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Neuromyelitis Optica - Causes, Signs, Symptoms, Diagnosis and Treatment

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Neuromyelitis optica (NMO), or Devic's disease, primarily affects the ocular nerves and the spinal cord. Continue reading the article to learn more.

Written by

Dr. Shikha

Medically reviewed by

Dr. Abhishek Juneja

Published At February 2, 2023
Reviewed AtFebruary 2, 2023

What Is Neuromyelitis Optica?

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare but severe autoimmune demyelinating inflammatory disorder that affects the central nervous system. It primarily damages the myelin, which serves as a protective layer around the nerves. The spinal cord and the optic nerves, which convey messages from the eyes to the brain, are primarily affected by neuromyelitis optica. The condition can induce paralysis and blindness as a result. Neuromyelitis optica is most commonly diagnosed in children. Adults in their 40’s are also susceptible. It is more commonly seen in young women, although it can also affect men. Neuromyelitis optica was once thought to be a kind of multiple sclerosis by experts. They now believe it is a different illness. Although the two disorders have the same kind of symptoms, neuromyelitis optica is frequently more severe. Multiple sclerosis vision difficulties typically affect a single eye at a time; however, neuromyelitis optica can impair both eyes at the very same time.

What Causes Neuromyelitis Optica?

The cause of Neuromyelitis optica is unknown to doctors. Although it does not appear to be inherited, many individuals who have it have several other autoimmune disorders, in which the body destroys healthy cells by mistake, for example, psoriasis, type 1 diabetes, vitiligo, and rheumatoid arthritis. The immune system targets myelin, the insulation that surrounds the nerves when one has neuromyelitis optica. Optic nerves and myelin cells in the spinal cord are specifically targeted. Neuromyelitis optica patients typically experience flare-ups of the condition that might occur months or years apart. People may be able to recover between flare-ups.

What Are the Signs and Symptoms of Neuromyelitis Optica?

  • Myelitis or optic neuritis are the two most common symptoms of neuromyelitis optica, and either of the two might arise first as the symptom. Optic neuritis is a disorder in which the optic nerve becomes inflamed, causing eye pain that is swiftly followed by vision loss (acuity). Only one eye is usually damaged (unilateral), although both eyes (bilateral) could also get impacted at the same time.

  • The other major syndrome is inflammation of the spinal cord, also known as transverse myelitis, which affects some, if not all, motor, sensory, and autonomic functions (bladder and intestine) below a particular level in the body; however, symptoms are often limited to one side of the body.

  • Pain in the spine or limbs, loss of bladder and bowel control, and moderate to severe paralysis of the lower limbs are all possible symptoms. Deep tendon reflexes can be excessive, or they can be absent or decreased at first and then become amplified.

  • There may be varying degrees of sensory loss. A rigid neck, limb, or back pain and headaches may be experienced by those who are affected. This illness may be difficult to distinguish from other idiopathic transverse myelitis patients.

  • It might be difficult to tell the difference between neuromyelitis optica and multiple sclerosis early on because both can develop myelitis and optic neuritis as symptoms. However, in neuromyelitis optica, optic neuritis and myelitis are more severe; brain magnetic resonance imaging (MRI) is more often normal, and spinal fluid examination does not frequently exhibit oligoclonal bands, which are markers that assist in differentiating it from multiple sclerosis.

  • In most cases of neuromyelitis optica, routine treatment with high-dosage corticosteroids improves the early symptoms of visual loss or paralysis, and partial recovery of motor, vision, sensory, or bladder function occurs. Neuromyelitis optica, on the other hand, frequently produces major permanent impairments in vision and spinal cord function, resulting in blindness or limited movement in recurrent cases. Patients with neuromyelitis optica may develop brain lesions in the later stages of the disease, but not always.

  • Intractable vomiting, often known as hiccups, is a well-known symptom of neuromyelitis optica. It is caused by inflammation in the medulla of the brainstem and may be the first sign of the disease. The brainstem and hypothalamus disorders are the most prevalent; however, inflammation can occur in the forebrain also, which is commonly accompanied by significant brain swelling or edema. Neuromyelitis optica can be accompanied by systemic or brain autoimmune illnesses, which can cause diagnostic confusion.

How Is Neuromyelitis Optica Diagnosed?

The doctor will assess the patient's intellect, eyesight, strength, speech, and reflexes. They may want to take blood and spinal fluid samples. A protein or an antibody is found in the blood of more than 70 % of patients with neuromyelitis optica. The doctor may also examine the spine to determine if it is irritated. A test called magnetic resonance imaging (MRI) is one way to do this, and it creates an image of the spinal cord using a big magnet and a computer. The patient may also need to consult an ophthalmologist and have an optical coherence tomography (OCT) scan to check for optic nerve damage.

What Is the Treatment for Neuromyelitis Optica?

Although there is no cure for neuromyelitis optica, there are medications and other therapies that can help the patient manage their symptoms. The doctor will most likely prescribe corticosteroids or steroids to aid with inflammation. Other medications may aid in preventing future attacks by preventing the body from killing off healthy cells. An intravenous (IV) method will be used to inject them into a vein in the arm. The exchange of plasma procedure, known as plasmapheresis, eliminates dangerous antibodies from the blood. The different components of the blood are separated by a machine, and then the plasma is replaced with a substitute. The new blood is then infused back into the patient's body. Preventative medications target faulty antibodies that assault healthy cells in the body, causing neuromyelitis optica. Other medicines to suppress the immune system may be prescribed by the doctor.

Conclusion:

The effects of neuromyelitis optica may worsen over time. The majority of persons with neuromyelitis optica have arm and leg weakness, and others may experience more serious symptoms. Many people with neuromyelitis optica will need to begin using a breathing machine or a ventilator. To address their difficulties, they may need to work with an interdisciplinary team of an occupational therapist or a social worker. If a person or his family member has been diagnosed with neuromyelitis optica, it is critical to create a support network that includes friends, family, support groups, and professionals.

Dr. Abhishek Juneja
Dr. Abhishek Juneja

Neurology

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neuromyelitis opticaoptic neuritis
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