概述
视神经脊髓炎(NMO)是一种中枢神经系统失调,造成眼神经和脊髓发炎。
NMO 也称为视神经脊髓炎谱系障碍(NMOSD)或德维克氏病。当身体的免疫系统对自己的细胞发生反应时,就会发生这种情况。其主要发生在连接眼睛视网膜和大脑的视神经,以及脊髓中。但有时也会发生在脑内。
这种疾病可能在感染后出现,或者可能与另一种自身免疫病有关。不规则抗体与中枢神经系统中的蛋白质结合并造成损害。
视神经脊髓炎常被误诊为多发性硬化症(MS)或被认为是 MS 的一种,但其实 NMO 是一种不同的状况。
视神经脊髓炎可导致单眼或双眼失明、腿部或手臂无力或瘫痪、疼痛性痉挛、感觉丧失、不受控制的呕吐和打嗝,以及脊髓损伤引起的膀胱或肠功能障碍。儿童患者可能出现意识模糊、癫痫发作或昏迷。
复发很常见。防止反复发作是避免失能的关键。视神经脊髓炎的急性发作或许可逆,但严重时可能导致永久性的视力丧失和行走问题。
症状
Symptoms of neuromyelitis optica are related to the inflammation that occurs in the nerves of the eye and spinal cord.
Vision changes caused by NMO are called optic neuritis. These may include:
- Blurred vision or loss of vision in one or both eyes.
- Not being able to see color.
- Eye pain.
Symptoms related to the spinal cord are called transverse myelitis. These may include:
- Stiffness, weakness or numbness in the legs and sometimes in the arms.
- Loss of feeling in the arms or legs.
- Not being able to empty the bladder or trouble managing bowel or bladder function.
- A tingling feeling or shooting pain in the neck, back or stomach.
Other symptoms of NMO may include:
- Hiccups.
- Nausea and vomiting.
Children can have confusion, seizures or coma. However, these symptoms in children are more common in a related condition known as myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Symptoms may get better and then worse again. When they get worse, it's known as a relapse. Relapses can happen after weeks, months or years. Over time, relapses can lead to total blindness or loss of feeling, known as paralysis.
病因
Experts don't know exactly what causes neuromyelitis optica. In people who have the disease, the immune system attacks healthy tissues in the central nervous system. The central nervous system includes the spinal cord, brain and optic nerves that connect the retina of the eye with the brain. The attack occurs because altered antibodies bind to proteins in the central nervous system and cause damage.
This immune system reaction causes swelling, known as inflammation, and leads to the damage of nerve cells.
风险因素
Neuromyelitis optica is rare. Some factors that may raise the risk of getting NMO include:
- Sex assigned at birth. Women have NMO more often than do men.
- Age. Most often, NMO affects adults. The average age of diagnosis is 40. However, children and older adults also can have neuromyelitis optica.
- Race or ethnicity. People of Hispanic, Asian, or African or Afro-Caribbean descent have NMO at higher rates than do people who are white.
Some research suggests that not having enough vitamin D in the body, smoking and having few infections early in life also may raise the risk of neuromyelitis optica.
Dec. 13, 2024