lecture 4: Amyotrophic lateral sclerosis Flashcards
what is another work from Amyotrophic Lateral Sclerosis (ALS)
lou gehrig disease
what kind of disorder is Amyotrophic Lateral Sclerosis (ALS)
progressive neurological disorder
what is the most common form of motor neuron disease in adults
Amyotrophic Lateral Sclerosis (ALS)
what is Amyotrophic Lateral Sclerosis (ALS) characterized by
the slow degeneration of. a motor neurons in the ventral horn (where motor neurons are) of the spinal cords and BS
what is amyotrophy
disorder of anterior horn cells of spinal cord and motor cranial nuclei
what does amyotrophy lead to
mm atrophy and weakness resulting from massive loss of alpha motor neurons
what is lateral sclerosis
demyelination and gliosis of the corticospinal tracts and corticobulbar tracts resulting from degeneration of the pyramidal cells in motor cortex , the cell bodies of the UMNd
lateral sclerosis is basically “scarring” which’s is what
type of motor neuron disease
post-polio syndrome, spinal muscular atrophy, Kennedy’s disease, primary lateral
sclerosis, progressive bulbar palsy
these are all what kind of diseases
motor neurons
what neurons are affects with Amyotrophic Lateral Sclerosis (ALS)
UMN and LMN without sensory loss
only motor neurons are affected
what is the average age , gender what is diagnosed with Amyotrophic Lateral Sclerosis (ALS)
40-70 and men
t/f: Amyotrophic Lateral Sclerosis (ALS) starts off either LMN or UMN but will progress to UMN and LMN
true
what kind of diagnosis is Amyotrophic Lateral Sclerosis (ALS)
diagnosis of exclusion
required a UMN and LMN symptoms as well as a decline in physical function
what are the initial symptoms of Amyotrophic Lateral Sclerosis (ALS)
very greatly but many ppl start w a single limb affected with different neural roots and nerves affected in that limb
what is common in Amyotrophic Lateral Sclerosis (ALS) patients
sense of faitgue and reduced exercise tolerance
what kind of onset can patients with Amyotrophic Lateral Sclerosis (ALS) have
Can have spinal onset (start in the limbs) or bulbar onset (cranial nerves).
Umpired reports that by the time most patients report weakness (ALS) they have lost approximately ___ of their motor neurons in the areas of weakness
80%
Despite the difference in onset, eventual course of the illness is ____ in most patients
(with different timings)
similar
what kind of UMN signs will a ALS patient have
- spasticity
- hyper reflex
- pathological reflexs
what is the LMN signs in a ALS patient
- mm weakness
- decrease endurance
- weakness neck extensors are common
- hyporeflexia
- hypotonicity
Pseudobulbar Palsy
• Emotional lability
i.e. inappropriate
or uncontrolled
laughing or crying
• Dysarthria
• Dysphagia
• Dysphonia
• Impairment of
voluntary
movements of the
tongue and facial
muscles
• Sialorrhea
these are all signs of what in ALS patients
bulbar signs
• Nocturnal
respiratory
difficulty
• Exertional
dyspnea
• Accessory muscle
use
• Paradoxical
breathing
these are all what kind of sings in ALS patients
respiratory signs
what are the 3 main things that someone with ALS will present with
faitgue ,
soasictity
mm weakness
the ALS Functional Rating Scale – Revised
(ALSFRS-R) is a ___ reported measures that is used for people with ALS and the higher the score the ____ the physcial function
patients
better
what is the primary movement dysfunction from a force production deficit with people with LA S
weakness
what are the associated sings with the force production deficit with people with ALS
movement:
mm tone:
sensation:
coordination:
movement: fractionated
mm tone: mixed (UMN and LMN)
sensation: normal
coordination: may be unable to test due to weakness
what is the mortality rate for ALS
3-5 years
what kind of intensity exercise should u use for ALS patients
moderate
- safe in early stages
- strengthening and cardiovascular exercises
- help maintain function
it is important to monitor what when exercising ALS patients
for overwork weakness
what is overwork waekness in patients with ALS
o Post exercise fatigue that interferes with activities
o Feeling weaker or pain >30 minutes post exercise
o Excessive soreness 24 – 48h post exercise
o Severe muscle cramping, heaviness in the extremities, prolonged SOB
what is key for exercise considerations in patients with ALS
exerccseis without excessive faitgue
