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Amyotrophic Lateral Sclerosis - Clin Med II

What does Amyotrophic translate to? Without muscle nutrition
What is ALS? "It is a degenerative disease of motor neurons. no sensory or cognitive problems"
Which areas of the nervous system does ALS affect? It affects both Upper and Lower Motor Neurons.
How is ALS characterized? "It is characterized by progressive atrophy of voluntary muscles. It affects arms, legs, speech, swallowing, and respiration."
When was ALS first identified? It was first identified in 1869.
What symptoms would you expect for LMN with ALS? "There would be weakness and atrophy of muscles, decreased DTRs, and fasciculations. Twitches become daily events and get progressively worse."
What would you expect from UMN with ALS? There will be spasticity, clonus & Babinski, and increased DTRs
What is DTR? Deep Tendon Reflex
What is the pathology of ALS? "There is degeneration of motor neurons in the brain and spinal cord. The loss of nervous system input to the muscles causes secondary degeneration of muscle fibers."
ALS is a primary _______ "neuropathy. (It is a neurological disease demonstrated through muscles.)"
What is the life expectancy for ALS? "It usually kills within decades. There are atypical cases where pts live much longer."
What is another name for ALS? Lou Gehrig's Disease is another name for it.
What is the incident rate of ALS? 4 out of every 100,000 Americans have this disease.
What are the demographics of ALS? "It is twice as common in men compare to women. 93% of pts are Caucasians."
What is the average age to be diagnosed with ALS? 55 yo is the average age to be diagnosed with this disease.
Is ALS inherited? 90% of cases are sporadic, 10% have a familial link.
What does it mean for ALS to be "sporadic"? It means this case of ALS is of unknown origin.
What is the breakdown for life expectancy with ALS? "50% of pts die within 3 yrs following dx, 20% live at least 5 yrs after dx, 10% live more than 10 yrs after dx"
What is key for ALS pts to live beyond 3 yrs? "pts live longer on a ventilator. It is a choice of ventilator or die. pts can expect a very poor quality of life, no good treatments, and no cure. (Debbi Downer says, ""hi"".)"
What are current theories as to the cause of ALS? "5-10% are familial. It could be excess glutamate (neurotransmitter) in CSF and serum. It could be a lack of protein (TDP-43) in the damaged cell's nucleus. It could be genetic, but 50 different genes have been found to be abnormal..."
How is ALS diagnosed? "It is diagnosed over time by looking for progressions of the disease and ruling out all other possible causes. There is no direct test."
What signs are they looking for to aid in diagnosis of ALS? They are looking for muscle weakness and atrophy (clumsiness), fasciculations (twitching), slurred speech, hyperreflexia, and spasticity
What diagnostic test results support a diagnosis of ALS? "CPK levels will be elevated (indicates cell death of muscle everywhere), EMG will be abnormal [wide spread fibrilations, fasciculations (nerve degeneration), and enlarged motor unit potentials]. We may also look at a Myelogram (spinal cord compression),
What conditions need to be ruled out in order to diagnose ALS? MS, tumor, myopathy, peripheral neuropathy, Lymes Disease, HIV, and CTS all need to be ruled out to confirm a diagnosis of this disease.
What is the clinical presentation of ALS? "The onset is rapid with progressive weakness. Mental status (intelligence, memory, personality) and sensation will be intact. pt feels and knows (aware) of everything, but can't move anything - tough."
What can be expected in the Early stage of ALS? "Expect patchy distribution of distal weakness (hands, feet, clumsiness, tripping). There will be muscle ""irritability"" in the form of cramping and spasms. It can progress within weeks to months to involve almost all muscles."
What can be expected in the Middle stage of ALS? "Distal muscle involvement will be more severe than proximal. There is a decline in functional status, and increased incidence of falls. pts will have complaints of ""heavy"" or ""clumsy"" legs. Fasciculations are prominent. There is an onset of hypperref
What can be expected in the Late stage of ALS? "There will be loss of speech (mutism), inability to swallow (aspiration pneumonia, feeding tube - G or J-tube). There will be respiratory failure (mechanical ventilation - less than 10% of pts in US elect this). Clinical trials for diaphragmatic electric
"What drug is available to treat ALS? What does it do?" Riluzole was the first approved drug in 1995. It reduces damage to neurons and prolongs survival by several month.
What is treatment ALS in the Late stage? "Treatment is palliative: comfort care while still trying to solve problems and improve quality of life. This includes G-tube, mechanical ventilation, motorized w/c, and communication devices."
What is the definition of palliative care? "Care to prevent or trea tsymptoms of the disease, minimized side effects, psychological and social problems related to the disease or treatment.
What is the definition of hospice? "Support and care, but no curable care or treatment, not trying to solve problems or prolong life"
What PT intervention is utilized for ALS? It is palliative and preventative. We want to minimize the effects of prolonged immobility. Provide functional training to maintain independence for as long as possible.
How much exercise is to be prescribed for ALS pts? "Want to exercise pt at moderate intensity (no more). Do not over exercise a neuropathic muscle. Start slowly and progress gradually."
What can flexibility training do for ALS pts? "It can help prevent contractures (difficult to bathe and care for) in ALS pts. Prevent frozen shoulder which is painful (they can feel pain - sensory and cognition intact)."
What can positioning do for ALS pts? "Positioning can prevent contractures and promote hygiene. It prevents skin breakdown (which leads to infection, then sepsis)."
How often should the positioning be changed for an ALS pt? Do frequent positional changes (at least every 2 hours). Encouraged to do every 15 min, but it's not realistic. Need to educate the family and caregiver on pressure relief and its importance.
What can PT do for respiratory management of an ALS pt? "Practice Deep breathing exercises, insectospirameter (IS), postural drainage, percussion/vibration, assisted cough, suctioning (saliva and phlegm - Yankauer and Nasotracheal or tracheal, not comfy but can't breathe without). Respiratory issues are usuall
What should we monitor for concerning respiratory in ALS pts? Monitor lung fields, and detect pneumonia early.
What supportive equipment can PT recommend for ALS pts? w/c (standard or electric), cushions to prevent ulcers (air or gel - foam doesn't last long), seating systems for pressure relief or support, a reclining back, hospital bed, hoyer lift, home adaptations (grab bars, stair lifts) may be recommended for this
What support can PT offer? "They can provide education to caregivers and family members about the process/progression of the disease. We can make referrals for support services (social services, psych, counseling, OT, ST, support groups, national/regional foundations for pt and fam
Created by: Susie234



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