Efficacy of the Ketogenic Diet for Infantile Spasms

Kossoff, Eric H.; Pyzik, Paula L.; McGrogan, Jane R.; Vining, Eileen P.G.; Freeman, John M.

doi:10.1542/peds.109.5.780

Cited by 210 publications

(145 citation statements)

References 25 publications

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“…Class 4 evidence supports the role of the ketogenic diet for infants younger than 1 year of age, and class 3 evidence for children aged 12-24 months. [12][13][14][15][16][17][18][19][20] There is evidence that ketosis can be sustained in infants.…”

Section: Approach To Therapy and Interventions In Infantile-onset Seimentioning

confidence: 99%

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

et al. 2015

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Summary Evidence‐based guidelines, or recommendations, for the management of infants with seizures are lacking. A Task Force of the Commission of Pediatrics developed a consensus document addressing diagnostic markers, management interventions, and outcome measures for infants with seizures. Levels of evidence to support recommendations and statements were assessed using the American Academy of Neurology Guidelines and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The report contains recommendations for different levels of care, noting which would be regarded as standard care, compared to optimal care, or “state of the art” interventions. The incidence of epilepsy in the infantile period is the highest of all age groups (strong evidence), with epileptic spasms the largest single subgroup and, in the first 2 years of life, febrile seizures are the most commonly occurring seizures. Acute intervention at the time of a febrile seizure does not alter the risk for subsequent epilepsy (class 1 evidence). The use of antipyretic agents does not alter the recurrence rate (class 1 evidence), and there is no evidence to support initiation of regular antiepileptic drugs for simple febrile seizures (class 1 evidence). Infants with abnormal movements whose routine electroencephalography (EEG) study is not diagnostic, would benefit from video‐EEG analysis, or home video to capture events (expert opinion, level U recommendation). Neuroimaging is recommended at all levels of care for infants presenting with epilepsy, with magnetic resonance imaging (MRI) recommended as the standard investigation at tertiary level (level A recommendation). Genetic screening should not be undertaken at primary or secondary level care (expert opinion). Standard care should permit genetic counseling by trained personal at all levels of care (expert opinion). Genetic evaluation for Dravet syndrome, and other infantile‐onset epileptic encephalopathies, should be available in tertiary care (weak evidence, level C recommendation). Patients should be referred from primary or secondary to tertiary level care after failure of one antiepileptic drug (standard care) and optimal care equates to referral of all infants after presentation with a seizure (expert opinion, level U evidence). Infants with recurrent seizures warrant urgent assessment for initiation of antiepileptic drugs (expert opinion, level U recommendation). Infantile encephalopathies should have rapid introduction and increment of antiepileptic drug dosage (expert opinion, level U recommendation). There is no high level evidence to support any particular current agents for use in infants with seizures. For focal seizures, levetiracetam is effective (strong evidence); for generalized seizures, weak evidence supports levetiracetam, valproate, lamotrigine, topiramate, and clobazam; for Dravet syndrome, strong evidence supports that stiripentol is effective (in combination with valproate and clobazam), whereas weak evidence supports that topiramate, zonisamide,...

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Section: Approach To Therapy and Interventions In Infantile-onset Seimentioning

confidence: 99%

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

et al. 2015

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“…Response to the diet was maintained for at least 6 months in seven of the 10 responders and for at least 1 year in two patients. In two patients with cryptogenic epilepsy, the ketogenic diet was stopped after 6 and 11 months of seizure freedom (7,11), but the epilepsy subsequently relapsed after 2 and 9 months respectively. The reintroduction of the diet controlled seizures again for one of them.…”

Section: Results (mentioning

confidence: 99%

“…1,2 Many investigators have confirmed the benefits that this treatment can offer to young patients with epilepsy, [3][4][5][6] including infants. 7,8 However, the best indications for using the diet have not been clearly defined since it was shown to be helpful in treating a large range of different seizure types and epilepsy syndromes. Special interest has been given to various conditions associated with epilepsy including myoclonic astatic epilepsy, 5,9 infantile spasms, 7 Dravet syndrome, 10 atypical absences, 11 acquired epileptic aphasia, 12 tuberous sclerosis, 13 and Rett syndrome.…”

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confidence: 99%

The ketogenic diet improves recently worsened focal epilepsy

Villeneuve

Pinton

Bahi‐Buisson

et al. 2009

Develop Med Child Neuro

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Aim We observed a dramatic response to the ketogenic diet in several patients with highly refractory epilepsy whose seizure frequency had recently worsened. This study aimed to identify whether this characteristic was a useful indication for the ketogenic diet. Method From the 70 patients who received the ketogenic diet during a 3‐year period at our institution, we retrospectively selected patients with focal epilepsy. There were 22 children, 13 females and nine males, aged from 5 months to 18 years 6 months (mean 6y 9mo, SD 5y 11mo). Fifteen had symptomatic and seven had cryptogenic focal epilepsy. Seizure frequency 1 week before initiating the ketogenic diet was compared with that at 1 month and at the last visit on the diet. Results Eleven patients were responders (defined as reduction of seizures by more than 50%) at 1 month. Responders were higher (p=0.046) in the group with a recent worsening of seizures than in those with stable seizure frequency. Seven patients were still seizure‐free at 6 months on the diet. Tolerability was excellent in 10 patients. Five patients stopped the diet because of early side effects. Interpretation The ketogenic diet may be a valuable therapeutic option for children with pharmacoresistant focal epilepsy, particularly those with a recent deterioration of seizure control and neurological status. Because of its rapid effect, the ketogenic diet may be a useful support to intravenous emergency drugs in such a situation.

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“…10,17 Treatment of infantile spasms with steroids, different AEDs, or ketogenic diet is rarely effective. [18][19][20][21] Even though therapeutic procedures in congenital CMV infection have yet to be established, 22,23 using an antiviral regimen in epilepsy caused by congenital HCMV infection was considered. Only one case report from Greece concerning unsuccessful treatment with GCV of a patient with infantile spasms caused by HCMV infection has been published so far.…”

Section: Discussionmentioning

confidence: 99%

Infantile spasms and cytomegalovirus infection: antiviral and antiepileptic treatment

Dunin-Wąsowicz

Kasprzyk-Obara

Jurkiewicz

et al. 2007

Develop Med Child Neuro

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From 1 January 1995 to 31 December 2004, 22 patients (13 males, nine females; age range 2‐12mo) with infantile spasms and cytomegalovirus (CMV) infection were treated with intravenous ganciclovir (GCV) and antiepileptic drugs. GCV was given for 3 to 12 weeks with a 1‐month interval (one, two, or three courses). Epileptic spasms occurred before (group A: eight patients), simultaneously (group B: eight patients), and after (group C: six patients) a diagnosis of human CMV (HCMV) infection and antiviral treatment. In 11 patients, DNA HCMV was found in cerebrospinal fluid by nested‐polymerase chain reaction method (neuroinfection). All infants excreted CMV in urine. DNA HCMV and specific immunoglobulin M and immunoglobulin G antibodies were present in blood. Ten patients, including four with neuroinfection, have been seizure‐free for at least the past 18 months. In two patients with neuroinfection, vigabatrin monotherapy was withdrawn after a 2 year 6 month seizure‐free period. Eighteen patients required antiepileptic drugs polytherapy, four of whom required additional adrenocorticotropic hormone (ACTH). Six patients on polytherapy were seizure‐free on follow‐up, two of whom were treated with ACTH, but no patient with hypsarrhythmia who required ACTH treatment was seizure‐free on follow‐up. In five patients, psychomotor development was normal, 16 had tetraplegia (Gross Motor Function Classification System [GMFCS] Level V), and one had diplegia (GMFCS Level III). Early antiviral and antiepileptic therapy could result in the long‐term cessation of seizures.

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Efficacy of the Ketogenic Diet for Infantile Spasms

Abstract: The ketogenic diet is a safe, well-tolerated, and possibly effective potential alternative to other therapies for infantile spasms.

Cited by 210 publications

References 25 publications

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

The ketogenic diet improves recently worsened focal epilepsy

Infantile spasms and cytomegalovirus infection: antiviral and antiepileptic treatment

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