Treating epilepsy with options other than antiepileptic medications

Article Figures & Data

Table 1

Treatable vitamin-responsive epilepsy.

Indication	Dose
Pyridoxine (B6)-dependent epilepsy	B6 15-30 mg/kg/d BID (up to 500 mg/day) or (50-100) mg IV with simultaneous EEG recording.
Pyridoxal 5’-phosphate-dependent epilepsy	Pyridoxal 5’-phosphate (30–50 mg/kg TID)
Folinic acid-responsive seizures	Folinic acid (3–5 mg/kg/dose)
Biotinidase deficiency	Biotin (10 - 20 mg/day)
Biotin-thiamine-responsive basal ganglia disease	Biotin (5–10 mg/kg/dose) and thiamine (300–900 mg/day)
Cerebral creatine deficiency syndromes	GAMT deficiency: Oral creatine monohydrate (dosage of 400-800 mg/kg/day TID or more) Ornithine: 100 mg/kg/day AGAT deficiency: Oral creatine monohydrate (dosage of 400-800 mg/kg/day TID or more) CRTR deficiency: Creatine monohydrate 100-200 mg/kg/day TID Arginine 400 mg/kg/day TID Glycine 150 mg/kg/day TID
GAMT - guanidinoacetate methyltransferase, AGAT - L-arginine:glycine amidinotransferase deficiency, CRTR - creatine transporter deficiency

Table 2

Mitochondrial cocktail supplements

Medication	Dose
Riboflavin (B2)	50–400 mg po OD in children and 50–400 mg po OD in adults
Ubiquinone (coenzyme Q10)	2–8 mg/kg po daily BID in children and 50–600 mg po OD in adults
L-Creatine	0.1 g/kg po OD; maximum 10 g/d in children and 5 g po OD or BID in adults
L-Arginine	150–300 mg/kg po BID to TID in children and adults
L-Carnitine	10–100 mg/kg daily po TID in children and 100–1000 mg per dose po BID to TID in adults
Vitamin E	1–2 IU/kg po daily in children and 100–200 IU po daily in adults
Vitamin C	5 mg/kg po daily in children and 50–200 mg po daily in adults
Alpha-lipoic acid	50–200 mg per day

Table 3

Review of Non-pharmacological options.

Conditions	Treatments	Dose/notes
Neuronal ceroid-lipofuscinoses type II	Cerliponase alfa	3 years or older: 300 mg by intraventricular infusion once every other week
Menkes disease	Copper histidine or copper chloride	Cooper histidine subcutaneous: Day 1: 50 µg/d Day 2: 100 µg/d Day 3 onward: 150 µg/d Copper chloride subcutaneous: Age <1 year: 250 µg 2x/day Age >1 year: 250 µg 1x/day
Tuberous sclerosis complex	Everolimus	5 mg/m² PO qd
Epileptic encephalopathies like CSWS, LKS, Doose, Rassmusen.. etc	Intravenous immunoglobulins	1- or 5-day course of 2 g/kg intravenous
Steroids	Oral prednisolone (up to 5 mg/kg/d) over 2–4 weeks. Methylprednisolone IV: 20–30 mg/kg/d for 3–5 days
Epileptic encephalopathies like Dravet syndrome and Lennox–Gastaut syndrome	Cannabidiol	5-25 mg/kg/d oral QD
Dravet syndrome	Fenfluramine	10 to 20 mg daily
Catamenial epilepsy, CSWS, LKS	Acetazolamide	10 to 15 mg/kg/d divided BID
Focal lesion like DNET, FCD, glioma	Focal lesionectomy, lobectomy or laser ablation
Mesial temporal sclerosis	Temporal lobectomy
Hemimegalencephaly or extensive unilateral cortical dysplasia, Rasmussen encephalitis, Sturge–Weber syndrome	Hemispherectomy
Lennox Gastatut syndrome	Corpus callosotomy
LKS	Multiple subpial transections
CSWS - continuous spike and wave during sleep, LKS - Landau Kleffner syndrome, DNET - Dysembryoplastic neuroepithelial tumors, FCD - focal cortical dysplasia