ALS

Question 1

ALS

Answer 1

amyotrophic lateral sclerosis
degenerative scarring of motor neurons in SC, brainstem, and cerebral cortex
Acquired
UMN symptoms

Question 2

Types

Answer 2

sporadic 90-95% no hereditary component

familial 5-10% more than one in lineage

Question 3

epidemiology

Answer 3

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

Question 4

Etiology

Answer 4

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

Question 5

Pathophysiology

Answer 5

motor neuron degeneration and death with gliosis (scarring) replacing lost neurons
loss of frontal or temporal cortical neurons (FTD) fronto-temporal dementia

Question 6

Motor neurons affected with ALS

Answer 6

spinal cord
brainstem (CN 5,7,9,10,12)
UMN in cortex/corticospinal tracts

Question 7

Usual sparing with ALS

Answer 7

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

Question 8

ALS Presentation

Answer 8

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

Question 9

Progression

Answer 9

spread in a contiguous manner (out then up/down)

UMN and LMN signs- problems vary secondary to level of damage

Question 10

UMN/LMN signs

Answer 10

UMN: weakness with slowness, hypereflexia, spasticity, clonus
LMN: weakness, atrophy, fasiculations, ms cramps, irritability of motor neurons
starts with vague symptoms

Question 11

signs/symptoms

Answer 11

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)

Question 12

UE weakness

Answer 12

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

Question 13

LE weakness

Answer 13

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

Question 14

Weakness in neck ms

Answer 14

weak neck extensors; inability to hold head up

cervical orthosis or SOMI to keep head up

Question 15

bulbar impairments

Answer 15

dysarthria- slow speech, slurred; becomes unintelligable
dysphagia- aspiration risk
sialorrhea- drooling, excess production saliva without ability to swallow; restrict socialization

Question 16

SLP consult

Answer 16

dysphagia, dehydration, malnutrition (feeding tube), speech/communication problems
long time to eat, choking, coughing, wet voice quality

Question 17

Respiratory impairments

Answer 17

-decrease respiratory ms strength; decrease VC

usually responsible for death

Question 18

Cognitive/behavioral impairments

Answer 18

-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%

Question 19

Diagnosis

Answer 19

presence of LMN signs (EMG)
UMN signs by clinical exam
progression of dx with no other evidence of pathology
diagnosis of exclusion

Question 20

Prognosis for ALS

Answer 20

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

Question 21

Medical management

Answer 21

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

Question 22

NIV

Answer 22

gently assists each breath
flow of air with inhalation, little resistance with exhalation
wearing schedule varies
keeps lungs open

Question 23

ALS functional rating scale

Answer 23

0-4 scale, monitor progression
addresses: speech, salivation, swallowing, writing, cutting food, hygiene, dressing, turning in bed, walking, stairs, dyspnea, orthopnea, respiratory

Question 24

PT Management

Answer 24

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

Question 25

Exercise

Answer 25

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

Question 26

UE management for Pt with ALS

Answer 26

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

Question 27

Cramping/Spasticity management

Answer 27

massage
cold
positioning- to oppose spasticity
moderate exercise to decreases spasticity

Question 28

equipment

Answer 28

walker or AD
bath bench
elevated commode seat
sliding board
hoyer or easy pivot
MAS mobile arm support for feeding 
BAO balanced arm orthosis

Question 29

Power chair

Answer 29

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

Question 30

Manual WC

Answer 30

if caregiver is going to push it
WC should still tilt with headrest
have supportive seating, high back
good pressure relieving cushion