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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