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Rare Epilepsies & ICD Codes

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About ICD Codes | ICD-10 Search Tools | Challenges | Additional Resources

About 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.

ICD codes may help:

  • accurately determine incidence, prevalence, comorbidity profile, and mortality

  • determine the impact of DEEs on the healthcare system

  • determine the best care for each patient

  • improve communication amongst healthcare providers

  • may decrease time to diagnosis

  • identify populations that may benefit from precision therapies

  • identify populations for care management and population health initiatives

  • identify patients for enrollment in clinical trials. 

ICD Code Search Tool

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

Rare Epilepsies with ICD Codes

Challenges with Obtaining an ICD Code

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.

GRIA1

GRIA2

GRIA3

GRIA4

GRIK2

GRIN1

GRIN2A

GRIN2B

SCN2A

SCN8A

SLC6A1

STXBP1

FOXG1

CACNA1A

CTNNB1

USP7

KABUKI SYNDROME

(KMT2D; KDM6A)

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

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