Opticomyelitis (Devik's disease)

Opticomyelitis (Devic's disease)

Devic's neuromyelitis optica (neuromyelitis optica, Devic's disease/syndrome) is a neurodegenerative process of an autoimmune nature, accompanied by the destruction of myelin sheaths.
Thus, this is one of the demyelinating diseases of the nervous system (more detailed information about pathology of this kind can also be found in the materials “Multiple sclerosis”, “Guillain-Barré syndrome”, “Subcortical atherosclerotic encephalopathy (Binswanger disease)”). Eugene Devic (France) was a practicing neurologist and researcher; His main publications concerned infantile chorea, cerebral glioma, tumors of the corpus callosum, and typhoid fever. In 1894, E. Devic, in collaboration with his graduate student Fernand Go, described a rare disease of the nervous system (by that time, only 16 cases were known in Europe and North America, to which Devic and Go added their own, seventeenth observation), in many ways similar to multiple sclerosis, but with a distinct specificity, namely almost isolated damage to the optic nerve and spinal cord; Such selectivity is not typical for demyelinating diseases.

It should be noted that descriptions of individual symptoms of neuromyelitis optica appeared long before the work of Devika-Go, back at the beginning of the 19th century. The outstanding English doctor and public figure, Sir Thomas Clifford Allbot, also contributed in 1870 and attracted the attention of the medical community to this specific disorder, but a detailed clinical description of neuromyelitis optica as a separate, independent disease belongs to E. Devic.

Throughout the twentieth century, despite a number of distinctive features, Devic's opticomyelitis was considered one of the atypically severe variants of multiple sclerosis, and some sources stubbornly continue to reproduce this hopelessly outdated interpretation. In the 2000s, mainly through the work of the research sector of the world-famous Mayo Clinic (Rochester, USA), this uncertainty was finally eliminated: it was possible to identify a humoral mechanism that is aggressive towards perivascular protein, and to find reliable biochemical markers of neuromyelitis optica (immunoglobulin antibodies), which do not occur in multiple sclerosis. According to the Mayo Clinic, this was the first time a specific molecular target for the autoimmune-inflammatory demyelinating process had been discovered. As of today, the presence of six types of antibodies is assumed, three of which have already been discovered.

Thus, Devic's neuromyelitis optica is indeed an independent nosological entity, and not a variant of multiple sclerosis.

Epidemiological estimates are difficult due to the relative rarity of this syndrome and the likely occurrence of numerous diagnostic errors. It is reported that "true" Devic's disease occurs approximately fifty times less frequently than classic multiple sclerosis, accounting for 1-2% of the total demyelinating diseases. The age range for initial diagnosis ranges from one year to 77 years; Most often, manifestation occurs in the period of 35-45 years. Women get sick much more often than men (3-9 times according to various sources). Between half and two-thirds of patients with Devic's neuromyelitis optica also have another autoimmune disorder.

3. Treatment options for Devic syndrome and prognosis

Devic's disease can have an acute course. Pathology in this form most often develops as a complication of acute respiratory viral infection and is diagnosed as post-infectious demyelinating encephalomyelitis. In this case, treatment includes intravenous administration of immunoglobulin, methylprednisolone, and corticosteroids. If the disease does not acquire a chronic form, then the prognosis is quite favorable - we can talk about the possibility of a complete cure or with the preservation of minor residual disorders.

Severe chronic forms of Devic syndrome are incurable. The pathology progresses in waves and leads to death. Therapy for such patients includes symptomatic and supportive treatment.

In rare cases, Devic syndrome is diagnosed in children. In this case, the disease, as a rule, has a single-phase acute course. After achieving remission, exacerbations are not typical. Timely treatment, including immunosuppressive therapy and corticosteroids, gives a favorable prognosis. Relapses in those affected in childhood are rare.

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Therapy

Treatment of this disease requires an integrated approach. It is complicated by the contradictory results of the use of immunosuppressive and immunomodulatory drugs. Of the 6 drugs registered in the world for the treatment of neuromyelitis optica, 3 are interferons with unproven clinical effectiveness. Plasmapheresis and high doses of corticosteroids can stop attacks. If there is no effect, mitoxantrone and rituximab may be prescribed. Prednisolone can prevent exacerbation.

Symptomatic therapy includes:

Antidepressants;
Muscle relaxants;
Intrathecal baclofen infusion;
Physiotherapeutic procedures: SMT, electrophoresis, paraffin therapy;
Massage;
Exercise therapy.

Treatment

Treatment of Devic's neuromyelitis optica is a difficult process. The main goal of doctors is to slow down the progression of pathology and improve the patient’s quality of life. For pathogenetic therapy, drugs that inhibit B-cell function (mitoxantrone) were previously popular. At the moment, monoclonal antibodies that suppress autoimmune inflammation are considered more effective. An example is rituximab.

To stop the exacerbation of the disease, glucocorticosteroids are used in large doses - prednisolone, hydrocortisone, dexamethasone. Partial blood purification from circulating immune complexes is carried out using plasmapheresis.

Symptomatic therapy is used to improve the patient's quality of life.

Groups of drugs used:

Muscle relaxants. Medicines reduce pathological hypertonicity. Representatives: dantrolene, tolperisone.
Antidepressants. Improves the emotional state of patients. Representatives: imipramine, amitriptyline.
Centrally acting painkillers – pregabalin.

Therapeutic exercise and physiotherapy are auxiliary techniques that help increase the effectiveness of basic therapy. The practice uses electrophoresis, massage, and paraffin baths.

Devic's neuromyelitis optica: causes, symptoms, diagnosis

Devic's neuromyelitis is considered a separate nosological entity, although the morphological substrate of the syndrome resembles multiple sclerosis.
Selection into a separate category is due to the need for unique treatment. Incorrect diagnosis makes early therapy impossible and irreversible changes occur, so we recommend immediately doing an MRI of the brain for early verification of the pathology. There are two forms of Devic's disease (DD):

Transverse myelitis - affecting the spinal structures (LETM);
Neuritis (acute, subacute) – accompanied by an inflammatory process of the optic nerve.

Correct treatment is possible only with timely diagnosis of the varieties of the disease.

Devic's opticomyelitis MRI in lateral projection

Symptoms

Neuromyelitis optica is a disease that is accompanied by a characteristic clinical picture. Symptoms of the pathology are associated with dysfunction of individual impulse-conducting fibers. Traditionally, the optic nerve (neuritis) and spinal cord tissue (myelitis) are involved in the process.

Depending on which structure is affected first, the manifestation of the clinical picture will differ. In 80% of patients, Devic's disease begins with visual impairment.

Possible symptoms:

Decreased vision clarity.
The appearance of interference, a “veil” before the eyes.
Pain in the eye sockets.

Progression of the disease without adequate treatment leads to complete loss of vision. Sometimes symptoms partially regress with incomplete restoration of eye function.

Myelitis debuts before neuritis in 20% of cases. Typical symptoms of spinal cord dysfunction occur, which manifest themselves in different ways:

Partial or complete loss of control of the lower limbs – paresis or paralysis. The violation may be one- or two-sided.
Deterioration in coordination of movements.
Pathological strengthening of the flexor muscles - hypertonicity.
Reduction or disappearance of sensitivity in the structures of the body located below the site of the spinal cord lesion.
Fecal incontinence, urinary retention.
Shooting pain radiating to the lower extremities.

The literature describes cases of neuromyelitis optica that occur against the background of a clinical picture that resembles ordinary acute respiratory diseases (ARI) or other infectious lesions of the body. Fever, general weakness, and the like came to the fore.

Sometimes Devic's disease can progress against the background of autoimmune pathologies:

Systemic lupus erythematosus.
Dermatomyositis.
Sjögren's syndrome.
Rheumatoid arthritis.
Idiopathic thrombocytopenic purpura.

In 75% of cases, it takes up to 3-4 months for the development of a characteristic clinical picture. Further progression of the pathology depends on the therapy used, the individual characteristics of the body and the aggressiveness of the disease.

Diagnostics

To make a diagnosis, a neurologist collects anamnesis, studies the results of a lumbar puncture and MRI. The disease must be distinguished from retrobulbar neuritis, acute disseminated encephalomyelitis, myelopathy, and recurrent myelitis.

To study the cerebrospinal fluid, a sample of cerebrospinal fluid is required. The acute phase of neuromyelitis optica is indicated by over 50 pleocytosis cells per 1 mm3. Oligoclonal bodies, which are typical for multiple sclerosis, can be found in only 20% of patients. Characteristic is the presence of NMO-IgG, antinuclear and antithyroid antibodies.

Consultation with an ophthalmologist is necessary, including testing of visual acuity and ophthalmoscopy. Examination of the fundus shows swelling of the optic nerve, pallor and blurring characteristic of neuromyelitis optica, even with an asymptomatic course of the disease. To clarify the diagnosis, a study of visual EP is indicated.

MRI will help visualize lesions in spinal segments. This disease is characterized by necrotic damage to two or more segments. MRI of the brain shows demyelination of the optic tracts. Pathology can be detected in the hypothalamus region while the remaining cerebral structures are intact. This picture helps differentiate neuromyelitis optica from REM and multiple sclerosis. They are characterized by necrotic lesions in the brain.

The absolute criteria include: acute myelitis, optic neuritis, and the absence of other lesions and the presence of NMO-IgG antibodies.

Forecast

Making predictions in the case of neuromyelitis optica is quite difficult. A complete recovery with stable remission may occur, residual paresis and visual disturbances may be observed. The most serious complication is amaurosis. In the most severe cases, death is possible. Death is caused by severe myelitis of the cervical segments of the spinal cord. This leads to respiratory failure. More unfavorable prognosis for monophasic course of the disease, accompanied by atrophy of the optic nerves and persistent motor deficit.