Table 2. Initial ACTH and topiramate treatment regimens, outcomes and clinical diagnosis.
HIE-hypoxic ischemic encephalopathy, HLHS-hypoplastic left heart syndrome, ARX-mutation in the Aristaless related homeobox gene, PVL-periventricular leukomalacia, CMV-cytomegalovirus, CDKL5-mutation in cyclin dependant kinase like 5 gene, IVH-intraventricular hemorrhage
| Initial Treatment | Highest Dose | Spasms duration prior to treatment (months) |
Time to Remission (months) |
Relapse (months) |
Primary Diagnosis |
|---|---|---|---|---|---|
| ACTH | 160U/M2/day | <1 | Cryptogenic | ||
| ACTH | missing | 1 | 0 | 2 | Rett syndrome |
| ACTH | 140U/M2/day | 1 | Aicardi syndrome | ||
| ACTH | 88U/M2day | 3 | Hermansky-Pudlak, HIE | ||
| ACTH | 170U/M2/day | 0.5 | 0 | Polymicrogyria | |
| ACTH | 150U/M2/day | <1 | 0 | 2.5 | Cryptogenic |
| ACTH | 180U/M2/day | <1 | 0 | Cystic brain lesions | |
| ACTH | 110U/M2/day | unknown | 0 | Neonatal stroke, HLHS | |
| ACTH | 150U/M2/day | <1 | 0 | 6 | Hydrocephalus |
| ACTH | High dose | 2 | ARX mutation | ||
| ACTH | 130U/M2/day | 1 | Cryptogenic | ||
| ACTH | 180U/M2/day | 1.5 | Hypotonia, developmental delay | ||
| Topirimate | 8.1 mg/kg/day | <1 | Hemimegencephally | ||
| Topirimate | 31 mg/kg/day | 3 | PVL | ||
| Topirimate | 20-30 mg/kg/day | <1 | Non-accidental trauma | ||
| Topirimate | 21.8 mg/kg/day | 2 | Congenital CMV | ||
| Topirimate | 7.2 mg/kg/day | <1 | Immediate Improvement; Multiple Relapses Resolution 69 |
HIE | |
| Topirimate | 14.3 mg/kg/day | <1 | CDKL5 mutation | ||
| Topirimate | 22.5 mg/day | >12 | Prenatal strokes | ||
| Topirimate | 35.1 mg/kg/day | <1 | Immediate Improvement Multiple Relapses |
Hypotonia, developmental delay | |
| Topirimate | 15.9 mg/kg/day | <1 | HIE, IVH | ||
| Topirimate | 5.6 mg/kg/day | <1 | Cryptogenic | ||
| Topirimate | 6.3 mg/kg/day | <1 | Pilocytic astrocytoma | ||
| Topirimate | 11.5 mg/kg/day | 1 | HIE | ||
| Topirimate | 18 mg/kg/day | <1 | Hypotonia, developmental delay | ||
| Topirimate | 1.3 mg/kg/day | 2 | 0 | Cryptogenic | |
| Topirimate | 3.4 mg/kg/day | <1 | 1 | Developmental delay, abnormal white matter |
|
| Topirimate | 9.3 mg/kg/day | 2 | Cryptogenic | ||
| Topirimate | 14.3 mg/kg/day | <1 | 8 | Premature/meningitis/PVL | |
| Topirimate | 22.5 mg/kg/day | <1 | Lisencephally | ||
| Topirimate | 10 mg/kg/day | Hypotonia, developmental delay | |||
| Topirimate | 20 mg/kg/day | <1 | Aicardi syndrome |