Lec 17 - ALS

What is amyotrophic lateral sclerosis (ALS)?

aka Lou Gehrig’s disease

• neurodegenerative

• destruction of motor neurons

  • localized paralysis

  • atrophy of muscles

• no cure

Who is ALS common in?

Common age: 40-70

• 20% more common in men.

• 93% of patients are Caucasian

• Average survival – 3-5 years (some 20+ years)

types of ALS? (3)

Sporadic

• most common form 90-95% of all cases

Familial

• genetic (5 to 10% of all cases)

Guamanian

• high incidence of ALS in Guam in 1950's

• Due to toxins in water and food

What are probable causes of ALS?(3)

Free radicals

• inherited form of ALS

• mutation in a gene that produces a strong antioxidant enzyme that protects your cells

Glutamate

• People with ALS typically have high levels of glutamate

• can be toxic to some nerve cells

Autoimmune response

• Trigger unknown – attack own nerve cells

early symptoms of ALS? (5)

Increasing muscle weakness

• Twitching

Cramping

• especially in arms and legs

Difficulty speaking

Trouble swallowing

Laboured breathing

late ALS symptoms? (7)

Extreme muscle atrophy

Reflexes are slow or non existent

Excessive drooling

Increased spasticity (muscle rigidity)

Weight loss

Choking

Respiratory and Cardiac failure

• Death

how to diagnose ALS?

X-rays

MRI

Blood and urine studies

Muscle and nerve biopsy

• electomyography (EMG)

nerve conduction velocity (NCV)

spinal tap

Still difficult to diagnose ALS from other neural diseases

What is symptomatic treatment for ALS?

Diazepam can relieve spasticity

Medical marijuana

Anti-inflammatories and analgesics

exercise as ALS treatment?

Increases anti-oxidants

Decreases spasticity

Reduces atrophy (early ALS)

• Resistive training important

Balance exercises reduce falls

What is multiple sclerosis (MS)?

Auto-immune

• neuro-degenerative/inflammatory

• break down of myelin sheath

  • affects nerve conduction

When is onset of MS?

Onset is usually between 20-40 years old

• Some childhood cases

• Relapsing/Remitting

  • Can stay stable for years

What is progressive MS?

Onset is usually after age 45

Worsens with time

• May or may not have periods of high severity

What is timeline for MS?

about 10 years

symptoms of MS? (9)

Fatigue

Muscle tingling

Numbness

Muscle weakness

Muscle spasticity

Incontinence

Vision problems

Mood swings

Falls

RF for MS? (6)

Latitude

• Further from Equator

• Temperate zones

• Vitamin D deficiency?

Obesity (esp. teenage)

Smoking

Genetics

Women>Men (3x)

Caucasian- more common

effect of aging on MS?

Aging may affect progession and severity

• More inflammatory markers

• Poor cell repair / Cell senescence

• Mitochondrial dysfunction

• Telomere shortening

• Lower estrogen and testosterone

• Changes in gut biome

Potential therapies target senescent cells and aging pathways

• May help with remyelination and neuroprotection

treatments for MS?

Medications

• Steroids

• Immuno-modulatory Meds

  • Change pattern or progress of auto-immune response

• Inflammatory meds

  • Change progression of inflammation

• Relapse-limiting meds

• Treat pain as needed

Exercise

What should exercise focus on for MS patients? (6)

Aerobic endurance

Muscle strength

Muscle endurance

Mobility / Prevent falls

Reduce risk of co-morbidities

• Cardiovascular disease / Diabetes

Better cognitive function