Dejerine-Spiller syndrome is a brainstem syndrome that belongs to the medulla oblongata syndromes and is characterized by alternating paralysis symptoms. Due to arterial occlusion, patients suffer from tongue paralysis associated with hemiplegia and sensory disturbances on the other side of the body.
What is Dejerine-Spiller syndrome?
The medulla oblongata corresponds to the medulla oblongata, which is the most caudal part of the brain. The structure is among the brainstem structures and is an important part of the central nervous system. Complete failure of the medulla oblongata usually results in death. The structure contains important reflex centers and autonomic functions such as respiratory function or circulatory functions. Reflexes such as the sneezing, vomiting and swallowing reflexes are also located in the medulla oblongata. In addition to medulla oblongata, the brainstem includes the midbrain and bridge. Proportional failures of brainstem structures are known as brainstem syndromes. Brainstem syndromes are characterized by alternating symptomatology and are divided into alternating pons, alternating midbrain, and alternating medulla oblongata syndromes depending on the localization of damage in the individual case. In addition to Jackson syndrome and Vernet syndrome, Dejerine-Spiller syndrome corresponds to an alternating medulla-oblongata syndrome. Of the ten classic medulla-oblongata syndromes, Dejerine-Spiller syndrome is one of the more severe symptom patterns, with alternating paralysis symptoms first described by neurologists Spiller and Dejerine in the early 20th century.
Causes
Like all brainstem syndromes, Dejerine-Spiller syndrome results from lesions in the brainstem area. The precise location of medulla oblongata syndrome is the medulla oblongata. Different conditions and disease events may result in injury to parts of the brain. In the case of Dejerine-Spiller syndrome, the primary cause of each symptom is usually arterial occlusion. Arteries carry oxygen-rich blood. In the human body, blood corresponds to a transport medium that supplies the tissues not only with vital oxygen but also with nutrients and messenger substances. The cerebral arteries supply the brain with oxygen and nutrients. Occlusions of these arteries result in ischemia and nutrient undersupply within the brain. When the supply of nutrients and oxygen is interrupted, cells automatically die. This cell death has serious consequences, especially in the brain, and can cause severe functional impairment. The arterial occlusions of Dejerine-Spiller syndrome are usually located in the anterior spinal or vertebral arteries. Pathophysiologically, a failure of the hypoglossal nucleus and a lesion of the medial lemniscus are present in addition to a medial medulla oblongata lesion. In addition, a lesion of the basal pyramidal tract may occur.
Symptoms, complaints, and signs
Patients with Dejerine-Spiller syndrome suffer from a complex of multiple symptoms. Characteristically, the syndrome is characterized by alternating paralysis symptoms. The left side of the brain is known to control the right side of the body and vice versa. However, this does not apply to the cranial nerves area. Thus, in the case of cranial nerve damage, the failure is not noticeable on the opposite side of the actual lesion, but on the same side. In Dejerine-Spiller syndrome, paralysis of the tongue occurs on the same side of the damage. The opposite side of the body shows hemiplegia. In most cases, the side of the hemiplegia also shows more or less severe sensory disturbances. The motor deficits of the tongue usually lead to a restriction of the ability to express oneself or to speak. In addition, the tongue paralysis can be associated with swallowing disorders or other problems with food intake. The severity of patients’ deficits depends on the duration of the causative arterial occlusion and thus the period of oxygen deprivation.
Diagnosis
The neurologist makes the diagnosis of Dejerine-Spiller syndrome using clinical symptoms and additional imaging of the brain. Slice images show lesions in the region of the medulla oblongata, which may be more or less severe.Differentially, the syndrome must be distinguished from related medulla oblongata syndromes. In addition, causes such as tumors and autoimmunological as well as bacterial inflammations must be excluded for the failure symptoms. The prognosis is relatively unfavorable for patients with Dejerine-Spiller syndrome. Complete regeneration does not occur in many cases.
Complications
Because of Dejerine-Spiller syndrome, various paralyses occur in the patient. In many cases, paralysis primarily affects the tongue. This results in speech disorders and comprehension problems. Targeted communication is often not possible for the patient. Other extremities or parts of the body may also be paralyzed, resulting in severe sensory disturbances and impaired perception. It is not possible for the patient to lead a normal everyday life. Often, the patient is dependent on the help of other people and suffers from depression and other psychological complaints. Not infrequently, this leads to exclusion from society. Due to the paralysis of the tongue, swallowing difficulties can also occur, which can further lead to underweight. It is also no longer possible to eat normal food and drink liquids. Dejerine-Spiller syndrome is not fully treatable. For this reason, symptoms are limited so that the patient can resume eating and expressing himself ordinarily. This treatment usually takes the form of therapy and does not lead to further symptoms or complications. However, in many cases, it is not possible to completely limit Dejerine-Spiller syndrome, so the patient must live with the limitations his or her entire life.
When should you see a doctor?
Since there is no self-healing in Dejerine-Spiller syndrome, a doctor must be consulted in any case. Usually, the syndrome is also not diagnosed immediately after birth, so a diagnosis by a doctor must be performed in any case. The doctor should be consulted if the patient suffers from paralysis of the face from time to time. These paralyses do not occur permanently and can, for example, affect the tongue or other muscles in the face. Often, only one side of the face is paralyzed. If these paralyses occur more often, a doctor must be consulted in any case. Difficulty swallowing can also indicate this syndrome. In severe cases, the syndrome can also lead to loss of consciousness, in which case an emergency physician should be called. The initial diagnosis may be made by a general practitioner. However, treatment involves the use of various therapies that can lighten the symptoms. Speech therapy care is also very useful for paralysis of the tongue and can restore normal development, especially in children.
Treatment and therapy
Causal therapy is not available for patients with medulla oblongata syndromes. Patients with Dejerine-Spiller syndrome receive purely symptomatic supportive treatment that leaves the underlying cause untouched. The main therapeutic measures are physiotherapy and occupational therapy. The nervous tissue within the brain is highly specialized. For this reason, the tissue of the central nervous system has only limited regenerative capacity. Although the damaged areas of the brain consequently do not regain full functional capacity, patients with brain lesion-related deficits can compensate for their deficits in individual cases through consistent training. This compensation is achieved by transferring functions from the damaged brain areas to healthy brain tissue. The hemiplegia of the affected person can thus at least be improved through targeted physiotherapy by stimulating neighboring brain areas to take over function from the damaged brain areas. If regeneration is not satisfactory, patients learn how to deal with their motor deficits on a daily basis in occupational therapy. In occupational therapy, for example, they learn about aids such as rollators that make everyday life easier for them. Since patients with Dejerine-Spiller syndrome also suffer from tongue paralysis, they also receive speech therapy sessions in addition to ergo- and physiotherapeutic care. Speech therapy sessions are particularly crucial in restoring patients’ expressive abilities and reducing their sense of helplessness.
Outlook and prognosis
In Dejerine-Spiller syndrome, affected individuals suffer from significant limitations in their lives, which significantly reduces their quality of life. Therefore, they are always dependent on treatment, as there is no self-healing and usually a worsening of symptoms in this disease. The patients suffer from various motor and cognitive deficits and are thus always dependent on the help of other people in their daily lives. Disturbances of the sensibility and likewise disturbances with the language occur. Furthermore, difficulties in swallowing can lead to difficulties in taking food and liquids. The severity of the symptoms of Dejerine-Spiller syndrome is strongly dependent on the interruption of the oxygen supply, so that no general prediction can be made. Therapy can usually only alleviate the symptoms of Dejerine-Spiller syndrome, but not cure it completely. Therefore, a positive course of the disease does not occur. The quality of life of the affected person can be increased again through various physiotherapy exercises. Whether Dejerine-Spiller syndrome has a negative effect on the patient’s life expectancy cannot be predicted in general.
Prevention
Dejerine-Spiller syndrome can be prevented only to the extent that occlusions of cerebral arteries can be prevented. Thus, in the context of the syndrome, the same preventive measures apply to a large extent as to strokes, circulatory disorders, and cardiovascular disorders.
Follow-up
In Dejerine-Spiller syndrome, the measures of follow-up care are usually severely limited. In this case, the affected person is first dependent on a rapid diagnosis with subsequent treatment so that no further complications can occur. This also prevents further worsening of the symptoms. The earlier the Dejerine-Spiller syndrome is recognized and treated, the better the further course of this disease usually is. The disease is often treated with the help of physiotherapy. Many of the exercises from such a therapy can also be performed at home, which may accelerate the healing process. The support and loving care of the patient by family or friends also has a positive effect on the course of the disease. Many patients also depend on this support in their daily lives, and psychological support may be necessary. However, it is not uncommon for professional psychological support to be necessary in order to prevent depression or further psychological upsets. In some cases, Dejerine-Spiller syndrome also reduces the life expectancy of the affected person, although no general course of the disease can be predicted.
Here’s what you can do yourself
Dejerine-Spiller syndrome currently cannot be treated causally. At best, patients can take self-help measures aimed at alleviating the symptoms of the disease or better managing daily life. Affected individuals are almost always prescribed physical therapy. Patients should seek out a physical therapist who has previous experience with this disease or a disorder with very similar symptoms. The exercise plan should also include occupational therapy elements. One of the most important self-help measures for patients is to consistently implement the exercise plan and practice regularly. Physiotherapy measures aim to slow muscle deterioration in the paralyzed areas of the body, thus improving motor function or at least maintaining it for as long as possible. Sensory function also benefits from adequate physiotherapy. Tongue paralysis is usually accompanied by difficulties in speech and food intake. In these cases, patients should also consult a speech therapist. Specific speech exercises can significantly improve articulation. Tongue paralysis also usually significantly impairs social interaction. In particular, patients who can hardly speak and cannot eat in company quickly feel isolated. This process can be counteracted by membership in a support group. Many of these groups are active online. In the case of severe mental suffering, a psychologist should be consulted.