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
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
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