ALS Flashcards
What is the most common adult onset MND?
motor neuron disease is ALS
What does ALS stand for?
Amyotrophic Lateral sclerosis
How is ALS characterized?
progressive
fatal
paralytic muscle wasting
What nuero system is involved in ALS?
UMN & LMN
What is age onset of ALS? and average onset
teens to 80’s average 50’s
What are the two types of ALS? and %?
SALS (sporadic) 90-95%
FALS (familial) 10-5%
What are the two onsets of ALS? and %?
limb onset 70-80%
bulbar onset 20-30%
What are limb and bulbar onset ALS?
limb onset: initial onset in the extremities and UMN>LMN
bulbar onset: initial involvement of bulbar muscles ie muscles of face and throat and LMN>UMN
Which onset if more detrimental?
bulbar affecting breathing
ALS male or female more common?
male ALS
What are some possible reasons for ALS?
oxidative stress
neurotoxicity (mercury, lead)
protein aggregation
lifestyle factors
What are common risk factors for ALS?
mutation: SOD1 Cluster of demographic: western Pacific male Gender: male>female Age Family hx
Disease course of ALS?
most die within 3-5 years due to respiratory failure
What is Pseudobulbar Palsy? S&S?
Corticobulbar tract affected
Speech, mastication, swallow
Pseudobulbar effect
What is Pseudobulbar effect?
sudden crying and laughing
What is Progressive Bulbar Palsy? S&S?
CN affected
Swallow, mastication, facial muscles
Fasciculation of tongue PROMINENT
What is PLS? S&S?
Primary lateral Sclerosis
Corticospinal tract, neuron loss in cortex
ONLY UMN affected: hyperreflexia/ spasticity
no fasciculation or atrophy
What is PMA? S&S?
Progressive Muscular Atrophy
loss of motor neurons in Anterior horn cells
ONLY LMN
progressive weakness, wasting, & fasciculation of hand muscles
MIX of what two forms results in the term ALS?
PLS & PMA= ALS
What are the best indicator to survival in ALS?
age at time of onset,
<35 y.o has best survival rate than older pt
and
limb-onset: they have better outcome than bulbar
What are some UMN signs?
spastic hyperreflexia clonus Babinski muscle weakness
What are some LMN signs?
weakness focal asymmetrical begins in UE, LE, or Bulbar muscles Begins distally in extremities hyporeflexia, hypotonia, fatigue
What is a typical weakness in LMN ALS?
C/S extensors
What are some Bulbar signs?
Dysarthria Dyphagia Sialorrhea Pseudobulbar effect respiratory dysfunction
