Sveinsson Olafur, Al Nimer Faiez, Piehl Fredrik
Department of Neurology, Karolinska University Hospital, Stockholm, Sweden.
Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
BMJ Case Rep. 2019 Feb 13;12(2):e226832. doi: 10.1136/bcr-2018-226832.
We describe a woman with both central and peripheral nervous system symptoms consistent with Morvan's syndrome who was successfully treated with immunosuppression including rituximab and the new antiepileptic drug lacosamide against peripheral nerve hyperexcitability. Despite being over 8 months in hospital and 4 months in an intensive care unit she recovered fully. It is also the first case where cerebrospinal fluid neurofilament-light (NfL) levels were followed during the disease course. The clinical course resembled that of anti-NMDA receptor encephalitis, where patients often recover surprisingly well despite severe symptoms and an extensive time in intensive care. A possible explanation is the comparatively low levels of NfL, indicating disease processes that are not characterised by extensive neuroaxonal degeneration.
我们描述了一名患有与莫旺综合征相符的中枢和周围神经系统症状的女性，她接受了包括利妥昔单抗在内的免疫抑制治疗以及针对周围神经兴奋性过高的新型抗癫痫药物拉科酰胺治疗，并取得了成功。尽管她住院超过8个月，在重症监护病房住了4个月，但她完全康复了。这也是第一例在病程中监测脑脊液神经丝轻链（NfL）水平的病例。临床病程类似于抗NMDA受体脑炎，尽管症状严重且在重症监护病房停留时间较长，但患者通常恢复得非常好。一个可能的解释是NfL水平相对较低，这表明疾病过程并非以广泛的神经轴突退变为特征。
