Zaid Muhammad, Saleemi Fatima, Usama Rana Muhammad, Tahir Ayman, Azhar Aima, Eljack Mohammed Mahmmoud Fadelallah, Tahir Muhammad Junaid
Department of Internal Medicine, Lahore General Hospital, Lahore, Pakistan.
Department of Neurology, Punjab Institute of Neurosciences, Lahore, Pakistan.
Radiol Case Rep. 2024 Oct 3;19(12):6648-6651. doi: 10.1016/j.radcr.2024.09.078. eCollection 2024 Dec.
Neuromyelitis optica spectrum disorder (NMOSD), also known as "Devic's syndrome", is an autoimmune demyelination disorder. It affects the optic nerve and spinal cord, causing optic neuritis and transverse myelitis. It is associated with anti-aquaporin 4 antibodies that target the aquaporin channel on astrocytes. An 18-year-old male with a history of appendectomy 6 months ago presented with neck pain, numbness in limbs up to the umbilicus, paresis, and spasticity in the left leg. The brain magnetic resonance imaging (MRI) was normal, while the spinal cord MRI showed hyperintense foci at the T2 level. Cerebrospinal fluid (CSF) analysis was in the normal range and negative for oligoclonal bands. The serological assay was positive for anti-aquaporin-4 antibodies (AQP4-IgG). The patient improved significantly after administering high doses of methylprednisolone and supplements. Due to the unavailability of eculizumab, he underwent plasmapheresis sessions to remove antibodies, which improved to a reasonable extent NMOSD most commonly targets older females, but in our report, it appeared in a young male. The patient only presented with transverse myelitis with no ophthalmologic problem. He made significant improvement with combination treatment of steroids, supplements, and plasmapheresis.
视神经脊髓炎谱系障碍(NMOSD),也被称为“德维克综合征”,是一种自身免疫性脱髓鞘疾病。它会影响视神经和脊髓,导致视神经炎和横贯性脊髓炎。它与靶向星形胶质细胞上的水通道蛋白通道的抗水通道蛋白4抗体有关。一名18岁男性,6个月前有阑尾切除术史,出现颈部疼痛、脐水平以下肢体麻木、左腿轻瘫和痉挛。脑部磁共振成像(MRI)正常,而脊髓MRI显示T2水平有高信号病灶。脑脊液(CSF)分析在正常范围内,寡克隆带为阴性。血清学检测抗水通道蛋白4抗体(AQP4-IgG)呈阳性。给予大剂量甲泼尼龙和补充剂后,患者病情显著改善。由于无法获得依库珠单抗,他接受了血浆置换以清除抗体,病情有了合理程度的改善。NMOSD最常发生于老年女性,但在我们的报告中,它出现在一名年轻男性身上。该患者仅表现为横贯性脊髓炎,无眼科问题。通过类固醇、补充剂和血浆置换的联合治疗,他有了显著改善。