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60 Cards in this Set

  • Front
  • Back
Describe the ALS disease classification and neuropathology.
an adult onset, multifocal neurodegenerative disease affecting the voluntary motor system

- relentless
- brainstem and SC affected
- attacks LMNs, corticospinal/corticobulbar (pyramidal)
What does the amyotrophic part of the name mean?
the muscle begins to degenerate or atrophy due to denervation (no innervation from MNs)

LMN
What is the essential neuropathological characteristic of ALS?
up to 80% of neurons die off before there is a clinically significant weakness
What does the lateral sclerosis part of the name refer to?
degeneration of corticospinal and corticobulbar tracts (that live in the lateral geniculus)
What is the "bottom line" when it comes to ALS?
selective apoptosis

the patient has already come a long way before being diagnosed
What is the incidence/prevalence of ALS?
- 20-30K live in the US with ALS (5K added yearly)

- most common motor neuron disease

- men > women
- 40-70 years old
What is the SOD1 gene?
it is a gene found in family transmitters

an enzyme that is a free-radical scavenger (anti-oxidant) -- we see a build up of free radicals in the motor neurons

5-10% of ALS is inherited, but only 20% of this 10% carry this gene
What percentage of ALS is unexplained or sporadic?
90-95% of ALS cases just happens
What does aging have to do with ALS?
there is an anti-oxidant build up in the neurons (take a lot of anti-oxidant vitamins)
What effect does glutamate have on ALS?
there tends to be an elevated amount of glutamate in CSF (possibly they die of excitotoxicity??)
What is the only approved drug to treat ALS?
a glutamate antagonist
What are LMN signs and symptoms?
- weakness
- atrophy
- hyporeflexia (or areflexia)
- cramps d/t muscle imbalances
- fasciculations (single MU twitching) -- 1 twitch every 3-4 seconds
- fatigue
Where are LMNs located?
ventral horn of the spinal cord
brainstem
Where are UMNs located?
pyramidal tract (corticospinal, corticobulbar tracts)
What are typical UMN signs and symptoms?
- spasticity
- hyperreflexia
- clonus
- weakness (not nerve root distribution)
- some disuse atrophy
- pathological reflexes/Babinski
What systems are affected by neuropathological processes?
1. respiration

2. skeletal (UE, LE, trunk), bowel and bladder sphincters are NOT affected

3. CN functions such as swallowing and eating

4. speech (form words, air control), non-verbal (facial muscles)
What is the function of the corticobulbar tract?
LMNs innervating muscles of the face, tongue, pharynx, and larynx
What is the function of the lateral corticospinal tract?
fractionated movements, controls fine/distal movements of ipsilateral limbs
What neuronal functions are typically spared during the early stages of ALS? (5)
1. cognition
2. sensation
3. ANS
4. CNs that move the eyes (3, 4, 6)
5. sphincter control of bowel and bladder

**HOWEVER these can become progressively worse
What are the most common symptoms reported INITIALLY by the patients (pre-diagnosis)?
1. weakness (located in different body regions, arm or leg)

2. problems start and then keep progressing, no pain/sensory changes as happens with a nerve root problem
Does fatigue or weakness occur first (as reported by an ALS patient pre-diagnosis)?
fatigue comes before weakness
Where does ALS typically start?
80% starts with arms, legs, and trunk

20% starts with CN signs (swallowing, hoarse voice, drooling)
What are 2 tests performed to rule in/out ALS?
1. needle EMG
2. NCS (nerve conduction study)
Why does it take so long to come up with a definative diagnosis?
- process of elimination (lots of tests performed)
- want to be certain before telling patient (3-5 years of life)
- must be progressive so our system fixes itself for a while
- people begin to adapt to problems
Describe the process of needle EMG testing.
1. stick needle EMG electrode into the muscle listening for insertional activity
2. resting activity
3. response to voluntary contration (quantitative EMG)
Does fatigue or weakness occur first (as reported by an ALS patient pre-diagnosis)?
fatigue comes before weakness
Where does ALS typically start?
80% starts with arms, legs, and trunk

20% starts with CN signs (swallowing, hoarse voice, drooling)
What are 2 tests performed to rule in/out ALS?
1. needle EMG
2. NCS (nerve conduction study)
Why does it take so long to come up with a definative diagnosis?
- process of elimination (lots of tests performed)
- want to be certain before telling patient (3-5 years of life)
- must be progressive so our system fixes itself for a while
- people begin to adapt to problems
Describe the process of needle EMG testing.
1. stick needle EMG electrode into the muscle listening for insertional activity
2. resting activity
3. response to voluntary contration (quantitative EMG)
What do we listen for with needle EMG to see if a patient has denervation of a muscle?
listen for crackling at rest (normally muscles are quiet)

spontaneous firing from a single muscle fiber (fibrillation)
What differs with a needle EMG for a patient with ALS than from a normal patient?
1. increased amplitude
2. increased size/duration
Will there be a change in nerve conduction study seen with an ALS patient?
there should be no change in NCV
What might we see with a patient who has ALS spinal muscle signs?
- LE > UE
- weaker distally vs. proximally
- bulbar signs come on later
What might we see with a patient who has ALS bulbar muscle signs?
- speech
- paresthesias
- respiratory
**faster course d/t life threatening onset
What are phases 1, 2, and 3 known as for ALS?
Phase 1: independent
Phase 2: partially independent
Phase 3: dependent
What are things can I expect to see with a patient in stage 1 of ALS?
- weakness
- clumsy
- I in ADLs
What are things can I expect to see with a patient in stage 2 of ALS?
begin to adapt behaviours
- moderate, selective weaknesses
- decreased ind in ADLs (stairs, buttoning)
What are things can I expect to see with a patient in stage 3 of ALS?
use more ADs
- severe weakness (ankles, wrists, hands)
- decreased ind in ADLs
- fatiguable
- increased repiratory effect
What are things can I expect to see with a patient in stage 4 of ALS?
- WC dependent
- severe LE weakness (spasticity?)
- some ADLs with inc fatigue
- shoulder pain
- edema in hand
What are things can I expect to see with a patient in stage 5 of ALS?
- severe LE weakness
- moderate to severe UE weakness
- WC
- skin breakdown
What are things can I expect to see with a patient in stage 6 of ALS?
- bedridden
- dep in ADLs
Describe the 3 phases of ALS.
phase 1: independent, may need AD even though they are funcitonally independent

phase 2: partially independent, assisted, more support from family, AD

phase 3: dependent, need respiratory support
What are treatments for stage 1 of ALS?
- strengthen
- ROM
- anticipate what they need to know
What are treatments for stage 2 of ALS?
- stretching
- strenghtening for muscles 3+ and above
- orthotics?
What are treatments for stage 3 of ALS?
- don't fatigue this patient
- deep breathing instruction
- keep physically I as long as possible
- WC
What are treatments for stage 4 of ALS?
- heat and massage for spasms
- anti-edema massage
- AA/PROM
- isometrics
What are treatments for stage 5 of ALS?
- educate family on transfers/positioning
- home modifications
- HMV
What are treatments for stage 6 of ALS?
- soft diet/tube foods
- suction of saliva
- airway clearance, trach
What are 4 problems seen in stage 6
1. dysphagia
2. dysarthria
3. saliva
4. breathing
What are 4 specific goals for PT in ALS?
1. preserve/promote functional independence
2. facilitate transition to functional dependence
3. preserve muscle strength, endurance and flexibility -- AVOID OVERWORK to vulnerable muscles
4. prevent secondary complications (disuse atrophy, pain, contractures, DVTs, skin breakdown, respiratory complications)
When taking a history, what are we concerned with about the medical condition?
the stage of disease by severity of symptoms

- onset date
- presentation pattern of symptoms
- date of definitive diagnosis
- rate/pattern of progression
What is a standardized tool I can use for an ALS patient to give me information about their disease?
daily activity log -- to give me a picture of how they live their life on a day by day basis
What are 3 physical examination/tests and measures?
1. ASL specific standardized measures
2. impairment tests and measures directly related to the underlying neuropathology and stage of disease
3. functional status
What are 4 assessment/evaulation/diagnosis/and specific goal setting factors to know or consider?
1. stage of the disease
2. primary problems for PT to address
3. secondary complications at risk for and why
4. will pt benefit from PT at this time or not?
What are 3 general PT interventions for ALS?
1. procedural -- exercises, ROM/stretching, functional skills, pain management, skin care, respiratory treatment

2. education/intervention -- pt/caregiver, energy conservation, functional training, ROM/stretching, postioning for skin and comfort, how to use adaptive equipment, environmental modifications

3. coordination/communications- equipment, braces, orthoses, pain management, consult/referals (speech, respiratory)
What sort of treatment strategies will I utilize during phase 1 (independent)?
- optimizing motor function (exercise, energy conservation)
- may need gait devices, orthoses and eventually a WC
- modified devices to make things easier
What sort of treatment strategies will I utilize during phase 2 (partially independent)?
geared towards providing assistive devices for independent function
- WC for mobility
- progress to needing assist for most functional mobility tasks
- problem solve with family for ease of mobility and self care tasks
What sort of treatment strategies will I utilize during phase 3 (dependent)?
physically dependent for all mobility and self care
- treatment focuses on family support/education/positioing
How does the FIM correlate with the phases/stages of ALS?
Stage 1: 7 (ind)
Stage 2: 6 (mod ind)
Stage 3: 5-6 (supervision)
Stage 4: 4 (min)
Stage 5: 2-3 (min, mod, max)
Stage 6: 1 (max, dependent)