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Eur J Pediatr. 2016 Oct;175(10):1267-76. doi: 10.1007/s00431-016-2765-z. Epub 2016 Sep 1.

The changing face of dietary therapy for epilepsy.

Author information

1
Department of Child Neurology and Psychiatry, IRCCS "C. Mondino" National Neurological Institute, Pavia, Italy.
2
Brain and Behaviour Department, University of Pavia, Via Mondino, 2, 27100, Pavia, Italy.
3
Makati Medical Center, Manila, Philippines.
4
Human nutrition and eating disorder center, department of public health, experimental and forensic medicine, University of Pavia, Pavia, Italy.
5
Department of Child Neurology and Psychiatry, IRCCS "C. Mondino" National Neurological Institute, Pavia, Italy. pierangelo.veggiotti@unipv.it.
6
Brain and Behaviour Department, University of Pavia, Via Mondino, 2, 27100, Pavia, Italy. pierangelo.veggiotti@unipv.it.

Abstract

Ketogenic diet is an established and effective non-pharmacologic treatment for drug-resistant epilepsy. Ketogenic diet represents the treatment of choice for GLUT-1 deficiency syndrome and pyruvate dehydrogenase complex deficiency. Infantile spasms, Dravet syndrome and myoclonic-astatic epilepsy are epilepsy syndromes for which ketogenic diet should be considered early in the therapeutic pathway. Recently, clinical indications for ketogenic diet have been increasing, as there is emerging evidence regarding safety and effectiveness. Specifically, ketogenic diet response has been investigated in refractory status epilepticus and encephalopathy with status epilepticus during sleep. New targets in neuropharmacology, such as mitochondrial permeability transition, are being studied and might lead to using it effectively in other neurological diseases. But, inefficient connectivity and impaired ketogenic diet proposal limit ideal availability of this therapeutic option. Ketogenic diet in Italy is not yet considered as standard of care, not even as a therapeutic option for many child neurologists and epileptologists.

CONCLUSIONS:

The aim of this review is to revisit ketogenic diet effectiveness and safety in order to highlight its importance in drug-resistant epilepsy and other neurological disorders.

WHAT IS KNOWN:

• Ketogenic diet efficacy is now described in large case series, with adequate diet compliance and side effects control. • Ketogenic diet is far from being attempted as a first line therapy. Its availability varies worldwide. What is New: • New pharmacological targets such as mitochondrial permeability transition and new epileptic syndromes and etiologies responding to the diet such as refractory status epilepticus are being pointed out. • Ketogenic diet can function at its best when used as a tailor-made therapy. Fine tuning is crucial.

KEYWORDS:

Dietary therapy; Drug-resistant epilepsy; GLUT1DS ketones; Ketogenic diet; Modified Atkins diet

PMID:
27586246
DOI:
10.1007/s00431-016-2765-z
[Indexed for MEDLINE]

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