Late-onset myoclonic epilepsy in Down syndrome
DS–associated AD spectrum
Epidemiology
-Age→ 40-50 yo
-Dementia→ AD (overlay path)
*Can occur w/o AD
Clinical Manifestations
-Szs types→ MCL & GTCs
-Three stage by Orsi (aka PME-like course)
1st) EEG abnormal during sleep
2nd) MCL on awake
3rd) Significant burden
DDx
-R/o metabolic causes and DIM
TTx
-DOC is LVT; otherwise, VPA
>If RFx, consider add-on PMP
>Prescribe, BZD rescue
-Avoid Na+ blockers
-OSA mgmt
Prognosis
-↑Mortality & ↑Cognitive decline
