Kodadhala Vijay, Devulapalli Saravana, Kurukumbi Mohankumar, Jayam-Trouth Annapurni
Department of Internal Medicine, Howard University Hospital, Washington, DC 20060, USA.
Department of Neurology, Howard University Hospital, 2041 Georgia Avenue, Washington, DC 20060, USA.
Case Rep Neurol Med. 2014;2014:291380. doi: 10.1155/2014/291380. Epub 2014 May 26.
Acute disseminated encephalomyelitis is a demyelinating disease, typically occurring in children following a febrile infection or a vaccination. Primary and secondary immune responses contribute to inflammation and subsequent demyelination, but the exact pathogenesis is still unknown. Diagnosis of acute disseminated encephalomyelitis is strongly suggested by temporal relationship between an infection or an immunization and the onset of neurological symptoms. Biopsy is definitive. In general, the disease is self-limiting and the prognostic outcome is favorable with anti-inflammatory and immunosuppressive agents. Locked-in syndrome describes patients who are awake and conscious but have no means of producing limb, speech, or facial movements. Locked-in syndrome is a rare complication of acute disseminated encephalomyelitis. We present a case of incomplete locked-in syndrome occurring in a 34-year-old male secondary to acute disseminated encephalomyelitis. Our case is unique, as acute disseminated encephalomyelitis occurred in a 34-year-old which was poorly responsive to immunosuppression resulting in severe disability.
急性播散性脑脊髓炎是一种脱髓鞘疾病，通常发生在儿童发热感染或接种疫苗之后。原发性和继发性免疫反应会导致炎症及随后的脱髓鞘，但确切的发病机制仍不清楚。感染或免疫接种与神经症状发作之间的时间关系强烈提示急性播散性脑脊髓炎的诊断。活检具有决定性意义。一般来说，该疾病具有自限性，使用抗炎和免疫抑制药物后预后良好。闭锁综合征描述的是患者清醒且有意识，但无法产生肢体、言语或面部运动。闭锁综合征是急性播散性脑脊髓炎的一种罕见并发症。我们报告一例34岁男性继发于急性播散性脑脊髓炎的不完全闭锁综合征病例。我们的病例很独特，因为急性播散性脑脊髓炎发生在一名34岁患者身上，对免疫抑制反应不佳，导致严重残疾。
