Lou-Gehrig's Disease (Amyotrophic Lateral Sclerosis) - Medtick
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Lou-Gehrig’s Disease (Amyotrophic Lateral Sclerosis)

What is it?

A progressive condition and is most common disease of the anterior horns of the spinal column, which hold the nerve roots responsible for control of the muscles of the trunk or head.

  • It causes progressive deterioration of both the upper motor neurons of the corticospinal tract, which conduct impulses from the brain to the spinal cord, and the lower motor neurons, which carry the impulses from the corticospinal tract to the individual muscles.
  •  As a result one’s voluntary muscles waste away causing muscle weakness, stiffness, speaking difficulties , swallowing difficulties and breathing difficulties .
  • It is caused by the dying of nerve cells, these cells process and transmit information from the brain/spinal cord to other parts of the body.
  • Amyotrophic lateral sclerosis (ALS) is the commonest form of Motor Neurone Disease affecting upper motor neuron  and lower motor neurons .
  • Symptoms most commonly develop between the ages of 55 and 75.
This condition can be fatal as it can cause respiratory failure.

Cause

  • Unknown
    • Maybe mutation occurs in the superoxide dismutase (SOD1) gene
    • The dysfunctional SOD1 enzyme forms a complex with copper and zinc ions within the mitochondria, leading to protein unfolding and aggregation. This accumulation disrupts the mitochondrial membrane potential and acts as a catalyst for further mitochondrial dysfunction. Cellular adenosine triphosphate (ATP) production is decreased and calcium homeostasis is disturbed, triggering apoptosis and leading to cell death.

Tradewell M, Cooper LA, Minotti S et al. Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis:mechanistic relationship and differential sensitivity to intervention.  Neurobiol Dis 2011;42:265–275.

  • Family history

Symptoms

  • Muscle twitches in the arm, leg, shoulder, or tongue?
  • Muscle cramps?
  • Tight and stiff muscles (spasticity)?
  • Muscle weakness affecting an arm, a leg, neck or diaphragm?
  • Slurred and nasal speech?
  • Difficulty chewing or swallowing?
  • problems with moving?
  • Problems with swallowing (dysphagia)?
  • Problems with speaking or forming words (dysarthria)?
  • Breathing difficulties (dyspnoea)?
  • Lose weight rapidly?

National institute of Neurological disorders and stroke

Complications /Information to beware of/General tips:

Medical Emergency Condition

And/or do not wait, phone for an ambulance if have or develop:

This condition can lead to:

  • Cognitive impairment and dementia-like symptoms, typically related to frontotemporal executive dysfunction, occur in up to 50% of ALS patients.
  • Parkinsonian symptoms, facial masking, tremor and postural instability may also develop.

Ringholz GM, Appel SH, Bradshaw M et al. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology 2005;65:586.

This condition may show similar symptoms to:

Detailed Information

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