Department of Neurology, Medical Park Goztepe Hospital, Bahcesehir University, Istanbul, Turkey.
Department of Neurology, Umraniye Training and Research Hospital, Health Sciences University, Istanbul, Turkey.
Int J Neurosci. 2021 Sep;131(9):914-918. doi: 10.1080/00207454.2020.1759584. Epub 2020 May 6.
PURPOSE/AIM OF THE STUDY: Subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the brain caused by a persistent measles virus infection occurring mostly in childhood or early adolescence. The spectrum of epileptic phenomena associated with SSPE is wide, varying from partial or generalized tonic-clonic seizures and atypical absences to myoclonic-atonic attacks. Tonic seizures are very rare in SSPE.
Herein, we discuss a case of 25 years old male that presented with adult-onset SSPE with tonic seizures accompanying myoclonic seizures.
Patient was treated with clonazepam 5 mg/day and an isoprinosine regimen with a dose of 70 mg/kg/day. This is the fourth case of SSPE presenting with myoclonic and tonic seizures and the first case of SSPE with myoclonic and tonic seizures reported in an adult-onset case in the English literature.
Adult-onset SSPE with tonic seizures is rare and may be confusing, thus, it is important to recognize the presence of this type of tonic motor seizures in SSPE patients.
目的/研究目的：亚急性硬化性全脑炎（SSPE）是一种由持续性麻疹病毒感染引起的大脑退行性疾病，主要发生在儿童期或青春期早期。与 SSPE 相关的癫痫现象谱很广，从部分或全面强直阵挛发作和非典型失神到肌阵挛-猝倒发作不等。强直发作在 SSPE 中非常罕见。
本文讨论了一例 25 岁男性患者，表现为成人起病的 SSPE，伴有强直发作伴肌阵挛发作。
患者接受氯硝西泮 5mg/天和异丁司特 70mg/kg/天的治疗方案。这是第四例以肌阵挛和强直发作为表现的 SSPE 病例，也是首例在成人起病的 SSPE 病例中报告的以肌阵挛和强直发作为表现的病例。
成人起病的伴有强直发作的 SSPE 较为罕见，可能具有混淆性，因此，重要的是要认识到 SSPE 患者存在这种类型的强直运动性发作。
