Jackson syndrome is a brainstem or alternans syndrome with crossed paralysis symptoms, also known as ventral paramedian oblongata syndrome. The cause of the manifestations is a stroke in the current area of the vertebral artery. Treatment is symptomatic supportive and consists mainly of physiotherapy as well as logopedic measures.
What is Jackson syndrome?
Damage to the brainstem causes so-called alternans syndromes. These syndromes are characterized by crossed paralysis symptoms and occur with unilateral lesions of brainstem structures. The syndromes fall into the group of brainstem syndromes. Characteristic is the ipsilateral loss of cranial nerves and hemiparesis of the opposite half of the body, which is associated with sensory disturbances. Depending on the exact location of the damage, different types of brainstem or alternans syndromes exist. One subtype is Jackson syndrome, which is associated with unilateral damage to the pyramidal tract and nucleus nervi hypoglossi. The damaged nerve nucleus is located in the ventral portion of the medulla oblongata, which is why Jackson syndrome is also called ventral paramedian oblongata syndrome. Jackson syndrome is marked by tongue paralysis on the side of the brainstem lesion and hemiplegia on the opposite side. The name Jackson syndrome is derived from the English neurologist J. H. Jackson, who first described the syndrome in the 19th century, citing hemorrhage in the medial medulla oblongata as the cause.
Causes
In most cases, Jackson syndrome is the result of a stroke. This stroke usually occurs within the current area of the vertebral artery. Due to the proximity to the pyramidal tract, this motor structure is affected, resulting in hemiparesis of the opposite side of the body. The two sides of the brain each supply the opposite half of the body from the pyramidal junction. However, the cranial nerve structures above the pyramidal tract junction are controlled by the cerebral hemispheres of the side they supply. For this reason, stroke in Jackson syndrome produces paralysis of the opposite extremities, but at the same time produces paralysis of the lesion-side cranial nerve structures. These nerves include the nucleus nervi hypoglossi, which is located in the ventral portion of the medulla oblongata and supplies the tongue with its branches. The cause of lesion in Jackson syndrome is always a local microangiopathic change or embolic occlusion in the terminal portion of the vertebral artery. Both events cause ischemia, which should thus be understood as the primary cause of the crossed paralysis symptomatology.
Symptoms, complaints, and signs
Like all other alternans syndromes, Jackson syndrome manifests clinically as crossed paralysis symptoms. Ipsilateral to the causative brainstem lesion, paralysis symptoms occur in the tongue, which is motor innervated by hypoglossal nerve and leads to the hypoglossal nucleus. Hemiparesis occurs on the body side of the brain lesion opposite the hemiparesis, with sensory disturbances due to pyramidal tract damage over the pyramidal junction. Sensory disturbances can manifest in Jackson syndrome in a variety of ways. In addition to numbness, tingling or burning may occur. Hyper-sensitivity is also conceivable, although not the rule. The severity of hemiplegia symptoms depends on the extent of brain damage. Due to the tongue paralysis, the affected persons sometimes have difficulties in eating and drinking. Pronunciation is also more or less alienated because of the unilateral tongue paralysis.
Diagnosis and course of the disease
Because of the characteristic symptoms, the neurologist usually makes the first tentative diagnosis of Jackson syndrome on the basis of the clinical picture. A differential diagnostic differentiation from other brainstem syndromes is nevertheless necessary in the diagnostic process. A much more frequent brainstem syndrome after brain damage in the region of the medial medulla oblongata is the Dejerine-Spiller syndrome, which deserves the highest differential diagnostic attention. Jackson syndrome is essentially a reduced subtype of Dejerine-Spiller syndrome.Full Dejerine-Spiller syndrome includes, in addition to the symptoms of Jackson syndrome, a substantial sensory disturbance on the hemiplegic side. The patient’s sensation of pain and temperature is preserved because of the lesion within the posterior cord. Patients with Jackson syndrome have a relatively favorable prognosis in comparison.
Complications
As a result of Jackson syndrome, most patients suffer from severe paralysis and other disturbances of sensibility, usually over the entire body. As a result, severe movement limitations occur, leading to a decreased quality of life. In most cases, these limitations also lead to psychological complaints or depression. It is not uncommon for patients to become dependent on the help of other people in their daily lives. Furthermore, there is the typical tingling and numbness in the affected areas. In some cases, hypersensitivity also occurs. Particularly due to paralysis of the tongue and the oral cavity, there may be difficulties with food intake and fluid intake, so that the affected person loses weight or suffers from dehydration. As a rule, Jackson syndrome cannot be treated causally. For this reason, only symptomatic treatment takes place. However, this does not always lead to a positive course of the disease. The life expectancy can be already limited by the causal disease. However, with the help of various therapies, some symptoms can be limited and treated.
When should one go to the doctor?
Patients who have suffered a stroke suffer from sequelae and lifelong health impairments in a large number of cases. Jackson syndrome is among the effects of the life-threatening condition and should be treated therapeutically. A physician should be notified if there is any discomfort in the mouth area. Paralysis of the tongue or loss of control over the tongue are considered worrisome and should be evaluated by a physician. If hemiplegia occurs, disturbances in sensation set in, or limitations in sensitivity are noted, the observations should be discussed with a physician. Tingling on the skin or burning sensations are among the indications that point to Jackson syndrome. A physician should be consulted to initiate appropriate therapeutic measures. Restrictions in mobility, changes in speech and disturbances in food intake must be presented to a physician. If weight loss or a reduced intake of fluids occurs as a result of the impairments, there is a risk of the organism being undersupplied. In order not to trigger another life-threatening situation, a doctor must be alerted in time. In severe cases, dehydration will occur without medical intervention, leading to an increased risk of premature death. If psychological discomfort sets in in addition to physical discomfort, a physician or therapist should be contacted.
Treatment and therapy
Causal treatment is out of the question for patients with Jackson syndrome. Brain damage has occurred and cannot be causally repaired. The human brain is incapable of complete regeneration after damage because its tissues are composed of highly specialized neurons. While other tissues of the body can achieve partial complete regeneration by taking up new cells, this is impossible for brain tissue. So although the brain defect cannot be reversed, patients with Jackson syndrome can certainly recover from their symptoms. Physiotherapy and speech therapy play a particularly important role in this recovery. These supportive treatment measures consistently challenge patients to perform the functions that should actually take place in the damaged brain areas. This training stimulates nerve cells in the immediate vicinity of the brain lesion to take over the functions of the defective region. At least an improvement in symptoms can therefore be expected. In many cases, patients even overcome the failure symptoms altogether. A reduction in stroke risk factors should be sought to prevent further brain damage in the future.
Outlook and prognosis
The prognosis of Jackson syndrome is unfavorable. The syndrome develops as a result of a stroke. In this, irreversible damage to the brain occurred.For this reason, symptomatic treatment of the individually occurring complaints becomes necessary. The aim of the therapy is to improve the existing quality of life. A cure is almost impossible. Exercises are carried out in targeted training sessions, which should lead to an alleviation of the symptoms. Acquired paralysis, however, cannot be completely regenerated despite all efforts. In many cases, the patient is dependent on daily help from others. The disease often leads to secondary symptoms. Since the mental and psychological burden is very high due to the existing symptoms, mental illness may develop. A large number of patients experience depressive states. These may be temporary or permanent in nature. Likewise, a severe loss of well-being is frequently documented. Coping with everyday tasks can no longer be fully accomplished by the patient. Restructuring of habitual routines is required. In the case of paralysis of the tongue, an adequate supply of fluids is necessary, otherwise a life-threatening condition will develop. People who have suffered a stroke and developed Jackson syndrome may have a reduced life expectancy.
Prevention
Jackson syndrome can be prevented by the same measures that apply to stroke prevention. In addition to abstaining from tobacco use, preventive measures include reducing obesity, diet, and adequate exercise. Reducing stress, in the broadest sense, can also be counted among the preventive measures.
Aftercare
In most cases, the person affected by Jackson syndrome has no or only a few options and measures of direct aftercare at his or her disposal. In this case, the disease must first and foremost be detected and treated by a physician quickly and, above all, at an early stage so that it does not lead to further complications or to other complaints. In Jackson syndrome, the focus is therefore on early detection and treatment of the disease so that further deterioration of the symptoms can be prevented. A medical professional should be contacted at the first symptoms of the syndrome. In most cases, the syndrome is treated by taking various medications. The affected person should always pay attention to a correct dosage and especially to a regular intake of the medication. If there are any uncertainties or questions, a doctor should be consulted first. Likewise, measures of physiotherapy or physiotherapy are often necessary. Many of the exercises can also be repeated in the patient’s own home, which may accelerate the healing of Jackson syndrome. Not infrequently, contact with other patients of the disease can also be very useful, as it comes to an exchange of information.
This is what you can do yourself
Accompanying the medical treatment measures, the symptoms of Jackson syndrome may also be treated independently. The sensory disturbances can be relieved, in support of physical therapy, by regular exercise with an exercise ball or other aids. Massages sometimes also help against numbness and tingling. Alternative measures such as acupuncture or Chinese medicine methods are also possible, but should be discussed in advance with the doctor in charge. Logopedic measures and specific speech exercises help against unclear pronunciation. Although the causal tongue paralysis cannot be treated in this way, the complaints can often be reduced. Since Jackson syndrome is usually also associated with psychological complaints, therapeutic support should be sought. In order to avoid another stroke, it is sometimes necessary to adjust the lifestyle. Those affected should avoid nicotine, alcohol and other stimulants and henceforth eat a healthy and balanced diet. A sufficient amount of exercise is an important preventive measure. Stress and physical or mental strain should be avoided if possible, or at least reduced. The responsible physician will support the patient in implementing these changes and can provide further self-help tips.