Frontotemporal dementia worsens cognitive and behavioral difficulties and lowers life expectancy in people with ALS. It can complicate communication, daily activities, and disease management.

Amyotrophic lateral sclerosis (ALS) is a progressive brain disorder that primarily affects nerve cells in the brain and spinal cord, leading to muscle weakness and loss of control.

When ALS occurs with frontotemporal dementia (FTD), a condition affecting the brain’s frontal and temporal lobes, its effect on life expectancy and quality of life can be significant.

Read on to learn how FTD affects people with ALS, possible treatment options, and more.

The presence of FTD in people with ALS typically leads to a more rapid progression of symptoms and a shorter life expectancy when compared with those with ALS alone.

Research suggests that people with ALS and FTD typically have a shorter survival time than those with ALS alone, about 2.5 years. But this can vary depending on individual circumstances.

This faster decline happens because the combination of muscle weakening from ALS and brain damage from dementia makes both conditions harder to manage together.

FTD adds to the challenges, including cognitive impairment and behavioral changes. These can complicate the management of ALS.

Cognitive decline in FTD can affect a person’s ability to:

  • understand and follow treatments
  • make decisions about their care
  • communicate their needs

Both motor and cognitive decline can increase the likelihood of infections, such as pneumonia, and other complications, further shortening life expectancy.

But, it’s important to remember that life expectancy can vary significantly based on the person’s overall health, the rate of disease progression, and the quality of care they receive.

FTD affects the quality of life for people with ALS in several ways, including:

  • Communication difficulties: FTD may lead to more communication difficulties in people with ALS. It affects their ability to understand and express language. Following instructions or expressing their needs might be challenging, causing frustration and misunderstandings.
  • Emotional blunting: People with FTD often experience reduced emotional expression. This can make them seem detached or indifferent to their loved ones. Lack of emotional connection may create emotional strain for both the person with ALS and their caregivers.
  • Behavioral changes: FTD can cause significant changes in behavior. A person may become impulsive, display socially inappropriate actions, or engage in repetitive behaviors, making it harder for caregivers to manage daily interactions.
  • Cognitive challenges: FTD affects planning, decision making, and judgment. This can complicate care routines, as people may have difficulty following instructions or understanding the importance of certain treatments.
  • Personality changes: FTD often alters personality traits, leading to irritability, apathy, or loss of empathy. This can affect relationships with family and friends, adding to the emotional challenges of managing ALS.

Common genetic factors link FTD and ALS. Half of people with ALS will develop FTD due to a common genetic mutation in the C9ORF72 gene.

This gene mutation is the cause of inherited ALS and FTD. So, researchers classify these two conditions as part of a spectrum of neurodegenerative diseases.

While not everyone with ALS develops FTD, those with a family history of neurodegenerative diseases, especially ALS or dementia, are at a higher risk.

Currently, no cure is available for ALS or FTD. Treatment is focused on managing symptoms and improving the quality of life. Possible treatment options include:

  • Medications for ALS: These include medications like riluzole (Rilutek), which can slightly slow disease progression, and edaravone (Radicava), which may delay some physical decline.
  • Medications for FTD: Antidepressants such as sertraline (Zoloft) or antipsychotics may help manage changes in mood, impulsivity, or aggressive behaviors.
  • Physical and occupational therapy: These therapies help maintain mobility, strength, and function as ALS progresses, supporting independence for as long as possible.
  • Speech therapy: ​​This therapy may assist with communication challenges from ALS and FTD. Tools like speech-generating devices or communication boards may be helpful.
  • Cognitive and behavioral therapy (CBT): CBT focuses on managing the emotional and cognitive symptoms of FTD, assisting with memory loss, decision making, and personality changes.
  • Caregiver support: Support groups, counseling, and respite care can help caregivers manage stress and provide optimal care.
  • Palliative care: As ALS and FTD progress, palliative care shifts the focus to ensuring comfort and the best possible quality of life. Palliative care aims to manage symptoms such as pain, breathing difficulties, and emotional distress.

In the final stages of ALS with FTD, a person may experience a severe decline in their physical and cognitive abilities. They might find it harder to move, speak, or breathe without assistance.

FTD can also cause severe memory loss, confusion, and personality changes, making communication difficult.

At this stage, a person will likely need full-time care and mechanical ventilation to help with breathing.

The focus will shift to palliative care, prioritizing comfort over extending life and ensuring that they spend their last days with dignity and ease.

This time can be challenging for loved ones. Planning ahead for end-of-life care, including discussions about wishes, advance directives, and hospice options, will help ensure preferences are respected.

ALS with FTD significantly affects life expectancy and quality of life due to its effects on physical abilities and cognitive function.

The progression of these diseases can feel overwhelming. While there’s no cure, treatments can help manage symptoms.

It’s crucial to have a strong support system, prepare early for future care needs, and prioritize maintaining the best possible quality of life throughout the disease progression.