The Garcin syndrome is the progressive paralysis of the V. to XII. Cranial nerve on one side of the brain. The disease has different causes.
What is Garcin Syndrome?
The Guillain-Garcin syndrome, Garcin syndrome for short, describes a neurological disease that is very rare in its full expression and is associated with the progressive, unilateral paralysis of the cranial nerves V – XII. Since the affected cranial nerves emerge at the base of the skull, Garcin syndrome is also called half -base syndrome.
The paralysis of the nerves occurs ipsilaterally, i.e. only on one side of the brain, in the area of the transition from the middle to the posterior cranial fossa. Garcin syndrome occurs as a complication of various diseases of the skull base. The symptoms that occur depend on the failing cranial nerves, although the sequence of paralysis can vary.
Other cranial nerves may also be affected. The syndrome was first described in 1926 by the French doctors Georges Guillain and Raymond Garcin, who also served as namesake.
Causes
Various tumors, swellings and inflammatory processes in the area of the skull base can be the cause of Garcin syndrome. The most common triggers of paralysis are malignant carcinomas and sarcomas, which are located in the roof of the pharynx, in the sphenoid sinus or in the ear and break into the base of the brain.
Also nasopharynx, ie tumors that are located in the nasopharynx can infiltrate into the cranial nerves. Tumors metastasizing to the spinal cord or brain are also possible causes. Inflammatory diseases are less common as a cause of Garcin syndrome.
The pachymeningitis, an inflammation of the outermost meninges can attack the brain nerves. This occurs as a result of a general inflammation of the middle ear and the paranasal sinuses, for example due to an untreated otitis media.
Fungal sinus infections can also spread to the brain in severe cases. This rhinocerebral form of mucormycosis can also lead to paralysis of passing cranial nerves. Carotid aneurysms are common around the base of the brain. In rare cases, these can also lead to semi-basal paralysis.
Symptoms, ailments & signs
The occurrence of symptoms in Garcin syndrome depends on the starting point of the source of the cause and its direction of spread. Depending on the sequence of paralysis of the cranial nerves, deficits occur in the head space.
Typical are visual and hearing disorders, balance problems, swallowing and speech difficulties, impaired taste perception and numbness of the facial muscles up to facial paralysis. The mobility of the head and shoulders can also be restricted. Depending on the cause of the appearance of the garcin symptom, a disease can be asymptomatic for a long time or with unspecific symptoms.
Often numbness, hypersensitivity or pain in the sciatica, as well as pain in the lower back and morning stiffness and also the sacralization of the fifth lumbar vertebra with the sacrum have been described. Disturbances of the sensory and motor neurons and the associated dysfunction of the extremities do not occur and there is no intracranial overpressure.
Diagnosis
In the course of Garcin syndrome, paralysis of the individual cranial nerves occurs one after the other in the direction of spread of the original disease. This is a slow process and does not damage the brain at first. Under certain circumstances it can take years for the dura mater to penetrate and for the causal disease to become life-threatening.
Only at this point in time can changes in the composition of the liquor be determined in the laboratory. In Garcin syndrome, the trigeminal nerve and abducens nerve are affected particularly early. In combination with changes in the skull base with a simultaneous lack of intracranial pressure and intact limb function, this represents an important parameter for differentiating the disease.
Radiological methods as well as magnetic resonance tomography and computed tomography, which enable the visualization of the pathological processes and often even allow a differential diagnosis, play a major role here. The gold standard for the diagnosis of pachymeningitis is the dural biopsy, accompanied by the determination of the laboratory values that are meaningful for the underlying disease.
Complications
Garcin syndrome paralyzes the cerebral nerve. This paralysis can lead to various complaints and complications. In most cases, however, there are impaired hearing and vision. In the worst case, the patient can go blind or lose all eyesight.
There are also problems with balance and coordination. Word finding disorders and language disorders also occur and make everyday life extremely difficult for the patient. In individual regions and extremities of the body, numbness can occur, so that movement is restricted. It is not uncommon for the symptoms to lead to psychological complaints and depression.
In most cases, reversible treatment of Garcin syndrome is unfortunately not possible. Only the symptoms can be limited, with no further complications. If eyes and ears have been damaged, it is usually no longer possible to restore them.
If a tumor is responsible for Garcin syndrome, it can be surgically removed if necessary. The further course, however, depends on the type and spread of the cancer, so that no general prediction is possible here. Life expectancy is reduced by Garcin Syndrome.
When should you go to the doctor?
A doctor’s visit is necessary if various kinds of functional disorders occur. A doctor should be consulted if there are impaired eyesight, impaired hearing or changes in taste perception.
In the event of unsteady gait, dizziness, balance disorders or problems with vocalization, the complaints must be clarified by a doctor. If symptoms of paralysis appear on the body, if the person concerned complains of sensory disorders or numbness on the skin, there is cause for concern.
A medical examination is necessary if the movement of the joints, the head or the extremities is restricted. A doctor is also required in the event of a general feeling of illness, reduced performance level and fatigue.
If complaints develop in the back area, muscle pain, tension sensations, one-sided physical stress or poor posture, a doctor should be consulted. If there is a feeling of pressure in the head, if there is a headache or if unusual swellings develop on the body, these changes are considered unusual.
If they last for several days or if they increase in intensity, a doctor is needed. Consultation with a doctor is recommended if you have a fever, restlessness, weakness or insomnia. If there is unfounded irritation or behavioral problems, the observations should be discussed with a doctor. If the person concerned suffers from symptoms such as morning stiffness or pain in the sciatic nerve, a visit to a doctor is advisable to clarify the cause.
Treatment & Therapy
The therapy of the Garcin syndrome takes place via the treatment of the causative illness. Direct therapy of the destroyed nerves cannot take place, but there is a chance that the nerve will regenerate again after the cause has been eliminated. However, this can take several years.
The causal disease has priority, as this can have life-threatening consequences for the patient. Tumors are traditionally treated by surgery, chemotherapy or radiation therapy or a combination of these. The chosen form of therapy depends largely on the location, size and metastasis of the tumor.
In all measures, however, the focus is always on the patient’s quality of life and the maintenance of as many bodily functions as possible. Inflammations or infections of the brain base are treated like their underlying disease, in the case of bacterial infections with antibiotics or in the case of fungal attack with antimycotics and surgical removal of the infected areas.
The paralysis of the cranial nerves is treated symptomatically in order to compensate for the loss or to alleviate the symptoms. With facial paralysis and the associated inability to close the affected eye, ointments, artificial tears or bandages must be used to ensure that the cornea does not dry out.
There is also the option of weighting the lid externally with lead weights or, in the long term, surgically with gold or platinum weights. Professionally performed physiotherapy is also important in order to strengthen the function of the facial muscles.
Movement exercises for the chewing and swallowing muscles can relieve symptoms and improve the patient’s quality of life. Inward strabismus can be counteracted either with suitable visual aids or orthoptic exercises. Speech therapy can help with speech difficulties.
Outlook & forecast
The prognosis for Garcin syndrome is tied to the causal disorder or disease. An independent therapy and thus the prospect of a cure is not given, since the syndrome develops as a result of existing health impairments. If the underlying disease can be treated and treated successfully, there is a good chance of relief from the symptoms. This depends on the overall condition of the patient.
Although no direct treatment of the affected nerves can take place, the nerve fibers regenerate immediately with the healing process of the underlying disease taking place. Various exercises to promote the mobility of the muscles are performed as a support. They improve well-being and should motivate the patient in dealing with the disease. Functions such as chewing are thus supported and contribute to an improvement in the quality of life.
If the causal disease cannot be cured or if it has a progressive course, a further increase in symptoms can be expected. In these cases, the prognosis is to be classified as unfavorable. This development can be particularly evident in the case of severe tumor diseases.
Treatment may focus on relieving pain, as disease progression can no longer be stopped with current medical options. For the overall prognosis, protection against infestation of other germs is important so that the immune system is not weakened any further.
Prevention
Since Garcin syndrome is the result of different initial diseases, only the individual causes can be prevented. Both cancer and the tendency to aneurysms are partly hereditary. Nevertheless, the risk of the disease can be reduced in both cases through a healthy lifestyle.
Smoking not only increases the risk of developing pharyngeal or nasopharyngeal carcinoma, it also significantly increases the risk of developing aneurysms in addition to high blood pressure, obesity, lack of exercise and diabetes mellitus. Inflammatory diseases of the head space should be treated medically.
Aftercare
Since Garcin syndrome is usually an incurable disease, there are only very limited options for follow-up care available to those affected. First and foremost, the early diagnosis and treatment of this disease is necessary. Garcin syndrome may also limit the patient’s life expectancy if the tumor spreads to other areas of the body.
However, no universal prediction can be made here. The person concerned is usually treated with chemotherapy or radiation therapy. He needs the support of friends and his own family, whereby psychological support is also very useful and necessary. In many cases, contact with other Garcin syndrome patients can also be useful, as information can be exchanged.
Furthermore, those affected are often dependent on taking antibiotics. It is important to ensure that it is taken correctly and regularly. Alcohol must be avoided when taking antibiotics, otherwise their effect will be reduced by the alcohol. Physiotherapy measures are also necessary for Garcin syndrome, although some of the exercises can also be performed in your own home.
You can do that yourself
Since the genesis and cause of Garcin syndrome have not yet been fully clarified, no information about the prevention of the syndrome can be given. The possibilities for self-help are limited, but a healthy lifestyle with a healthy diet and exercise always has a positive effect on illness.
If the person concerned suffers from inflammation in the head area, this should always be examined and treated at an early stage. Those affected themselves are often dependent on outside help in their lives. Above all, the help of people close to you, such as friends and family, has a very positive effect on the course of the disease.
When taking antibiotics, attention should be paid to a ban on alcohol and possible interactions with other drugs. The doctor usually points this out to the patient. Also, you need when it is in the range of what is possible, affected at different physio – or ergothrapeutischen exercises or take part in physiotherapy. These exercises can also be repeated at home to further strengthen mobility.
If you have difficulty swallowing, friends or relatives can also help with the intake of food and liquids. Contact with other sufferers of Garcin syndrome can also alleviate psychological complaints and contribute to an exchange of information.
