Thomas Alissa A, Stommel Elijah W
Department of Neurology, Dartmouth Hitchcock Medical Center, Lebanon, NH.
J Clin Neuromuscul Dis. 2014 Mar;15(3):112-6. doi: 10.1097/CND.0000000000000025.
Demyelinating disease can acutely affect the central or peripheral nervous system in the forms of acute disseminated encephalomyelitis, acute inflammatory demyelinating polyradiculoneuropathy, and related disorders. Rarely, demyelination can affect the central and peripheral nervous system simultaneously in a disorder that has been described as acute severe combined demyelination. We describe a 54-year-old woman who presented with rapidly progressive ascending paralysis and areflexia with abnormalities in cerebrospinal fluid, electrodiagnostic studies, and magnetic resonance imaging of the brain and cervical spine. She had antibodies to GD1a and responded to treatment with intravenous immunoglobulin and corticosteroids. Our case illustrates that, even in severe form, acute combined central and peripheral demyelination may be considered a treatment responsive and potentially reversible disease.
脱髓鞘疾病可急性影响中枢或周围神经系统,表现为急性播散性脑脊髓炎、急性炎症性脱髓鞘性多发性神经根神经病及相关疾病。罕见的是,脱髓鞘可在一种被称为急性严重联合脱髓鞘的疾病中同时影响中枢和周围神经系统。我们报告一名54岁女性,她出现快速进展的上行性麻痹和无反射,脑脊液、电诊断检查以及脑和颈椎磁共振成像均有异常。她有抗GD1a抗体,对静脉注射免疫球蛋白和皮质类固醇治疗有反应。我们的病例表明,即使是严重形式的急性中枢和周围联合脱髓鞘,也可被视为一种对治疗有反应且可能可逆的疾病。