aml ten minute
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Ten Minutes of Medicine on Amytrophic Lateral Sclerosis Diagnosis, Symptoms, Treatment,& Research for a Hospice Home TeamTRANSCRIPT
SUNFLOWER HOSPICE Home Team
TEN MINUTE MEDICINE
Amyotrophic Lateral Sclerosis11.25.2014
Neurodegenerative Disease
• Usually Starts At Age 60
• If Inherited Starts At Age 50
• Muscle Spasticity
• Rapid Progression To Muscle Weakness
• Difficulty Swallowing, Speaking , Breathing
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
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
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
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
BCV-Biphasic Cuirass Ventilation
BiPAP-Bilevel Positive Pressure Airway
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
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
Treatment
• Riluzole (Rilutek) Only Treatment-Lengthens Survival by Several Months
• Extends Time Before Person Needs VentilSupport
• Antiglutaminergic-Reduces Damage to Motor Nerons
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
Sunflower Thought
The EndWe Bloom Where We Are Planted