Aml ten minute
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Ten Minutes of Medicine on Amytrophic Lateral Sclerosis Diagnosis, Symptoms, Treatment,& Research for a Hospice Home Team
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Aml ten minute
- 1. SUNFLOWER HOSPICE Home Team TEN MINUTE MEDICINE Amyotrophic Lateral Sclerosis 11.25.2014
- 3. Neurodegenerative Disease • Usually Starts At Age 60 • If Inherited Starts At Age 50 • Muscle Spasticity • Rapid Progression To Muscle Weakness • Difficulty Swallowing, Speaking , Breathing
- 4. Occurrence • In US 5,600 Diagnosed Each Year • 30,000 Americans Currently Affected • In Europe 2.2/100,000 People • Unknown Rate In Rest Of World • First Described By Charles Bell in 1824 • 5-10% Inherited From Parents • Diet Rich in Branch Chain Amino Acids
- 5. Onset • 25% Bulbar- First Difficulty Speaking & Swallowing- Quicker Course • Cognitive Function, Bladder, Bowel, Eye, Movement Generally Spared • 5% Frontotemporal Dementia • Majority Maintain, Hearing, Sight, Touch, Smell
- 6. Earliest Symptom • Weakness &/Or Muscle Atrophy • Others Trouble Swallowing, Cramps, Stiffness • Slurred Speech, Nasal Speech • Leg Onset, Dropped Foot • Arm Onset, Cant Button Shirt, Use Key
- 7. Progression • Eventually Cant Walk or Use Hands, Arms • Lose Ability to Speak, Swallow Food • Slower Progression in Obese or Younger -40 • In Limb Onset Uss Affects One Them, then next before Moving to Other Body Region • Bulbar Uss Arms First
- 9. BiPAP-Bilevel Positive Pressure Airway
- 10. Diagnosis • No Test Can Provide Dx • Upper & Lower Motor Neuron Signs In Single Limb Is Suggestive • Dx Based On Sx & Signs MD Observes • EMG May Show Conductivity Problem • MRI Uss Normal
- 11. Prognosis • Median Onset From Dx-Death 39 Months • Less Than 4% Survive Longer Than 10 Years • Physicist Stephen Hawking Survived >50yrs • Uss Death In 3-4 years • Uss Death From Respiratory Failure Or Pneumonia
- 12. Treatment • Riluzole (Rilutek) Only Treatment-Lengthens Survival by Several Months • Extends Time Before Person Needs Ventil Support • Antiglutaminergic-Reduces Damage to Motor Nerons
- 13. Research • Stem Cell In Israel, Bone Marrow Stem Cells Taken & Grown To Prod Neurotrophic Factors Injected Back-tendency to Stabalize ALS • Ozanezumab- Monoclonal Ab to Stabilize Connection Between Motor Neuron & Muscle Phase II, Small Group Some Promise
- 16. The End We Bloom Where We Are Planted