Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
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) |