July 25, 2019 e-Pearl of the Week: Late-onset myoclonic epilepsy in Down syndrome (LOMEDS)

Late-onset myoclonic epilepsy in Down syndrome (LOMEDS)

Late-onset myoclonic epilepsy in Down syndrome (LOMEDS) is a progressive epilepsy characterized by development of myoclonus, tonic/clonic seizures, and gradual cognitive and functional decline in patients with Down syndrome (DS) over age 40¹.  Prevalence reaches 46% in patients over age 50². Accumulation of beta-amyloid plaques contributes to the development of myoclonic epilepsy in both DS with Alzheimer Disease (AD) and the general AD population². Differential diagnosis includes Lance-Adams syndrome and Unverrich-Lundborg disease.  EEG findings may show generalized spike-wave, polyspike-wave, or diffused slowing¹. Treatment suggestions are levetiracetam and valproate¹˒².

References

1. J. Möller, H. Hamer, W. Oertel, F. Rosenow.  Late-myoclonic epilepsy in Down’s syndrome. Seizure 2001; 10: 303-305. doi:10.1053/seiz.2000.0500

2. R. Simone, X. Puig, P. Géllisse, A.Grespel, P.Genton. Senile myoclonic epilepsy: Delineation of a common condition associated with Alzheimer’s disease in Down’s syndrome. Seizure 2010; 19: 3 83-389. doi:10.1016/j.seizure.2010.04.008

Submitted by Angela Welty, MSN, RN, ACNP-BC, Acute Care Nurse Practitioner, Sugar Land Neurology and Sleep, Texas, and Faisal Khan, MD, D, ABSM, DABPN, Consultant Neurologist, Sugar Land Neurology and Sleep, Texas.

Angela Welty and Dr. Khan report no disclosures.