Blomme Lies, Van de Velde Kirsten
Neurology Department, ZNA Middelheim Antwerp, Antwerp, Belgium.
Neurology Department, AZ Nikolaas, Sint-Niklaas, Belgium.
Case Rep Neurol. 2019 Apr 18;11(1):132-136. doi: 10.1159/000499448. eCollection 2019 Jan-Apr.
In this report we present a clinical case of trismus. The patient in question showed symptoms of trismus for 3 days, rapidly leading to respiratory insufficiency. Afterwards she developed myoclonus and progressive encephalopathy. Neurological workup showed no relevant abnormalities. A CT of the abdomen revealed a mass in the lower abdomen, which turned out to be an ovarian teratoma. Progressive encephalopathy with rigidity and myoclonus (PERM) was diagnosed clinically. Treatment with corticosteroids, benzodiazepines, and levetiracetam did not ameliorate the patient's condition. Only after the introduction of plasmapheresis was there a spectacular improvement in her clinical state. In this case we could not detect associated antibodies. The most likely cause of PERM is paraneoplastic disease secondary to ovarian teratoma. This type of tumor has been associated with multiple paraneoplastic neurological conditions, but this is the first case associated with PERM. To date there is only one publication on trismus as a sole presenting sign, with a quite similar disease course.
在本报告中，我们介绍了一例牙关紧闭的临床病例。该患者出现牙关紧闭症状3天，迅速导致呼吸功能不全。此后，她出现了肌阵挛和进行性脑病。神经系统检查未发现相关异常。腹部CT显示下腹部有一个肿块，结果是卵巢畸胎瘤。临床上诊断为伴有强直和肌阵挛的进行性脑病（PERM）。使用皮质类固醇、苯二氮䓬类药物和左乙拉西坦治疗未能改善患者的病情。仅在采用血浆置换后，她的临床状态才有了显著改善。在这个病例中，我们未能检测到相关抗体。PERM最可能的原因是卵巢畸胎瘤继发的副肿瘤性疾病。这种类型的肿瘤与多种副肿瘤性神经系统疾病有关，但这是第一例与PERM相关的病例。迄今为止，仅有一篇关于牙关紧闭作为唯一表现体征的报道，其病程与之颇为相似。
