Neuromyelitis optica (NMO)
This is a rare autoimmune condition in which the immune system attacks the body's own nervous tissue — above all the optic nerves, the spinal cord and certain…
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Medicines commonly prescribed for Neuromyelitis optica (NMO)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: INJECTABLE, 15 mg injectable 0.6 mlActive substance: methotrexateManufacturer: Nordic Group B.V.Prescription requiredDosage form: INJECTABLE, 30 mgActive substance: methotrexateManufacturer: Ebewe Pharma Ges.M.B.H. Nfg.KgPrescription requiredDosage form: INJECTABLE, 50 mg/mlActive substance: methotrexateManufacturer: Medac Gesellschaft Für Klinische Spezialpräparate GmbhPrescription required
This is a rare autoimmune condition in which the immune system attacks the body's own nervous tissue — above all the optic nerves, the spinal cord and certain areas of the brainstem. In most people the target is a protein called aquaporin-4, found on the surface of the cells that surround nerve fibres. The same condition also goes by the names Devic's disease and neuromyelitis optica spectrum disorder (NMOSD). It runs in attacks: a relapse builds up over hours or days, and between relapses things are usually stable. The cause cannot be removed, but treatment does the thing that matters most — it cuts down the number of attacks, because it is the attacks that leave lasting loss of sight and mobility.
What a relapse looks like
An attack involves one of three areas, less often several at once. Symptoms build over hours or days and last for weeks.
- Optic nerve. Pain deep in the eye that gets worse on moving it, then blurred vision, colours losing their strength (red especially) and a patch appearing in the centre of the visual field. In this condition sight is affected more severely than in other kinds of optic nerve inflammation, and often both eyes are involved, either together or one after the other.
- Spinal cord. Weakness and numbness in the legs, sometimes the arms too, a sensation of a tight band around the chest or abdomen, sharp burning or shooting pain, painful muscle spasms and a stubborn itch over one patch of skin. Passing urine is often disturbed, from retention to incontinence, and so are the bowels. Sexual function can be affected as well.
- Brainstem. There is a very characteristic pattern: intractable hiccups, nausea and vomiting lasting days or weeks. They are usually put down to a stomach problem when in fact they may be the only sign of a relapse. Less often there is double vision, dizziness, drowsiness or difficulty breathing.
Sometimes an attack is confined to the eyes (optic neuritis) or to the spinal cord alone (transverse myelitis). Relapses come back after weeks, months or years, and it is these, rather than any slow decline, that determine what is left behind.
How it differs from multiple sclerosis
Both are inflammatory conditions, both involve the optic nerves and the spinal cord, and neuromyelitis optica used to be regarded as a form of multiple sclerosis. It is now clear that they are separate diseases, and the difference is not an academic one.
- Attacks are more severe and recovery is poorer. Lasting damage accumulates attack by attack, not through a slow steady worsening.
- On MRI the spinal cord lesion is usually long, running over three vertebrae or more, whereas in multiple sclerosis it is short.
- In the spinal fluid, the oligoclonal bands typical of multiple sclerosis are usually absent.
- Most important of all: the medicines used to treat multiple sclerosis do not work in this condition, and some of them — beta interferons, natalizumab, fingolimod — can trigger a severe relapse. So if the picture is even slightly atypical for multiple sclerosis, the antibody test should be done before treatment starts, not afterwards.
There is a third, similar condition linked to antibodies against a different protein, MOG. It behaves differently and is treated differently, so it is tested for as well.
Which tests confirm the diagnosis
The key investigation is a blood test for antibodies against aquaporin-4. It is done by a cell-based assay, which is considerably more accurate than the older methods; the sample is blood, not spinal fluid. It is best taken before plasma exchange and before starting any treatment that suppresses the immune system, otherwise the result can be falsely negative.
- If it is negative, antibodies against the MOG protein are looked for — a different condition with similar attacks.
- MRI of the spinal cord shows an extensive area of inflammation; MRI of the brain and optic nerves shows damage to the visual pathway and the areas typical of this condition around the ventricles and in the brainstem.
- Optical coherence tomography and visual evoked potentials measure how much the optic nerve has suffered, including where the person has not noticed it.
- A lumbar puncture is used to rule out infection and to look at the oligoclonal bands.
- Tests for other autoimmune conditions: neuromyelitis optica often keeps company with lupus, Sjögren's syndrome and autoimmune thyroid disease.
A negative antibody test does not rule the diagnosis out: a seronegative form exists, and the diagnosis is then made from the combination of the clinical picture and the MRI. It is a neurologist who makes it, not a scan report read in passing.
When waiting is not an option
Here the speed of treatment directly affects whether sight and movement come back. An attack treated in the first few days leaves considerably less damage than the same attack three weeks on.
Contact a doctor the same day if:
- vision in one or both eyes has worsened over hours or days, particularly with pain on moving the eye;
- weakness or numbness has appeared in the legs, all the more so if it is creeping upwards;
- there is a sensation of a tight band around the chest or abdomen;
- passing urine has become difficult, or control of the bladder or bowel has been lost;
- hiccups, nausea or vomiting have gone on for several days in a row.
Call an ambulance — in Spain, Italy, Portugal, Poland and Ukraine on the single European number 112 — if:
- breathing becomes difficult or there is breathlessness at rest;
- choking occurs and liquids cannot be swallowed;
- weakness in the legs or arms is visibly progressing over a matter of hours;
- consciousness is disturbed, with confusion or marked drowsiness.
How an attack is brought under control
During a relapse there is a single aim: to stop the inflammation as quickly as possible, while the nerve fibres are still alive.
- High-dose corticosteroids given into a vein — a course of several days in hospital, usually followed by tablets with a gradual reduction. This is almost always the first step.
- Plasma exchange: some of the blood is passed through a machine that removes antibodies from the plasma and returns it to the body. It is used when steroids have not been enough, and where sight or the spinal cord is severely affected it is often started alongside them from the outset. The earlier it begins, the better the chance of getting vision back.
- Intravenous immunoglobulin is an option where plasma exchange is unavailable or poorly tolerated.
Rehabilitation does not start after discharge but as soon as the person is well enough: the longer a muscle goes unused, the harder it is to bring it back.
How relapses are kept away
After the first confirmed attack, continuous treatment is started to damp down the overactive immune system. It is taken for years and is not a course: stopping it on your own is one of the commonest causes of a severe relapse.
- Conventional immunosuppressants (azathioprine or mycophenolate mofetil, for example) are taken as tablets. They do not reach full effect straight away, so for the first months they are usually combined with a low dose of steroid. Regular blood counts and liver tests are needed.
- Medicines that clear B lymphocytes from the blood (rituximab and similar agents) are given by infusion to a schedule, most often every few months.
- Modern targeted treatments developed specifically for this condition: complement system blockers, interleukin-6 receptor blockers and newer anti-B-cell agents. They are used first and foremost in people with confirmed aquaporin-4 antibodies.
Any of these treatments raises the risk of infection. Hence several rules worth agreeing with a doctor in advance: vaccinations are given before treatment begins (with complement blockers, protection against meningococcus is essential), live vaccines are not given during treatment, and any fever is a reason to ring the doctor rather than sit it out at home.
Pregnancy is possible with this condition, but it is planned in advance: some of the medicines are incompatible with conceiving and with breastfeeding, and the risk of a relapse after delivery is higher. That conversation is worth having beforehand, not afterwards.
Living with what the attacks leave behind
Between relapses there are symptoms treated separately from the disease itself, and here a great deal depends on getting the details right.
- Nerve pain and itching. Ordinary painkillers work poorly against a burning pain that follows the line of a nerve. Medicines from the anticonvulsant group and certain antidepressants help; they are built up gradually from low doses.
- Muscle spasms. Muscle relaxants are used together with stretching and regular exercise; tablets alone, without movement, achieve little.
- Bladder. Do not hold on, and do not cut down on drinking as a precaution: retention needs a timed voiding routine, sometimes intermittent self-catheterisation, which is taught, and prompt treatment of urinary infections. Cloudy urine with a fever in someone on immunosuppression is a reason to seek advice quickly.
- Bowels. A regular routine, enough fluid, fibre, and laxatives if needed.
- Movement. Physiotherapy preserves what remains; an occupational therapist helps adapt the home and choose a walking aid without losing independence.
- Vision. Where sight is permanently reduced, magnifiers, strong contrast around the home, good lighting and low-vision training all help.
- Fatigue and mood. Heat temporarily worsens the symptoms, so effort is best planned for the morning. Low mood and anxiety are common here, and taking them to a professional is every bit as sensible as taking a painful leg.
Online consultation
With this condition many questions come up between neurology appointments: whether what started today looks like a relapse, whether it can wait for the scheduled visit, what the antibody result means, how to handle vaccinations or plans for pregnancy while on treatment. In an online consultation a doctor can go through your situation, help pull the reports into a coherent picture and point you towards whoever should be seen next.
It is a convenient way to discuss test results and to prepare for the conversation with a specialist. If any of the signs in the urgent section are present, though, the place to start is emergency care, not an online appointment.
This material is for information only and does not replace medical advice.
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