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 |