What is the complexity of the diagnosis and treatment of Devic’s disease – a dangerous demyelinating pathology?
- 1 Opticomyelitis Devika
- 2 Development mechanism and causes
- 3 Symptoms
- 4 Diagnostics
- 5 Differentiation with other diseases
- 6 Treatment
- 7 Complications
Disease manifests itself in different ways, so making an accurate diagnosis is quite difficult. However, only the early determination of the diagnosis will help save the life and health of the sick person.
The disease averages 12% of all pathologies associated with the destruction of the myelin sheath of nerve cells, and Europeans, according to statistics, suffer less frequently. Opticomyelitis is considered an autoimmune pathology. It affects women several times more often. The first signs usually appear at the age of 30-40 years, but can be observed in other age groups.
The course of pathology allows us to talk about its two types:
- Monophasic At the same time, damage to the optic tracts, loss of vision, demyelination processes develop. Sometimes the symptoms of Devika’s disease appear within 30 days. It occurs in 15-25% of cases.
- Recurrent Between episodes may take several months or years. Diagnosed often. However, the prognosis is more favorable.
The course may be similar to multiple sclerosis, Sjogren syndrome, infectious diseases.
Development mechanism and causes
When Devik opticomyelitis affects the proteins of the membranes of two types of cells – astrocytes and oligodendrocytes.
Astrocytes support and share neurons, regulate the supply of nutrients, perform the function of the blood-brain barrier, can turn into nerve cells themselves. The main task of oligodendrocytes is the myelination of the long processes of neurons, their separation from other cells.
The key points in the development of pathology consider the formation of antibodies to the aquaporin-4 protein of the astrocyte plasma membrane and proteins of the myelin sheath. Antibodies penetrate the blood-brain barrier, cause inflammation and, ultimately, damage to the cell membrane. As a result, the process of conducting nerve impulses is disturbed, the protection of nervous tissues from toxins, microorganisms, and antibodies circulating in the blood is reduced.
The exact reason for the development of opticomyelitis is still unknown. It is assumed that this occurs as a result of autoimmune processes. According to one version, viral diseases play a provocative role.
In most cases, the onset of Devic’s disease is associated with damage to the optic nerves. This reduces visual acuity from one or two sides, there is pain in the eyes as they move. In a monophasic course, signs of destruction of myelin quickly join the visual impairment. With a relapsing course, spinal symptoms appear later.
The symptoms of pathology of the optic nerve include the occurrence of the sensation of veil before the eyes, blurring, blurred vision. Soreness develops when the eyeballs move. The study does not show fundus changes. Swelling, blanching of the optic discs is detected. In some cases – their atrophy. Symptoms appear on one side or two, depending on the severity of the process.
Sight can recover over time. Sometimes there is complete blindness associated with irreversible damage to the optic nerve. In some cases, vision loss is transient.
Spinal tissues are damaged longitudinally and transversely. In the latter case, there may be a partial or complete violation. Inflammation captures several sites, accompanied by edema, circulatory disorders, demyelination. As a result, the conduction of impulses along the fibers of the cells that carry out motor and sensory innervation is disturbed.
Signs indicating spinal cord injury appear below the site of injury. Among them are:
- Movement disorders. Affect all limbs or only part of them.
- Decreased sensitivity. A person does not feel pain, vibration, temperature changes, touch.
- Muscle weakness.
- Spasticity. There is a combination of hyper tone, weakness in the muscles, a feeling of resistance when trying to perform a movement, and increased fatigue.
- Coordination disorders. A person can not regulate movement when walking, while trying to maintain body position.
- Symptom Lermitta. It feels like a sharp pain, piercing the back, arms, legs when bending, movements of the head, neck, cough.
- Vegetative disorders. Devika’s disease is accompanied by increased sweating, shortness of breath, increased heart rate.
- The defeat of the pelvic organs. Manifested in urinary incontinence, feces, or, conversely, urinary retention and constipation.
Determining Devic’s disease is not so easy; it needs to be separated from other autoimmune diseases associated with demyelination.
Examination begins with clarifying the symptoms, checking reflexes. A test for the symptom Lermitta.
Patients are assigned magnetic resonance imaging of the head and spine. The images obtained show signs of damage to the spinal canal in three or more segments. This is the main difference from multiple sclerosis, in which 1-2 segments are affected. On the pictures of the head, only violation of the optic nerves is noted, and all along. With damage to the nerves on both sides, a “gull symptom” is noted. There are signs of demyelination of foci in the brain in the hypothalamus and periventricular areas.
With the difficulties of diagnosis, the use of MRI with MR spectroscopy is shown. The method allows to differentiate gray and white matter, the processes of transportation of various substances, the place where the blood-brain barrier increases.
Spinal fluid is punctured. At the same time, neutrophils, cell debris, antibodies, proteins, leukocytes are detected in the analysis.
In the study of blood, antibodies to aquaporin-4 are detected. This is a characteristic feature of Devic opticomyelitis, which distinguishes it from other demyelinating pathologies.
It is mandatory, even with complete blindness, an ophthalmoscopy. The condition of the fundus of the eye, blood vessels, disk.
Differentiation with other diseases
Carefully carried out thorough diagnostics makes it possible to distinguish Devic’s disease from autoimmune diseases, tuberculosis, viral damage of spinal structures, multiple sclerosis.
Read also … Dangerous disease – disseminated encephalomyelitis: causes, symptoms and prognosis
For a long time, it was believed that Devic syndrome and multiple sclerosis is one disease. Indeed, they share a common pathological process. However, later research data allowed to distinguish between these diseases.
With Devic opticomyelitis, specific antibodies are found in the blood that are not inherent in multiple sclerosis or other pathologies. In the liquor are found in a large number of leukocytes.
MRI reveals signs characteristic of opticomyelitis. A consistent lesion of more than three segments of the spinal structure is detected. Areas of demyelinating brain lesions are few, insignificant.
In opticomielitis, optic nerve damage and blindness are among the most common symptoms. They accompany sclerosis much less frequently.
Other differences distinguish features of multiple sclerosis, which are not characteristic of Devic’s disease:
- cognitive impairment;
- emotional lability;
- weakness of muscles in arms and legs;
- burning and tingling sensation in the fingers.
Currently, there is no medicine that is able to stop the destruction of the protein components of the cells. The main goal is to reduce the rate of development of opticomielitis. Monoclonal antibodies, whose action is aimed at suppressing autoimmune inflammations, are considered effective. Used rituximab, Mitoxatron.
In acute exacerbations, glucocorticosteroid therapy is prescribed. Dexamethasone, Prednisolone is used. Plasmapheresis is often used to purify the blood from antibodies.
Interferon is used in the treatment of Devika syndrome, but the effectiveness of these drugs has not been proven.
Symptomatic treatment includes the use of muscle relaxants. They weaken the muscle tone, reduce the frequency and intensity of spasms. Tolperisone appointed. Improve the emotional state of antidepressants help, for example, Amitriptyline. To relieve pain pregabalin prescribed.
Physiotherapeutic methods – electrophoresis, UHF, complement the treatment. Paraffin baths are used. Conducted phonophoresis with hydrocortisone. The purpose of the procedures is to improve the blood flow and normalize the metabolism. Massages and physiotherapy exercises are considered to be useful for Devic opticomyelitis. Exercise allows you to restore the ability to move, remove the limited movement, sensitivity.
The duration and quality of life with Devic opticomyelitis depends on its course, timely diagnosis and treatment. With the defeat of the respiratory centers can stop breathing and death within one to two years from the onset of the first symptoms.
Other complications include blindness, paralysis, organ dysfunction in the pelvic region.
In some situations, especially if treatment began at the first signs of the disease, complete recovery, restoration of vision is possible.
Devic’s disease is a severe demyelinating disease. It is difficult to diagnose and cure in time. Among the main symptoms are visual impairment, decreased sensitivity and ability to move. With a delay in the appointment of therapy opticomielitis leads to serious complications associated with the development of blindness, the complete lack of movement. Timely diagnosis and strict adherence to the recommendations of the doctor will significantly improve the quality of life and achieve a stable remission.
The following sources were used to prepare the article:
Martynova O. A. Opticomyelitis (Devik’s Disease): Differences in Differential Diagnosis // Journal of the Bulletin of the Council of Young Scientists and Specialists of the Chelyabinsk Region – 2016.
Isaikin A.I., Shmidt T.E., Yakhno N.N., Kavelina A.V., Fateeva T.G. Opticomyelitis // Neurological journal – 2014.
Kotov A.S., Andryukhina O.M., Matyuk Yu.V., Bunak M.S., Borisova M.N., Panteleeva M.V., Shatalin A.V. Optical neuritis and myelitis in a teenager: Devic opticomyelitis or multiple sclerosis? // The Neurological Journal – 2015.