Foramen Jugular Syndrome: Causes, Symptoms & Treatment

Foramen jugulare syndrome is also known as Vernet syndrome and corresponds to a failure of the three cranial nerves IX, X and XI, which manifests in complaints the dysphonia and dysphagia. In most cases, the cause is a tumor in the middle region of the forman jugulare. Treatment is by excision, as radiation therapy has been found to be particularly harmful in this area.

What is foramen jugulare syndrome?

The foramen jugulare is also known as the throttle hole and corresponds to a large opening at the base of the skull through which nerve pathways descend to the neck area. The anatomic structure is located posterior to the exit of the internal carotid artery. The anterior border of the area is formed by the petrous bone. The posterior boundary is represented by the occipital bone. Overall, the jugular foramen consists of three distinct compartments. The anterior portions are called the pars anterior and contain the inferior petrosal sinus. The middle portion is called the pars intermedialis and is home to the IX, X, and XI cranial nerves with the glossopharyngeal nerve, the vagus nerve, and the accessorius nerve. In addition, the posterior meningeal artery for blood supply to the meninges is located in this section. The last compartment corresponds to the pars posterior, which is home to the sigmoid sinus. Foramen jugulare syndrome is a symptom complex preceded by damage to the anatomic structure. The leading symptom is a partial or complete loss of the cranial nerves IX, X and XI. The clinical picture is also referred to as Vernet syndrome in the specialist literature. This name is derived from the first describer of the disease: the French neurologist Maurice Vernet, who documented the syndrome in the early 20th century.

Causes

The causes of Vernet syndrome lie in damage to the jugular foramen. The middle portion of the anatomic structure is particularly important in this context because the three cranial nerves pass through the foramen at this point. The primary cause of the impairment of the cranial nerve structure may be due to a glomus tumor, for example. This cause is the most common. The glomus tumor corresponds to a paraganglioma, which is usually benign and neuroendocrine in nature. Such tumors arise from autonomic ganglia originating in the parasympathetic or sympathetic nervous system. Meningiomas or schwannomas such as acoustic neuroma may also be involved in the development of foramen jugulare syndrome. Other possible causes include metastases within the cerebellopontine angle. Rather rarely, the syndrome is caused by cholesteatoma or results from mechanical damage after trauma.

Symptoms, complaints, and signs

Patients with foramen jugulare syndrome show varying degrees of cranial nerve deficits. Usually, the deficits are not partial but total, causing complete paralysis in the motor supply area of the cranial nerves. Such paralyses manifest themselves in detail in dysphonia, for example in the form of pathological hoarseness. Equally common are complaints such as dysphagia or sensory disturbances and sensory disorders in the posterior third of the tongue. In sensory disorders, patients often recognize tastes as bitter, among other things, even though the food consumed is not a bitter food. Beyond the symptoms mentioned above, paralysis of the affected side of the soft palate often occurs, causing a deviation from the healthy side. In many cases, parotid secretion decreases bit by bit. Due to the sensitivity and sensory disturbances, the gag reflex may also experience impairments. In addition, failures of the sternocleidomastoid and trapezius muscles often occur.

Diagnosis and course

The diagnosis of Vernet syndrome is usually made on the basis of clinically characteristic criteria. Initial suspicion can be confirmed by imaging of the skull. MRI is particularly useful in this case. Causative tumors show a typical image in the slice images and thus allow classification as foramen jugulare syndrome. The prognosis for patients with Vernet syndrome depends primarily on the degree of malignancy. Operability may also have a crucial impact on prognosis.

Complications

Foramen jugular syndrome presents with a variety of complications, depending on the affected region in the brain.In most cases, the loss of cranial nerves leads to paralysis, disability and other disturbances of the body. Sensory disturbances occur to an increased extent, which can strongly influence the patient’s everyday life. In many cases, the perception of tastes is also disturbed, with food sometimes tasting more bitter than it actually is. Sensory function and muscle movements are also disturbed due to foramen jugulare syndrome. Complications occur primarily when the cranial nerves have been permanently damaged. In this case, it is not possible to restore the damaged nerves and the patient must live with the complications that occur. If the nerves have not yet been damaged and the tumor can be successfully removed, no complications occur. In many cases, chemotherapy is also used. In the case of a benign tumor, treatment is not mandatory. Often, treatment is not necessary as long as there are no complications. Life expectancy is reduced by the foramen jugulare syndrome if the tumor spreads in a malignant form. In this case, death occurs in the worst case.

When should you see a doctor?

Because foramen jugulare syndrome does not heal itself and, in most cases, worsens, a doctor must always be consulted. A visit to the doctor should be made if the affected person suffers from paralysis or chronic hoarseness. The paralysis occurs mainly in the face or mouth and tongue and can lead to difficulty swallowing or discomfort when taking food. In many cases, a bitter taste in common foods may also indicate foramen jugulare syndrome, so an examination by a physician is necessary. Many sufferers of foramen jugulare syndrome also suffer from severe retching, causing them to vomit frequently. Diagnosis of foramen jugulare syndrome can be made by a general practitioner or an ENT specialist. However, further treatment always depends on the underlying disease, and a complete cure is not possible in many cases. In the case of psychological complaints or depression, a psychologist should always be consulted, as a healthy psyche can also accelerate the healing process. Psychological treatment may also be necessary for the patient’s parents or relatives.

Treatment and therapy

Vernet syndrome is generally treated causally. Causal therapy addresses the primary cause of the failure symptoms and attempts to resolve the primary cause. With the elimination of the primary trigger, the individual symptoms also subside, as long as the cranial nerves have not taken irreparable damage due to the tumor. Minor impairments may nevertheless remain even if the causative tumor has been removed. The therapy of choice is a more or less complete excision of the tumor. However, surgical excision of intracranial glomus tumors in particular is difficult due to blood richness and infiltrative growth of the tumor type. Alternatives to invasive procedures include radiation therapy or chemotherapy. However, these forms of therapy are considered controversial in the context of foramen jugulare syndrome. The cranial nerves suffer irreparable damage, especially with radiation therapy in this area. If the tumor is benign, the benefits of any therapy must be weighed against the anticipated risks. Benign tumors in the area of the jugular foramen do not necessarily have to be removed. If the tumor causes little discomfort, at least a wait-and-see approach can be taken in this case to avoid risking unnecessary worsening of symptoms.

Outlook and prognosis

The prognosis of foramen jugulare syndrome is tied to the cause of the presenting disorders and the applicable treatment options. If the cranial nerve failure at the foramen is caused by a tumor in the brain, the patient may die prematurely in an unfavorable case. Depending on the location of the tumor, a decision is made as to whether surgical intervention and cancer therapy can be undertaken. If the tumor is benign and can be removed without further complications, the patient has a chance of recovery. If there are no further impairments, the patient is considered to have recovered following follow-up treatment.In the coming months and years, control examinations are performed at regular intervals. If no new tissue changes form, the patient is considered permanently cured. If there is a malignant tumor, the chances of recovery deteriorate immensely. If further growth cannot be prevented despite irradiation of the tumor, there is no prospect of a cure with the current medical options. Metastases form and the cancer gradually spreads. If the foramen jugulare syndrome is caused by a fall or accident, the prognosis must be judged by the extent of damage to the skull. In the case of smaller defects or tears, repair is possible. Large-scale damage, on the other hand, leads to lifelong impairment.

Prevention

Foramen jugular syndrome cannot be prevented. The tendency to develop paragangliomas is in the genes, as the tumors have historically been associated with familial clustering. The only preventive measure in this case would be for patients with a known predisposition to the tumor type not to have children of their own.

Follow-up

Options for follow-up care are not available to the affected person in most cases of foramen jugulare syndrome, or are very limited. Because it is a genetic condition that is inherited, it is also not possible to provide causative therapy, only symptomatic therapy. A complete recovery is not possible. If the patient wishes to have children, genetic testing can be performed to prevent inheritance of the foramen jugulare syndrome. In most cases, treatment is carried out with the help of medications. The patient is primarily dependent on the correct and regular intake of the medication in order to permanently alleviate the symptoms. Since the foramen jugulare syndrome also leads to the appearance of various tumors, regular examinations should be performed by a physician in order to detect the tumors at an early stage and also to remove them. Self-healing cannot occur with the foramen jugulare syndrome. Intensive and loving care of the affected person also has a positive effect on the further course of the disease, whereby they are often dependent on intensive conversations in order to prevent psychological upsets or depression. Possibly, the disease reduces the life expectancy of the affected person.

What you can do yourself

With cranial nerve failure, the affected person has few options to bring about relief or cure of the disease through his or her own efforts. Patients help themselves most by seeing professional physicians they trust and can work well with when they have foramen jugular syndrome. It is important and very helpful if the sufferer is fully informed and educated about the condition. This is beneficial in coping with the symptoms in everyday life. Even if on the physical level the possibilities to improve the situation are limited, the affected person can take good care of his psychological stability. A positive attitude to life and optimism are beneficial during medical treatment and the subsequent healing process. With a stable social environment at his side, the sufferer can draw new strength and maintain confidence in difficult phases. If the emotional problems increase, it is helpful to consult a therapist. The way of life should be arranged in such a way that well-being and joy of life are achieved. In spite of altered or limited possibilities for shaping one’s life, there are various approaches to planning leisure time, which can be used according to individual preferences. This brings new impetus and has a positive effect on the general state of health.