Dados do Trabalho

Título

KETOGENIC DIET AS THERAPY FOR SEIZURES REDUCTION IN NON-KETOTIC HYPERGLYCINEMIA

Apresentação dos casos

We describe two children who presented refractory seizures in the neonatal period, hypotonia, poor feeding, and severe developmental delay. Patients of both genders were identified. A 4-year-old boy and an 8-year-old girl. Symptoms began in the first days of life and were confirmed at 2 and 6 years of age, respectively. Ketogenic diet started at 3 and 7 years, on proportion 4:1 and 3:1, respectively. A dramatic decrease in seizure frequency was observated after introduction of the ketogenic diet., Their EEG presented improvements. Their neurological examination showed an improvement in alertness, and reduction in spasticity.

Discussão

Nonketotic hyperglycinemia is a severe neurometabolic disorder characterized by increased glycine levels, and progressive degenerative lesions in central nervous system. Current glycine reduction therapy uses high doses of sodium benzoate and antiepileptic drugs. These cases showed that the ketogenic diet may represent an alternative method of a clinical control.

Comentários finais

The ketogenic diet may be considered a valuable treatment to control ou to reduce the refractory seizures in Nnonketotic hyperglycinemia .

Referências

Genç Sel, Ç.; Kılıç, M.; Yüksel, D.; Aksoy, A.; Kasapkara, Ç.S.; Ceylaner, S.; O ˘guz, K.K. Nonketotic hyperglycinemia: Clinical
range and outcome of a rare neurometabolic disease in a single-center. Brain Dev. 2018, 40, 865–875.
Nowak M, Chuchra P, Paprocka J. Nonketotic Hyperglycinemia: Insight into Current Therapies. J Clin Med. 2022 May 27;11(11):3027. doi: 10.3390/jcm11113027. PMID: 35683414; PMCID: PMC9181064.

Palavras Chave

refractory epilepsy; therapy; Diet