Table 1.
Patient characteristics and outcome
| ID/Sex | Age (y) | On KD before | Duration ketogenic PN (d) | AED at trial start | Etiology | Epilepsy syndrome | Indication for PN | Ketosis >2 mmol/L | Long‐term outcome response at 3 mo |
|---|---|---|---|---|---|---|---|---|---|
| 01/M | 0.03 | No | 4 | Ca‐folic acid, vitamin B6 | Unknown | Ohtahara syndrome | Gradual enteral feeding | No | No response |
| 02/F | 0.15 | No | 3 | Ca‐folic acid, vitamin B6 | Unknown | Ohtahara syndrome | Gradual enteral feeding | Yes | 50% seizure reduction |
| 03/M | 1.79 | 1 y | 2 | LEV, TPM | Perinatal asphyxia | West syndrome | Surgery (PEG implantation) | Yes | 82% seizure reduction |
| 04/F | 3.27 | 16 wk | 10 | CLB, TPM | Alpers disease | Convulsive status epilepticus | Ventilation | No | No response |
| 05/M | 6.49 | 2 y | 4 | TPM | Prematurity, IVH | Lennox‐Gastaut syndrome | Illness, vomiting | No | 56% seizure reduction |
| 06/M | 0.72 | 25 wk | 3 | TPM, LEV | Sturge‐Weber syndrome | Focal epilepsy | Epilepsy surgery | Yes | Seizure free |
| 07/M | 3.35 | No | 1 | TPM | Unknown | Lennox‐Gastaut syndrome | Food refusal at start | Yes | 86% seizure reduction |
| 08/M | 10.80 | No | 41 | LEV, MDZ | Mitochondriopathy (POLG mutation) | Convulsive status epilepticus | Ventilation | No | 10% seizure reduction |
| 09/M | 0.46 | 2 wk | 19 | LEV, TPM | Unknown | Partial migrating seizures | Prolonged generalized seizures | Yes | No response |
| 10/M | 4.16 | 2.5 y | 2 | TPM, LEV, CLB | Sturge‐Weber syndrome | Focal epilepsy | Epilepsy surgery | No | 50% seizure reduction |
| 11/M | 0.78 | 16 wk | 2 | VGB | Gaba‐transaminase deficiency | West syndrome | Surgery (PEG implantation) | No | 90% seizure reduction |
| 12/F | 1.98 | 1.5 y | 4 | PGB, VitB6, TPM | PDHC‐deficiency | West syndrome | Illness, vomiting | Yes | 75% seizure reduction |
| 13/M | 3.95 | 3.5 y | 2 | TPM, LEV, VBG | Unknown | West syndrome | Surgery (PEG implantation) | Yes | 90% seizure reduction |
| 14/M | 1.84 | 1 y | 3 | TPM, LEV, NZP | Unknown | West syndrome | Surgery (PEG implantation) | Yes | Seizure free |
| 15/M | 1.58 | 7 mo | 5 | LEV, VGB | Prematurity, HIE | West syndrome | Epilepsy surgery | Yes | Seizure free |
| 16/F | 1.97 | 8 mo | 2 | LEV | FCD | West syndrome | Epilepsy surgery | No | No response |
| 17/F | 0.15 | 1 mo | 1 | FBM, LEV | Vanishing white matter disease | Ohtahara syndrome | Surgery (PEG implantation) | Yes | 80% seizure reduction |
AED, antiepileptic drugs; CBZ, carbamazepine; F, female; FCD, focal cortical dysplasia; HIE, hypoxic ischemic encephalopathy; ID, identification; IVH, intraventricular hemorrhage; KD, ketogenic diet; LEV, levetiracetam; M, male; mo, months; NZP, nitrazepam; PB, phenobarbital; PDHC, pyruvate dehydrogenase complex; PEG, percutaneous endoscopic gastrostomy implantation; PN, parenteral nutrition; TPM, topiramate; VGB, vigabatrin; vit B6, vitamin B6; VPA, valproic acid; wk, weeks; y, years; ZS, zonisamide.