Optic atrophy is the medical term for the degradation of optic nerve cells that can be present as part of various primary diseases. The autoimmune disease multiple sclerosis is one of the most common causes of optic nerve atrophy and resulting ocular atrophy. Treatment of atrophy depends on the cause.
What is optic atrophy?
Optic atrophy is a degenerative disease of the optic nerve. Along the optic nerve, nerve cells break down as part of the disease. The cell loss can lead to blindness. Optic atrophy is thus one of the most common causes of blindness. In adolescents and children, the disease usually progresses much more rapidly and severely than in older individuals. Several forms of atrophy are distinguished according to cause and appearance. Simple optic atrophy is characterized by sharp borders and is usually non-inflammatory. In contrast, the secondary forms are often preceded by inflammation. Incomplete atrophy degrades the optic nerve only in sections and is characterized by temporal optic disc blanching. To be distinguished from these three forms is the hereditary form, which can occur in the context of various hereditary diseases. Either the nerve tissue atrophy is unilateral or bilateral. The nerve fibers may be affected by the degradation processes as well as the surrounding myelin sheath.
Causes
In principle, all lesions and diseases of the optic nerve can cause atrophy. Increased intracranial pressure with congestive papillae is as likely to be a trigger as a traumatically induced optic nerve lesion or glaucoma. Just as often, optic neuritis or ischemia precedes the degenerative manifestation. Nerve compressions are also conceivable, such as those associated with superior optic nerve fissure syndrome, optic nerve sheath meningioma, or tumor in the optic nerve junction. Toxic causes due to alcohol, tobacco, quinine, arsenic, lead, or bromine should be distinguished from these causes. In turn, the hereditary form of optic atrophy may occur in the setting of conditions such as Behr syndrome I, motor-sensitive neuropathy VI, limb-girdle dystrophy 2O, or Tay-Sachs syndrome, Cohen syndrome, and metachromatic leukodystrophy. When primary atrophy is present, dysfunction in subcellular tissues is the cause.
Symptoms, complaints, and signs
The symptomatology of optic atrophy differs depending on the cause. For example, the cause determines whether the optic nerve degenerates unilaterally or bilaterally. Subjective perception of symptoms also varies, ranging from subjectively perceived lack of symptoms to visual loss and visual field loss. Visual field loss is the most characteristic symptom of the disease. These deficits vary in severity. In most cases, they are due to a progressive loss of central visual acuity, which may progress to blindness. The color perception of those affected is often disturbed. The same applies to the dark adaptation of the eyes. Sometimes there is an additional disturbance of the pupil reaction. Especially hereditary disorders show a progressive course, which often leads to amaurosis in the terminal phase. Patients usually do not complain about pain. However, mild pain is not excluded, especially in the case of atrophies due to inflammation.
Diagnosis and course of the disease
Diagnosis of optic atrophy includes a history, visual acuity testing, and perimetry. Funduscopy can also help the physician make a diagnosis. The optic nerve and orbital or intracranial structures are visualized with an imaging technique such as MRI. Possible causes such as space-occupying lesions can be seen from the imaging. The back of the eye is examined via ophthalmoscopy. In this procedure, optic atrophy usually shows a fade associated with discoloration of the optic disc. The course of the disease depends on the cause. Hereditary progressive forms of atrophy often have a rather unfavorable prognosis. This form particularly often leads to blindness. Optic nerve damage that has already occurred is irreversible. However, depending on the cause of the disease, it may be possible to prevent further damage in the future. For the physician, optic atrophy sometimes has diagnostic value as a symptom of various diseases.
Complications
In general, the complications and also the symptoms of optic atrophy depend very much on its cause. However, in any case, those affected by this disease suffer from visual complaints. The optic nerve and the associated cells are degraded, so that visual complaints or, in the worst case, complete loss of vision can occur. Losses in the visual field can also occur due to optic atrophy and continue to significantly reduce the patient’s quality of life. Similarly, impaired perception of different colors may occur during blindness. However, no particular pain or other complications occur. Especially in young people or in children, blindness can lead to severe psychological discomfort and also to depression. For this reason, these are also dependent on psychological treatment in the case of optic atrophy. However, the life expectancy itself is not affected by optic atrophy. A direct treatment is not possible. Especially after blindness, visual acuity cannot be restored. If optic atrophy occurs due to an inflammation, it can possibly be treated with the help of antibiotics. Tumors are removed by radiation therapy in this case.
When should you see a doctor?
When there is a fading of nerve cells on the optic nerve in one eye, a visit to the doctor is essential. The right person to see is an ophthalmologist. Optic atrophy causes visual field loss in both mild and severe cases. WEs comes to a loss of visual acuity. Regardless of whether the sequelae are severe or not, a visit to the doctor is advisable because optic atrophy is progressive. The diagnosis cannot be made without a specular examination of the back of the eye. Then the search for the cause of the disease begins. Optic atrophy is caused by such diverse causes as inflammation of the optic nerve, increased intracranial pressure, and also chronic alcohol or tobacco intoxication. It is important to treat the causes of optic atrophy as well. Therefore, they must be determined if possible. Whether the atrophy of the nerve cells occurred as a result of or during another disease is secondary for treatment. An MRI scan may provide information on how far the optic atrophy has progressed. At the end of the progressive degeneration of the nerve cells, blindness occurs. A problem is that optic atrophy can also occur in children. In this case, the damage to the nerve cells is usually rapid. Therefore, the ophthalmologist should be consulted even earlier in children than in adults. Early detection and prompt treatment play a supporting role in optic atrophy.
Treatment and therapy
Because central nerve damage is irreversible, no complete cure option exists for optic atrophy that has already occurred. Treatment depends on the causative disease. While some hereditary forms of atrophy are not treatable, other causes of degeneration can be treated causally or even cured. Optic neuritis in particular is usually at least symptomatically treatable. In most cases, the primary cause of optic neuritis is the autoimmune disease multiple sclerosis, which has not yet been treatable causally. However, an inflammatory MS attack on the optic nerve can often be interrupted by drugs such as cortisone in order to save the nerve. However, this does not rule out the recurrence of the inflammation. In the case of bacterial infections of the optic nerve, the physician administers liquid antibiotics to control the damage to the optic nerve. Causative tumors are removed as far as possible or treated by radiation therapy. If, on the other hand, increased intracranial pressure has caused the optic atrophy, a reduction of the pressure is induced, for example by draining the cerebrospinal fluid. For causative glaucoma, drug therapies and laser treatments are therapeutic options.
Outlook and prognosis
In optic atrophy, it is important to treat the causative underlying disease as soon as possible. This is the only way to avoid irreparable damage. As a result, the prognosis is unfavorable in the absence of treatment. Damage to the optic nerve is irreparable. They ensure that the ability to see is permanently severely limited.However, the question now is, by which factors optic atrophy has occurred. All treatment options, and also the expected prognosis, are defined by the triggering underlying disease. This can be tumors, increased intracranial pressure, alcohol poisoning, viral diseases of the eye or other triggers. Because of the diversity of triggers, an identical prognosis cannot be given for all cases. The prognosis is initially based on the general health of the eye. In addition, it depends on the fast and successful treatment of the triggering disease, which affects the optic nerve. The therapy of a viral disease is protracted. However, according to a study, Leber’s hereditary optic neuropathy can be successfully treated with the drug Idebenon. This is evidence that an underlying disease treated early and consistently can keep the extent of the optic atrophy triggered by it low. The death of the nerve cells on the optic nerve cannot be prevented. But if therapy is started early, the patient’s usual vision can be largely preserved.
Prevention
Optic atrophy cannot usually be prevented. This is especially true when the degenerative phenomenon occurs in the setting of hereditary diseases or multiple sclerosis.
Aftercare
Affected individuals have very few aftercare measures available in most cases of optic atrophy. First and foremost, a physician must be contacted early in this disease to prevent other compilations or further worsening of symptoms. As a rule, self-healing cannot occur, so that those affected by this disease are always dependent on medical examination and treatment. The earlier a doctor is consulted, the better the further course of the disease usually is. Those affected are dependent on taking various medications. The correct dosage with regular intake must always be observed in order to alleviate the symptoms permanently and, above all, in the long term. Regular checks and examinations by a doctor are also very important, as this monitors and controls the current condition of the body. In many cases, however, those affected are dependent on the help and care of their own family. Here, loving conversations are also very important, as this can prevent depressive moods or other mental illnesses.
This is what you can do yourself
Patients with optic neuropathy need to wear appropriate visual aids. To preserve or even improve the eyes’ ability to see, vision training and targeted eye protection are recommended. Anyone who sits in front of a screen for several hours a day should take a break once or twice an hour. Eye drops can help to relax the eyes. Affected individuals should talk to their doctor about supportive measures and implement them in a targeted manner. Although optic atrophy cannot be treated causally, the above-mentioned measures can stabilize visual acuity and thus also improve well-being in the long term. People who suffer from vision problems or other complaints such as headaches or dizziness despite wearing glasses should inform their doctor. There may be another condition that needs to be diagnosed. In the case of optic atrophy, the eyes should also not be exposed to strong external stimuli such as direct sunlight or drafts. Hygiene measures such as regular dusting and a weekly change of bed linen help to protect the eyes and prevent further deterioration of vision. In addition to glasses, other options include contact lenses or surgery to improve visual acuity via laser treatment. After such an operation, it is essential to follow the doctor’s instructions to avoid permanent damage to the eye and to ensure optimal recovery.