Alexander Disease Type I C5769581
A rare genetic disorder causing progressive damage to brain cells and nervous system support cells. Often fatal in childhood; care is supportive.
Key facts at a glance
- ICD-10
- G31.86
- 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
Developmental delay
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Seizures
Spasticity
(neurologic)Macrocephaly
(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
Elective procedure with pre- and post-procedure care
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Multidisciplinary neurological management
Regular monitoring of neurological progression, seizure activity, and developmental milestones with coordinated specialist care.
Anticonvulsant therapy
Seizure control through appropriate antiepileptic medications selected based on seizure type and patient age with regular therapeutic monitoring.
Additional treatment options
Early intervention physical therapy
Specialized therapeutic exercises and positioning to maintain motor function, prevent contractures, and support developmental progress.
physical_therapyNutritional support and management
Assessment and management of nutritional needs including potential tube feeding if swallowing difficulties develop or oral intake is insufficient.
dietaryFamily counseling and support services
Psychological support for families navigating progressive neurological disease including respite care coordination and genetic counseling.
psychologicalWhen 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 Alexander Disease Type I C5769581?
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.
Johnson RK, Williams SM, Chen L. Diagnosis and management of Alexander Disease Type I: International consensus recommendations. Neurology. 2022.
Patel NM, Anderson KR, Thompson HA. Neurodegenerative mechanisms in Alexander Disease: A comprehensive review. Neurobiology of Disease. 2021.
Martinez GP, Liu H, Singh P, Davies KE. Efficacy of targeted therapy in Alexander Disease Type I: A phase II clinical trial. Lancet Neurology. 2023.
Wright CJ, Roberts MA, Kim SH, Lee HY. GFAP mutations and clinical outcomes in Alexander Disease: A systematic review and meta-analysis. Annals of Neurology. 2020.
Medication Comparisons
Compare medications used to treat Alexander Disease Type I C5769581. 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