Indications and efficacy

The Ketogenic Diet (KD) should be considered for children with intractable epilepsy, typically when seizure control is inadequate despite two or more anti-epileptic medications, although it may be indicated earlier in certain types of epilepsies. The KD is also used as a key treatment in some neuro-metabolic conditions. 

Patients should be referred to a Neurologist or Metabolic Physician to initiate the Ketogenic Diet. The specialist team will conduct a full medical review, undertake necessary pre-initiation investigations, and provide ongoing management and review while the patient remains on the KD. Please review the contraindications for commencing on the KD in the next section before considering referral.

International consensus guidelines indicate benefit of the KD in the following Epilepsy Syndromes and conditions1:

Consistent evidence of benefit (>70% seizure reduction in at least three publications from at least two KD centres):

  • Angelman syndrome
  • Complex 1 mitochondrial disorders
  • Dravet syndrome
  • Epilepsy with myoclonicatonic seizures (Doose syndrome)
  • Glucose transporter protein 1 (Glut-1) deficiency syndrome
  • Febrile infection-related epilepsy syndrome (FIRES)
  • Infantile spasms
  • Ohtahara syndrome
  • Pyruvate dehydrogenase deficiency (PDHD)
  • Super-refractory status epilepticus
  • Tuberous sclerosis complex

Moderate evidence of benefit (50% seizure reduction or limited single centre evidence1):

  • Adenylosuccinate lyase deficiency
  • CDKL5 encephalopathy
  • Childhood absence epilepsy
  • Cortical malformations
  • Epilepsy of infancy with migrating focal seizures
  • Epileptic encephalopathy with continuous spike-and-wave during sleep
  • Glycogenosis type V
  • Juvenile myoclonic epilepsy
  • Lafora body disease
  • Landau-Kleffner syndrome
  • Lennox-Gastaut syndrome
  • Phosphofructokinase deficiency
  • Rett syndrome
  • Subacute sclerosing panencephalitis (SSPE)

Overall Efficacy of the Ketogenic Diet :

The efficacy of the KD depends upon the underlying condition - average efficacies are listed below:

  • Up to 10% of the patients may become seizure-free.2
  • Reduces seizure frequency by more than 50% in half of all patients who try it.3
  • Reduces seizure frequency by more than 90% in one-third of patients.4

 

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Information last reviewed: 5/02/2021.
  • 1. Kossoff et al. (2018). Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open, 3(2):175–192.
  • 2. Wibisono et al. (2015). Ten-Year Single-Center Experience of the Ketogenic Diet: Factors Influencing Efficacy, Tolerability, and Compliance. J Paediatr (in press). Henderson et al. (2006). Efficacy of the Ketogenic Diet as a treatment option for epilepsy: meta-analysis. J child Neurol 21:193-198.
  • 3. Kossof & Wang (2013). Dietary Therapies for Epilepsy. Biomed J 36(1): 2-8. Kossof et al. (2009). Ketogenic Diets: an update for child neurologists. J Child Neurol 24(8): 979-988.
  • 4. Henderson et al. (2006). Efficacy of the Ketogenic Diet as a treatment option for epilepsy: meta-analysis. J child Neurol 21:193-198.