Garcin Syndrome: Causes, Symptoms & Treatment

Garcin syndrome is the progressive paralysis of the Vth to XIIth cranial nerves. Cranial nerve of one side of the brain. Various causes underlie the condition.

What is Garcin syndrome?

Guillain-Garcin syndrome, or Garcin syndrome for short, describes a neurological disorder that is very rare in full expression and is associated with progressive hemiplegia of cranial nerves V – XII. Because the affected cranial nerves exit at the base of the skull, Garcin syndrome is also called semibase syndrome. The paralysis of the nerves occurs ipsilaterally, that is, only on one side of the brain, in the area of the transition from the middle to the posterior fossa. Garcin syndrome occurs as a complication of various diseases of the skull base. The symptoms that occur depend on the cranial nerves that fail, and the order of paralysis may vary. In some circumstances, other cranial nerves may be affected. The syndrome was first described in 1926 by French physicians Georges Guillain and Raymond Garcin, who also served as the namesakes.

Causes

Garcin syndrome may be caused by various tumors, growths, and inflammatory processes at the base of the skull. The most common triggers of paralysis are malignant carcinomas and sarcomas, which are located in the pharyngeal roof, sphenoid sinus, or ear and invade the base of the brain. Nasopharyngeal carcinomas, which are tumors located in the nasopharynx, can also infiltrate into the cranial nerves. Tumors metastasizing to the spinal cord or brain are also possible causes. More rarely, inflammatory diseases are found to be the cause of Garcin syndrome. Pachymeningitis, an inflammation of the outermost meninges, can affect the cranial nerves. This occurs as a result of overlapping inflammation of the middle ear and paranasal sinuses, for example, due to an untreated middle ear infection. Fungal infections of the paranasal sinuses can also spread to the brain in severe cases. This rhinocerebral course of mucormycosis can also lead to the paralysis of passing cranial nerves. Aneurysms of the carotid artery often occur in the area of the base of the brain. In rarer cases, these can also lead to hemiplegia.

Symptoms, complaints, and signs

The occurrence of symptoms in Garcin syndrome depends on the starting point of the causative lesion and its direction of spread. Depending on the order of paralysis of the cranial nerves, deficiency symptoms occur in the head region. Typical are visual and hearing disturbances, balance problems, swallowing and speech difficulties, disturbed taste perception and numbness of the facial muscles up to facialis paresis. Mobility of the head and shoulders may also be limited. Depending on the cause of the onset of the Garcin’s symptom, a disease may be asymptomatic for a long time or may present with nonspecific symptoms. Numbness, hypersensitivity or pain at the sciatica, as well as lower back pain and morning stiffness, and also sacralization of the fifth lumbar vertebra with the sacrum have also been frequently described. Disturbances of sensory and motor neurons and associated limb dysfunction do not occur, and intracranial hypertension is also absent.

Diagnosis

In the course of Garcin syndrome, paralysis of the individual cranial nerves occurs sequentially in the direction of propagation of the initial disease. This is a slow process, and the brain is not initially harmed in this process. Under certain circumstances, it may take years until the dura mater is penetrated and the causative disease becomes life-threatening. Only at this point can changes in the cerebrospinal fluid composition be detected by laboratory diagnosis. In Garcin’s syndrome, the trigeminal nerve and abducens nerve are affected particularly early. In combination with changes in the skull base in the absence of intracranial pressure and intact limb function, this represents an important parameter for differentiating the disease. Radiographic procedures play a major role here, as do magnetic resonance imaging and computed tomography, which allow visualization of the pathological processes and often even permit a differential diagnosis. The gold standard for the diagnosis of pachymeningitis is durabiopsy, accompanied by the determination of laboratory values indicative of the underlying disease.

Complications

Garcin syndrome causes paralysis of the cranial nerve.These paralyses can lead to various complaints and complications. In most cases, however, hearing and vision are impaired. In the worst case, the patient can go blind or lose all sight. There are also problems with balance and coordination. Word-finding and speech disorders also occur and make the patient’s daily life extremely difficult. Deafness can occur in individual regions and extremities of the body, resulting in restricted movement. Not infrequently, the symptoms also lead to psychological complaints and depression. In most cases, unfortunately, no reversible treatment of Garcin syndrome is possible. Only the symptoms can be limited, with no further complications. If eyes and ears have been damaged, it is usually not possible to restore them. If a tumor is responsible for Garcin’s syndrome, it can be surgically removed if necessary. However, the further course 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 see a doctor?

A visit to the doctor is necessary when functional disorders of various kinds develop. If there are impairments in vision, disturbances in hearing, or changes in taste perception, a doctor should be consulted. In case of gait unsteadiness, dizziness, balance disorders as well as problems with phonation, the complaints should be clarified by a doctor. If there are signs of paralysis on the body, or if the affected person complains of sensory disturbances or numbness on the skin, there is reason for concern. A medical examination is necessary in case of movement restrictions of the joints, head or extremities. If there is a general feeling of illness, reduced performance level and fatigue, a doctor is also needed. If complaints develop in the back area, if there are muscle pains, tension sensations, one-sided physical strain or incorrect posture, a visit to the doctor should be made. If a feeling of pressure develops in the head, headaches occur or unusual swellings develop on the body, these changes are considered unusual. If they persist for several days or increase in intensity, a doctor is needed. Consultation with a physician is recommended in the event of fever, inner restlessness, weakness, or sleep disturbances. If unfounded irritability occurs or behavioral abnormalities are present, the observations should be discussed with a physician. If the affected person suffers from phenomena such as morning stiffness or pain in the sciatic nerve, a visit to the doctor is advisable to clarify the cause.

Treatment and therapy

Therapy of Garcin’s syndrome involves treatment of the causative disease. There is no direct therapy for the destroyed nerves, but there is a chance that the nerve will regenerate after the cause is removed. However, this may take several years. Priority is given to the causative disease, as this can have life-threatening consequences for the patient. Tumors are classically treated by surgery, chemotherapy or radiotherapy, or a combination of these. The form of therapy chosen depends largely on the location, size and metastasis of the tumor. However, the quality of life of the patient and the preservation of as many bodily functions as possible are always in the foreground of all measures. Inflammations or infections of the brain base are treated in the same way as their underlying disease, i.e. in the case of bacterial infections via antibiotics or in the case of fungal infections via antimycotics and surgical clearance of the infected areas. Cranial nerve palsy is treated symptomatically to compensate for the loss or to relieve symptoms. In the case of facial paralysis and the accompanying inability to close the affected eye, care must be taken to prevent the cornea from drying out by using ointments, artificial tears or bandages. Furthermore, it is possible to weigh down the eyelid externally with lead weights or, in the long term, surgically with inserted gold or platinum weights. Professionally performed physiotherapy is also important to strengthen the function of the facial muscles. Movement exercises for chewing and swallowing muscles can alleviate discomfort and raise the patient’s quality of life.Inward strabismus can be counteracted either by suitable visual aids or orthoptic exercises. Speech difficulties can be helped by logopedic therapy.

Outlook and prognosis

The prognostic outlook of Garcin syndrome is tied to the causative disorder or disease. There is no independent therapy and thus no prospect of cure because the syndrome develops as a result of existing health impairments. If the underlying disease can be successfully treated and therapized, there is a good chance of alleviating 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 directly with the healing process of the underlying disease taking place. Supportive exercises are performed to promote the mobility of the muscles. 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 quality of life. If the causative disease cannot be cured or if it has a progressive course, a further increase in symptoms is to be expected. In these cases, the prognosis is to be classified as unfavorable. In particular, severe tumor diseases may show this development. The focus of treatment may be directed toward alleviating pain, since disease progression can no longer be stopped with current medical options. For the overall prognosis, protection against infestation by additional germs is important to prevent further weakening of the immune system.

Prevention

Because Garcin syndrome is a consequence of different initial diseases, only prevention of each cause is possible. Both cancer and the tendency to aneurysms are partly hereditary. Nevertheless, the risk of disease in both cases can be reduced by a healthy lifestyle. Smoking not only increases the risk of developing pharyngeal or nasopharyngeal carcinoma, but 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 headspace should be treated medically.

Follow-up

Because Garcin syndrome is a mostly incurable disease, the affected person has very limited options for aftercare. First and foremost, this involves early diagnosis and treatment of this disease. Garcin’s syndrome may also limit the patient’s life expectancy if the tumor spreads to other parts of the body. However, no universal prediction can be made in this regard. The affected person is usually treated by chemotherapy or radiotherapy. He needs the support of friends and his own family, and psychological support is also very useful and necessary. In many cases, contact with other patients of Garcin syndrome can also be useful, as an exchange of information can occur. Furthermore, those affected are often dependent on taking antibiotics. It is important to ensure that they are taken correctly and regularly. Alcohol must be avoided when taking antibiotics, as their effect is otherwise reduced by the alcohol. Measures of physiotherapy are also necessary in Garcin syndrome, although some of the exercises can be performed at home.

This is what you can do yourself

Since the origin and cause of Garcin syndrome are not yet fully understood, no information can be given on how to prevent the syndrome. The possibilities of self-help are limited, however, a healthy lifestyle with a healthy diet and sports activities always has a positive effect on a disease. If the affected person suffers from inflammations in the area of the head, these should always be examined and treated at an early stage. The affected persons themselves are often dependent on outside help in their lives. In particular, the help of close people, such as friends and family, has a very positive effect on the course of the disease. When taking antibiotics, attention must be paid to a ban on alcohol and to possible interactions with other medications. As a rule, the doctor will point these out to the patient.Likewise, if it is within the realm of possibility, affected individuals should participate in various physical or ergothrapeutic exercises or physiotherapy. These exercises can also be repeated at home to further strengthen mobility. If swallowing difficulties occur, friends or relatives can also help with the intake of food and liquids. Contact with others affected by Garcin syndrome can also relieve psychological discomfort and contribute to an exchange of information.