Lecture 9: management og amyytophiv lateral sclerosis

Question 1

no known specific etiology

Question 2

Question 3

goal w/ als

the primary goal of sall health care professionals involved with threating pts with als is maintaining patients qol and their indepdence in functional mobility/adls

Question 4

fatigue happens w/ this = we dont want to overwork them

resp impacted

dyspahgia/swallowing impacted

profound drooping of head = neck muscle weakness against gravity - not tight in a motion - they’re weak - cant hold hd up
* we probs cant get this stronger for them

this posture impacts
* eating
* swallowing
* breathing
* articulation
* vision

Question 5

limb onset worse than bulbar

Question 6

cervical collars helpful for hd drooping
* headmaster collar best

Question 7

disease specific outcome measures

Function: ALS function rating scale and the revised version
* self reported/clincian-interview scale rating function
* 4 (normal function) to 0 (unable to attempt task)
* revised version adds resp status

QOL: ALS assessment questionaire
* self reported/clinician interview scale measuring qol
* original scale: 40 items that represent five distinct areas of health: mobility (10 items), adl (10 items), eatinh and drinking (3 items), communication (7 items and emotional functioning (10 items)

now shortened to 11 items - had 40
* looks at function + emotional functioning etc
* mainly important for monitoring disease progression
* want to do a qol goal instead of mmt goal. use thi really for later stages, earlier stages can be more function based

Question 8

pt interventions
* depdent on the pattern and severity of the disease
* multidisplinary
* multisystem by every discipline
* avoid overuse fatigue (specifics depend on the person, but in general a weak/denervated m is more suscepitble to damage)

Question 9

in als motor neurons in the brain and spinal cord gradually degenerate and die

Question 10

things that well use at some point in als

constant reeval every few months because it is progressive and things change quickly

Question 11

stages of ALS
* broken down same way as pd
* note restorative is only in early
* preventitive/compensatory more at end/middle

only certain m groups imacted early on

promote aerobic to slow ex progression

dont want secondary impairments

vet=dvt

start convo about adaptive equipment etc…

note a lot of shit happens in early stage - have symptoms quickly

preventitive any stage - think prevent skin breakdown

late stage in wheel chair / restricted to bed

Question 12

another look

needs multidisplinary approach

Question 13

knowledge check: what stage of als would you prioritize prevention
* early

Question 14

ALS abd EX

Limited research specifically on ALS and EX - because ltos of peeps w/ als dont want to ex
* however similar neuromuscular disease have been studied

overuse weakness does not occur in m w/ an MMT of grade 3 or greater (works against gravity
* meaning if the m isnt denervated passted grade 3 - meaning we dont have to worry as much about fatiguing these m’s out
* wait until m has antigravity strength before stressing
* mod resistance ex can increase strengh in muscles w/ an mmt grade of 3 or greater - so you actually can help these muscles
* strength gains are proportional to m strength - meaning if their baseline was shit they probs won’t get much better
* heavy eccentriv ex should be avoided
* ex may produce functional benefits
* avoid neuromuscular eletrical stimulation - so no estem - too much for - the impaired motor unit! - test - especially nmes (tens maybe for pain control but id pick not 2)
* monitor fatigue, dont overwork
* psychological benefits yet to be determined

grip strength good for these pts

Question 15

bulbar = trunk impacted
limb onset = typically hands first

Question 16

ALS - what we know
* EX may be benefical especially in early stages if muscle
* already weakened muscles may not improve strength (esp below grade 3)
* appropriate: general arom/stretching, resitive strengthening (unaffected muscles) with low to mod weights, aerobic acivities (swimming, walking, biking) at submax levels

have them do arom if capable - thats an ex

stretching is fine

resistance in unaffected muscles (above 3/5)

nothing done at max levels

Question 17

als doesnt always affect everyone the same way. some poeple get lucky

change can happen in weeks or years dpending on the diagnosis

Question 18

knowledge check: what grade of strength should we have the pt demo before exercsing that m
* 3/5
* can move against gravity