Eddie's pathology. Ectodermal dysplasia is the main signs of a genetic disease. Educational video on the anatomy of the trigeminal nerve and its branches

They are gaining great popularity. This is due to poor ecology, heredity, a large number of different infections, and so on. Among these defects is Adie syndrome (Holmes-Adie), which is most often observed in people with damage to the postganglionic fibers of the eye, which occurs due to a bacterial infection. In this case, the eye pupil partially or completely loses the ability to narrow. It takes on an oval shape or becomes uneven, and segmental damage to the iris is observed.

Description

Holmes-Ady syndrome - a disorder of a neurological nature, which is characterized by constant dilation of the pupil, the reaction to light of which is very slow, but a pronounced reaction is observed with a bright close dissociation. So, when approaching, the pupil narrows quite slowly or does not narrow at all, and returns even more slowly to its original size, sometimes within three minutes. When staying in the dark for a long time, the patient experiences pupil dilation. Bound Eydie syndrome in neurology with the fact that the bacterial infection contributes to damage to neurons in the cluster of nerve cells that are located in the part of the orbit that is located behind, and control the narrowing of the eye. In addition, people with this disease experience disruption of the body's autonomic control, which is associated with damage to nerve cells in the spinal cord. A person's ankle is twitching and sweating is impaired. The disease happens:

1. Congenital, which is characterized by dysfunction of the Achilles reflex, visual impairment when examining an object close up is not observed.
2. Acquired, which is caused by visual impairment when viewed closely, which is corrected by instilling miotic agents. Occurs after injuries, infections and poisoning.

Etiology and epidemiology

Adey's syndrome has not been fully studied to date; in some cases it is considered to be a symptom at the stage of development of autonomic failure. Most often, the disease occurs in women aged about thirty-two years. There are known cases of familial disease. National and racial characteristics do not play any role in this.

In most cases, the disease manifests itself unilaterally as mydriasis. This disease is quite rare; some sources indicate one case of pathology per twenty thousand people. These are most often single cases, but sometimes the anomaly can occur in entire families. Children very rarely get this disease. A large number of patients seek help between the ages of twenty and fifty. The origin of the syndrome is unknown; it appears in people who do not have any eye pathologies. In rare cases, the disease occurs due to trauma to the orbit, as well as as a result of metastasis of cancerous tumors into the orbit of the eye.

Causes

Eydie syndrome causes is unclear at present. What is known is that the ciliary ganglion, which is located in the orbit, is affected; this provokes an anomaly in the functioning of the muscles responsible for the ability to clearly see objects at a distance. The ability to see sometimes returns, but the pupil does not react to light. Doctors believe that the main reasons for this pathology are:

vitamin deficiency and infectious diseases;

congenital myotinia;

herpes eyes;

atrophy of the eye muscles, which progresses;

inflammation of the brain or its membranes.

Symptoms

Signs of the disease are three criteria:

1. One pupil is pathologically dilated and does not respond to light.
2. Loss of tendon reflexes
3. Sweating disorder.

Other symptoms of a disease such as Eydie syndrome may manifest themselves in photophobia, farsightedness, decreased vision, headache, and decreased leg reflexes. The size of the pupils may change throughout the day. At first one eye is affected, but after a few years the other eye may also be affected.

The disease manifests itself immediately after a severe attack of headache, then mydriasis begins to develop, vision deteriorates, and blurring appears when looking at objects that are close to each other. In most cases, loss of tendon reflexes and hyperthermia are observed.

Eydie syndrome: diagnosis

Diagnosis is possible using a slit lamp. A test is also carried out using Pilocarpine, a solution of which is instilled into the eyes and the pupils are observed for the next twenty-five minutes. In this case, the healthy eye does not show any reaction; in the affected eye, the pupil narrows. MRI and CT examinations are often performed. The ciliary nerves may become inflamed in the presence of diphtheria, especially in the third week of the disease. Then the pupils are restored. Eydie's syndrome is very similar to A. Robertson's syndrome, which is characteristic of neurosyphilis, so diagnostic measures are carried out to make an accurate diagnosis.

If a pathology of the pupil is observed, and the causes are unknown, an examination is performed using a slit lamp to exclude mechanical injuries to the iris, the presence of a foreign body, injury, inflammation, as well as adhesions, and glaucoma.

Treatment

Usually with an illness such as Eydie syndrome, treatment not developed. The therapy does not have the desired effect. Patients are prescribed glasses that correct disorders. Pilocarpine drops are also prescribed to correct an eye defect. Sweating disorders are treated using thoracic sympathectomy. This disease cannot be completely cured.

Prognosis and prevention

All pathological changes in a disease such as Eydie syndrome are irreversible, so the prognosis is somewhat unfavorable. But the disease is not life-threatening and does not affect a person’s ability to work. But in some cases, the disappearance of tendon reflexes can develop even further over time. Then both pupils are affected, become small in size and practically do not react to light. Treatment with Pilocarpine drops does not bring obvious results.

Preventive measures are aimed at timely treatment of infectious diseases, which often lead to complications. It is necessary to eat right and follow a daily routine. A healthy lifestyle helps reduce the risk of developing various pathological conditions in the human body.

Thus, this disease is incurable, only a decrease in the manifestation of symptoms is possible; over time, the disease can progress and lead to bilateral eye anomalies. But the disease is not life-threatening, since no deaths have been observed with this disease.

Dexter Fletcher's film has been released in Belarus - a film adaptation of a real story about one of the most famous losers and at the same time sports heroes.

Eddie Edwards is a chronic loser who once decided at all costs to compete at the Winter Olympics as a ski jumper, having virtually no training. Having overcome all difficulties, Edwards finally got to the games in 1988 and, as expected, took last place. Despite this, the clumsy guy with glasses and a funny mustache became the favorite of all fans and almost a national hero.

The creators of Eddie the Eagle make no secret of the fact that their film is only partially based on the beautiful story of Eddie Edwards' downfall. After watching the film and studying the real information in detail, we tried to figure out what from the film actually happened and what was made up for the sake of greater drama.

Is Hugh Jackman's character based on a real person?

No. The biography of Eddie Edwards never mentions ski jumper Bronson Pirie (Hugh Jackman), who was kicked out of the team and later became Eddie's coach. This character is mostly fictional. The film says that Peary was a promising athlete until he became addicted to alcohol. In reality, Eddie Edwards learned ski jumping in Lake Placid under the tutelage of two Americans, John Wiscombe and Chuck Berghorm. The film's screenwriter Sean McAuley said that Jackman's character was inspired by the stories of Eddie's trainers, but there is no direct connection between them and Bronson Pirie.

Is it true that Eddie Edwards had no experience in ski jumping?

No. The real Eddie was a much more experienced ski jumper than the guy we saw in the film. Edwards actually worked as a plasterer, but for some time he was interested in stunt jumping over cars and buses. In addition, he had extensive experience in alpine skiing, so at first he wanted to compete at the Olympics in this sport. By 1986, Eddie was practically broke, so he was desperately looking for a new type of activity. “I had no money at all, so I had to choose a sport that did not require expenses,” Eddie said in an interview. “I drove past the jumps in Lake Placid and thought: this looks good.” In the film, Eddie (played by Taron Egerton) was inspired to become a ski jumper by a poster hanging on the wall in his room.

Did Eddie really almost become disabled as a child?

Not really. Eddie was always a fearless child and constantly paid for his bravery. At the age of ten, he played on the football team as a goalkeeper and his hard playing style led to serious damage to his knee joint. As a result, he had to wear a cast for three whole years. Then Eddie began to learn to ski and joined the British national team at the age of thirteen.

Was the real Eddie an only child?

No. The real Eddie Edwards has a younger sister, Lisa. In 2007, Eddie saved her life by donating the bone marrow that the girl needed for a donor transplant.

Is it true that Eddie lived in a mental hospital for some time?

Yes. Although this was not shown in the film, the real story of the athlete does mention the fact that, in order to save money, he checked into a ward in a Finnish mental hospital. While there, Eddie learned that he had qualified for the British Olympic team. His haters later joked that his home suited him perfectly.

To earn some money, Eddie constantly took part-time jobs as a gardener, nanny, hotel porter and even a cook. The athlete drove his mother’s car, wore a helmet given to him by the Italians, and took skis from the Austrian team (in the film, Hugh Jackman’s character robbed a lost and found office to find equipment for Eddie). He wore six pairs of socks to keep his oversized ski boots on his feet. One day Eddie broke his jaw and instead of going to the hospital, he tied it with a pillowcase and continued to do his business.

Where did the nickname "Eddie the Eagle" come from?

When Eddie arrived at the Calgary Olympics in 1988, fans at the airport greeted him with signs that read, "Welcome to Calgary, Eddie the Eagle." This inscription was caught on Canadian television, so the nickname quickly became popular among the people. At the same time, the name Eddie appeared: in fact, the ski jumper’s name is Michael Edwards.

Was Eddie really as clumsy as he is portrayed in the film?

Yes. One of the funny incidents happened when he tried to go out to the fans greeting him in Calgary. “I walked towards the posters, but didn’t notice the glass door and crashed into it as hard as I could, breaking my skis,” Eddie said.

Is it true that Edwards only placed last in all competitions?

Yes. Eddie the Eagle finished last in all three jumps at the Calgary Olympics, but managed to break his own record. But more importantly, he completed the competition without a single injury, which can already be considered a success. “You all won our hearts. Some of you flew like eagles,” said games organizer Frank King at the closing ceremony.

Despite his defeats, was Eddie really a favorite of the public and the press?

Yes. This moment in the film is shown most accurately in comparison with the real story. Despite Eddie's lack of athletic talent, many Olympic spectators expressed sincere sympathy for him, because they saw him not as some kind of superhero, but as an ordinary British boy, just like the rest of them. “I think that I have become a kind of symbol of the sports spirit - a simple amateur guy made it to the Olympics only thanks to his sincere love for sports,” Eddie said. As in the film, this caused outrage in the circles of professional Olympians who devoted almost their entire lives to their sport. They believed that Edwards was unworthy of competing at this level.

How was Eddie Edwards' life after the Olympics?

After performing at the games in 1988, Eddie received invitations to various television shows, including the popular The Tonight Show with Johnny Carson. According to Eddie, thanks to contracts for these performances alone, he earned about 600,000 pounds sterling in a year. A plasterer by trade, Edwards returned to construction work for a while, but then there was a boom in motivational seminars and the former ski jumper also began making a living from it. He eventually sold the film rights to his life story. Eddie Edwards has long become part of British folklore as the heroic underdog who never gave up on his dreams.

The film "Eddie the Eagle" can be seen in all Minsk cinemas until April 20.

Anton Kolyago,ByCard

» , » Adi's syndrome

Adi syndrome

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Publication date: February 14, 2017

Adie's syndrome is a neurological disorder characterized by a tonic pupil that is slow to respond to light. Adie's syndrome is characterized by three main symptoms and signs, namely, a single abnormal pupil (mydriasis) that does not shrink in response to light, loss of deep tendon reflexes, and impaired sweating. Other signs may include farsightedness due to accommodative paresis, photophobia, and difficulty reading. Adi syndrome is not progressive and is not a life-threatening disorder.

In most people with Adi syndrome, the affected pupil is larger than normal and may not constrict normally in response to light stimulation. In some people with the syndrome, however, the pupil remains smaller than normal. Most people with Adi syndrome have poor or absent reflexes. Other symptoms and signs in a person with this syndrome include:

• Headache
• Facial pain
• Blurred vision
• Emotional fluctuations

The disease does not progress and usually does not cause severe disability. In Adi syndrome, the muscles that control the pupil remain tense. Lesions in a certain part of the third nerve cells can cause loss of reflexes.

Causes of Adi syndrome

Although the exact cause of Adi syndrome remains unknown, the disorder may be inherited in an autosomal dominant manner.

Treatment of Adi syndrome

The diagnosis of Adi syndrome can be made based on the results of the use of pilocarpine drops. The pupil of people with Adi syndrome, which does not contract in response to light, will constrict in response to pilocarpine. Other forms of treatment may include glasses, diluted pilocarpine therapy, and several other methods.

Eydie-Holmes syndrome (Eydie syndrome) is characterized by paralysis of the eye muscles, in which the ability of the pupil to respond normally to a light stimulus is completely lost. As a rule, Eydie syndrome manifests itself as unilateral mydriasis.

Women are susceptible to Eydie-Homes syndrome much more often than men. As a rule, it is observed in middle-aged people. There are often family cases.

Signs of the disease

A distinctive feature of Eydie syndrome is a decrease, and sometimes complete disappearance, of the pupil's ability to constrict in response to light. At the same time, the size of the pupils becomes different, with dilation and deformation of the affected pupil. With convergence (reduction of the visual axes to the center to view nearby objects), the affected pupil very slowly narrows and immediately expands when convergence stops. This effect is called pupillotonia. Vision in Eydie-Homes syndrome is also reduced.

Often this condition occurs after a severe attack of headache. At the beginning of the disease, the size of the pupil is very large, although over time it may become smaller. It is not uncommon for the pupil size to change throughout the day.

As a rule, Eydie's syndrome initially affects only one eye. However, there is a high probability of damage to the second eye over the next few years.

Often, Eydie-Homes syndrome is accompanied by impaired reflexes of the lower extremities.

Causes

The disease is characterized by damage to the cell bodies of the ciliary ganglion, localized in the orbit, and postganglionic fibers. As a result, there is a disruption of the innervation of the ciliary muscle, as well as the sphincter of the iris, which leads to paralysis of accommodation (with loss of the ability to clearly see objects at different distances) and the inability to constrict the pupil.

True, over time, the ability to accommodate sometimes returns, but the ability of the pupil to react to light is completely lost.

The exact cause of the disease is not clear to this day, just as the exact moment of development of the disease is not clear. Among the factors leading to the occurrence of Eydie-Homes syndrome, experts call vitamin deficiency, as well as infectious diseases. A similar condition was observed in diabetes mellitus, segmental hypohidrosis, Shy-Drager syndrome, amyloidosis, and diphtheria.

Diagnosis of Eydie-Homes syndrome

A diagnosis of Eydie-Holmes syndrome can be made by examining the eye using a slit lamp and performing a test with Pilocarpine. Performing this test involves instilling a pre-diluted solution of Pilocarpine (at a rate of 1:10) into both eyes, with further observation of the reaction of the pupils for 25 minutes. At this dilution, the Pilocarpine solution has no effect on a healthy eye, but a constriction of the pupil may be observed on the affected eye. In Eydie syndrome, the tonic pupil narrows due to the high sensitivity of the denervated sphincter to Pilocarpine.

Differential diagnosis of Eydie-Homes syndrome is carried out with Argyll Robertson syndrome, characteristic of neurosyphilis.

Prognosis and treatment

The prognosis of the disease is unfavorable due to the fact that such pathological changes of a functional and neuromuscular nature are irreversible.

To date, no specific treatment regimen for Eydie-Homes syndrome has been developed. Symptomatic therapy, in most cases, does not have the desired effect. In order to correct the cosmetic defect of a dilated pupil at least to some extent, the use of Pilocarpine is indicated.

At the Moscow Eye Clinic medical center, everyone can undergo an examination using the most modern diagnostic equipment, and based on the results, receive advice from a highly qualified specialist. We are open seven days a week and work daily from 9 a.m. to 9 p.m. Our specialists will help identify the cause of vision loss and provide competent treatment for identified pathologies. Experienced refractive surgeons, detailed diagnostics and examination, as well as the extensive professional experience of our specialists allow us to ensure the most favorable result for the patient.

This syndrome was first described by a British neurologist named William John Adie, who noticed that some people had a very slow reaction of the pupils to light and other stimuli, or it was completely absent.

A characteristic feature of Adie's syndrome (also known as Adie's syndrome or tonic pupil) is that the neurons of the ciliary ganglia degenerate. This leads to deviations in the innervation of the pupil and eyelash muscle. Which entails a deterioration in the connection between the organ and the central nervous system.

Reasons for violation

Eydie syndrome can be congenital or acquired. The causes that cause both variants of the syndrome are considered the same. The disorder most often manifests itself in middle-aged women, on average hovering around 30-35 years.

At the moment, not everything is known about Holmes-Eydie syndrome, but it is believed that the main reasons for its development are the following diseases:

• congenital;
• ocular herpes;
• progressive atrophy of the eye muscles;
• possible inflammation of the brain and its membranes (,).

The difference between the work of diseased pupils and a normally functioning organ is that they can react very slowly or not at all to changes in the light conditions around them. Also, the development of the syndrome leads to deterioration and blurred vision.

Mechanism of occurrence and development of the syndrome

As mentioned above, at the moment doctors cannot give a clear answer to the question of what exactly causes this syndrome. Based on numerous observations, it was concluded that its characteristic features are very similar to cases when a person experiences degradation in the functioning of the ciliary ganglia, which are responsible for connecting the muscles of the eye with the nerves located in it. The most likely causes of the appearance of a tonic pupil were listed above.

That is, the problem of the disease is clearly that the nerves and muscles in this system cease to perform their function correctly and are either unable to perceive signals from the outside world adequately, or are unable to transmit them to the central nervous system.

When a person becomes infected with infections that negatively affect the functioning of the ciliary ganglia, or they are exposed to destructive mechanical effects, for example, all kinds of injuries, then similar symptoms are observed, as in the case of Eydie syndrome.

Difference between Adie and Argyle syndromes

In the field of eye diseases of a neurological nature, the syndrome is also widely known. It is also characterized by the fact that the pupils lose the ability to respond to the presence of light sources. However, it still retains the ability to focus on objects at different distances and the ability to react to an approaching object.

Most often, the syndrome is diagnosed in patients who are ill. In the early stages of the disease, it can manifest itself rather weakly, when syphilis acquires a late, developed form and becomes obvious.

Adie and Argyll syndromes should not be confused with each other, as they are not the same thing. In the case of Argyle syndrome, the cause of the problem is clearly infection of the body with syphilis, that is, an infection. It is this that gradually develops and spreads throughout the body that leads to this result.

If we talk about Addie's syndrome, then the reasons for its occurrence lie on a completely different plane, although they have a very similar end result.

Clinic and diagnostics

The main symptoms that indicate the presence of a tonic pupil are:

1. Very slow reaction of pupils to changes in lighting. Even if you shine a bright light source, for example, a flashlight, directly into a person’s eyes, the same picture of reaction inhibition will be observed. In some cases, there may also be a complete absence of any reaction.
2. On the side where the pupil is susceptible to disease, it may be expanded and even deformed.
3. Slow response during convergence. There is no ability to focus the gaze on objects located at different distances. When the patient looks at a nearby object, his pupil narrows rather slowly.
4. Upon detailed examination, the following phenomena are observed: increased sensitivity to vagotropic poisons, paralysis of the iris sphincter.

The photo shows a clear lack of pupil reaction to light

If you have such symptoms, it is advisable to contact specialists who will diagnose and check whether the disease is actually present.

Two methods are used for diagnosis:

1. Slit lamp examination.
2. Use of the drug Pilocarpine. It is dripped into the eyes and the reaction of the pupils is observed after about 25-30 minutes. A healthy eye ignores this substance, but a sick eye will give itself away by constriction of the pupils.

These are the main methods for diagnosing this disease.

Principles of treatment

To treat the syndrome and reduce the manifestations of its symptoms, the following methods are used.

Treatment with the already mentioned Polycarpine. By regularly putting drops in their eyes, you can achieve some improvement in their condition. Doctors prescribe special reading glasses to the patient, which correct existing disorders.

If there are additional symptoms of the disease and associated abnormalities, other means and methods of therapy may be used.

It should be noted that the prognosis for this disease is positive - Eydie syndrome is safe and does not interfere with a person’s normal life. No deaths have been reported among patients with this disorder.