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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2021-01-01
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| Series: | Epilepsy & Behavior Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589986421000307 |
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doaj-6b50ea02e169471fb0ee12a016ddbf312021-05-22T04:38:20ZengElsevierEpilepsy & Behavior Reports2589-98642021-01-0116100456Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticusChristopher Martin Allen0Christiana Avye Hall1Naomi Elizabeth Cox2Hayley Ryan3Thearina De Beer4Michael Francis O'Donoghue5Department of Neurology, Queen’s Medical Centre, Nottingham, United Kingdom; Corresponding author at: Department of Neurology, Queen’s Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom.Department of Neurology, Queen’s Medical Centre, Nottingham, United KingdomDaisy Garland Ketogenic Dietitian, Queen’s Medical Centre, Nottingham, United KingdomDepartment of Critical Care, Queen’s Medical Centre, Nottingham, United KingdomDepartment of Critical Care, Queen’s Medical Centre, Nottingham, United KingdomDepartment of Neurology, Queen’s Medical Centre, Nottingham, United KingdomThe 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.http://www.sciencedirect.com/science/article/pii/S2589986421000307Status EpilepticusIntensive CareNeurogenetics |
| collection |
DOAJ |
| language |
English |
| format |
Article |
| sources |
DOAJ |
| author |
Christopher Martin Allen Christiana Avye Hall Naomi Elizabeth Cox Hayley Ryan Thearina De Beer Michael Francis O'Donoghue |
| spellingShingle |
Christopher Martin Allen Christiana Avye Hall Naomi Elizabeth Cox Hayley Ryan Thearina De Beer Michael Francis O'Donoghue Adjunctive use of the ketogenic diet in a young adult with UBE2A deficiency syndrome and super-refractory status epilepticus Epilepsy & Behavior Reports Status Epilepticus Intensive Care Neurogenetics |
| author_facet |
Christopher Martin Allen Christiana Avye Hall Naomi Elizabeth Cox Hayley Ryan Thearina De Beer Michael Francis O'Donoghue |
| author_sort |
Christopher Martin Allen |
| title |
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_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_sort |
adjunctive use of the ketogenic diet in a young adult with ube2a deficiency syndrome and super-refractory status epilepticus |
| publisher |
Elsevier |
| series |
Epilepsy & Behavior Reports |
| issn |
2589-9864 |
| publishDate |
2021-01-01 |
| 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. |
| topic |
Status Epilepticus Intensive Care Neurogenetics |
| url |
http://www.sciencedirect.com/science/article/pii/S2589986421000307 |
| work_keys_str_mv |
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