Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus

The ketogenic diet (KD) may have a role in treating super-refractory status epilepticus (SRSE). Predominantly used in paediatric epilepsy, there are few reports of its use in adults. We describe a 19-year-old man with UBE2A deficiency syndrome, drug resistant generalized epilepsy, and severe intelle...

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Autores principales: Allen, Christopher Martin, Hall, Christiana Avye, Cox, Naomi Elizabeth, Ryan, Hayley, De Beer, Thearina, O'Donoghue, Michael Francis
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8164013/
https://www.ncbi.nlm.nih.gov/pubmed/34095805
http://dx.doi.org/10.1016/j.ebr.2021.100456
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author Allen, Christopher Martin
Hall, Christiana Avye
Cox, Naomi Elizabeth
Ryan, Hayley
De Beer, Thearina
O'Donoghue, Michael Francis
author_facet Allen, Christopher Martin
Hall, Christiana Avye
Cox, Naomi Elizabeth
Ryan, Hayley
De Beer, Thearina
O'Donoghue, Michael Francis
author_sort Allen, Christopher Martin
collection PubMed
description The ketogenic diet (KD) may have a role in treating super-refractory status epilepticus (SRSE). Predominantly used in paediatric epilepsy, there are few reports of its use in adults. We describe a 19-year-old man with UBE2A deficiency syndrome, drug resistant generalized epilepsy, and severe intellectual disability, who developed SRSE. Initiation of the KD on day 81 of his intensive care unit stay and achieving a state of ketosis seven days later resulted in SRSE resolution and discharge from hospital and recovery to his normal cognitive state. Initiating the KD required a multidisciplinary team for diet initiation and carer education. The KD requires a prospective study of efficacy for SRSE and this should include adult patients.
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spelling pubmed-81640132021-06-04 Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus Allen, Christopher Martin Hall, Christiana Avye Cox, Naomi Elizabeth Ryan, Hayley De Beer, Thearina O'Donoghue, Michael Francis Epilepsy Behav Rep Case Report The ketogenic diet (KD) may have a role in treating super-refractory status epilepticus (SRSE). Predominantly used in paediatric epilepsy, there are few reports of its use in adults. We describe a 19-year-old man with UBE2A deficiency syndrome, drug resistant generalized epilepsy, and severe intellectual disability, who developed SRSE. Initiation of the KD on day 81 of his intensive care unit stay and achieving a state of ketosis seven days later resulted in SRSE resolution and discharge from hospital and recovery to his normal cognitive state. Initiating the KD required a multidisciplinary team for diet initiation and carer education. The KD requires a prospective study of efficacy for SRSE and this should include adult patients. Elsevier 2021-05-07 /pmc/articles/PMC8164013/ /pubmed/34095805 http://dx.doi.org/10.1016/j.ebr.2021.100456 Text en © 2021 The Author(s) https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Allen, Christopher Martin
Hall, Christiana Avye
Cox, Naomi Elizabeth
Ryan, Hayley
De Beer, Thearina
O'Donoghue, Michael Francis
Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title_full Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title_fullStr Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title_full_unstemmed Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title_short Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus
title_sort adjunctive use of the ketogenic diet in a young adult with ube2a deficiency syndrome and super-refractory status epilepticus
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8164013/
https://www.ncbi.nlm.nih.gov/pubmed/34095805
http://dx.doi.org/10.1016/j.ebr.2021.100456
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