Horner Syndrome: Causes, Symptoms & Treatment

Horner syndrome refers to specific nerve damage that affects various eye muscles. The condition consists of a three-part symptom complex (a so-called symptom triad): this symptom triad includes a drooping upper eyelid, significant pupil constriction, and an eyeball that sinks into the eye socket.

What is Horner syndrome?

The trisection described above of pupil constriction, drooping eyelid, and sunken eye is characteristic of Horner syndrome. In addition to these directly visible symptoms, there may be impaired sweat production in the various regions of the upper half of the body. Horner’s syndrome is not considered a disease in its own right, but rather a partial symptom of many other diseases. Nevertheless, some findings indicate that various nerve damages are responsible for the occurrence of the symptom. The damaged nerves may be located throughout the body and may be caused by the different diseases. Familial and genetic causes are also no longer ruled out.

Causes

The hypothalamus (an important area in the human brain) is responsible for controlling muscles, which can lead to the symptoms typical of Horner syndrome. In order to travel from the hypothalamus to the eye and vice versa, the nerve pathways must travel a complicated circuit through the spinal cord. During this journey, the cranial nerves can be damaged at any time. Often, damage to the nerve pathways is due to circulatory problems (this is most often localized in the brainstem) or a brain tumor, which can act on and damage the affected nerves. Cancerous tumors at the top of the lungs and pathological cavities in the cervical medulla can also lead to nerve damage and thus trigger Horner’s syndrome. In addition, inflammation, localized in the middle cranial fossa, can also lead to the symptom triad of Horner syndrome.

Symptoms, complaints, and signs

In most cases, Horner syndrome is manifested by symptoms in the eye area on one side of the body, but not on both sides. The disease is noticeable in the area of the pupil, orbit, and eyelid. Thus, the function of the pupil is disturbed in Horner’s syndrome. Regardless of brightness, the pupil is always constricted. Therefore, patients complain that their vision is impaired in the dark because ambient light does not fall sufficiently on the retina. Due to the failure of the orbital muscle, the eyeball is slightly sunken into the skull. In healthy individuals, the muscle ensures that the eye protrudes slightly. Depending on the severity of the disease, this retraction of the eyeball may be more or less visible. Many patients suffering from Horner’s syndrome complain of a drooping eyelid. This symptom is due to a dysfunction of the Müller’s muscle. In addition to these three classic symptoms, there are other manifestations. They also occur only on one side of the face. Different colorations of the iris, pigment disorders or dilated vessels are indications of Horner’s syndrome. In some cases, patients do not sweat properly on one side of the face. This is referred to as a disorder of sweat secretion.

Diagnosis and course

The tripartite symptom complex is easy to recognize. In patients with Horner’s syndrome, the damaged pupil is proportionally smaller to the intact pupil, and the eyelid droops and can be lifted only minimally even with effort. In response to light, the pupil dilates slowly and usually incompletely in Horner’s syndrome. The other symptoms can also be clearly seen, for example, increased sweat secretion can be clearly observed on the skin in some regions of the body, while some regions have no sweat production at all. By administering cocaine and amphetamine eye drops, the damage can be localized as well as its extent. The physical impairment affects only the eye and the eye muscles. For example, due to the constriction of the pupil, the perceptual behavior of the eye can change in a negative sense, the visual field is reduced due to the drooping eyelid, and three-dimensional vision can be significantly impaired.Mostly, patients with Horner’s syndrome suffer especially from psychological stress, because the facial expression and the expression of the face can change significantly due to the symptomatology. In the further course, it must be concluded for what reason the symptomatology of Horner’s syndrome occurs and which other disease is responsible for it. Because only in this way can the symptomatology be treated effectively.

Complications

Horner syndrome causes significant discomfort to the eyes, which can have an extreme impact on the life of the affected person. In most cases, the pupils are greatly enlarged and the eyeball is retracted. Furthermore, the upper eyelids may also droop, negatively affecting the patient’s aesthetics. As a result of the cosmetic complaints, it is not uncommon for patients to develop inferiority complexes or a lowered self-esteem. It is not uncommon for patients to feel ashamed of the symptoms. In severe cases, it can also lead to visual impairment or other limitations of vision. These usually have a negative impact on the daily life of the affected person and also reduce the quality of life. Direct treatment of Horner’s syndrome and its symptoms does not take place in most cases. Instead, the underlying disease is always investigated and treated, with no particular complications. The complaints usually disappear when the underlying disease has also been defeated. Life expectancy is not altered or reduced by Horner’s syndrome. The disease does not disappear on its own in most cases, so symptoms increase without treatment.

When should you see a doctor?

If Horner’s syndrome is suspected, a doctor must be consulted promptly. Typical warning signs that need to be clarified are constricted pupils and a sunken eyeball, often associated with visual disturbancesm, pain in the area of the eye and excessive sweating. When these symptoms occur, there is most likely a serious underlying condition that needs to be investigated and treated if necessary. It is not always Horner’s syndrome, but medical clarification is necessary in any case. This is especially true if the symptoms seem to occur for no reason and persist over a long period of time. Individuals who have had a brain tumor in the past are particularly susceptible to developing Horner’s syndrome. Nerve disorders and syringomyelia are also possible causes of the syndrome. Those who consider themselves to be in these risk groups are best advised to talk to their doctor. In addition to the family doctor, an ophthalmologist or neurologist may also be called in. In the event of a medical emergency, emergency medical treatment is required.

Treatment and therapy

No specific therapy has been developed to treat Horner syndrome. This is mainly because many different causes can cause the suffering of Horner’s syndrome. With treatment of the actual disease – which is the cause of Horner syndrome – the tripartite symptoms are also relieved. Thus, the treatment is always individually tailored to the actual suffering of the patient and goes hand in hand with the treatment of the causative disease. If the treatment results in an improvement of the patient’s state of health, then in most cases there is also an improvement of the Horner’s syndrome. If relief of the symptomatology of Horner’s syndrome fails to occur, other causes for this symptomatology must be identified.

Outlook and prognosis

To date, no treatment method exists that can specifically and completely cure Horner syndrome. Patients and physicians must therefore broaden their perspective and search for causes. The causes of Horner syndrome can often be eliminated. If this is successful, the three typical symptoms of miosis, ptosis and enophthalmos usually disappear. In general, the probability of a positive outcome increases if the disease is treated early. If no therapy is given, the symptoms increase. Horner’s syndrome itself does not affect life expectancy. Sometimes the nerve is severed by the underlying disease. Affected persons must then cope with Horner’s syndrome for the rest of their lives. A cure is impossible. This prospect plunges many patients into a deep inner conflict. As facial expressions change, they often suffer from lowered self-esteem.The aesthetic effects, which among other things cause the drooping eyelid, can hardly be hidden. In this situation, there is a risk of developing a long psychosis. The quality of life suffers. To start from this situation back to everyday life is impossible for many without outside help.

Prevention

There is little the patient can do to prevent Horner’s syndrome. Horner’s syndrome develops due to another disease, the development of which patients usually have no influence on. After all, a tumor cannot be predicted and eventually avoided, nor can a circulatory disorder or a motorcycle accident with damage to the arm nerve plexus, which can also lead to the symptomatology of Horner syndrome.

Follow-up

In Horner syndrome, the options and measures of follow-up care are very limited in most cases. Since it is also a congenital disease, this disease can also not be treated causally, but only purely symptomatically. If the patient wishes to have children, genetic counseling can also be performed to prevent inheritance of the disease. The main focus in Horner’s syndrome is therefore the early detection of the disease, so that it does not lead to a further worsening of the symptoms. The treatment of Horner’s syndrome depends primarily on the exact underlying disease. It is not always possible to achieve an improvement. If the disease leads to psychological upsets or depression, the loving care of one’s own family and friends has a very positive effect on these complaints. Regular check-ups by a doctor are also very important. If the syndrome is treated by taking medication, it is important to ensure that it is taken correctly and regularly. Possible interactions with other medications should also be taken into account. In children, especially the parents should pay attention to a correct intake.

What you can do yourself

The patient cannot contribute to the treatment of Horner’s syndrome himself. In any case, the affected person is dependent on medical treatment to limit the symptoms of the syndrome. Preventing the syndrome is also usually not possible. In many cases, patients need support and psychological treatment. This can come from their own friends and family, but should also be accompanied by visits to a psychologist. In the case of children, education about the disease and its possible consequences must be provided to ensure the child’s psychological stability. Conversations with other sufferers can also help in this regard. Due to the visual complaints, the patients are dependent on the help of other people in their everyday lives. In this context, warm and cordial care has a very positive effect on the course of Horner’s syndrome. Due to the disturbed sweat production, patients should wear airy and light clothing, especially in summer, to avoid sweating and thus unpleasant situations. The disturbances of blood circulation can be limited in some cases by massages or heat applications. However, a complete cure for Horner’s syndrome is not possible.