Department of Psychiatry, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08901, USA.
Epilepsy Behav. 2010 Aug;18(4):494-7. doi: 10.1016/j.yebeh.2010.05.020. Epub 2010 Jun 26.
Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) is a progressive neurodegenerative disorder associated with polygenetic, maternally inherited, mitochondrial DNA mutations. MELAS has multisystem presentation including neurological, muscular, endocrine, auditory, visual, cardiac, psychiatric, renal, gastrointestinal and dermatological symptoms. Clinical course and prognosis are variable, often leading to cognitive decline, disability, and premature death. Both convulsive status epilepticus (CSE) and nonconvulsive status epilepticus (NCSE) are reported with MELAS. This report illustrates a case of MELAS with recurrent complex partial seizures, NCSE, confusion, aggressive behaviors, hallucinations, and paranoid delusions. Rapid video/EEG confirmation of diagnosis and aggressive antiepileptic drug intervention are required. Further education of medical professionals regarding this disorder, its appropriate management, and the significance of NCSE is indicated to avoid delay of treatment.
线粒体脑肌病、乳酸酸中毒和卒中样发作（MELAS）是一种与多基因、母系遗传、线粒体 DNA 突变相关的进行性神经退行性疾病。MELAS 具有多系统表现，包括神经、肌肉、内分泌、听觉、视觉、心脏、精神、肾脏、胃肠道和皮肤症状。临床病程和预后各不相同，常导致认知能力下降、残疾和过早死亡。MELAS 可出现惊厥性癫痫持续状态（CSE）和非惊厥性癫痫持续状态（NCSE）。本报告介绍了一例 MELAS 患者，其反复发作复杂部分性发作、NCSE、意识混乱、攻击性行为、幻觉和偏执性妄想。需要快速进行视频/EEG 以确诊，并积极使用抗癫痫药物进行干预。需要进一步对医疗专业人员进行有关这种疾病的教育，对其进行适当的管理，并认识到 NCSE 的重要性，以避免治疗延误。
