The so-called clivus edge syndrome describes a main clinical feature as a result of a horizontal displacement of the brainstem in the upper region. In the tentorial slit, the oculomotor nerve is thereby damaged by increased pressure. The cause of this is subdural hematoma following cerebral hemorrhage or cranial trauma.
What is clivus edge syndrome?
Clivus edge syndrome represents the main symptom of brain injury that occurs due to a rapidly increasing intracranial increase in pressure caused by subdural hematomas. Causes are various skull traumas or also cerebral hemorrhages of all kinds, which can occur, for example, in tumors. The so-called oculomotor nerve is pressed laterally by the hippocampal gyrus against the Blumenbachian clivus, which is located at the sella turcica. In this way, irritation of the focal oculomotorius occurs, which manifests itself in a transient irritant miosis as well as a homolateral constriction of the pupils. Later, oculomotor nerve palsy develops, accompanied by absolute pupillary rigidity and unilateral mydriasis. Finally, there is a total loss of all external oculomotoric branches. This clinical picture is then referred to as complete oculomotor nerve palsy.
Causes
Causes of clivus edge syndrome include traumatic brain injury and tumors. Craniocerebral trauma usually occurs due to brain injury caused by external force. This often results in cerebral hemorrhage, which in turn can be considered the cause of clivus edge syndrome. Tumors can also trigger this clinical picture. In most cases, these are malignant tumors in the neuroectodermal tissue, which is located in the central nervous system. All other so-called intracranial tumors, such as meningioma, are considered only secondary causes of clivus edge syndrome. However, due to their rapid growth, they also belong to the group of brain tumors that can at least trigger the clinical picture, as they significantly alter the brain structure. However, the main cause is damage to the oculomotor nerve, the so-called third cranial nerve. This nerve has numerous external eye muscles, the function of which can be impaired in complex ways depending on the extent of the damage. In this case, the mobility of the eyes as well as the perception are severely reduced. Here, the cause of clivus edge syndrome is called external or internal oculomotor palsy.
Symptoms, complaints, and signs
In clivus edge syndrome, there is a contusion of the brainstem due to increased pressure in the cranial cavity. This can be caused by brain tumors or epidural hematomas, for example. In this way, the brain stem is pressed downward against the bones of the skull, so that the so-called oculomotor nerve is also pressed holistically against the bone structure of the clivus. The first symptom to appear during the course is ipsilateral pupillary dilation, which occurs due to the stretching and irritation of the affected nerve in the so-called tentorial slit. Later, there is paralysis of the oculomotor nerve, which is accompanied by a dilated and light-rigid pupil. In the later stages of the disease, complete oculomotor nerve paralysis occurs and additional dilation of the contralateral pupil develops. This is due to the displacement of the brainstem, which is now directly at the edge of the clivus.
Diagnosis and course
A variety of medical options can be used to diagnose clivus edge syndrome. The clinical picture manifests itself in different symptoms and manifestations. Thus, it is absolutely necessary to have the suspicious complaints, which point to the clivus edge syndrome, adequately clarified neurologically. This includes, for example, a thorough strabological examination, which should definitely be followed by a report. If the patient’s condition allows this, it should be done immediately after the first symptoms appear. In order to make a diagnosis, the attending physician will perform analyses of the movements and carry out complex procedures to measure squint angles from different viewing directions. This also serves to detect any combined paralysis of the eye muscles. The motor function of the pupils is also assessed. Clivus edge syndrome may also present with accompanying symptoms such as headaches and neck pain, as well as various ataxias.
When should you go to the doctor?
Unfortunately, the symptoms of clivus edge syndrome are not particularly clear-cut, so the syndrome is recognized late or only by chance. As a rule, a doctor should be consulted whenever there are severe and prolonged headaches that cannot be attributed to a specific cause. Paralysis in certain parts of the body may also be indicative of clivus edge syndrome and should also be investigated if it occurs over a long period of time. Furthermore, sudden discomfort in the eyes can indicate the disease, so that here, too, examination by a doctor is advisable. As a rule, the first diagnosis is made by a general practitioner. A detailed examination is then carried out with the help of an MRI or an X-ray. Pain in the neck can also indicate clivus edge syndrome and should be described to the doctor. Further treatment is by surgical intervention. Eye complaints can be treated by an ophthalmologist. Usually, there is a positive course of the disease.
Complications
Due to clivus edge syndrome, the patient suffers from bleeding in the brain and cranial trauma. Due to the increased pressure, many patients experience headaches and dizziness. The patient’s pupils dilate for no apparent reason. If the disease progresses severely, complete paralysis of the pupils eventually occurs. In most cases, the affected person suffers from the formation of malignant tumors that are difficult to treat with complications. If the clivus edge syndrome is not caused by a tumor, but by circulatory disorders, there are usually no complications. In this case, surgical intervention can also take place, during which the disease is treated. Clivus edge syndrome leads to a compulsive posture of the head in most patients. This posture can promote neck pain and significantly affect the life of the affected person. Also, due to the constant headaches, the quality of life comes off. The disease of the eye muscles can be treated with the help of prism glasses and does not lead to further complications. As a result, the vision is usually also strengthened again.
Treatment and therapy
Since the clivus edge syndrome is a neurological disorder, the clarification of the causes should be carried out in any case by a specialist in neurology. Basically, the prognosis for this clinical picture as well as its sequelae is rather poor, since mostly severe traumas, aneurysms or malignant tumors are the underlying causes. Furthermore, during the regeneration phase, faulty innervations often occur, which also cause an unfavorable course of the disease. If the cause of the clivus edge syndrome lies in general disturbances of the blood circulation, improved chances of recovery can be expected. In this case, however, the situation should change positively within one year, otherwise a so-called strabismus operation can be considered. This then allows the patient to return the field of binocular single vision to the so-called primary position. Thus, a displacement and forced posture of the head is permanently prevented. The present findings ultimately provide information as to whether the affected eye muscles must be treated first. If the existing paresis is only slightly pronounced, so-called prismatic lenses can be used. These then help to improve the patient’s vision and compensate for pupillary motility.
Outlook and prognosis
As a rule, the further course of clivus edge syndrome depends relatively strongly on the severity of the symptoms and also on the cause of the disease. In this regard, a general prediction is not possible. However, the symptoms can only be alleviated by direct treatment. If left untreated, paralysis due to the syndrome cannot be stopped. In the case of tumors, the prognosis is relatively poor, since the symptoms can no longer be alleviated in this case. If the clivus edge syndrome only occurs due to a circulatory disorder, these can be cured or at least alleviated in many cases. In this case, patients may need surgical intervention to correct the strabismus. The forced posture of the head can also be significantly alleviated by this procedure.In mild cases of clivus edge syndrome, the symptoms are relieved by prismatic lenses. To avoid complications, a doctor should therefore be consulted at the first signs of this condition. Early diagnosis always has a positive effect on the course of the disease. In order not to aggravate the symptoms, the affected person should therefore always protect his head from injury.
Prevention
There are no direct medical measures available to prevent clivus edge syndrome. Due to this fact, it is absolutely necessary to consult a specialist at the slightest disturbances and suspicious symptoms regarding vision. Since the causes are very diverse, a thorough examination should definitely take place, especially from a neurological point of view. This is also necessary because the clivus edge syndrome is usually based on serious causes such as tumors or hemorrhages, which if left untreated would result in the death of the patient. When clivus edge syndrome occurs as a result of a cerebral hemorrhage, a space-occupying and inoperable brain tumor, an aneurysm, or traumatic brain injury, follow-up after acute treatment or surviving surgery is essential.
Follow-up care
Even the triggers of clivus edge syndrome usually require treatment and monitoring. The consequences of oculomotor nerve damage are even more so. To make matters worse, however, the symptoms of clivus edge syndrome are often misinterpreted or not recognized early enough. If severe headaches occur after acute treatment of cerebral hemorrhage or tumors and persist for a prolonged period of time, this could be a clivuscant syndrome. The same is true when paralysis, dizziness or sudden visual problems occur. It makes sense, because of the severity of the symptoms and the underlying triggering disease, for patients to attend regular follow-up examinations after acute treatment. The chances of timely diagnosis of clivus edge syndrome are then higher. Follow-up can be done by the general practitioner as well as by the ophthalmologist, a neurologist or the former surgeon in the hospital context. If caused by circulatory disturbances, the clivus edge syndrome can be treated relatively well. On the other hand, if a space-occupying and inoperable brain tumor, traumatic brain injury, or aneurysm is present, the outlook for the patient is poor. In these cases, only symptomatic help can be offered as follow-up for the discomfort caused by clivus edge syndrome.
This is what you can do yourself
In the case of clivus edge syndrome, the affected person should protect his or her body, and in particular his or her head, from jerky movements or exposure to other external influences. Falls or blows to the head aggravate the symptoms, so care must be taken to provide adequate head protection. To avoid jolting, jumping, running or hopping should be completely avoided during the healing process. Slow and steady movements are helpful. Head posture should be checked regularly and stress to the head minimized as much as possible. In the course of the day, it is beneficial for recovery if the head is laid down after a while or if the affected person lies down during breaks, if possible. This relieves the muscles, tendons and nerves connected to the head. In addition, the head is moved less during the rest period. At the first sign of discomfort and dizziness, the affected person should adopt a gentle posture until the symptoms are reduced. When riding a bicycle, motorcycle or car, care should be taken to slow down. The head should not be subjected to unnecessary jarring, so driving over bumps or potholes must be avoided. Strenuous cognitive tasks or intensive work on the PC should also be avoided to reduce overall brain activity.
