Slowly progressive or subacute visual impairment

Slowly progressive or subacute visual impairment

I. For one eye

1. 1. Neuropathy of the optic nerve or retrobulbar neuritis
2. 2. Ischemic neuropathy
3. 3. "Alcohol-Tobacco" (B12-deficient) optical neuropathy.
4. 4. Tumor of anterior cranial fossa and orbit, pseudotumor of orbit.
5. 5. Diseases of the eye (uveitis, central serous retinopathy, glaucoma, etc.)

II. On both eyes

1. Ophthalmic causes (cataracts, some retinopathies).
2. Hereditary optic neuropathy Leber and Wolfram syndrome (Wolfram).
3. Uremic optic neuropathy.
4. Mitochondrial diseases, in particular, the syndrome of Kirns-Seyr (more often - pigment retinopathy, rarely - neuropathy of the optic nerve).
5. Distiroidal orbitopathy (optic neuropathy due to compression of the optic nerve with expanded rectus muscles at the apex of the orbit).
6. Nutritional neuropathy.
7. Neurofibromatosis Recklinghausen type I.
8. Degenerative diseases of the nervous system, involving the involvement of the optic nerves and the retina.
9. Chronic increase in intracranial pressure.
10. Iatrogenic (levomitsetin, amiodarone, stethomycin, isoniazid, penicillamine, digoxin).

[1], [2], [3], [4], [5], [6], [7],

I. Slowly progressive or subacute deterioration of vision in one eye

Neuropathy of the optic nerve or retrobulbar neuritis. Subacute monocular vision impairment in young people without a headache and a normal ultrasound picture suggests the development of the optic nerve neuropathy.

A tumor can be suspected if the optic nerve disc protrudes. With edema of the optic disc, vision also worsens gradually. In the case of retrobulbar neuritis, the inflammatory process takes place in the retroorbital part of the nerve. Consequently, nothing is revealed during the acute stage of ophthalmoscopy. Conduction of visual evoked potentials reveals functional disturbances in the optic nerve. In more than 30% of cases, retrobulbar neuritis is the first manifestation, the manifestation of multiple sclerosis, but it can also occur in later stages of the disease. If it is known that the patient has multiple sclerosis, then diagnostic problems do not arise. If not, it is necessary to carefully question the patient about the typical symptoms and signs of the disease and fully examine the clinical and paraclinical methods. If the optic neuritis appears in the initial stage of multiple sclerosis, then clinical search for other focal symptoms may be unsuccessful. In this case, a complete program of electrophysiological research, including bilateral visual evoked potentials (II pair of cranial nerves), a blinking reflex (V and VII cranial nerves), somatosensory evoked potentials with stimulation of the medial and tibial nerves, and a neurovisual examination should be performed.

Ischemic retinopathy. In the elderly, ischemic damage to the optic nerve can be the reason for the slow development of similar symptoms. Fluorescent angiography is required to demonstrate disturbed arterial perfusion. Often revealed atherosclerotic narrowing of the internal carotid artery.

"Alcohol-Tobacco" optical neuropathy (vitamin B12-deficiency) can begin with a deterioration of vision in one eye, although damage to both eyes is possible. The timing of development is rather uncertain. The cause of the disease is probably not the toxic effect of tobacco or alcohol, but a lack of vitamin B12. The presence of vitamin B12 deficiency is often observed with alcohol abuse. Insufficiency B12, which causes subacute combined degeneration of the spinal cord, also leads to scotomas and optical atrophy.

The concentration of alcohol in the blood is examined, a general and neurological examination is carried out. Often there is a decrease in sensitivity by the type of "gloves and socks," the lack of reflexes on the legs, and the electrophysiological data of the demyelinating process, mainly in the spinal cord. This is demonstrated by some disturbance of the SSVP (somatosensory evoked potentials) with normal or almost normal conduction of the peripheral nerves preserved. Deficiency of absorption of vitamin B12 is detected by means of blood analysis and urinalysis.

Tumor. Tumors of the anterior cranial fossa and orbit may manifest a steadily increasing deterioration of vision in one eye. In young patients, it is usually a matter of glioma of the optic nerve (compression optic nerve neuropathy). In addition to loss of vision, it is difficult to distinguish any other symptoms at first. Then the compression of the optic nerve or chiasma is manifested by the pallor of the optic nerve disk, often by various defects in the visual fields of both eyes, a headache. The disease progresses over several months or years. The causes of compression include a tumor (meningioma, optic glioma in children, dermoid tumors), an aneurysm of the carotid artery (leading to a violation of eye movements), carotid calcification, etc.

Often, children do not even complain of a headache. Planned X-ray examination can reveal the expansion of the optical channel. Neuroimaging (CT, MRI) makes it possible to identify a tumor.

In adult patients, anywhere in the anterior cranial fossa may appear tumors that can eventually cause compression optic neuropathy (meningioma, metastatic tumor, etc.).

Often, a change in personality is added to the impaired vision. Patients become inattentive to their work and family, do not follow their appearance, the sphere of interests is changing. Others notice a decline in initiative. The degree of these changes is tolerable. Patients rarely themselves seek medical help about this.

When performing a neurologic examination, palpation of the optic nerve disk and a decrease in the direct and friendly reaction of the pupils to light are detected. Other "anterior cranial fossa findings" may include a one-sided anosmia that does not change the sense of smell and taste in the patient but is detected by special research methods, sometimes a congestive nipple of the optic nerve on the other side (Foster-Kennedy syndrome).

Slow development of compression neuropathy is observed with aneurysm, arteriovenous malformation, craniopharyngomy, pituitary adenoma, pseudotumor cerebri.

Ocular (orbital) pseudotumor, due to the increase in one or more muscles in the orbit, is accompanied by a violation of eye movements, mild exophthalmos and conjunctiva injection, but visual acuity reduction is rare. This syndrome is one-sided, but sometimes another eye is involved. Ultrasound reveals the expansion (increase in volume) of the muscles of the orbit, as in the syndrome of distyroid orbitopathy.

Some ophthalmic diseases (uveitis, central serous retinopathy, glaucoma, etc.) can lead to slow deterioration of vision in one eye.

II. Slowly progressive or subacute deterioration of vision in both eyes

Ophthalmic causes (cataracts, some retinopathies, including paraneoplastic, toxic, nutritive) lead to a very slow decrease in visual acuity in both eyes; they are easily recognizable by the oculist. Diabetic retinopathy is one of the common causes of this decline in vision. Retinopathy can develop with systemic (systemic lupus erythematosus), hematological (polycythemia, macroglobulinemia) diseases, sarcoidosis, Behcet's disease, syphilis. Older people sometimes develop so-called senile macular degeneration. Pigmented degeneration of the retina accompanies many accumulation diseases in children. Glaucoma with inadequate treatment can lead to an increasing decrease in vision. Volumetric and inflammatory diseases of the orbit can be accompanied not only by reduced vision, but also by pain.

Hereditary optic neuropathy Leber and Wolfram syndrome (Wolfram). Hereditary neuropathy of the optic nerves of Leber is a multi-system mitochondrial disease caused by one or more mutations of mitochondrial DNA. Less than half of these patients have a family history of a similar disease. The onset of the disease is usually between 18 and 23 years with a decrease in vision per eye. The other eye is inevitably involved in a few days or weeks, that is, subacute (rarely - in a few years). When examining the field of vision, a central scotoma is revealed. On the fundus there is a picture of characteristic microangiopathy with capillary telangiectasias. This picture is sometimes accompanied by dystonia, spastic paraplegia and ataxia. In some families, these neurological syndromes can occur without optical atrophy; in other families - optical atrophy without concomitant neurologic syndromes.

Tungsten syndrome also refers to mitochondrial diseases and is manifested by a combination of sugar and diabetes insipidus, optical atrophy and bilateral neurosensory hearing loss (DID-MOAM syndrome). Diabetes mellitus develops in the first decade of life. The decrease in vision progresses in the second decade, but does not lead to complete blindness. Diabetes is not considered the cause of optical atrophy. Sensorineural hearing loss also progresses slowly and rarely leads to severe deafness. At the heart of the disease is a progressive neurodegenerative process. Some patients describe concomitant neurological syndromes, which include: anosmia, autonomic dysfunction, ptosis, external ophthalmoplegia, tremor, ataxia, nystagmus, epileptic seizures, diabetes insipidus of central origin, endocrinopathy. Often there are various mental disorders. The diagnosis is established clinically and by DNA-diagnostic methods.

Uremic optic neuropathy - bilateral swelling of the disc and reduced visual acuity, sometimes reversible with dialysis and corticosteroids.

The syndrome of Kearns-Seyr (a variant of mitochondrial cytopathy) is caused by the deletion of mitochondrial DNA. The disease begins at the age of up to 20 years and is manifested by progressive external ophthalmoplegia and pigmented degeneration of the retina. In addition, the diagnosis should be at least one of the following three manifestations:

1. violation of intraventricular conduction or complete atrioventricular block,
2. increase in protein in liquor,
3. cerebellar dysfunction.

Dysthyreoidal orbitopathy rarely leads to optic neuropathy due to compression of the optic nerve with expanded rectus muscles at the apex of the orbit. Nevertheless, such cases occur in neurological practice. For the diagnosis resort to ultrasound of the orbit.

Nutritive neuropathy of the optic nerve is known for alcoholism, B12 deficiency. The literature describes a similar so-called Jamaica neuropathy and the Cuban epidemic neuropathy.

Neurofibromatosis Recklinghausen type I - multiple brown spots on the skin of the color "coffee with milk," an iris of the iris, multiple neurofibromas of the skin. This picture can be accompanied by optical glioma, neurofibromas of the spinal cord and peripheral nerves, macrocephaly, neurological or cognitive deficits, scoliosis and other bone anomalies).

Degenerative diseases of the nervous system involving optic nerves and retina (mucopolysaccharidosis, abelatipoproteinemia, ceroid lipofuscinosis, Niman-Pick disease, Refsum's disease, Barde-Biddle syndrome, etc.) In these diseases, a slowly progressive decline in vision is observed in the context of massive polysystemic neurologic symptoms , which determines the clinical diagnosis.

Chronic increase in intracranial pressure, regardless of its cause, can lead to a slowly progressive decrease in vision even in the absence of a local effect on the visual pathways. These diseases are accompanied by a headache, edema of the optic nerve disk, an increase in the size of the blind spot. Focal neurological symptoms associated with a decrease in vision depend on the location and cause of the pathological process (tumors of the occipital or temporal lobe, other voluminous processes of this localization, pseudotumor cerebri).

Iatrogenic optic neuropathy can develop with prolonged use of certain drugs (levomycetin, cordarone, streptomycin, isoniazid, penicillamine, digoxin).

Here, such rare causes of acute and (or) chronically progressive decline in vision as the disease of Behçet are not described; radiation damage to the optic nerve; sinus thrombosis, fungal lesions, sarcoidosis.

Diagnostics

Clarification of the cause of slowly progressing visual impairment requires measurement of visual acuity, examination of the oculist to exclude eye disease, clarification of the nature of the limitation of the visual fields, neuroimaging inspection, study of cerebrospinal fluid, evoked potentials of different modalities, somatic examination.

[8], [9], [10], [11]