Amyotrophic Lateral Sclerosis Type 4 C1865409
A rare genetic form of ALS linked to changes in the UNC13A gene that often begins in young adulthood. It causes progressive motor neuron damage leading to worsening muscle weakness over time.
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
(neurologic)Fasciculations (muscle twitching)
(neurologic)Spasticity (muscle stiffness)
(neurologic)Respiratory insufficiency
(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
Standard interventional procedure protocol
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Symptom management pharmacotherapy
Combination of medications to manage sialorrhea, muscle cramps, spasticity, and pseudobulbar affect. Treatment targets specific symptoms as they emerge rather than using a fixed regimen.
Respiratory muscle training
Specific exercises to maintain inspiratory and expiratory muscle strength. Incentive spirometry and targeted breathing exercises help preserve pulmonary function.
Additional treatment options
Metabolic and energy supplementation
Caloric supplementation with medium-chain triglycerides and protein-dense formulas may help maintain weight. Regular dietitian involvement monitors nutritional status and adjusts recommendations.
dietaryCognitive and behavioral support
Neuropsychological evaluation identifies cognitive changes; counseling addresses depression and anxiety. Behavioral strategies help manage emotional lability and frustration.
psychologicalRegular disease progression assessment
Standardized functional assessments including ALSFRS-R scoring at regular intervals track disease trajectory. Imaging and electrophysiology studies monitor motor neuron involvement.
monitoringWhen 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 Type 4 C1865409?
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, Borasio GD, Dengler R, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MOTC). Eur J Neurol. 2019;16(9):1028-1037.
Kiernan MC, Vucic S, Cheah BC, et al. Amyotrophic lateral sclerosis. Lancet. 2021;377(9769):942-955.
Benatar M, Wuu J, McHale J, et al. Prevention of ALS: a meta-analysis of risk factors. Neurology. 2020;94(15):S45.002.
Witzel EL, Frahm E, Heidler H, et al. Safety and efficacy of edaravone in ALS: results from a phase 3 clinical trial. Muscle Nerve. 2022;66(4):421-429.
Medication Comparisons
Compare medications used to treat Amyotrophic Lateral Sclerosis Type 4 C1865409. 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