2.9 ALS Intro Flashcards
What is the clinical presentation of ALS? What neuroscience structures are affected?
Combination of upper and lower motor neuron lesions. Presents as spasticity, hyperreflexia, generalized weakness, muscle atrophy, and paralysis.
Selective dysfunction and death of neurons in the motor pathway.
Motor neurons controlling bladder and eye movements are spared.
Clinical Signs: Asymmetric tongue protrusion, cranial nerve deficit. Drooping of head, muscle cramps, fasciculations – involuntary muscle twitches due to deneravted muscles.
What is the disease course of ALS?
Genetic mutation and excitocity
Fast relentless
Dominant LMN impairment, then UMN masks LMN neuron involvement
Women decline faster than men
What is medical management of ALS?
Disease modifying therapies, cognition and behavior, communication and education.
Nutrional management, respiratory management 65% increases survival, symptom management
What are the prognostic factors related to ALS?
Ventilation less than 75% decreases survival time.
Multi-disciplinary clinic – increases quality of life and survival rates.
Age – more than 80 years : poor likelihood of surviving
Gender – women decreases survival due to increased rates of bulbar onset
What outcome measures are used in individuals with ALS?
ALS Functional Rating Scale – is a 4 point scale
Increased score indicates increased level of function
Items on it include bulbar function, fine motor function, gross motor function, gross motor function, respiratory function.
ALS Depression Inventory – includes respiratory system, chest expansion, fatigue, mobility.
What is general rehabilitation management of ALS?
Respiratory therapy – greater than 75% longer survival rate, 65% recommended consideration point for NW, less than 50% tube feeding minimize risk of aspiration.
