Lyutfi Emran, Kasabova Eva, Matev Boyko, Shangova Kaloyana, Kaprelyan Ara
First Clinic of Neurology, University Hospital "St. Marina", Varna, BGR.
Neurology and Neurosciences, Medical University of Varna, Varna, BGR.
Cureus. 2024 Dec 9;16(12):e75410. doi: 10.7759/cureus.75410. eCollection 2024 Dec.
Neuromyelitis optica spectrum disorder (NMOSD) includes conditions with autoimmune genesis, which are manifested by attacks of optic neuritis (ON) and transverse myelitis (TM), and also express aquaporin 4 (NMO-IgG) or myelin oligo-endocytic glycoprotein (MOGAb) antibodies. In rare cases, the disease may also have a clinical presentation with only TM, without ON or with ON, without TM. These conditions are also included in the spectrum. We present a case of a 50-year-old patient with complaints of blurry vision in the left eye, neck pain, and numbness of the hands a few months prior to hospitalization. After a consultation with an ophthalmologist, magnetic resonance imaging (MRI) of the brain was performed, and findings showed left ON. Serological findings showed antibodies for aquaporin 4 with a ratio of 1:100, which is a pathognomic marker for NMOSD. Upon admission to the clinic, the complaints persisted. The neurological status revealed decreased vision of the left eye, pain in the Valleix points in the cervical region, and hyperesthesia including dermatomes C5-C8, more prominent for the right arm. Routine blood tests showed leukocytosis, neutrophilia, and lymphocytosis. Additional virology studies were performed to rule out neuroborreliosis, neurosyphilis, and human immunodeficiency virus (HIV). MRI of the cervical spine revealed dural sac compression from degenerative spinal disease. Electromyography (EMG) showed radiculopathy at the C5-C8 levels and neuropathy of n. ulnaris sinistra and n. medianus dextra. Consultation with an ophthalmologist revealed a decrease in the left eye visus. The treatment plan consisted of methylprednisolone 1000 mg for five days and gastroprotection with famotidine 40 mh p.o. Further consultations with the ophthalmologist were done in the following days to monitor the visual status of the patient. After completion of the diagnostic and treatment plan, slight recovery of the visus and reversal of the pain symptomatology was reported. Based on the history, clinical findings, ophthalmologic examination, brain and neck imaging (MRI), and laboratory results (NMO-IgG ratio 1:100 ), NMOSD with left retrobulbar neuritis (Devic's disease) was concluded as the final diagnosis. Multiple sclerosis (MS; retrobulbar neuritis) first attack, systemic connective tissue disease (systemic lupus erythematosus, Sjögren's syndrome, and antiphospholipid syndrome), and neuro-infection were discussed. Clinical suspicion of intracranial tumors was ruled out. Corticosteroids and gastroprotectors were prescribed for oral use under the supervision of a neurologist. Regular check-ups were performed. The reported case of NMOSD with unilateral optic nerve involvement is a rare condition in neurological practice. It requires an individualized approach to diagnose and apply treatment. Patient follow-up is a key starting point toward gathering more information about autoimmune processes in the central nervous system (CNS) and toward finding faster, more reliable, and cheaper clinical and instrumental methods of diagnosis, which in turn may lead to more timely initiation of treatment, proportionally leading to improvement in the patient's quality of life.
视神经脊髓炎谱系障碍(NMOSD)包括自身免疫性起源的疾病,其表现为视神经炎(ON)和横贯性脊髓炎(TM)发作,并且还可检测到水通道蛋白4抗体(NMO-IgG)或髓鞘少突胶质细胞糖蛋白抗体(MOGAb)。在罕见情况下,该疾病也可能仅表现为TM,而无ON,或者仅表现为ON,而无TM。这些情况也包括在该谱系中。我们报告一例50岁患者,在住院前几个月出现左眼视物模糊、颈部疼痛和手部麻木的症状。在咨询眼科医生后,进行了脑部磁共振成像(MRI)检查,结果显示为左眼视神经炎。血清学检查结果显示水通道蛋白4抗体比例为1:100,这是NMOSD的一个特征性标志物。入院时,患者的症状持续存在。神经系统检查发现左眼视力下降、颈部瓦勒氏点疼痛以及包括C5 - C8皮节的感觉过敏,右臂更为明显。常规血液检查显示白细胞增多、中性粒细胞增多和淋巴细胞增多。进行了额外的病毒学研究以排除神经莱姆病、神经梅毒和人类免疫缺陷病毒(HIV)。颈椎MRI显示退行性脊柱疾病导致硬脊膜囊受压。肌电图(EMG)显示C5 - C8水平神经根病以及左侧尺神经和右侧正中神经病变。眼科会诊显示左眼视力下降。治疗方案包括静脉滴注甲泼尼龙1000mg,连用五天,并口服法莫替丁40mg进行胃保护。在接下来的几天里,进一步咨询眼科医生以监测患者的视力状况。在完成诊断和治疗方案后,报告显示视力略有恢复,疼痛症状有所缓解。根据病史、临床表现、眼科检查、脑部和颈部成像(MRI)以及实验室检查结果(NMO-IgG比例1:100),最终诊断为伴有左侧球后视神经炎的NMOSD(德维克病)。讨论了多发性硬化(MS;球后视神经炎)首次发作、系统性结缔组织病(系统性红斑狼疮、干燥综合征和抗磷脂综合征)以及神经感染。排除了颅内肿瘤的临床怀疑。在神经科医生的监督下,开具了口服皮质类固醇和胃保护剂。进行了定期检查。所报告的伴有单侧视神经受累的NMOSD病例在神经科临床实践中较为罕见。它需要个体化的诊断和治疗方法。患者随访是获取更多关于中枢神经系统(CNS)自身免疫过程信息以及寻找更快、更可靠和更便宜的临床及仪器诊断方法的关键起点,这反过来可能导致更及时地开始治疗,相应地改善患者的生活质量。