NMO stands for Neuromyelitis optica. Neuromyelitis optica is an autoimmune inflammatory disease that affects the central nervous system and results in degradation of certain insulating nerve sheaths (medical term demyelination). As a result, inflammation of the optic nerve develops over a period of a few months to two years. This occurs either unilaterally or bilaterally. The spinal cord also becomes inflamed (myelitis).
What is neuromyelitis optica?
In numerous cases of neuromyelitis optica, there are special antibodies against a specific water channel, the so-called aquaporin-4 channel. The pathophysiological significance of these substances has not yet been fully clarified and is the subject of current medical research. The disease is synonymously referred to in some cases as Devic’s syndrome or with the abbreviation NMO. See AbbreviationFinder for abbreviations related to NMO.
It is a relatively rare disease of the central nervous system. Neuromyelitis optica accounts for about one percent of all demyelinating diseases. There is also a debate as to whether neuromyelitis optica is a special form of multiple sclerosis or whether it forms an independent disease.
The disease was first scientifically described at the beginning of the 19th century. Subsequently, Eugène Devic and Fernand Gault researched the disease, so that neuromyelitis optica is sometimes also referred to as Devic’s syndrome. There is currently a study group dedicated to researching neuromyelitis optica. The topic is the networking of scientific and clinical findings to investigate the clinical course of the disease and its frequency.
According to the current state of knowledge of medical research, there is still no complete clarity about the causes that lead to the development of neuromyelitis optica. However, it is suspected that special antibodies against the so-called aquaporin-4 channels play a central role in the development of the disease. Because these antibodies are found in numerous patients who are affected by neuromyelitis optica.
Symptoms, Ailments & Signs
Neuromyelitis optica is associated with numerous typical symptoms and complaints. The first characteristic of the disease is that the myelin layer of central nerves is broken down. In connection with this, there is also a degradation of the neuritis nervi optici and the spinal cord.
In the further course of the disease, visual disturbances appear, which in some cases lead to blindness (medical term amaurosis). Blindness manifests itself in either one or both eyes and develops over a few hours or days. In addition, in the context of neuromyelitis optica, a paraplegic syndrome is possible, which is associated, for example, with sensory disorders, paralyzed extremities or disorders of the bladder function.
Basically, the disease shows either a monophasic or a multiphasic course. In addition, it may progress chronically. In histological examinations, some demyelination foci can be detected that are similar to multiple sclerosis. Irreversible necrosis is also possible.
Diagnosis & course of disease
If symptoms typical of neuromyelitis optica appear, a suitable specialist should be consulted immediately. Together with the affected patient, he discusses his medical history (anamnesis) as well as the individual complaints. This enables a clinical diagnosis to be made.
In connection with this, various neurological examinations are necessary, in which, for example, inflammation in the optic nerves and spinal cord is searched for. Damage to the brain, which in some cases shows similar symptoms, can also be ruled out. To ensure the diagnosis, the aquaporin-4 antibodies are determined.
In addition, magnetic resonance imaging of the skull and spine is required. Because within the framework of differential diagnostics, multiple sclerosis and retrobulbar neuritis, for example, must be ruled out. It should be noted that, especially at the beginning of the disease, an exact differentiation from multiple sclerosis is not always possible. Retrobulbar neuritis is often associated with visual disturbances similar to neuromyelitis optica, but does not involve the spinal cord.
Neuromyelitis optica usually causes inflammation of the optic nerve. Therefore, if this disease is not treated, it can also lead to a complete loss of vision in the patient. Especially in children or young people, vision loss can lead to severe psychological damage or depression.
In children, neuromyelitis optica also clearly disrupts development. However, whether the loss of vision occurs on both sides or on one side cannot generally be predicted universally. Furthermore, due to neuromyelitis optica, other sensory disorders or paralysis can also occur in different parts of the body. The function of the bladder may be disturbed as a result, so that the patient develops incontinence.
Neuromyelitis optica can be treated with medication. If the person has lost vision, it cannot be restored and the vision loss is usually irreversible. If there are psychological problems, the person concerned is dependent on psychological treatment. Usually there are no complications. The patient’s life expectancy is also not negatively affected by neuromyelitis optica.
When should you go to the doctor?
Difficulty seeing or pain in one or both eyes indicates an eye disease. A doctor must determine whether it is neuromyelitis optica and, if necessary, initiate further measures. The help of a doctor is needed at the latest when the well-being is impaired or further physical complaints in the area of muscles, intestines and bladder occur. People who are already suffering from a chronic nerve disease should contact the doctor responsible as soon as possible if the symptoms mentioned occur.
It is also best for people with gastrointestinal diseases or neurological disorders to speak to a specialist promptly so that optic neurmyelitis can be identified and treated before further complications arise. Apart from the family doctor, you can also talk to an ophthalmologist. Accompanying bladder and bowel incontinence should be treated by a gastroenterologist or other internist. If there is an additional muscle disease, an orthopedist or a sports doctor should also be consulted. Patients often need long-term therapeutic support.
Treatment & Therapy
There are various options for treating neuromyelitis optica. These are used on a case-by-case basis and depend on the individual symptoms and the severity of the complaints. In numerous cases, neuromyelitis optica progresses in one attack, i.e. it is monophasic.
On the other hand, a multiphasic or chronic course of the disease is also possible. The demyelinated foci often regress. However, permanent damage due to tissue death is also possible. In the event of a relapsing course, therapy begins with the administration of cortisone. In some cases, however, the affected patient does not respond to the cortisone.
The treatment differs from that of multiple sclerosis, where mainly special immune modulators are used. The long-term therapy of the disease is based on the administration of immunosuppressors, for example the active ingredient azathioprine.
Studies indicate that the antibody rituximab could also show good efficacy in neuromyelitis optica. In addition to cortisone, relapses in neuromyelitis optica can also be treated with plasmapheresis.
Outlook & Forecast
The prognosis of neuromyelitis optica is favorable with early diagnosis and successful drug therapy. The prerequisite for this is that the active ingredients of the prescribed medicines are well absorbed by the organism and lead to a regression of the inflammation. Within a few weeks, significant improvement or recovery is documented in these patients.
If medical support is not sought, the otherwise favorable prognosis deteriorates. The ability to see decreases continuously and can lead to permanent blindness in those affected. Due to the emotional burden of the symptoms, the risk of psychological complications is significantly increased. The prognosis also worsens if a chronic course of the disease develops. Irreversible damage to the optic nerve can occur. As a result, the eyesight of those affected is impaired for life.
People who already suffer from previous illnesses and therefore have a weakened immune system also experience a delay in the healing process or persistent health problems. In these cases, psychotherapeutic support should take place, since an interaction of emotional and physical factors can often be observed. Both areas influence each other and can therefore bring about an improvement in the overall situation if appropriate support is provided. Patients who have a relapsing or recurring course of the disease require regular drug treatment over the course of their lives.
Effective measures to prevent neuromyelitis optica are not yet known according to the current state of medical research. On the one hand, there is a lack of clarity about the exact causes of the disease, on the other hand, it is an autoimmune disease.
Diseases of this kind can generally hardly be prevented. It is therefore particularly important to consult a specialist quickly at the first suspicion of the disease or the occurrence of typical symptoms in order to initiate appropriate therapy.
In the case of neuromyelitis optica, follow-up examinations should take place even if symptoms are no longer recognizable. Follow-up tests include visual field (field of vision) testing, visually evoked cortical potentials (VEP), and brain magnetic resonance imaging (MRI). The visual field is examined by ophthalmologists. Both eyes are checked individually.
It is tested which area the respective eye perceives when looking straight ahead. The neurologist performs a VEP and an MRI is created in a radiological practice. A reduced reaction time with VEP can indicate a remaining or renewed inflammation in the area of the visual pathway, a circulatory disorder or degenerative processes. An MRI of the brain then provides an image of this.
The treating neurologist decides which of the procedures should be performed. If there are no symptoms, a one-time check is usually sufficient. In some cases, annual follow-up is recommended.
Based on the examination results, a decision is then made as to whether and in what form further treatment is necessary. For this purpose, it must be checked whether the optic neuritis is part of another disease. Depending on the result, further treatment is then carried out. There is no special medication for the follow-up treatment of neuromyelitis optica.
You can do that yourself
The disease is accompanied by a gradual deterioration of the optic nerve. Since self-help measures are not sufficient to achieve an improvement in vision, a doctor should be consulted as early as possible.
In addition, the eyes are not exposed to heavy loads. Do not look directly into the sun or other bright light sources. This can lead to an immediate deterioration in vision. In addition, when reading or working on the screen, there should be sufficient light sources in the area so that the optic nerve is not subjected to further overload. There is always a risk of irreparable damage, which should be avoided.
If there are complaints in the back area, the permanent adoption of protective or incorrect posture is not beneficial. These can lead to irreversible impairments of the skeletal system and trigger secondary diseases. Restrictions on mobility would be the result. In the case of inflammatory symptoms in the back, pain or tension, cooperation with a doctor is essential.
In order for the organism to have sufficient defenses for the healing process, it is important to ensure a stable immune system. The body’s self-healing system can be supported with a vitamin-rich diet, avoiding obesity and sufficient exercise in the fresh air.