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Experience with the Ketogenic Diet in a Boy with CLCN4 Related Neurodevelopmental Disorder Cover

Experience with the Ketogenic Diet in a Boy with CLCN4 Related Neurodevelopmental Disorder

By: G Sager,  U Yukselmis,  O Güzel,  A Turkyilmaz and  M Akcay  
Open Access
|Mar 2024

References

  1. He H, Guzman RE, Cao D, Sierra-Marquez J, Yin F, Fahlke C, et al. The molecular and phenotypic spectrum of CLCN4-related epilepsy. Epilepsia. 2021;62(6):1401–1415.
  2. Xu X, Lu F, Zhang L, Li H, Du S, Tang J. Novel CLCN4 variant associated with syndromic X-linked intellectual disability in a Chinese girl: a case report. BMCPediatr. 2021;3;21(1):384.
  3. Weinert S, Gimber N, Deuschel D, Stuhlmann T, Puchkov D, Farsi Z, et al. Uncoupling endosomal CLC chloride/proton exchange causes severe neurodegeneration. EMBO J. 2020;4;39(9):e103358.
  4. Palmer EE, Pusch M, Picollo A, Forwood C, Nguyen MH., et al. Functional and clinical studies reveal pathophysiological complexity of CLCN4-related neurodevelopmental condition. Mol Psychiatry. 2023 Feb;28(2):668–697.
  5. Bough KJ, Rho JM. Anticonvulsant mechanisms of the ketogenic diet. Epilepsia. 2007 Jan;48(1):43–58.
  6. Kossoff EH, Zupec-Kania BA, Auvin S, Ballaban-Gil KR, Christina Bergqvist AG., et al. Charlie Foundation; Matthew’s Friends; Practice Committee of the Child Neurology Society. Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open. 2018 May 21;3(2):175–192.
  7. Zhou P, He N, Zhang JW, Lin ZJ, Wang J, Yan LM, et al. Novel mutations and phenotypes of epilepsy-associated genes in epileptic encephalopathies. Genes Brain Behav. 2018;17(8):e12456.
  8. Archila R, Papazian O. Síndrome de Lennox-Gastaut [Lennox-Gastaut syndrome]. Rev Neurol. 1999;16–31;29(4):346–9. Spanish.
  9. Accardi A, Miller C. Secondary active transport mediated by a prokaryotic homologue of ClC Cl- channels. Nature. 2004:26;427(6977):803–7.
  10. Guzman RE, Alekov AK, Filippov M, Hegermann J, Fahlke C. Involvement of ClC-3chloride/proton exchangers in controlling glutamatergic synaptic strength incultured hippocampal neurons. Front Cell Neurosci. 2014;23;8:143.
  11. Veeramah KR, Johnstone L, Karafet TM, Wolf D, Sprissler R, Salogiannis J, et al. Exome sequencing reveals new causal mutations in children with epileptic encephalopathies. Epilepsia. 2013;54(7):1270–81.
  12. Fernández-Marmiesse A, Roca I, Díaz-Flores F, Cantarín V, Pérez-Poyato MS, Fontalba A, Laranjeira F, et al. Rare Variants in 48 Genes Account for 42% of Cases of Epilepsy With or Without Neurodevelop-mental Delay in 246 Pediatric Patients. Front Neurosci. 2019:8;13:1135.
  13. Tassinari CA, Dravet C, Roger J, Cano JP, Gastaut H. Tonic status epilepticus precipitated by intravenous benzodiazepine in five patients with Lennox-Gastaut syndrome. Epilepsia. 1972 Jul;13(3):421–35.
  14. Sankar R. GABA(A) receptor physiology and its relationship to the mechanism of action of the 1,5-benzodiazepine clobazam. CNS Drugs. 2012;26(3):229–44.
  15. Macdonald RL, Kelly KM. Antiepileptic drug mechanisms of action. Epilepsia. 1995;36l 2:S2–12.
  16. Palmer EE, Stuhlmann T, Weinert S, Haan E, Van Esch H, Holvoet M, et al. De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females. Mol Psychiatry. 2018;23(2):222–230.
Language: English
Page range: 77 - 82
Published on: Mar 12, 2024
Published by: Macedonian Academy of Sciences and Arts
In partnership with: Paradigm Publishing Services
Publication frequency: 2 issues per year

© 2024 G Sager, U Yukselmis, O Güzel, A Turkyilmaz, M Akcay, published by Macedonian Academy of Sciences and Arts
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.