Table 4.

General and specific recommendations on diagnosis, treatment and follow-up of subjects with epilepsy, LOMEDS or not. Recomendations based on clinical practice experience. (AED: antiepileptic drugs; LEV: levetiracetam; VPA: valproic acid; OSA: obstructive sleep apnea).

Epilepsy in DS: General Recommendations

Specific Recommendations in LOMEDS

❑Structured anamnesis with patient and caregivers with neurological exam. ❑Check for potential triggers: Metabolic disturbances, infections, sleep deprivation, medication failure, brain traumatic injury, e.g., ❑Video-EEG as soon as possible after episode or relevant changes in seizure semiology. ❑Request home videos of episodes. ❑Chronic treatment with AED based on risk of seizure recurrence. ❑Unless clearly established focal origin of seizures use broad-spectrum AED. ❑Monotherapy is preferred. ❑Monitoring seizure frequency, treatment compliance, tolerance and possible interactions. ○Blood test monitoring if required.

❑Myoclonus not always have an epileptic etiology (e.g., metabolic origin). ○Attention to both underdiagnosis and overdiagnosis. ❑Use broad spectrum drugs (first choice: LEV, alternative: VPA). ○Monotherapy is preferred. ○Start low and go slow. ○Avoid sodium channel blockers and gabaergic antiepileptic drugs (worsening of myoclonic seizures). ❑Epilepsy is not a criterion either for withdrawing or not initiating treatment with anticholinesterase drugs. ❑Evaluate the coexistence of associated sleep pathology (mainly OSA), which can aggravate seizure control.