, cutting food, hygiene, dressing, turning in bed, walking, stairs, dyspnea, orthopnea, respiratory
PT Management
teach pt deep breathing and coughing exercises, even in absence of current problems- increase VC, relaxation
general mobility- stay active, decrease fall risk (balance, DF STR, strategy to get back up, footwear)
exercise
Exercise
little evidence; may improve function and psychological well being
balance between overuse fatigue and disuse atrophy
can prevent premature cardiopulmonary deconditioning
Start with stretch
most beneficial in early stages
avoid heavy eccentric exercise
whole body exercise at submax level (swim, walk)
train at moderate to low intensities
UE management for Pt with ALS
teach AROM, AAROM, PROM, esp for SH, hands
avoid overhead exercises and activities without scap stability (poor ms stabilizers)
educate pt about now pulling on UE to transfer, dress
Keep UE supported at all times
resting (night) hand splint for good jt position
teach to massage hands
adaptive equipment as needed
Cramping/Spasticity management
massage
cold
positioning- to oppose spasticity
moderate exercise to decreases spasticity
equipment
walker or AD bath bench elevated commode seat sliding board hoyer or easy pivot MAS mobile arm support for feeding BAO balanced arm orthosis
Power chair
plan ahead
pt may need to use different drive options as disease progresses
if power, make sure pt can transport with van
power tilt with headrest
supportive seating
good pressure relieving cushion
Manual WC
if caregiver is going to push it
WC should still tilt with headrest
have supportive seating, high back
good pressure relieving cushion
