expectancy with Charcot’s disease

The symptoms of amyotrophic lateral sclerosis (ALS), or Charcot’s disease, typically include complete paralysis of the muscles in the arms, legs and throat, over time leading to an inability to walk, eat, talk and even breathe. Amyotrophic lateral sclerosis (ALS) begins in adulthood between the ages of 40 and 80, and will progress within 3 to 5 years to complete paralysis and death, usually due to paralysis of the patient’s respiratory muscles.

Depending on the location of the motor neurons affected at disease onset, the initial symptoms of Charcot’s disease will differ:

• In most cases, the disease begins with motor weakness in the upper or lower limbs. This is known as the spinal form of the disease. The affected limbs may atrophy and become flaccid when the peripheral motor neuron is most affected, or become stiff when the central motor neuron is most affected. Patients typically experience cramping and fasciculations (small, brief and involuntary muscle contractions without joint movement). Muscle weakness is localised at first, but then becomes worse and gradually spreads to other regions of the body.
• In around 30% of cases, Charcot’s disease begins with damage to the motor neurons in the brain stem (bulbar-onset ALS), and the first symptom is difficulty articulating words. Most often, this is soon combined with difficulties in swallowing, with silent aspirations. In this form of the disease, there is also excessive saliva in the mouth, as well as high emotional sensitivity.
• In rarer cases (around 10%), the disease may begin with damage to the musculature of the neck, causing ‘drooped head syndrome’ and difficulty remaining upright when walking, and/or shortness of breath.
• In most cases, the disease begins in one region of the body and spreads to others, so that patients with a spinal form of the disease may later develop bulbar-onset symptoms, or vice versa.
• All patients will experience impairments in their breathing muscles, causing respiratory failure in the later stages of the disease. It is this respiratory failure that will affect patients’ prognosis and eventually result in death.