From the Guidelines
The ketogenic diet may be a valuable treatment option for patients with New-Onset Refractory Status Epilepticus (NORSE), but its effectiveness is not clearly established in the provided evidence. When considering the management of NORSE, it is essential to note that the provided study 1 does not directly address the relationship between a ketogenic diet and NORSE. However, in the context of refractory status epilepticus, the study highlights the challenges in making definitive recommendations due to a lack of prospective controlled randomized trials. Some key points to consider in the management of NORSE include:
- The importance of early intervention with effective treatments to prevent long-term neurological damage
- The potential role of newer agents, such as levetiracetam and propofol, in refractory status epilepticus, as mentioned in the study 1
- The need for careful consideration of the potential benefits and risks of any treatment, including dietary therapies like the ketogenic diet Given the lack of direct evidence on the ketogenic diet's effectiveness in NORSE, a conservative approach would be to consider the ketogenic diet as a potential treatment option for NORSE patients who have failed conventional antiepileptic medications, under strict neurological and nutritional supervision. It is crucial to weigh the potential benefits of the ketogenic diet against its potential risks and to carefully monitor patients for any adverse effects. In real-life clinical practice, the decision to initiate a ketogenic diet in NORSE patients should be made on a case-by-case basis, taking into account the individual patient's circumstances and the availability of other treatment options.
From the Research
Relationship Between Ketogenic Diet and New-Onset Refractory Status Epilepticus (NORSE)
- The ketogenic diet has been shown to be a feasible and effective treatment option for adults with refractory and super-refractory status epilepticus, including those with NORSE 2, 3, 4.
- A case study described a 29-year-old woman with cryptogenic NORSE who was treated with a ketogenic diet, which was challenging to maintain in the intensive care unit due to inconspicuous carbohydrates in commonly administered medications and poor nutritional state 2.
- The ketogenic diet was restarted in a non-ICU unit, facilitating antiseizure drug tapering without re-emergence of status epilepticus 2.
- Practical considerations for implementing ketogenic dietary therapy in adults with super-refractory status epilepticus include dietary composition, potential drug-diet interactions, and monitoring during ketogenic treatment 3.
- The control of super-refractory status epilepticus is critical in reducing morbidity and mortality, and there is emerging evidence that the ketogenic diet may be a safe and effective treatment option for these patients 3, 4.
- In patients with NORSE, the ketogenic diet should be started within seven days if there is no improvement with first-line immunotherapy, according to published consensus recommendations 5.
Treatment Approaches for NORSE
- Management of seizures in the initial 24-48 hours should be like any case of refractory status epilepticus, with first-line immunotherapy starting within 72 hours using steroids, intravenous immunoglobulins, or plasmapheresis 5.
- If there is no improvement, the ketogenic diet and second-line immunotherapy should start within seven days, with rituximab recommended as the second-line treatment if there is a strong suggestion or proof of an antibody-mediated disease 5.
- Other treatment options, such as tocilizumab, may also be considered, especially in cases with possible neuroinflammation 6.