ALS Flashcards
what is ALS
a neurodegenerative disease that effects the motor neuros of the BS, brain and the spinal cord
what is the most common neuro disease in the adult population
ALS
what age does ALS occur at
at any age but normally after 50s
when does death occur for these indivduals
3-5 from onset
what type of ALS is more prominent limb or bulbar
limb onset
70% - 80%
how prevent is the bulbar onset of ALS
20 - 30%
Limb-onset ALS presentation
Initial involvement in extremities
Bulbar-onset presentation
initial involving the bulbar muscle - diffucty speaking, chewing, and swallowing
what pop is the bulbar more prevalent in
middle aged women
how many people with ALS have a family presentation with it
5 -10%
Autosomal dominant (in most cases)
hat are known risk factors for ALS
disease causing mutations
clusters - western pacific ALS
family history
age
gender (male > females)
possible risk factors
neurotoxiant disorders
certian occupations
diet
lifestyle factors
vigours physical axtivity
what is the pathophysiology of ALS
progressive degeneration and loos of motor neurons in the spinal cord, motor cortex, and motor cortex
early versus late in the disease progression neurosn
early - healthy intaxct neurons can replace the degenerated neurons
later - there are not more healthy neuron to replace the degenerated ones
what areas impact with ALS
UMN
corticospinal tract
brainstem nuclei for CN 5, 7, 9, 10, 12
anterior horn of the spinal cord
what is normally spared with ALS
brainstem nuclei that are controlling the extral ocular movemnts
motor nuclei that are controlling the pelvic floor striated muscles - anal and external sphincter
sensory system
what is the most frequently presenting impairment
focal assymetrical weakness
muscle weakness is isolated to. a specific muscle
what is the caridnal sign of ALS
muscle weakness
LMN path
muscle weakness
hyporeflexia
hypotonicity
atrophy
muscle cramp
faciulations
what is a fasciculation
a muscle twitch
UMN patho
hyperreflexia
hypertonicty - spacity
muscle weakness
patho relfexes
bulbar patho
dysphalgia
dysarrthia
sialorrhea
what is sialorrhea
excessive saliva flow
what is what is pseudobulbar affect
Inappropriate involuntary laughing and crying due to a nervous system disorder.
Respiratory impairments
seen with ALs
Inspiratory/expiratory muscle weakness,
dyspnea/DOE,
nocturnal respiratory difficulty, orthopnea,
hypoventilation,
secretion retention, ineffective cough
what is orthopneas
Discomfort when breathing while lying down flat;
neck issues and ALS
neck stiffness
heavy head sensation
hard time stablizing the head
what are 2nd impairment that you might see with muscle weakness
contracture
sublexation
tendon shortening
decreased ROM
adhesive capcilitus
amb difficulties
foot drop
what symptoms do we look for the diagnosis of ALS
LMN degradation - neuroexamination
UMN degration
progressive spread if signs within the specfic region
what must be absent for the issue to be ALS
Electrophysiologic evidence of other disease processes that might explain the signs of LMN or UMN degeneration
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiologic signs
how does ALS spread
initialing the sym spread withing the segment of onset then to other regions
spread for unilateral arm onset
contra arm
isp leg
contra leg
bulbar muscles
spread for uni leg
contra leg
isp arm
contra arm
bulbar muscles
spread for bulbar onset
one arm
contra arm
what is death normally due to
respiratory failure
do younger people have better survial rate then older people
yes
Delay between symptom onset and diagnosis leads to what
increase mortality
what does the presence of executive dementia lead to
worse prognosis
Less severe involvement at time of diagnosis leads to what kind of prognosis
better prognosis
what goes into the management of ALS
pt and family edu
management of symptoms
nutrition management
respiratory decisions
cog
drug therapies
pallative care
early stage ALS what are we working on as a PT
preventative
restorative
compensatory
middle/late stage ALS what are we working on as a PT
compensatory
preventative
resortative
are there drig therapies for ALS
yes
Riluzole, Edarvone, Relyvrio
what do you prioritize on the PT neuro exam
CN
Motor
function
balance
what do you screen on the PT neuro exam
cognition
sensory
coordination - should be intact since the cere is spared
what is the The Revised ALSFRS (ALSFRS-R)
0 - worst
48 - best
correlate significantly with quality of life as measure
what is the ALS Assessment Questionnaire
Measure subjective health status (impairments and disabilities)
Physical mobility, ADL’s, eating and drinking, communication and emotional functioning
0-100 scale (worst - best)
Restorative PT interventions
improve impairments and activity modifications
when is it good to use restrorative methods
middle/early stages
these are only temp solutions
Compensatory PT interventions
lost something so use this instead
modify activity, task, or enviroment to minimize activity limitation and participation restrictions
what is preventative PT interventions
min potential impairments - loss of ROM or strengt, pneumonia,
what restorative/preventative activities do we do in the early stages of ALS
strengthing
endurance
stretching - ROM , AAROM
wheel chair use in the middle stage of ALS
WC is needed for longer distances and all of the time towards the end of this stage
muscle weakness seen in the middle stage
serve muscle weakness in some group and minor in other groups
late stage WC use
WC dependent
late stage ADLs
completely dependent
lates stage muscle strength
weakness everywhere -repirtory compromise
what is the main roll of the PT throughout the stage of ALS
promoting functional independence
promoting health and wellness
providing alternative means for carrying out difference functional tasks
min complications
edu and support
through what stages is the person with ALS able to amb
stage 1 -3
AD come in at stage 2
stage 3 weakness
server weakness in certain groups
at what stage is the ALS pt dep
stage 5
at what stage is the ALS bedridden
stage 6
intervention progress - early stage
edu
aerobic and strengthening activity
energy conservation stage
intervention progress - middle
DME if needed
transition to PROM/AROM plan from a resitive plan
education
energy conservation
family training
intervention progress - late
edu
DME
PROM for comfort
prevention of PNA and pressure injuries
what to do for cervical weakness
neck braces
supportive seating (high backs)
elevated reading materials
tilit in space WCs
education about good arm support for long sitting periods
for dsyarthria and dysmetria what to do
SLP
heand and trunk control
chin tuck for swallowing
edu about aspiration risk
what to do about respiratory muscle weakness
energy conservation tech
sign of apiration
sign for resp infection
oral secreation and choking episodes
why are Orthoses good
support weak muscle
reduce stress on functional muscles
energy conservation
mim fatigue
what are some interventions for muscle cramps and spascity
soft tissue mobilization
stretching
cold (short term reduction in spac)
edu about slow prolonged stretches
postural positioning
splints
what is normally included in a PT treatment plan for ALS
ROM and stretching
low to moderated resistance training to functional muscles
submax aerobic activities
what exercises should we avoid with ALS
highly rep or high resistant exercises
can you strengthen muscle that have been weakened by ALS
no
no pain no gain does not work here
how often should the exercise routine be done
daily
can be broken into part to avoid aftigue
what level of exercise should be perfromed
mod intensity
low resistance
