AOCCN2017

講演情報

Poster Presentation

[P2-1~135] Poster Presentation 2

2017年5月12日(金) 10:00 〜 15:40 Poster Room A (1F Navis A・B・C)

[P2-63] A case of CDKL5 disorder with the intractable epileptic seizures: improved ADL by simple treatment strategy

Tatsuhiko SHIKE1, 2 (1.Department of Pediatrics, Yokohama Municipal Citizens Hospital, Japan, 2.Department of Pediatrics, Keio University School of Medicine, Japan)

[Introduction]Reduction of seizure burden achieved through anti-epileptic treatment usually provides improved quality of life in epileptic patients. However, overly-aggressive antiepileptic treatment can cause adverse effects, sometimes compromising patient’s quality of life. [Case Report]The presently reported patient is a 5-year-old girl with history of CDKL5-related epilepsy. At two weeks of age, she started to exhibit multiple daily seizures that were characterized by sudden motion arrest, tonic-clonic movement and cyanosis. Despite an aggressive antiepileptic treatment using multiple anticonvulsants, her epilepsy remained refractory, and evolved into various types of seizures including multifocal and myoclonic ones. As a result of such an aggressive antiepileptic treatment, her quality of life was severely compromised to the point where she was overly-sedated and required parenteral feeding. Dosing adjustment of the anticonvulsants, eventually leading to valproate monotherapy, successfully reverted her quality of life with increased wakefulness and oral intake, without increasing seizure frequency.[Discussion]The present observation exemplifies a spontaneous remission of epilepsy in CDKL5-related epilepsy, and disadvantages due to an overly-aggressive antiepileptic treatment. In epileptic patients, proper dosing of anticonvulsants based on a careful evaluation whether the therapeutic benefit outweighs the potential adverse effects is warranted.