Amyotrophic Lateral Sclerosis

see also Motor Neuron Disease

  • ALS (also called Lou Gehrig’s disease) a rapidly progressive and fatal neurodegenerative disease.
  • Most common type of motor neuron (MN) disease.
  • Characterised by gradual degeneration and death of MNs.
  • Most people who develop ALS are 40-70 years old.
  • The average age for the onset of ALS is 55.
  • ALS occurs throughout the world with no racial, ethnic, or socioeconomic boundaries.

ALS can be classified as

  • Sporadic: most common form.  Random and no known cause
  • Familial: Inherited form (rarer occuring in ~5%)
  • Guamanian: An extremely high incidence of ALS was observed in Guam in the 1950s

Symptoms

Usually begins with a weakness of one limb, which progresses to two/more limbs.  Can also present as dysarthria/dysphagia.

As it progresses, symptoms can include twitching, cramping, partial/complete paralysis, falls/instability, lack of fine motor control/coordination, persistent fatigue etc.  Eventually, ALS will effect the respiratory system and cause difficulty breathing.  Combined with swallowing difficulties, pneumoniae/choking is the most common cause of death.

Pathology

The main feature of ALS is degeneration of the upper and lower motor neurons in the motor cortex, brain stem and spinal cord.  Proteinous inclusions are found in both motor neurons and astrocytes in human ALS.

Familial ALS

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

%d bloggers like this: