Horner's syndrome: what is it, symptoms, treatment, prognosis

What is Horner's Syndrome?

Horner's Syndrome (Bernard-Horner syndrome, oculosympathetic syndrome) Is a rare disease characterized by miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), agidrosis (absence sweating of the face) and enophthalmos (deeper than normal immersion of the eyeball into the bone cavity (orbit), protecting the eye). These are the four classic hallmarks of the disorder.

The syndrome is caused by damage to the sympathetic nerves in the face. The underlying causes of Horner's syndrome vary greatly and can include anything from a snake or insect bite to a neck injury. blunt force, swelling, stroke and underlying diseases affecting areas surrounding sympathetic nerves.

In rare cases, oculosympathetic syndrome is congenital (present from birth) and may be associated with a lack of pigmentation in the iris (the colored part of the eye). Treatment for Horner's syndrome depends on the underlying cause.

Symptoms and Signs

The characteristic physical signs and symptoms associated with oculosympathetic syndrome usually affect only one side of the face. These include:

• drooping upper eyelid;
• constriction of the pupil;
• dryness (no sweating) on ​​the same side of the face (ipsilateral) as on the affected eye;
• and deeper than normal position of the eyeball in the orbit.

If Horner's syndrome occurs before the age of two, the colored parts of the eyes (iris) can be different colors (heterochromia). In most cases, the iris on the affected side lacks color (hypopigmentation).

Causes of Horner's Syndrome

Oculosympathetic syndrome can be caused by any of a variety of factors. The main reasons are described in the table below:

Lesions of the central (first order) nerve	Preganglionic (second order) nerve lesions	Lesions of the postganglionic (third order) nerve
Cerebrovascular injury.	Apical lung tumors (eg, Pancost's tumor).	Cluster headaches or migraines.
Multiple sclerosis.	Lymphadenopathy (lymphoma, leukemia, tuberculosis, mediastinal tumors).	Shingles.
Tumors of the pituitary gland or basal skull.	Injury to the lower brachial plexus or cervical rib.	Internal carotid artery dissection can be traumatic.
Basal meningitis (for example, as a result syphilis).	Aneurysms of the aorta, subclavian or common carotid artery.	Raeder's syndrome (paratrigeminal syndrome).
Neck injury (eg, dislocation of the cervical spine or dissection of the vertebral artery).	Trauma or surgical injury (neck or chest).	Carotid-cavernous fistula.
Syringomyelia.	Neuroblastoma.	Temporal arteritis.
Arnold Chiari Syndrome.	Dental abscess lower jaw.
Spinal cord tumors.

In most cases, the physical signs associated with Horner's syndrome develop from the interruption of the sympathetic nerve supply to the eye due to injury or swelling. The lesion develops somewhere along the path from the eye to the region of the brain that controls the sympathetic nervous system (hypothalamus). The sympathetic nervous system (in combination with the parasympathetic nervous system) controls many of the involuntary functions of the glands, organs, and other parts of the body.

Some cases of Horner's syndrome occur for no apparent or known cause (idiopathic). In other cases, some clinical researchers believe that the disorder may be inherited as an autosomal dominant genetic trait.

Chromosomes present in the nucleus of human cells carry the genetic information of every person. The cells of the human body usually have 46 chromosomes. Pairs of human chromosomes are numbered 1 through 22, and the sex chromosomes are labeled X and Y. Men have one X and one Y chromosome, while women have two X chromosomes. Each chromosome has a short arm, labeled "p", and a long arm, labeled "q". Chromosomes are further subdivided into many numbered bands. For example, "chromosome 11p13" refers to lane 13 on the short arm of chromosome 11. The numbered stripes indicate the location of the thousands of genes present on each chromosome.

Genetic diseases are defined by a combination of genes for a specific trait found on chromosomes obtained from the father and mother.

All humans carry several abnormal genes. Parents who are close relatives (by blood, i.e. if they are siblings) are more likely than unrelated parents, have the same abnormal gene, which increases the risk of having children with recessive genetic disorder.

Dominant genetic disorders occur when only one copy of an abnormal gene is needed for a disease to appear. An abnormal gene can be inherited from either parent or be the result of a new mutation (gene change) in a sick person. The risk of passing the abnormal gene from the affected parent to the offspring is 50% for each pregnancy, regardless of the sex of the child.

Affected populations

Oculosympathetic syndrome is a rare disease that affects men and women in equal numbers and occurs at any age among any ethnic group in any geographic location.

Diagnostics

Bernard-Horner syndrome is suspected by a doctor based on symptoms.

To confirm the diagnosis of Horner's syndrome and to find out the location of the injury, the doctor conducts a study, which consists of two parts:

1. First, the doctor instills drops with a small amount of cocaine or apraclonidine into both eyes.
2. If oculosympathetic syndrome is possible, then after 48 hours the doctor will conduct another study. In both eyes, he instills drops of hydroxyamphetamine.

The reaction of the pupils to the drug can help determine if oculosympathetic syndrome is likely and help locate the lesion.

To rule out tumors and other serious diseases that can destroy the nerve fibers that connect the brain to the eye, the patient undergoes magnetic resonance imaging (MRI) or computed tomography (CT) of the brain, spinal cord, chest, or neck.

Symptomatic Disorders

Symptoms of the following disorders may be similar to those of Horner's Syndrome. Comparisons can be useful for differential diagnosis.

• Adi syndrome (also called Holmes-Adi syndrome or Adi's pupil tonic) is a rare neurological disorder that affects the pupil of the eye. Symptoms of the syndrome are a large (dilated) pupil and a slow response to light or focus on nearby objects. In some patients, the pupil may be smaller rather than dilated. Lack or poor reflexes are also associated with the disorder.
• Wallenberg-Zakharchenko syndrome (dorsolateral medullary syndrome, lateral medullary infarction syndrome) is a rare disease caused by a blood clot (blood clot). It is characterized by difficulty in pronouncing words due to a disease of the central nervous system, difficulty swallowing, staggering gait, dizziness, low fluid pressure in the eyeball, giving it a rounded shape, lack of coordination during voluntary movements, rapid involuntary movement the eyeball, signs of Horner's syndrome on the side where the lesion is present, and loss of pain and temperature sensations on the side of the body opposite defeat.

Horner's syndrome treatment

In general, the appropriate treatment for Horner's syndrome depends on the underlying cause. The goal of treatment is to eliminate the underlying disease, injury. However, in many cases, there is no effective treatment. Rapid recognition of the syndrome and appropriate referral to appropriate specialists is vital.

Whether surgery is required and what type of surgery is needed depends on the specific cause of Horner's syndrome. Potential surgical interventions include neurosurgical treatment of oculosympathetic syndrome associated with with aneurysm, and vascular surgery for causative conditions such as dissection of the carotid artery or aneurysms.

Genetic counseling is helpful for patients and their families if they have a genetic form of the disorder. Other treatments are symptomatic and supportive.

Forecast

The prognosis of Horner's syndrome also depends on the underlying cause. The characteristic symptoms of oculosympathetic syndrome usually do not significantly affect quality of life or vision. However, they can indicate the presence of a serious or even life-threatening medical condition that requires immediate medical attention.