Bulbar disorders consist of the following: Bulbar syndrome: what is it? The difference between pathology and pseudobulbar palsy

Bulbar syndrome is a disorder accompanied by simultaneous failure of the function of several nerves - glossopharyngeal, sublingual and vagus. This leads to disruption of the processes of chewing and swallowing, and speech dysfunction. Among the dangerous consequences of the pathology are changes in the functioning of the heart and respiratory system. Treatment of bulbar syndrome is based both on the use of various groups of drugs and on surgical interventions. The prognosis for the disease is usually cautious. The outcome depends on the etiology of the lesion.

Causes of development of bulbar syndrome

Many unfavorable factors can provoke disruptions in the functioning of the nervous system and reflex arc. These include both infectious agents and non-infectious pathologies. The main causes of bulbar syndrome in children and adults are:

1. Congenital genetic abnormalities. A number of diseases are accompanied by damage to the normal structure and functions of the medulla oblongata, which leads to the appearance of specific symptoms of the lesion. Amyotrophy Kennedy and porphyrin disease can provoke the occurrence of such disorders. Pseudobulbar syndrome in children, often noted against the background of cerebral palsy, is also one of the hereditary pathologies. The peculiarity of the problem is the absence of atrophy of the affected muscles, that is, the paralysis is peripheral.
2. A common cause of bulbar syndrome in newborns is bacterial and viral infections. They lead to inflammation of the membranes of the brain and spinal cord. Meningitis and encephalitis are accompanied by the formation of paralysis, both central and peripheral. Common agents leading to the disorder are Lyme disease and botulism.
3. Neurology describes the occurrence of bulbar and pseudobulbar syndromes against the background of defects in bone structures, for example, due to injuries or osteochondrosis affecting the neck area. The mechanism of formation of clinical signs is associated with compression of nerve structures, which prevents their normal functioning.
4. Disorders of the central nervous system also arise during the formation of oncological processes. Tumors prevent adequate nutrition and blood supply to the affected organs, which leads to the formation of problems.
5. Vascular defects are a common cause of bulbar syndrome. Characteristic disturbances in the functioning of the muscles of the tongue and pharynx occur as a result of a stroke, since with this problem there is a severe degree of ischemia of the nervous tissue. Congenital anomalies in the structure of arteries and veins can also lead to the formation of the disease.
6. Autoimmune problems are included in a separate group of etiological factors that provoke the occurrence of bulbar syndrome. A common cause of nervous system disorders is multiple sclerosis, which is accompanied by damage to the membranes of the fibers that form the spinal cord and brain.

Main symptoms of pathology

Bulbar syndrome has specific clinical manifestations. Classic signs include:

1. Changes in facial expressions in patients. The facial muscles atrophy, which leads to the formation of characteristic symptoms. Patients appear emotionless.
2. Due to damage to the glossopharyngeal nerve, the swallowing process is disrupted. Patients have difficulty chewing food and can easily choke.
3. There is abundant salivation, which occurs due to pathological changes in the functioning of the vagus. The inhibitory effect of the vagus nerve weakens, which leads to active secretion of the secretion of the digestive glands. Due to the paralysis of several structures at once, it is difficult for the patient to completely close the jaws, so saliva may flow out of the corners of the mouth.
4. Damage to the hypoglossal nerve provokes severe speech disorders. Patients find it difficult to speak because the muscles are weakened. Pronounced sounds are slurred and drawn out. Similar problems are diagnosed in both adult patients and children.
5. The most dangerous symptoms of bulbar syndrome are associated with a violation of automaticity in the functioning of vital systems. Patients with the disease suffer from changes in heart function and respiratory failure. Respiratory problems are exacerbated by the high risk of aspiration. In severe cases, apnea is observed, requiring emergency medical attention.

Necessary diagnostic tests

Bulbar syndrome is not an independent disease. Therefore, confirming the presence of a problem comes down to identifying the cause of its occurrence, as well as establishing the nature of the pathological disorders. For this purpose, standard diagnostic tests are carried out - blood, urine and stool tests, as well as a general examination of the patient and medical history.

In many cases, the use of visual methods is required, in particular, magnetic resonance imaging, which makes it possible to detect the presence of tumor and ischemic processes in the cranial cavity. Electromyography, used to differentiate central and peripheral paralysis, is also informative.

Therapy methods

Treatment of bulbar syndrome should be aimed at the cause of its occurrence. In this case, patients often require emergency care, especially against the background of acute cardiac and respiratory failure. Therapy is also symptomatic in nature and is aimed at improving the general condition of the patient. Both conservative methods, traditional and folk, and radical ones are used.

Drug treatment

1. When an infectious agent that triggers the development of symptoms is identified, antibacterial drugs of various groups are used.
2. To reduce the intensity of inflammatory processes in injuries, as well as as a palliative treatment of pseudobulbar syndrome in oncology, hormonal agents are used, for example, Prednisolone and Solu-Medrol.
3. In order to restore the function of autonomic innervation, Atropine is prescribed. It helps reduce the active secretion of saliva and also prevents bradycardia.
4. In a number of emergency conditions associated with the development of severe arrhythmia, the use of Lidocaine, which is administered to patients intravenously, is justified.

Physiotherapy

Massage has a pronounced effect in bulbar syndrome. It helps relax muscles and restore normal blood supply to the affected area. Kinesiotherapy improves trophism and the functioning of the nervous system, that is, special training that has positive reviews. Exercises are used to help restore the functioning of the facial muscles. Gymnastics is also used to improve the function of the muscles involved in the swallowing process.

Once the patient's condition has stabilized, work with a speech therapist will be required. It is necessary for the correction of established speech disorders. Electrophoresis with Hydrocortisone and Lidocaine has a pronounced therapeutic effect during rehabilitation after brain damage, for example, as a result of a stroke.

Surgery

In some cases, the use of more radical methods is required. They are used both to alleviate the condition of patients and to influence the cause of the disease. Some patients have a tracheostomy installed to maintain adequate breathing. Many also require a nasogastric tube. Surgical intervention is necessary for patients with operable tumors in the cranial cavity, as well as for those injured as a result of various injuries.

In many cases, treatment is only symptomatic. No exact protocols have been developed to combat bulbar syndrome, since therapy is aimed at the cause of the disease. At the same time, there are recommendations that are used in most European countries to alleviate the condition of patients with amyotrophic lateral sclerosis. The same principles can be used to treat bulbar syndrome:

1. Medications that help relieve spasms, as well as anticonvulsants that provide relaxation of the affected muscles, are widely used.
2. The use of heat and water helps to improve the well-being of patients.
3. Moderate physical activity is also important. In this case, it is recommended to use both general exercises and special ones aimed at training the affected muscle groups.
4. To maintain adequate body weight, as well as meet the body's nutritional needs, you will need to consult a doctor to create the right diet.
5. Classes with a speech therapist, both group and individual, are used not only for the purpose of correcting speech dysfunctions. They also contribute to the social rehabilitation of patients, which greatly facilitates the further treatment process.

Nutritional Features

In many cases, patients with bulbar syndrome cannot eat normally. For this purpose, a special tube is installed through which food is supplied. Such features require the use of liquid products. The diet should be balanced and provide adequate nutrition for the body.

Prognosis and possible complications

The outcome of the lesion largely depends on the cause of the disease. Timely provision of medical care is also important, although even with adequate therapy it is not always possible to cure the disease. Moreover, the prognosis for pseudobulbar syndrome is much better, since the pathology is not accompanied by dysfunction of vital organs.

The greatest danger in the disorder is cardiac and respiratory failure. Patients often die due to severe arrhythmia, aspiration pneumonia, and asphyxia when food enters the respiratory tract.

Prevention

Preventing the development of the problem is based on preventing the occurrence of infections and non-communicable diseases that can lead to the formation of pathology. To do this, it is important to lead a healthy lifestyle, as well as regularly undergo preventive examinations with doctors.

Bulbar syndrome, called bulbar palsy, is a syndrome of damage to the hypoglossal, glossopharyngeal and vagus cranial nerves, the nuclei of which are located in the medulla oblongata. Bulbar syndrome occurs with bilateral (less often with unilateral) damage to the nuclei of the X, IX, XI and XII cranial nerves, which belong to the caudal group and are located in the medulla oblongata. In addition, the roots and trunks of nerves inside and outside the cranial cavity are damaged.

With bulbar syndrome, peripheral paralysis of the muscles of the pharynx, lips, tongue, soft palate, epiglottis and vocal folds occurs. The result of such paralysis is dysphagia (impaired swallowing function) and dysarthria (impaired speech). characterized by a weak and dull voice, up to complete aphonia, nasal and “blurred” sounds appear. The pronunciation of consonant sounds that differ in the place of articulation (front-lingual, labial, posterior-lingual) and in the method of formation (frictional, stop, soft, hard) becomes the same type, and vowels become difficult to distinguish from each other. Speech in patients diagnosed with bulbar syndrome is slow and very tiring for patients. Depending on the severity of muscle paresis and its prevalence, bulbar dysarthria syndrome can be global, partial or selective in nature.

Patients with bulbar palsy choke even on liquid food, because... unable to perform swallowing movements, in especially severe cases, patients develop disorders of cardiac activity and breathing rhythms, which very often leads to death. Therefore, timely emergency care for patients with this syndrome is extremely important. It consists of eliminating the threat to life and subsequent transportation to a specialized medical facility.

Bulbar syndrome is characteristic of the following diseases:

Genetic diseases such as Kennedy's disease and porphyria;

Vascular diseases (myelencephalon infarction);

Syringobulbia, motor neuron disease;

Inflammatory-infectious Lyme, Guillain-Barré syndrome);

Oncological diseases (brain stem glioma).

The basis for diagnosing bulbar palsy is the identification of characteristic signs or clinical symptoms. The most reliable methods for diagnosing this disease include electromyography and direct examination of the oropharynx.

There are bulbar and pseudobulbar syndromes. The main difference between them is that with pseudobulbar syndrome, paralyzed muscles do not become atrophied, i.e. paralysis is peripheral, there are no fibrillary twitching of the tongue muscles and a degeneration reaction. Pseudobulbar palsy is often accompanied by violent crying and laughter, which are caused by disruption of connections between the central subcortical nodes and the cortex. Pseudobulbar syndrome, unlike bulbar syndrome, does not cause apnea (cessation of breathing) and cardiac arrhythmias. It is observed mainly in diffuse brain lesions of vascular, infectious, intoxication or traumatic origin.

Bulbar syndrome: treatment.

Treatment of bulbar palsy is aimed mainly at eliminating the underlying disease and compensating for functions that are impaired. To improve swallowing function, drugs such as glutamic acid, proserin, nootropic drugs, galantamine and vitamins are prescribed, and for increased salivation, the drug atropine. Such patients are fed through a tube, i.e. enterally. If respiratory function is impaired, artificial ventilation is prescribed.

Such a problem as the development of pseudobulbar syndrome in a child is a real test for parents. The fact is that the symptoms of this disease manifest themselves quite clearly and, if the reaction is untimely, take a long time to overcome.

What is pseudobulbar syndrome

The essence of this disease comes down to the appearance of multiple large and small foci of hemorrhage, which lead to damage in both hemispheres of the fibers connecting the motor nuclei of the cerebral cortex with the brain stem.

This type of lesion can develop due to repeated strokes. But there are cases when pseudobulbar syndrome (PS) makes itself felt even without previous cases of hemorrhage.

With such a problem, as a rule, bulbar functions begin to suffer. We are talking about swallowing, chewing, articulation and phonation. Violation of such functions leads to pathologies such as dysphagia, dysphonia, and dysarthria. The main difference between this syndrome and the bulbar syndrome is that there is no development of muscle atrophy and reflexes of oral automatism are observed:

Increased proboscis reflex;

Oppenheim reflex;

Astvatsaturov's nasolabial reflex;

Distant-arial and other similar reflexes.

Pseudobulbar syndrome - causes

The development of this syndrome is a consequence of atherosclerosis of cerebral vessels and the resulting foci of softening, which can be localized in both hemispheres.

But this is not the only factor leading to such a syndrome. The vascular form of syphilis of the brain, as well as neuroinfections, degenerative processes, infections and tumors affecting both hemispheres, can also have a negative impact.

In fact, pseudobulbar syndrome occurs when, against the background of some disease, the central pathways running from the motor centers of the cerebral cortex to the motor nuclei of the medulla oblongata are interrupted.

Pathogenesis

The development of such a syndrome manifests itself through severe atheromatosis of the arteries of the base of the brain, which affects both hemispheres. In childhood, bilateral damage to the corticobulbar conductors is recorded, resulting in cerebral palsy.

If you have to deal with the pyramidal form of pseudobulbar syndrome, the tendon reflex increases. With the extrapyramidal form, slow movements, stiffness, anemia and increased muscle tone are first recorded. The mixed form implies the total manifestation of the above-described signs indicating pseudobulbar syndrome. Photos of people affected by this syndrome confirm the seriousness of the disease.

Symptoms

One of the main symptoms of this disease includes impaired swallowing and chewing. In this condition, food begins to get stuck on the gums and behind the teeth, liquid food can flow out through the nose, and the patient often chokes during meals. Moreover, changes occur in the voice - it takes on a new shade. The sound becomes hoarse, consonants drop out, and some intonations are completely lost. Sometimes patients lose the ability to speak in a whisper.

With a problem such as pseudobulbar syndrome, symptoms can also be expressed through bilateral paresis of the facial muscles. This means that the face takes on a mask-like, anemic appearance. It is also possible to experience attacks of violent convulsive laughter or crying. But such symptoms are not always present.

It is worth mentioning the tendon reflex of the lower jaw, which can increase sharply during the development of the syndrome.

Often pseudobulbar syndrome is recorded in parallel with a disease such as hemiparesis. Extrapyramidal syndrome may occur, which leads to stiffness, increased muscle tone and slowness of movement. Intellectual impairment, which can be explained by the presence of multiple foci of softening in the brain, is also possible.

Moreover, unlike the bulbar form, this syndrome excludes the occurrence of disorders of the cardiovascular and respiratory systems. This is explained by the fact that pathological processes do not affect the vital centers, but develop in the medulla oblongata.

The syndrome itself can have either a gradual onset or an acute development. But if we consider the most common indicators, it can be argued that in the vast majority of cases, the appearance of pseudobulbar syndrome is preceded by two or more attacks of cerebrovascular accident.

Diagnostics

To determine pseudobulbar syndrome in children, it is necessary to differentiate its symptoms from nephritis, parkinsonism, bulbar palsy and nerves. One of the distinguishing features of the pseudoform will be the absence of atrophy.

It is worth noting that in some cases it can be quite difficult to distinguish PS from Parkinson-like paralysis. This disease progresses slowly, and in the later stages apoplectic strokes are recorded. Moreover, signs similar to the syndrome appear: violent crying, speech disorder, etc. Therefore, a qualified doctor must determine the patient’s condition.

Development of the syndrome in children

A problem such as pseudobulbar syndrome can manifest itself quite clearly in newborns. Already in the first month of life, signs of this disease become noticeable.

When examining a baby with pseudobulbar syndrome, fibrillation and atrophy are not detected, but the reflex of oral automatism is recorded. Also, such a syndrome can lead to pathological crying and laughter.

Sometimes doctors diagnose combined forms of pseudobulbar and bulbar syndrome. This form of the disease is a consequence of amyotrophic lateral sclerosis, thrombosis in the vertebrobasilar artery system, inoperable malignant tumors of the trunk or demyelinating processes.

Treatment of the syndrome

To influence pseudobulbar syndrome in children, you must initially take into account the stage of its occurrence. In any case, the treatment will be more effective the sooner the parents take the child to the doctor.

In the event that this syndrome progresses, drugs are usually used that are aimed at normalizing lipid metabolism, coagulation processes and reducing cholesterol in the blood. Drugs that improve microcirculation, bioenergetics of neurons and metabolic processes in the brain would be useful.

Such drugs as Encephabol, Aminalon, Cerebrolysin, etc. have a similar effect. In some cases, doctors may prescribe drugs that have an anticholinesterase effect (Proserin, Oxazil).

Considering what disorders pseudobulbar syndrome causes in children, it is extremely important to know the signs indicating its development. After all, if you ignore the obvious symptoms and do not start the treatment process on time, then you may not be able to completely neutralize the disease. This means that the child will suffer from swallowing dysfunction for the rest of his life, and not only that.

But if you react in a timely manner, the chances of recovery will be quite high. Especially if stem cells are used in the treatment process. Their administration in a disease such as pseudobulbar syndrome can have the effect of physically replacing the myelin sheath and, moreover, restoring the functions of cells that have been damaged. Such a restorative effect can return the patient to full functioning.

How to influence the condition in newly born children

If pseudobulbar syndrome has been diagnosed in newborns, treatment will involve an integrated approach. First of all, this is a massage of the orbicularis oris muscle, feeding through a tube and electrophoresis with proserin on the cervical spine.

Speaking about the first signs of recovery, it is worth noting that they include the appearance of reflexes of the newborn, which were previously absent, stabilization of the neurological status and positive changes in the deviations recorded earlier. Also, with successful treatment, there should be an increase in motor activity against the background of physical inactivity or an increase in muscle tone in the case of severe hypotension. In children with long gestational ages, a meaningful reaction to contact and emotional tone improves.

Recovery period in the treatment of newborns

In most cases, if you do not have to deal with incurable severe lesions, the early recovery period begins within the first 2-3 weeks of the child’s life. When dealing with a problem such as pseudobulbar syndrome, treatment at week 4 and beyond includes recovery therapy.

At the same time, for children who have had to endure seizures, drugs are selected more carefully. Cortexin is often used, the course of treatment is 10 injections. In addition to these measures, Pantogam and Nootropil are administered orally to children during treatment.

Massage and physiotherapy

Regarding the use of massage, it is worth noting that it has a predominantly tonic and, in rare cases, relaxing effect. It is also carried out for all children. For those newborns who have spasticity of the limbs, massage is indicated earlier - on the 10th day of life. But it is important not to exceed the current norm - 15 sessions. Moreover, this method of treatment is combined with taking Mydocalm (twice a day).

Physiotherapy, in turn, is focused on electrophoresis of magnesium sulfate with aloe or lidase on the cervical spine.

Pseudobulbar dysarthia

This is one of the diseases resulting from pseudobulbar syndrome. Its essence comes down to disruption of the pathways connecting the nuclei of the bulbar group with the cerebral cortex.

This disease can have three degrees:

- Lightweight. The violations are minor and manifest themselves in the fact that children have difficulty pronouncing growling and hissing sounds. When writing a text, the child sometimes confuses letters.

- Average. It occurs more often than others. In this case, there is virtually a complete absence of facial movements. Children have difficulty chewing and swallowing food. The tongue also moves poorly. In this state, the child cannot speak clearly.

- Severe (anarthria). Facial movements are completely absent, as well as the mobility of the muscles of the speech apparatus. In such children, the lower jaw droops, but the tongue remains motionless.

For this disease, drug treatment methods, massage and reflexology are used.

It is not difficult to conclude that this syndrome is a fairly serious threat to the child’s health, so the disease requires parents to quickly respond to symptoms and be patient during the treatment process.

Dysfunction of the cranial nerves, the nuclei of which are located in the medulla oblongata, is called bulbar syndrome. The main characteristic of the disease is general paralysis of the tongue, muscles of the pharynx, lips, epiglottis, vocal cords and soft palate. Often the consequence of bulbar syndrome (paralysis) is a disorder of the speech apparatus, the functions of swallowing, chewing and breathing.

A less pronounced degree of paralysis is manifested in cases of unilateral damage to the nuclei of the IX, X, XI and XII (caudal group) nerves, their trunks and roots in the medulla oblongata, but a severe degree of bulbar syndrome is more common with bilateral damage to the same nerves.

Pseudobulbar syndrome is a bilateral interruption of the corticonuclear pathways, leading to a neurological syndrome. It has a similar clinical picture to bulbar syndrome, but has a number of distinctive features and occurs due to damage to other parts and structures of the brain.

A significant difference between bulbar and pseudobulbar syndrome is that in the second there is no disturbance in the rhythm of cardiac activity, atrophy of paralyzed muscles, or cessation of breathing (apnea). It is often accompanied by unnatural forced laughter and crying of the patient, resulting from a disruption of the connection between the central subcortical nodes and the cerebral cortex. Most often, pseudobulbar syndrome occurs with diffuse brain lesions of traumatic, vascular, intoxication or infectious origin.

Bulbar syndrome: causes

The list of possible causes of paralysis is quite wide, it includes genetic, vascular, degenerative and infectious factors. Thus, genetic causes include acute intermittent porphyria and Kennedy bulbospinal amyotrophy, and degenerative causes include syringobulbia, Lyme disease, polio and Guillain-Barre syndrome. The cause of bulbar syndrome can also be a stroke of the medulla oblongata (ischemic), which is much more likely than other diseases to lead to death.

The development of bulbar syndrome occurs with amyotrophic lateral sclerosis, paroxysmal myoplegia, spinal amyotrophy Fazio-Londe, diphtheria, post-vaccination and paraneoplastic polyneuropathy, as well as due to hyperthyroidism.

Other probable causes of bulbar syndrome include diseases and processes in the posterior cranial fossa, brain and craniospinal region, such as:

• Botulism;
• Tumor in the medulla oblongata;
• Bone abnormalities;
• Syringobulbia;
• Granulomatous diseases;
• Meningitis;
• Encephalitis.

Against the background of paroxysmal myoplegia, myasthenia gravis, dystrophic myotonia, oculopharyngeal myopathy, Kearns-Sayre syndrome, psychogenic dysphonia and dysphagia, paralysis can also develop.

Symptoms of bulbar syndrome

With paralysis, patients have problems with the consumption of liquid food, they often choke on it, and sometimes they cannot reproduce swallowing movements, which is why saliva may flow from the corners of their mouth.

In especially severe cases of bulbar syndrome, a disorder of cardiovascular activity and respiratory rhythm may occur, which is due to the close location of the nuclei of the cranial nerves of the caudal group with the centers of the respiratory and cardiovascular systems. Such involvement of the cardiac and respiratory systems in the pathological process often leads to death.

A sign of bulbar syndrome is loss of the palatal and pharyngeal reflexes, as well as atrophy of the tongue muscles. This occurs due to damage to the nuclei of the IX and X nerves, which are part of the reflex arcs of the above reflexes.

The most common symptoms of bulbar syndrome are the following:

• The patient lacks facial expressions, he cannot swallow or chew food fully;
• Violation of phonation;
• Getting liquid food into the nasopharynx after consumption;
• Heart dysfunction;
• Nasal and slurred speech;
• In the case of unilateral bulbar syndrome, there is a deviation of the tongue to the side unaffected by paralysis, its twitching, as well as drooping of the soft palate;
• Breathing disorders;
• Absence of palatine and pharyngeal reflex;
• Pulse arrhythmia.

Symptoms of paralysis in each individual case can be of varying degrees of severity and complexity.

Diagnosis of bulbar syndrome

Before proceeding with direct treatment, the doctor must examine the patient, especially the oropharynx area, identify all the symptoms of the disease, and conduct an electromyography, according to which it is possible to determine the severity of paralysis.

Treatment of bulbar syndrome

In some cases, to save the life of a patient with bulbar syndrome, preliminary emergency care is required. The main goal of such assistance is to eliminate the threat to the patient’s life before he is transported to a medical facility, where adequate treatment will then be selected and prescribed.

The doctor, depending on the clinical symptoms and the nature of the pathology, can predict the outcome of the disease, as well as the effectiveness of the proposed treatment for bulbar syndrome, which is carried out in several stages, namely:

• Resuscitation, support of those body functions that were impaired due to paralysis - artificial ventilation to restore breathing, the use of Proserin, adenosine triphosphate and vitamins to trigger the swallowing reflex, the appointment of Atropine to reduce salivation;
• This is followed by symptomatic therapy aimed at alleviating the patient’s condition;
• Treatment of the disease against which the development of bulbar syndrome occurred.

Patients with paralysis are fed enterally, using a feeding tube.

Bulbar syndrome is a disease that occurs due to disruption of the cranial nerves. Often, even with adequate treatment, it is possible to achieve 100% recovery of the patient only in isolated cases, but it is quite possible to significantly improve the patient’s well-being.

Gradually developing dysfunction of the bulbar group of the caudal cranial nerves, caused by damage to their nuclei and/or roots. A triad of symptoms is characteristic: dysphagia, dysarthria, dysphonia. The diagnosis is made based on examination of the patient. Additional examinations (cerebrospinal fluid analysis, CT, MRI) are carried out to determine the underlying pathology that caused the bulbar palsy. Treatment is prescribed in accordance with the causative disease and existing symptoms. Urgent measures may be required: resuscitation, mechanical ventilation, combating heart failure and vascular disorders.

General information

Bulbar palsy occurs when the nuclei and/or roots of the bulbar group of cranial nerves located in the medulla oblongata are damaged. The bulbar nerves include the glossopharyngeal (IX pair), vagus (X pair) and hypoglossal (XII pair) nerves. The glossopharyngeal nerve innervates the muscles of the pharynx and provides its sensitivity, is responsible for the taste sensations of the posterior 1/3 of the tongue, and provides parasympathetic innervation to the parotid gland. The vagus nerve innervates the muscles of the pharynx, soft palate, larynx, upper digestive tract and respiratory tract; gives parasympathetic innervation of internal organs (bronchi, heart, gastrointestinal tract). The hypoglossal nerve provides innervation to the muscles of the tongue.

The cause of bulbar palsy may be chronic cerebral ischemia, which develops as a result of atherosclerosis or chronic vascular spasm in hypertension. Rare factors causing damage to the bulbar group of cranial nerves include craniovertebral anomalies (primarily Chiari malformation) and severe polyneuropathies (Guillain-Barré syndrome).

Symptoms of progressive bulbar palsy

The clinical manifestations of bulbar palsy are based on peripheral paresis of the muscles of the pharynx, larynx and tongue, which results in disturbances in swallowing and speech. The basic clinical symptom complex is a triad of signs: swallowing disorder (dysphagia), articulation disorder (dysarthria) and speech sonority (dysphonia). Difficulty swallowing food begins with difficulty taking liquids. Due to paresis of the soft palate, fluid from the oral cavity enters the nose. Then, with a decrease in the pharyngeal reflex, swallowing disorders of solid foods develop. Limitation of tongue mobility leads to difficulty chewing food and moving the food bolus in the mouth. Bulbar dysarthria is characterized by slurred speech and a lack of clarity in the pronunciation of sounds, which is why the patient’s speech becomes incomprehensible to others. Dysphonia manifests itself as hoarseness of voice. Nasolalia (nasality) is noted.

The patient's appearance is characteristic: the face is hypomimic, the mouth is open, there is drooling, difficulty chewing and swallowing food, and food falling out of the mouth. Due to damage to the vagus nerve and disruption of the parasympathetic innervation of somatic organs, disorders of respiratory function, heart rate and vascular tone occur. These are the most dangerous manifestations of bulbar palsy, since often progressive respiratory or heart failure causes the death of patients.

When examining the oral cavity, atrophic changes in the tongue, its folding and unevenness are noted, and fascicular contractions of the tongue muscles may be observed. The pharyngeal and palatal reflexes are sharply reduced or not evoked. Unilateral progressive bulbar palsy is accompanied by drooping of half of the soft palate and deviation of its uvula to the healthy side, atrophic changes in 1/2 of the tongue, deviation of the tongue towards the affected side when it protrudes. With bilateral bulbar palsy, glossoplegia is observed - complete immobility of the tongue.

Diagnostics

A neurologist can diagnose bulbar palsy by carefully studying the patient’s neurological status. The study of the function of the bulbar nerves includes assessment of the speed and intelligibility of speech, timbre of voice, volume of salivation; examination of the tongue for the presence of atrophies and fasciculations, assessment of its mobility; examination of the soft palate and checking the pharyngeal reflex. It is important to determine the frequency of breathing and heart contractions, and study the pulse to detect arrhythmia. Laryngoscopy allows you to determine the lack of complete closure of the vocal cords.

During diagnosis, progressive bulbar palsy must be distinguished from pseudobulbar palsy. The latter occurs with supranuclear damage to the corticobulbar tracts connecting the nuclei of the medulla oblongata with the cerebral cortex. Pseudobulbar palsy is manifested by central paresis of the muscles of the larynx, pharynx and tongue with hyperreflexia (increased pharyngeal and palatal reflexes) and increased muscle tone characteristic of all central paresis. Clinically it differs from bulbar palsy in the absence of atrophic changes in the tongue and the presence of oral automatism reflexes. Often accompanied by violent laughter resulting from spastic contraction of the facial muscles.

In addition to pseudobulbar palsy, progressive bulbar palsy requires differentiation from psychogenic dysphagia and dysphonia, various diseases with primary muscular damage causing myopathic paresis of the larynx and pharynx (myasthenia gravis, Rossolimo-Steinert-Kurshman myotonia, paroxysmal myoplegia, oculopharyngeal myopathy). It is also necessary to diagnose the underlying disease that led to the development of bulbar syndrome. For this purpose, a study of cerebrospinal fluid, CT and MRI of the brain is carried out. Tomographic studies make it possible to visualize brain tumors, demyelination zones, cerebral cysts, intracerebral hematomas, cerebral edema, displacement of cerebral structures during dislocation syndrome. CT or radiography of the craniovertebral junction can reveal abnormalities or post-traumatic changes in this area.

Treatment of progressive bulbar palsy

Treatment tactics for bulbar palsy are based on the underlying disease and leading symptoms. In case of infectious pathology, etiotropic therapy is carried out; in case of cerebral edema, decongestant diuretics are prescribed; in case of tumor processes, the issue of removing the tumor or performing shunt surgery to prevent dislocation syndrome is decided together with a neurosurgeon.

Unfortunately, many diseases in which bulbar syndrome occurs are a progressive degenerative process occurring in cerebral tissues and do not have effective specific treatment. In such cases, symptomatic therapy is carried out to support the vital functions of the body. Thus, in case of severe respiratory disorders, tracheal intubation is performed and the patient is connected to a ventilator; in case of severe dysphagia, tube feeding is provided; vascular disorders are corrected with the help of vasoactive drugs and infusion therapy. To reduce dysphagia, neostigmine, ATP, and vitamins are prescribed. B, glutamic acid; for hypersalivation - atropine.

Forecast

Progressive bulbar palsy has a highly variable prognosis. On the one hand, patients may die from cardiac or respiratory failure. On the other hand, with successful treatment of the underlying disease (for example, encephalitis), in most cases, patients recover with complete restoration of swallowing and speech function. Due to the lack of effective pathogenetic therapy, bulbar palsy associated with progressive degenerative damage to the central nervous system (with multiple sclerosis, ALS, etc.) has an unfavorable prognosis.