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CSR - Central Serous Retinopathy

Adapted from: Central Serous Retinopathy

What is CSR?

CSR is a condition which causes temporary or permanent impairment of vision. The symptoms are loss of sensitivity in dim light, usually in an oval shaped gray or brown area, blurring or distortion of the visual image. It is a result of the detachment of most of the layers of the retina (the back of the eye) from its supporting tissue as a result of the buildup of fluid. The buildup of fluid appears to be due to one or more small breaks in the retinal pigment epithelium.

The term "idiopathic CSR" which you may see used simply means that the cause is not known. It affects primarily males between 20-45 and is associated with stress. For example, airline pilots suffer from it at a high rate, and some people find that attacks go away if they go on vacation. There have been some attempts to classify different forms of the disease but they are tentative and not linked to different causes or treatments.


Usually the fluid build up disappears after a few weeks to a few months with little long term damage. During this time the condition often fluctuates wildly on a daily or hourly basis. It particularly gets worse when under stress, tired or ill.

However recurrences are quite common and damage can accumulate over time. Significant, disabling damage can accumulate over time. If the detachment persists for too long the retina is starved of nutrients and can be damaged permanently, with a permanent gray area or a blind spot. The likelihood of recurrences is higher if the initial attack is more severe.

There are wide variations in the estimates of recurrence rates. Opinions also vary on the risk of the condition occurring in the other eye. Often patients are told that recurrences are rare, and that there is no need for concern. While this may be true in many cases, there is reason to doubt that this complacent attitude is correct.

A long term study of patients with CSR found that 50% of patients get the severe and extensive form of the disease after 12 years of evolution (Reference 1). It may be that the complacent attitude of clinicians is a result of the fact that they provide no effective treatment, so patients drift away and there is no long term visibility to the evolution of the condition.

Standard Treatment

There is no effective clinical treatment for the condition.

Laser treatment has been used but research results suggest that the treatment does not improve the long term outcomes. The laser therapy effectively 'burns' the leak shut, but can cause damage.

It can be used to overcome the immediate problem, for example if an attack is preventing studying for examinations. This may also be useful if an attack goes on for a long time - more than a couple of months, depending on how severe the attack is. A long standing attack can cause permanent damage to the retina and in this case laser treatment may be a good idea as a way to prevent permanent damage.

Recently more refined laser treatments have been used as certain frequencies of light cause less damage than standard frequencies.

Stress and CSR

CSR is associated with stress. Individuals who report being under stress and people who are in objectively stressful occupations such as airline piloting are susceptible to CSR. Stress includes pain, infection, excessive heat and cold, low blood sugar, excessive exercise, hunger and crash diets, jet lag, lack of sleep, psychological stress and also very intensive exercise.

Stress causes the adrenal cortex to secrete cortisol and this may be the link between stress and CSR. There have been some studies which suggest that the link is instead due to the increased adrenaline that comes with stress but these studies appear to be flawed due to lack of control for blood pressure.

High endorphin levels (a result of pain or excess exercise) or opiate levels (from use of heroin or morphine as drugs or as pain killers) are also associated with CSR.

CSR sufferers have usage rates for tranquillizers much higher than the general population, are more susceptible to high blood pressure, and are more likely to be users for cortisone steroid medications.

High cortisol levels are often but not always a result of stress. See below for the linkage between cortisol and CSR.

Cortisol and CSR

What is Cortisol?

Cortisol is a hormone secreted by the adrenal cortex which assists the body to deal with various stresses. It reduces inflammation and immune system function and triggers the breakdown of protein into sugars.

A certain amount of cortisol is necessary for life. Without cortisol even a small amount of stress will kill you. Addison's disease is a disease which causes low cortisol levels, and which is treated by cortisol replacement therapy.

Cortisol Associated with CSR

CSR is associated with high cortisol levels. That is, people with high cortisol levels are more likely to suffer from CSR, and people with CSR generally have high levels of cortisol.

CSR sufferers have high levels of cortisol made by their own adrenal gland (50-80% higher than the average, and outside the normal range) (Reference 3).

CSR is also associated with treatment by corticosteriods ("cortisone") for other conditions such as allergy and inflammation. These drugs go under names like Hydrocortisone, Cortisone and Prednisolone. There have been several cases where CSR has recurred during each of several courses of treatment with cortisone drugs and gone away each time the treatment was stopped. Usually, doctors will claim that nasal sprays and skin creams are not absorbed into the body and will not therefore cause adverse side effects. However in a number of cases this information has turned out to be unreliable and CSR attacks have ceased when the treatment was terminated. Doctors do sometimes have a psychological tendency to believe that their treatments are more effective, less painful and less damaging than they really are.

CSR is also associated with pregnancy (which generates very high cortisol levels in some cases). The high levels are cortisol in pregnancy and the body's need to protect against this may explain why women are less prone to getting CSR than men.

The association of objective and subjective stress with CSR also points to cortisol because stress raises cortisol levels. In addition, a high level of CRF, the hypothalamic hormone which drives cortisol levels, causes a subjective experience of stressfulness.

The incidence of CSR in people suffering from Cushing's syndrome is about 5%, a very high level. Cushing's syndrome consists of very high cortisol levels usually caused by a tumor in the pituitary gland or in the adrenal glands. Again this confirms the linkage between cortisol and CSR, but also suggests that high cortisol is not enough to cause the disease on its own. Presumably some other weakness plays a part.

Adverse Effects of Excess Cortisol

Cortisol is a powerful chemical and has numerous adverse effects in excess. It is therefore recommended that cortisone medication be taken in as small a dose as possible for as short a time as possible. A partial list of the adverse effects of cortisol follows:

Controlling Cortisol Levels

Cortisol Regulation by the body

The body's control of cortisol levels is complex. The PVN area within the hypothalamus secretes a substance called CRF (corticotropin releasing factor). This is picked up by the pituitary gland which then secretes ACTH (adrenocorticotropic hormone). In turn this causes the adrenal cortex to secrete cortisol.

The hypothalamus acts as the body's stress detector and drives cortisol production in this way as a response to stress.

Negative feedback mechanisms exist between the body's cortisol levels and the pituitary and hypothalamus to keep the cortisol levels within reasonable bounds in normal circumstances. The negative feedback is more effective in controlling high cortisol that results from psychological stress than high cortisol that results from physical damage such as blood loss or illness.

One unfortunate fact about the body is its tendency to resist any change. For example if you go in a diet the body increases subjective hunger and decreases metabolic rate, thus 'helping' you not to lose weight. The same thing applies with many of the techniques to reduce cortisol levels. They may work for a while but then the body adapts and levels return to the original values. So it may be necessary to keep revising your strategies.

Factors that elevate cortisol

Drugs like caffeine and nicotine tell the hypothalamus that you are under stress, leading to increased cortisol levels, as well as increased adrenaline levels. The increase in cortisol levels from consuming 4-5 cups of coffee per day may be of the order of 50-60% and the increase is highest in people who are already prone to high levels of cortisol. There are anecdotal reports that other stimulants are also associated with CSR attacks e.g. ephedrine (found in decongestants and herbal weight loss preparations).

Stress increases cortisol levels. This includes all the forms of stress described above. However the body is better able to control cortisol levels that result from psychological stress than those that result from physical stress.

Cortisone steroid drugs mimic the effect of high cortisol levels.

There is a lot of evidence that a stressful childhood leads to a permanently increase susceptibility to stress and increased CRF levels from the hypothalamus leading to higher cortisol levels. The stresses in childhood can range from separation from the mother through to physical and sexual abuse, hunger and disease. This can sensitize the hypothalamus and make it "trigger happy".

Stress in CSR Sufferers

While many CSR sufferers live objectively stressful lives as pilots etc, others suffer from high levels of subjective stress although their lives do not appear to be inherently stressful. For these people, it is not entirely clear why they would have high levels of subjective stress.

This may be due to an inherent metabolic tendency to over-produce adrenaline and cortisol which produce subjective feelings of stress via brain receptors for those hormones.

Another explanation is that a stressful childhood can lead to an overactive hypothalamic stress detector, leading to the production of adrenaline and cortisol in relatively normal circumstances. There is considerable animal research to suggest this does occur.

It is also possible that the person may have poor strategies for dealing with stresses. Studies have suggested that many CSR sufferers have 'Type A' personality which describes a tendency to be unable to relax. Do not assume that the cause is psychological though. In the past many conditions thought to be of psychological causes have turned out to have significant or dominant physical causes, for example severe depression and schizophrenia. In a sense then, psychological explanations are the explanation of last resort for the clinician who has no answers, and often amounts to a strategy of 'blame the patient'. The so-called 'schizophrenogenic mother' is a notorious example of this syndrome.

Having said that, there are a lot of effective stress management techniques available. See below.

Finding Someone Qualified

The condition is fairly unusual so it is difficult to find someone who is fully qualified to treat it, or even to diagnose it. It is quite common for serious and obvious illnesses to go undiagnosed for years. A recent study found that the average time to diagnose Cushing's Syndrome, a very serious illness sometimes associated with CSR, was 3 1/2 years.

There are several conditions that can be confused with CSR, such as Age Related Macular Degeneration and diabetic retinopathy, which are more common. It is important to have the diagnosis confirmed and tests are available to verify the leakage of fluid that is characteristic of CSR.

A retinologist, not just an ordinary ophthalmologist, can diagnose the condition. Because the condition initially causes a quite subtle loss of vision, an ophthalmologist may even tell you that there is nothing wrong.

You should then be able to get your cortisol tested without too much trouble to confirm the link with high cortisol in your case, unless you are using cortisone medication or are already aware of a high cortisol level.

In theory the best person for dealing with the high cortisol is an endocrinologist. However the levels of cortisol in CSR are generally not high enough to qualify as a case of Cushing's syndrome. Cushing's syndrome is the classic high cortisol disease. As anti-cortisol drugs can have adverse side-effects, you are likely to find a high degree of reluctance to provide any treatment.

This is very frustrating. Doctors prescribe cortisone treatments quite freely, even though it has severe side effects. On the other hand there is a high degree of reluctance to prescribe anti-cortisol treatments even when cortisol levels are high, in the name of 'accepted clinical practice'.

The other problem with endocrinologists is that in most cases of CSR the root cause of the high cortisol seems to be the hypothalamus which is part of the brain, and so is not in the jurisdiction of the endocrinologist. The brain is also far more complicated than the endocrine system.

Potentially a neurologist may be able to help if, as appears often to be the case, the condition is driven in many cases by an overactive hypothalamus. However you will probably find an even greater reluctance to do anything about the hypothalamus than is the case for the adrenal gland and its cortisol. The hypothalamus is very complex and plays a critical role in many vital body functions.

If you have high cortisol levels but do not have the tumor characteristic of Cushing's disease, your condition is likely to be labeled as "pseudo-Cushing's syndrome". The term is unfortunate because the high cortisol and the damage that results, is just as real as in the real thing. Pseudo-Cushing's syndrome is believed to be due to excessive alcohol consumption or psychological depression or stress. However a recent study has established that a significant proportion of the population has a genetic predisposition to high cortisol levels, so it may not all be in your mind after all.

On the positive side, practitioners will most likely find your condition interesting.

What you can do

Drug Treatment

There are not many good drugs to control cortisol levels. They all seem to have side effects. The most benign seem to operate on the adrenal, but have the problem that they indirectly cause the pituitary to emit larger amounts of ACTH, which may not be good because ACTH drives the production of other hormones, which may cause fluid retention or masculinization. There is some research going on in this area because high cortisol levels seem to speed the progress of AIDS.

The following drugs have been suggested as possible aids to CSR treatment. Note that none can be considered proven treatments and all may have side effects, and should be used only with great caution if at all.



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