Symptoms and Signs of Dementia in ALS

Medical Author: John P. Cunha, DO, FACOEP
Medically Reviewed on 11/15/2021

Doctor's Notes on Dementia in ALS

Amyotrophic lateral sclerosis (ALS, also called Lou Gehrig's disease) is a type of motor neuron disorder that affects the part of the nervous system that controls voluntary movements. The muscles become progressively weaker, and the disease eventually leads to paralysis and death. In most people with ALS cognitive processes (such as thinking, learning, memory, and speech) are not affected. Occasionally, a person with ALS does experience dementia, a severe brain disorder that interferes with a person’s ability to carry out everyday activities.

Symptoms of dementia in amyotrophic lateral sclerosis (Lou Gehrig disease) include

  • apathy (lack of interest, withdrawal),
  • lack of emotion,
  • reduced spontaneity,
  • loss of inhibition,
  • restlessness or overactivity,
  • social inappropriateness,
  • mood swings,
  • memory loss,
  • loss of speech and/or language (partial or complete), or
  • loss of reasoning or problem-solving ability.

What Is the Treatment for Dementia in ALS (Lou Gehrig Disease)?

There is no cure for amyotrophic lateral sclerosis (ALS).  Two medications are currently available to try to slow the progression of the disease and one is used to stabilize mood symptoms:

  • Riluzole (Rilutek
    • Has been shown to slow the progression of ALS for some patients and increase survival
  • Rasagiline (Azilect)
    • A monoamine oxidase inhibitor (MAOI) that demonstrates neuroprotective action
    • Appears to increase survival
  • Nuedexta (dextromethorphan HBr and quinidine sulfate)
    • Used for pseudo-bulbar effects such as inappropriate crying or laughing

Patients with ALS diagnosis usually experience a rapid decline in both physical and cognitive abilities over the course of 1 to 3 years (in some cases it is slower). It is important for the patient and the family to surround themselves with a multi-disciplinary team to help with the long-term management issues for the ALS patient. Teams helping in the care of patients with ALS may include:

  • Speech therapists
  • Occupational therapists
  • Physical therapists
  • Respiratory therapists
  • Neuropsychologists
  • Nurses (especially home-care nursing)
  • Other home care professionals (home health aides, caretakers)
  • Social workers

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Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.