Obara Koji, Ono Tomoko
Neurology, National Hospital Organization Akita National Hospital, Yurihonjo, JPN.
Cureus. 2022 Feb 10;14(2):e22099. doi: 10.7759/cureus.22099. eCollection 2022 Feb.
Febrile infection-related epilepsy syndrome (FIRES) is a rare disease, whereby refractory status epilepticus (a severe epileptic syndrome) occurs in previously healthy individuals following a febrile illness. Here, we report a patient with FIRES who received ketogenic diet (KD) therapy initiated in the chronic phase. A 21-year-old man presented with status epilepticus, following fever and headache. In the acute phase, his seizures were refractory to conventional antiepileptic drugs and were suppressed only by intravenous anesthetics. In the chronic phase, he showed frequent seizures with concurrent severe cognitive decline. Twenty-seven months after onset, the patient was started on KD. Consequently, his seizure frequency rapidly reduced while his cognitive function slowly improved, albeit incompletely. Recently, KD has been shown to both reduce seizures and improve cognitive prognoses in children with FIRES. Although early KD may help in both seizure control and cognitive prognosis, it is likely that KD can be applied to adult patients with chronic FIRES.
发热感染相关癫痫综合征（FIRES）是一种罕见疾病，此前健康的个体在发热性疾病后会出现难治性癫痫持续状态（一种严重的癫痫综合征）。在此，我们报告一名FIRES患者，其在慢性期开始接受生酮饮食（KD）治疗。一名21岁男性在发热和头痛后出现癫痫持续状态。急性期时，他的癫痫发作对传统抗癫痫药物难治，仅通过静脉麻醉剂得以抑制。慢性期时，他频繁发作癫痫，同时伴有严重的认知功能衰退。发病27个月后，该患者开始接受生酮饮食治疗。结果，他的癫痫发作频率迅速降低，而认知功能虽未完全恢复但缓慢改善。最近研究表明，生酮饮食对患有FIRES的儿童既能减少癫痫发作，又能改善认知预后。虽然早期采用生酮饮食可能有助于控制癫痫发作和改善认知预后，但生酮饮食很可能也适用于患有慢性FIRES的成年患者。
