Table 3.
Summary of published studies of adjunctive KDT for refractory and super-refractory status epilepticus in adults (age ≥ 18 years)
| First author (year) |
Subjects, n |
Etiology | KDT | Time to diet start (days) |
Time to ketosis (days) |
Time to SE response (days) |
Response % |
Outcome | AE |
|---|---|---|---|---|---|---|---|---|---|
| Bodenant (2008)87 | 1 | Epileptic encephalopathy, PNA | KD | 31 | NR | 7 | 100 | Death | 0 |
| Villeneuve (2009)63 | 1 | Ito syndrome | KD | 2–14 | NR | 3 | 100 | NR | NR |
| Wusthoff (2010)88 | 2 | Rasmussen encephalitis; viral encephalitis | KD | 20, 101 | 8, 10 | 6, 11 | 100 | Home by 1 year | 2 acidosis |
| Nam (2011)67 | 1 | Encephalitis | KD | 15 | NR | 7 | 100 | Functional baseline | 0 |
| Martikainen (2012)90 | 1 | POLG | LGIT | 4 | NR | 4 | 100 | Home | 0 |
| Strzelczyk (2013)91 | 1 | Lafora disease | KDa | 16 | 4 | 4 | 100 | Home | 0 |
| Thakur (2014)92 | 10b | 4 NORSE, 2 NMDA, 1 LGI1, 1 anoxia, 1 FCD, 1 neurocysticercosis | KD | 2–60 (median 22) | 1–7 | 1–31 (median 3) | 90 | 7 ARF, 1 SNF, 1 VRU, 1 death | 1 acidosis, 2 ↑TG |
| Matsuzono (2014)93 | 1 | Encephalitis | KD | 155 | NR | 25 | 100 | ARF | NR |
| Amer (2015)94 | 1 | NMDA | KD | 21 | NR | 14 | 100 | SNF | NR |
| Uchida (2017)95 | 1 | NMDA | KD + STP | NR | NR | 60 | 100 | NR | NR |
| Cervenka (2017)96 | 15 | 5 NORSE, 2 LGS, 3 ICH, 2 anoxia, 1 GBM, 1 encephalitis, 1 NAT | KD | 2–21 (median 10) | 0–16 | 0–10 (median 5) | 73 | 1 home, 8 ARF, 2 SNF, 4 death | 4 acidosis, 2 GI, 2 HLD, 2 hypoglycemia, 1 hyponatremia, 1 weight loss |
| Blunck (2018)97 | 1 | ASD change in epilepsy patient | KD + SGLT2 inhibitorc | 107 | 7 | NA | 0 | Death | 0 |
| Park (2019)85 | 1d | FIRES | KD | 37 | 2–6 | 7 | 0, 100e | Ambulatory | Nausea/vomiting |
| Francis (2019)98 | 11 | 3 TBI, 2 ICH, 2 anoxia, 1 stroke, 1 NMDA, 1 EtOH, 1 ASD nonadherence | KD | 0–3 (median 1) | 0–5 (median 1) | NR | 73 | 2 home, 4 LTACH, 2 ARF, 2 SNF, 1 CM | 7 acidosis, 2 hypoglycemia, 1 hyponatremia, 1 transaminitis |
| Prasoppokakorn (2019)99 | 1 | Autoimmune encephalitis | MCT-KDf | 56 | NA | 6 | 100 | NR | 0 |
Abbreviations: AE, adverse events deemed related to KD use; ASD, antiseizure drug; ARF, acute rehabilitation facility; CM, comfort measures; EtOH, alcohol withdrawal; FCD, focal cortical dysplasia; FIRES, febrile infection related epilepsy syndrome; GBM, glioblastoma multiforme; GI, gastrointestinal side effects (including constipation); HLD, hyperlipidemia; ICH, intracranial hemorrhage; KD, classic or modified ketogenic diet; LGI1, leucine-rich, glioma-inactivated 1 encephalitis; LGIT, low glycemic index treatment; LGS, Lennox–Gastaut syndrome; LTACH, long-term acute care hospital; MCT-KD, medium-chain triglyceride KD; NA, not achieved; NAT, remote nonaccidental trauma resulting in epilepsy; NMDA, N-methyl D-aspartate receptor encephalitis; NORSE, new-onset refractory status epilepticus of unknown etiology; NR, not reported; PNA, pneumonia; POLG, mitochondrial polymerase γ related epilepsy; response %, proportion of patients who had resolution of SE; SE, status epilepticus; SNF, skilled nursing facility; STP, stiripentol; TBI, traumatic brain injury; TG, triglycerides; VRU, ventilatory rehabilitation unit.
The patient received a parenteral 4:1 ketogenic diet treatment for 12 days, then switched to an enteral preparation administered via a gastrostomy tube.
Includes one patient previously reported in Cervenka et al (2011)89 which has been omitted from this table to avoid redundancy.
The patient initially received a KD for 16 days without achieving ketosis so an SGLT2 inhibitor was added with consistent ketosis achieved 7 days later.
Excludes one patient previously reported in Nam et al (2011).67
Proportion of patients with ≥50% reduction in seizures.
The patient initially received a KD but was switched to MCT-KD due to persistently elevated TGs after 2 weeks.