Short answer · Medically reviewed summary · Last updated: 2026-05-08
Unverricht-Lundborg disease is classified under ICD-10 code G40.311 (Generalized idiopathic epilepsy and epileptic syndromes, intractable, with status epilepticus) and historically under ICD-9 code 345.11. As a form of Progressive Myoclonus Epilepsy type 1 (EPM1), accurate coding is essential for clinical documentation and insurance navigation. What exactly is Unverricht-Lundborg disease? Unverricht-Lundborg disease is a rare, autosomal recessive neurological disorder characterized by stimulus-sensitive myoclonus and tonic-clonic seizures.
ICD10 code of Unverricht-Lundborg Disease and ICD9 code
ICD-10 and ICD-9 codes for Unverricht-Lundborg Disease, with classification details for clinicians, coders and patients.
Unverricht-Lundborg disease is classified under ICD-10 code G40.311 (Generalized idiopathic epilepsy and epileptic syndromes, intractable, with status epilepticus) and historically under ICD-9 code 345.11. As a form of Progressive Myoclonus Epilepsy type 1 (EPM1), accurate coding is essential for clinical documentation and insurance navigation.
What exactly is Unverricht-Lundborg disease?
Unverricht-Lundborg disease is a rare, autosomal recessive neurological disorder characterized by stimulus-sensitive myoclonus and tonic-clonic seizures. It is the most common form of progressive myoclonus epilepsy, typically manifesting in children between the ages of 6 and 15. For the 19 community members on DiseaseMaps.org living with Unverricht-Lundborg disease, managing the progressive nature of the condition requires a multidisciplinary approach.
How is Unverricht-Lundborg disease diagnosed?
Diagnosis of Unverricht-Lundborg disease relies on a combination of clinical presentation, electroencephalogram (EEG) findings showing generalized spike-wave discharges, and molecular genetic testing. Physicians look for the hallmark expansion of a dodecamer repeat in the CSTB gene on chromosome 21q22.3. Because Unverricht-Lundborg disease symptoms often overlap with other epilepsies, genetic confirmation is the gold standard for diagnosis.
What are the primary clinical features?
The clinical progression of Unverricht-Lundborg disease varies, but common features include:
- Action-induced myoclonus (involuntary muscle jerks triggered by movement or stress).
- Generalized tonic-clonic seizures that may decrease in frequency over time.
- Ataxia and mild cognitive changes that may emerge as the condition progresses.
- Sensitivity to light and sound, which can exacerbate seizure activity.
Next steps
- Consult a specialized neurologist or epileptologist familiar with progressive myoclonus epilepsies.
- Engage with the DiseaseMaps.org community to share experiences and coping strategies with others managing Unverricht-Lundborg disease.
- Request a referral for genetic counseling to discuss the autosomal recessive inheritance pattern and family planning.
- Keep a detailed seizure and myoclonus diary to assist your medical team in optimizing medication dosages.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Unverricht-Lundborg disease profile.
- Orphanet: EPM1 (Unverricht-Lundborg disease) classification and clinical data.
- OMIM (Online Mendelian Inheritance in Man): Entry #254800 (EPM1).
- International League Against Epilepsy (ILAE): Clinical diagnostic criteria for progressive myoclonus epilepsies.
