Takahashi Hiroka, Imai Katsumi, Takayama Rumiko, Mine Jun, Ohtani Sanae, Ikeda Hiroko, Kubota Yuko, Takahashi Yukitoshi, Inoue Yushi, Fujiwara Tateki
National Epilepsy Center Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka.
No To Hattatsu. 2011 Jul;43(4):305-8.
A modified ketogenic diet was demonstrated to be remarkably effective in a child with intractable symptomatic focal epilepsy with combined seizures of focal seizures and epileptic spasms (ES) in a cluster (ESC). ES started at 8 months of age and disappeared with ACTH therapy. At the age of 13 months, the child began to have intractable focal seizures that, later, were followed by ESC 10 times a day. Brain MRI showed only a non-specific diffuse cerebral atrophy. Interictal EEG showed high amplitude diffuse disorganized slow waves with prominent sharp waves predominant over the bilateral occipital region. We started a modified ketogenic diet (mKD) treatment without fasting or a water/calorie limitation. Since the 20th day of mKD, the patient has been seizure free (6 months) without adverse effects. EEG showed remarkable improvement and he has some improvement in the developmental milestones. A modified ketogenic diet is easier to start and continue compared to the classic ketogenic diet, and should be tried in intractable epilepsies that are not treatable surgically early in life from the developmental prognosis point of view.
改良生酮饮食被证明对一名患有难治性症状性局灶性癫痫的儿童非常有效，该患儿同时出现局灶性发作和癫痫性痉挛（ES）成簇发作（ESC）。ES在8个月大时开始出现，并通过促肾上腺皮质激素（ACTH）治疗消失。13个月大时，该患儿开始出现难治性局灶性发作，随后每天出现10次ESC。脑部磁共振成像（MRI）仅显示非特异性弥漫性脑萎缩。发作间期脑电图显示高幅弥漫性紊乱慢波，双侧枕区有明显的尖波。我们开始了改良生酮饮食（mKD）治疗，无需禁食或限制水/热量摄入。自mKD治疗第20天起，患者已无癫痫发作（6个月）且无不良反应。脑电图显示明显改善，其发育里程碑也有一些改善。与经典生酮饮食相比，改良生酮饮食更容易开始和持续，从发育预后的角度来看，对于无法通过手术治疗的难治性癫痫，应在生命早期尝试使用。
