Amyotrophic Lateral Sclerosis 26 With Or Without Frontotemporal Dementia C5436882
A genetic form of ALS that may cause thinking and behavior changes similar to frontotemporal dementia alongside movement symptoms. It affects adults and worsens over time, with treatment focused on symptom management.
Key facts at a glance
- ICD-10
- G12.21
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- fatal
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Progressive muscle weakness
Frontotemporal dementia
Respiratory dysfunction
Muscle atrophy
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Disease-modifying pharmacotherapy
Riluzole, an FDA-approved glutamate inhibitor, represents the standard disease-modifying treatment approach. May modestly extend survival in ALS patients with genetic forms of the disease.
Standard interventional procedure protocol
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Multidisciplinary ALS care clinic
Comprehensive care through a specialized ALS clinic involving neurology, pulmonology, gastroenterology, and mental health professionals. Regular assessments every 3 months or as symptoms change.
Additional treatment options
Neuropsychiatric management
Management of behavioral symptoms associated with frontotemporal dementia using non-pharmacological approaches first, including behavioral modification strategies and caregiver education.
psychologicalRespiratory function surveillance
Regular monitoring of vital capacity and nocturnal oximetry to anticipate respiratory failure. Early discussion of non-invasive ventilation options before crisis develops.
monitoringSymptomatic physical therapy
Goal-oriented physical and occupational therapy focusing on maintaining function, preventing contractures, and adapting to progressive weakness through assistive devices.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Amyotrophic Lateral Sclerosis 26 With Or Without Frontotemporal Dementia C5436882?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) - revised report of an EFNS task force. Eur J Neurol. 2012.
Burrell JR, Halliday GM, Kril JJ, et al. The frontotemporal dementia-motor motor neuron disease continuum. Lancet Neurol. 2016.
Benatar M, Wuu J, Andersen PM, et al. Design of a randomized, placebo-controlled, phase 2/3 trial of antisense oligonucleotide therapy for C9orf72-associated amyotrophic lateral sclerosis (C9ORF72-ALS). Neurology. 2020.
Gordon PH, Meininger V. How can we improve clinical trials in amyotrophic lateral sclerosis? Nat Rev Neurol. 2011.
Medication Comparisons
Compare medications used to treat Amyotrophic Lateral Sclerosis 26 With Or Without Frontotemporal Dementia C5436882. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy