Table 5.

Recommendations for Screening, Assessment, and Management of Seizures in Children With a Leukodystrophy.

	Presymptomatic	Early Symptomatic	Intermediate Symptomatic	Advanced Symptomatic
Screening	Review seizure types Anticipatory guidance Seizure “first aid” Address parental anxiety relating to seizure presentations	Anticipatory guidance relating to expected changes in seizure types/presentationsFirst seizure Description of seizure event Review of possible/subtle seizure presentations to determine true incidence Recurrent seizures / epilepsy Determine seizure types, frequency, and syndromes
Assessment		Consider common etiologies: Seizures with fever Infection Recent medication change Staring spells Electroencephalographs (EEGs) only when there is reasonable clinical suspicion for a seizure When considering repeat brain imaging, consider need for sedation and weigh risk against possible insights to be gained Seizure syndrome (or change in semiology) may dictate management more than the specific leukodystrophy
		First seizure EEG (consider sleep-deprived EEG if child tolerates) Recurrent seizures / epilepsy Prolonged EEG Antiseizure drug levels
Management		Seizure syndrome (or change in semiology) may dictate management more than the specific leukodystrophy Avoid polypharmacy when seizures are not intractable Consider the use of clonazepam or lorazepam for “bridges” only in the setting of illness, to avoid long-term/chronic drug use Consider adding gabapentin when sedative effects are preferable Weigh risks and benefits of potentially invasive interventions First seizure Prevent triggers Consider antiseizure medications Recurrent seizures / epilepsy Prevent triggers Consider antiseizure medications Consider ketogenic diet Intractable seizures / epilepsy Prevent triggers Consider antiseizure medications Consider ketogenic diet Consider surgical interventions (eg, vagus nerve stimulation)