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Rare Epilepsies & ICD Codes
ICD CODES are critical for rare epilepsies. ICD codes have the potential to:
(1) accurately determine incidence, prevalence, comorbidity profile, and mortality
(2) determine the impact of DEEs on the healthcare system
(3) determine the best care for each patient
(4) improve communication amongst healthcare providers
(5) may decrease time to diagnosis
(6) identify populations that may benefit from precision therapies
(7) identify populations for care management and population health initiatives
(8) identify patients for enrollment in clinical trials. ​​
"This code is more than just a formality - it's the critical key to unlocking so much for our community. Without it, our patients remain invisible, scattered across different medical systems, and uncounted. This lack of recognition leads to a cascade of issues - underreported data, lack of funding, missed opportunities for research, and, most critically, no clear path to better treatments. Having a code means being counted, and being counted means access to better care, more precise treatments, and the attention that CTNNB1 patients deserve. It means we can finally build a clearer picture of the prevalence and impact of this rare disease, making the journey less lonely for each family."
Effie Parks, CTNNB1
To search for codes for epilepsy or by disorder, Visit: https://icd10cmtool.cdc.gov/?fy=FY2022
To promote the utilization of codes, REN is tracking rare epilepsies with codes and maintaining a list here. If you are aware of missing codes, please contact: info@rareepilepsynetwork.org
Conditions & Corresponding ICD-10 Code
SECURING CODES HAS BEEN A CHALLENGE FOR MANY DISORDERS
Across the rare epilepsies and neurodevelopmental disorders, it has been a challenge to secure codes. As of September 2024, the following disorders were under consideration by the National Center for Health Statistics(NCHS) for codes. Some disorders have been waiting for more than a year.
Articles and podcasts on this topic include:
ICD-11 Is Coming Soon and We Need Your Help!, Mike Graglia, Syngap Research Fund, October 16, 2021
Fight Like a Mother: When an ICD-10 Code Determines Life or Death, Frieda Wiley, Inside Precision Medicine, April 1, 2024
Once Upon a Gene - Episode 224, Amber Freed, Once Upon a Gene, April 4, 2024
FOR ADDITIONAL BACKGROUND ON ICD CODES
ICD-10, the International Classification of Diseases, 10th Revision, is a globally recognized system for coding diseases, symptoms, abnormal findings, and mortality. Developed by the World Health Organization (WHO), ICD-10 standardizes health condition reporting and enables better tracking of global disease and mortality statistics. In the U.S., a modified version called ICD-10-CM is used for diagnosis coding, and ICD-10-PCS for inpatient procedure coding. Notably, there is often a significant delay in the U.S. adoption of each ICD revision; ICD-10, endorsed by WHO in 1990, was only adopted by the U.S. in 2015. While WHO has already released ICD-11, it may still be some time before it is adopted in the U.S., highlighting variable adoption timelines across countries.
What Does The Format of ICD-10 Mean?
ICD Code Letters Relating To Epilepsies
FOR EXAMPLE: G93.81
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G stands for Diseases of the nervous system
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93 stands for a subcategory for Other disorders of the brain.
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.81 add specificity to the diagnosis. In this case, .81 refers to Acute necrotizing encephalopathy
ADDITIONAL RESOURCES
ICD-10-CM, Centers for Disease Control and Prevention (CDC)
WHO Mortality Database, World Health Organization (WHO)
2.05: ICD-10-CM, MedicalBillingandCoding.org
Structure of an ICD-10-CM Code, National Cancer Institute (NCI)
Understanding ICD-10 Codes: Information for Consumers, AHIMA Foundation