OPTA 222 (Amytrophic Lateral Sclerosis)
Cause of ALS
5-10% inheratance
90-95% sporadic: oxidative stress, abnormalities in immune system, glutamate toxicity
Pathology
-Affect upper motor nueron and lower motor neuron
-neuron death in corticospinal and corticobular tracts: degeneration of betz cells
death usually in 3 years
upper motor neuron
lesion are diagnosed by reflex testing
- A positive Hoffman sign: abdution of thumb
-a positive Babinski reflex: toe turns out
Motor Lower Neuron
lesion are diagnosed by
-loss of muscle tone
-flaccidity
-week or absent deep tendon reflexes
First present of ALS
- Fatigue
- upper and lower motor nueron signsin 2 spinal regions
- pregression of signs and symptoms
- bulbar signs; difficulty swallowing, speech sensation in throut
Thing you don't see in ALS
-Presence of sensory
- autonomic nervous system disfuncton
- sphincter contro issue
-ocular motor nerve pathways abnormalities
-cog deter
Diagnosis and prognosis
D: EMG(eletrical activity in muscle), Gene Testing, nerve conduction studies(how quickly and electical signalcan be transmitted down a nerve),
P: eventual death from respitory failure
Early Stages
-Muscle weaknessboth spasticity or flaccid
-loss of muscle bulk
-Fasciculation(muscle contractions, visible under skin)
- Fatigue, balance issues, tripping
- footdrop
- hand weakness
- slurred speech
- dysphasia
Middle Stages
-swallowing muscles
- some muscle might be fully paralized
- breathing weakened
- emotional(pseudobulbar affect)
End Stages
- extreme limited mobility
- may not be eating by mouth
- breathing limited(lack of o2
- death
theraputic goals physical
Ex