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PubMed This is a summary of 20 peer-reviewed journal articles Updated
Neurology

Standard of Care and Your Team

At a Glance

FTD-ALS has no cure, so care combines ALS-directed medicines such as riluzole and edaravone with support for swallowing, breathing, and behavior; these medicines do not treat FTD's cognitive or behavior changes. A multidisciplinary team and early capacity planning help families manage new needs.

Managing the FTD-ALS spectrum requires more than just treating muscle weakness; it requires a team that understands how the brain and the motor system interact. While there is currently no cure, the standard of care focuses on slowing the disease progression, managing symptoms, and supporting the family through a multidisciplinary care model [1][2].

Standard ALS-Directed Medications

Two main medications are used as ALS-directed therapies. It is important to note that these therapies do not treat the FTD (cognitive and behavioral) component. Because this spectrum involves both thinking and moving, your doctor will tailor these treatments to your loved one’s specific needs.

  • Riluzole: This is a widely used treatment for ALS. It works by reducing levels of glutamate, a chemical messenger that can become toxic to nerve cells if there is too much of it [3].
    • Formulations: For those with dysphagia (swallowing difficulty), a liquid oral suspension or oral film is available [4]. Whether a tablet can be crushed or given through a tube depends on the exact product and must be confirmed with a pharmacist [5].
    • Monitoring: Because Riluzole can affect the liver, blood tests to check liver enzymes (ALT and AST) are required, following local product labels [6]. These are usually checked more frequently during the first six months of treatment [3].
    • Side Effects: Common issues include nausea, dizziness, and fatigue [3].
  • Edaravone: This medication acts as an antioxidant to reduce “oxidative stress” in the nerves [7].
    • Administration: It can be given as an intravenous (IV) infusion or an oral suspension taken following strict fasting instructions [8][9].
    • Considerations: Evidence for edaravone and other ALS trial results in people with significant FTD/ALS-FTSD is limited because such patients are often excluded from trials [10][11]. Eligibility, expected benefit, monitoring burden, route, and local approval differ.

The Multidisciplinary Care Team

Research shows an association between multidisciplinary clinic care and improved quality of life and survival for ALS patients [12][1]. In these clinics, you see several specialists in one visit. A complete team should include:

  1. Neurologist: The team leader who specializes in neuromuscular and cognitive disorders.
  2. Speech-Language Pathologist (SLP): Evaluates swallowing safety and helps with communication strategies or devices [13].
  3. Physical and Occupational Therapists (PT/OT): Provide equipment like braces or walkers and suggest home modifications to prevent falls [14].
  4. Registered Dietitian: Monitors weight and nutrition, which is vital because weight loss can worsen both cognitive and physical symptoms [15][16].
  5. Pulmonologist/Respiratory Therapist: Tracks breathing strength and manages tools like non-invasive ventilation [17][18].
  6. Social Worker & Palliative Medicine: Provides emotional support, complex symptom management, and helps navigate insurance and long-term care planning [19][2].
  7. Pharmacy and Neuropsychology: To assist with complex medication regimens and behavioral expertise.

Preparing for Your Visit

Visits to a multidisciplinary clinic can be long and exhausting. To make the most of them:

  • Bring a Second Set of Eyes: A family member or friend can help take notes, especially if the patient is experiencing cognitive changes.
  • Track Behavioral Changes: Because the patient may not realize their behavior or personality has changed (anosognosia), the caregiver’s report is the most important tool the doctor has [13][20].
  • Prioritize Your Questions: Write down your top three concerns for each specialist before you arrive. Identify a designated contact for urgent daytime changes.
  • Discuss “Capacity” Early: As the disease progresses, it may become harder for the patient to make complex medical decisions. Use early visits to discuss their wishes for future care [17][18].

If your current care team does not include these specialists, consider seeking a second opinion at a certified ALS or FTD Center of Excellence [1].

Common questions in this guide

What is the standard treatment for FTD-ALS?
There is currently no cure for FTD-ALS. Care aims to slow disease progression, manage symptoms such as weakness, swallowing or breathing problems, and support the patient and family. Riluzole and edaravone may be considered for the ALS component, but they do not treat the cognitive or behavioral features of FTD.
Does riluzole treat the frontotemporal dementia part of FTD-ALS?
No. Riluzole is an ALS-directed medicine and is not expected to treat the cognitive, behavioral, or language changes of FTD. It can affect the liver, so the care team generally checks liver enzymes and may monitor them more often during the first six months, following the product instructions.
How is edaravone given for someone with FTD-ALS?
Edaravone may be given by intravenous infusion or as an oral suspension taken with strict fasting instructions. Evidence from ALS trials is limited for people with significant FTD or ALS-FTSD because they are often not included. The neurologist should discuss expected benefit, eligibility, monitoring, route, and local approval before starting it.
Who should be part of an FTD-ALS care team?
A multidisciplinary team commonly includes a neurologist, speech-language pathologist, physical and occupational therapists, dietitian, pulmonologist or respiratory therapist, social worker, palliative care clinician, pharmacist, and neuropsychology specialist. These professionals address movement, swallowing, breathing, nutrition, communication, behavior, medicines, emotional needs, and planning. The exact team may vary by clinic and patient needs.
How can caregivers prepare for a multidisciplinary clinic visit?
Bring a trusted family member or friend to take notes, track changes in behavior, swallowing, breathing, and function, and write down the top concerns for each specialist. Because anosognosia can limit awareness of behavior or personality changes, caregiver observations can be especially valuable. Discuss who to contact for urgent changes and address future decision-making capacity early.
When should capacity and future care wishes be discussed in FTD-ALS?
It is helpful to discuss decision-making capacity and future care wishes early, while the patient can clearly express preferences and participate in complex decisions. The neurologist and care team can explain assessment options and document the patient’s goals. Planning can be revisited as symptoms and abilities change.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How often will my loved one’s liver enzymes (ALT/AST) and bilirubin be checked while they are on Riluzole?
  2. 2.If swallowing becomes more difficult, what is the process for switching from Riluzole tablets to the liquid suspension?
  3. 3.Does our clinic include a speech-language pathologist, genetic counselor, and a social worker who specifically understand the behavioral side of FTD-ALS?
  4. 4.Given the dual diagnosis, what is the specific rationale for starting Edaravone, and how will we monitor its effectiveness?
  5. 5.Can we schedule a formal capacity assessment to ensure my loved one is involved in care decisions while they are still able?
  6. 6.Who is the specific person on the team I should contact for urgent symptom changes?

Questions For You

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References

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This page is for informational purposes only and does not constitute medical advice. Treatment choices, medication monitoring, and capacity planning for FTD-ALS should be individualized with the patient’s neurologist and multidisciplinary care team.

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