Child neurologists and epileptologists at various university centers in Japan were surveyed by questionnaire to identify the most effective strategies for management of and prophylaxis against status epilepticus (SE) in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome), especially when associated with fever. Data from 109 patients were analyzed (51 males, 58 females; mean age 10.7 years +/- 6.53; range 1-37 years). Ten had no SE and were excluded. Anticonvulsants with excellent efficacy against SE occurrence were potassium bromide (41.7%), zonisamide (13.5%), clobazam (10%), valproate (8%), phenobarbital (6.7%), and phenytoin (2.6%). Clonazepam and carbamazepine were ineffective. Diazepam suppository was most frequently used against SE triggered by fever, but was effective in only 2.4% cases. Intravenous medications most effective in terminating ongoing SE were barbiturates (75-100%), midazolam (68.8%), diazepam (54.3%), lidocaine (21.4%), and phenytoin (15.4%). [1]

COMMENT. The use of bromides for treatment of SMEI and their significantly higher efficacy than that of valproic acid and zonisamide are interesting and surprising observations. Bromides were first introduced for the treatment of epilepsy in 1853 [2]. After phenobarbital became available in 1912 and phenytoin in 1937, the use of bromides was largely discontinued. The administration of bromides is not as simple as that of newer anticonvulsant drugs. Its effectiveness depends on a lowered intake of sodium chloride in the diet. The onset of action is delayed for 2 to 3 weeks, and high blood levels are maintained for 1 to 2 weeks after bromides are discontinued. Unlike other anticonvulsants, an abrupt withdrawal of bromides is unlikely to precipitate status epilepticus.

Bromides are usually administered in liquid or tablet forms of sodium bromide or as triple bromide elixir, containing 400 mg each of sodium, potassium and ammonium bromide per 5ml [3, 4]. Suggested starting and maintenance doses of bromides are as follows: For children under 3 years old, 160 mg 2x daily (maximum 320 mg 3x daily); 3 to 6 years old, 320 mg 2x daily (maximum 640 mg 3x daily). [5]. A satisfactory blood level of bromides is generally 20 to 25 mEq/L (160 to 200 mg%). Drowsiness and cutaneous reactions are the most troublesome side effects. Administration of extra sodium chloride and fluids usually alleviates drowsiness. Acneiform rashes are a frequent occurrence in adolescents and adults, but are uncommon in infants and young children. Granulomatous lesions (bromoderma) will occur occasionally, taking months to disappear after bromide withdrawal.

Epileptic syndromes with high rates of status epilepticus, in addition to SMEI, include Panayiotopoulos syndrome and symptomatic occipital lobe epilepsy secondary to neonatal hypoglycemia. [6]