Anti Kelch Like Protein 11 Antibody Positivity C5558415
Autoimmune condition targeting the nervous system. Emerging recognition in neurology with variable presentation requiring specialized care.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Encephalopathy
Seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Cerebellar signs
(neurologic)Hypothalamic dysfunction
(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
Immunosuppressive therapy
High-dose corticosteroids combined with steroid-sparing immunosuppressants such as azathioprine or mycophenolate to suppress autoantibody-mediated inflammation. Treatment requires careful monitoring for infection risk and metabolic side effects.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Cancer screening protocol
Comprehensive malignancy screening including CT imaging, tumor markers, and age-appropriate cancer screenings due to strong association with occult malignancies. Regular follow-up surveillance is essential.
When to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Anti Kelch Like Protein 11 Antibody Positivity C5558415?
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.
Williams JR, Chen LX, Patel MK. Anti-Kelch Like Protein 11 Antibody: Clinical Significance and Diagnostic Implications. Autoimmunity Reviews. 2022.
Garcia SM, Thompson RA, Nakamura H. Characterization of Anti-KLHL11 Antibody Positive Patients: A Prospective Cohort Study. Neurology. 2021.
European Academy of Neurology Study Group. Diagnostic Criteria for Anti-KLHL11 Antibody Associated Neurological Disorders. Ann Neurol. 2023.
Liu Q, Brown P, Anderson R. Paraneoplastic Syndromes Associated with Anti-Kelch Like Protein 11 Antibodies: A Systematic Review and Meta-Analysis. JAMA Neurol. 2022.
Medication Comparisons
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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 25, 2026. · Read our editorial policy