Pseudobulbar Syndrome: Causes, Symptoms and Treatment

With various brain lesions, complexes of characteristic disorders, called syndromes, develop. One of them is pseudobulbar paralysis. This condition occurs when the nuclei of the medulla oblongata cease to receive sufficient regulation from the overlying centers. This does not represent an immediate danger to the life of the patient, in contrast to bulbar paralysis. Correct differential diagnosis between these two syndromes not only allows the doctor to determine the level of damage, but also helps to estimate the prognosis.

How does pseudobulbar syndrome occur?

In the brain there are "old" departments that can work autonomously, without the regulatory influence of the cortex. This supports life activity, even with damage to the higher parts of the nervous system. But such basic activity of neurons is accompanied by violation of complex motor acts. After all, for the coordinated work of different muscle groups, a clear coordination of various nerve impulses is needed, which only the subcortical structures can not provide.

Pseudobulbar syndrome (paralysis) occurs when the higher regulation of motor (motor) nerve nuclei in the medulla oblongata is disturbed. These include centers 9, 10 and 12 pairs of cranial nerves. Many ways in the brain have a partial or complete cross. Therefore, the complete disappearance of control impulses occurs with bilateral lesion of the frontal cortex of the brain or with extensive damage to the subcortical neural pathways.

Those who remain without the "commander" of the nucleus begin to work autonomously. The following processes occur:

• the function of the respiratory muscles and the heart is preserved, that is, the vital acts remain unchanged;
• changes articulation (pronunciation of sounds);
• because of the paralysis of the soft palate, swallowing is disturbed;
• there are involuntary stereotyped grimaces with imitation of emotional reactions;
• the vocal cords become inactive, the background is broken;
• activated deep unconditioned reflexes of oral automatism, which can normally be detected only in infants.

The nuclei of the medulla oblongata, the cerebellum, the brain stem, the limbic system and the many paths between the individual subcortical structures of the brain participate in the formation of such changes. There is their disinhibition and discoordination of work.

The difference between bulbar syndrome and pseudobulbar

Bulbar syndrome is a complex of disorders that occur when the nuclei themselves are damaged in the medulla oblongata. These centers are located in a thickening of the brain, called a bulb, which gave the name of the syndrome. And with pseudobulbar paralysis, the nuclei continue to function, although coordination between them and other nerve formations is impaired.

In both cases articulation, phonation and swallowing suffers. But the mechanism of development of these violations is different. With bulbar syndrome, there is a pronounced peripheral paralysis of the muscles of the pharynx and soft palate with degeneration of the muscles themselves, often accompanied by a disruption of vital functions. And with pseudobulbar syndrome, paralysis is central, the muscles remain unchanged.

Causes of pseudobulbar syndrome

Atherosclerosis can lead to neuronal damage in the cerebral cortex and, as a consequence, to pseudobulbar syndrome.

Pseudobulbar syndrome is a consequence of neuronal damage in the frontal cortex of the brain or the nerve pathways from these cells to the nuclei in the medulla oblongata. The following states can lead to this:

• hypertensive disease with the formation of multiple small ischemic or hemorrhagic foci, repeated strokes;
• atherosclerosis of small vessels of the brain;
• one-sided lesion in the brain of arteries of medium and small caliber, which leads to the syndrome of stealing (blood supply deficiency) in symmetric areas;
• vasculitis with systemic lupus erythematosus, tuberculosis, syphilis, rheumatism;
• degenerative diseases (amyotrophic lateral sclerosis, Pick's disease, supranuclear palsy, Creutzfeldt-Jakob disease, multiple sclerosis and others);
• consequences of traumatic brain injury;
• the consequences of brain damage in utero or as a result of birth trauma;
• the consequences of severe ischemia after clinical death and coma;
• pronounced metabolic disturbances against long-term use of valproic acid preparations;
• tumors of the frontal lobes or subcortical structures;
• inflammatory process in the brain tissues.

Most often, pseudobulbar syndrome occurs in chronic progressive states, but it can also occur with acute oxygen starvation in many parts of the brain.

Clinical picture

Such patients can laugh or cry against their own will.

Pseudobulbar syndrome is characterized by a combination of swallowing disorders, speech, the appearance of violent grimaces (laughter and crying) and symptoms of oral automatism.

The appearance of choking and pauses before swallowing is explained by the weakness of the pharyngeal muscles and the soft palate. In this case, the disturbances are symmetrical and not as gross as in bulbar paralysis. There is no atrophy, no twitching of the affected muscles. A pharyngeal reflex may even be elevated.

Speech disorders in pseudobulbarnom syndrome capture only pronunciation - speech becomes fuzzy and, as it were, blurred. This is called dysarthria, it can be caused by paralysis or spastic muscle tonus. In addition, the voice becomes deaf. Such a sign is called dysphonia.

The syndrome necessarily includes symptoms of oral automatism. And the patient himself does not notice them, these signs are revealed during special tests during a neurological examination. The slight irritation of certain zones leads to a reduction in the chin or perioral muscles. These movements resemble sucking or kissing. For example, such a reaction appears after touching the palm or corner of the mouth, when tapping on the back of the nose. And with a light blow to the chin, chewing muscles are shortened, closing the slightly open mouth.

Often there are violent laughter and crying. This is the term characteristic short-term contraction of facial muscles, similar to emotional reactions. These involuntary grimaces are not associated with any impressions and can not be stopped by effort of will. Violated and arbitrary movements of the facial musculature, because of which a person can open his mouth at the request to screw up his eyes.

Pseudobulbar syndrome is not isolated, it appears against the background of other neurological disorders. The overall picture depends on the root cause. For example, the defeat of the frontal lobes is usually accompanied by emotional-volitional violations. A person at the same time can become inactive, uninitialized, or, conversely, disinhibited in their desires. Dysarthria is often combined with a decrease in memory and speech disorders (aphasia). With the defeat of the subcortical zones, there are often various motor disorders.

How to treat

When pseudobulbar syndrome is detected, the main disease must be treated first. If this is a hypertensive disease, prescribe antihypertensive and vascular therapy. With specific vasculitis (syphilitic, tuberculosis) necessarily use antimicrobial agents, antibiotics. In this case, treatment can be carried out together with narrow specialists - dermatovenereologist or phthisiatrist.

In addition to specialized therapy, drugs are prescribed to improve microcirculation in the brain, normalize the work of nerve cells and improve the transmission of nerve impulses. For this, various vascular, metabolic and nootropic agents, anticholinesterase drugs are used.

There is no universal remedy for the treatment of pseudobulbar syndrome. The doctor selects the scheme of complex therapy taking into account all available disorders. In addition to taking medications, special exercises for the affected muscles, the Strelnikova breathing gymnast, and physiotherapy can be used. If speech is disturbed, children are required to have classes with a speech therapist, this will greatly improve the child's adaptation in society.

Unfortunately, it is usually not possible to completely get rid of pseudobulbar syndrome, because such disturbances occur when expressed bilateral brain lesions, which are often accompanied by the death of many neurons and the destruction of nervous pathways. But the treatment will compensate for violations, and rehabilitation exercises will help a person adapt to the problems that have arisen. Therefore, do not neglect the recommendations of the doctor, because it is important to slow the progression of the underlying disease and keep the nerve cells.