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    Use of the ketogenic diet to manage refractory epilepsy in CDKL5 disorder: Experience of >100 patients

    Access Status
    Fulltext not available
    Authors
    Lim, Z.
    Wong, K.
    Olson, H.
    Bergin, A.
    Downs, Jennepher
    Leonard, H.
    Date
    2017
    Type
    Journal Article
    
    Metadata
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    Citation
    Lim, Z. and Wong, K. and Olson, H. and Bergin, A. and Downs, J. and Leonard, H. 2017. Use of the ketogenic diet to manage refractory epilepsy in CDKL5 disorder: Experience of >100 patients. Epilepsia. 58 (8): pp. 1415-1422.
    Source Title
    Epilepsia
    DOI
    10.1111/epi.13813
    ISSN
    0013-9580
    School
    School of Physiotherapy and Exercise Science
    URI
    http://hdl.handle.net/20.500.11937/58453
    Collection
    • Curtin Research Publications
    Abstract

    Wiley Periodicals, Inc. © 2017 International League Against Epilepsy Objective: Pathogenic variants involving the CDKL5 gene result in a severe epileptic encephalopathy, often later presenting with features similar to Rett syndrome. Cardinal features of epilepsy in the CDKL5 disorder include early onset at a median age of 6 weeks and poor response to antiepileptic drugs. The ketogenic diet (KD) was first introduced in the 1920s as a treatment option for refractory epilepsy in children. This study investigated use of the KD in the CDKL5 disorder and its influences on seizures. Methods: The International CDKL5 Disorder Database, established in 2012, collects information on individuals with the CDKL5 disorder. Families have provided information regarding seizure characteristics, use, and side effects of the KD treatment. Descriptive statistics and time to event analyses were performed. Clinical vignettes were also provided on patients attending Boston Children's Hospital. Results: Data regarding KD use were available for 204 individuals with a pathogenic CDKL5 variant. Median age of inclusion in the database was 4.8 years (range = 0.3–33.9 years), with median age of 6 weeks (range = 1 day–65 weeks) at seizure onset. History of KD use was reported for 51% (104 of 204) of individuals, with a median duration of use of 17 months (95% confidence interval = 9–24). Changes in seizure activity after commencing KD were reported for two-thirds (69 of 104), with improvements in 88% (61 of 69). Nearly one-third (31.7%) experienced side effects during the diet. At ascertainment, only one-third (32%) remained on the diet, with lack of long-term efficacy as the main reason for diet cessation (51%, 36 of 70). Significance: Benefits of KD in the CDKL5 disorder are in keeping with previous trials on refractory epilepsies. However, poor long-term efficacy remains as a significant barrier. In view of its side effect profile, KD administration should be supervised by a pediatric neurologist and specialist dietician.

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    • Ketogenic diet to manage refractory epilepsy in the cdkl5 disorder
      Lim, Z.; Downs, Jennepher; Wong, K.; Leonard, H. (2017)
      Objectives: Mutations involving the CDKL5 gene have been identified as a cause of severe epileptic encephalopathy, often later presenting with features similar to Rett syndrome.1 Cardinal features of epilepsy in the CDKL5 ...
    • Seizure variables and their relationship to genotype and functional abilities in the CDKL5 disorder
      Fehr, S.; Wong, K.; Chin, R.; Williams, S.; De Klerk, N.; Forbes, D.; Krishnaraj, R.; Christodoulou, J.; Downs, Jennepher; Leonard, H. (2016)
      Objective: To investigate seizure outcomes and their relationships to genotype and functional abilities in individuals with the cyclin-dependent kinase-like-5 (CDKL5) disorder. Methods: Using the International CDKL5 ...
    • Prevalence and onset of comorbidities in the CDKL5 disorder differ from Rett syndrome
      Mangatt, M.; Wong, K.; Anderson, B.; Epstein, A.; Hodgetts, S.; Leonard, H.; Downs, Jennepher (2016)
      Background: Initially described as an early onset seizure variant of Rett syndrome, the CDKL5 disorder is now considered as an independent entity. However, little is currently known about the full spectrum of comorbidities ...
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