home Diseases Bulbar palsy. More than just a speech disorder: bulbar syndrome. Treatment of progressive bulbar palsy

Bulbar palsy. More than just a speech disorder: bulbar syndrome. Treatment of progressive bulbar palsy

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-Barre 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 arrhythmia. 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.

Dysfunction of the medulla oblongata causes bulbar palsy, which is also called bulbar syndrome. This disease is expressed in violation of the swallowing, chewing and respiratory functions of the human body. This occurs as a result of paralysis of various parts of the oral cavity.

The cause of the development of bulbar palsy is damage to the nuclei of the cranial nerves, as well as their roots and trunks. There are unilateral and bilateral lesions, as well as bulbar and pseudobulbar palsy.

Main parts of the brain

The difference between unilateral and bilateral nuclear damage is that the symptoms are less pronounced in the first case and the symptoms are more pronounced in the second.

As for this type of disease, such as bulbar palsy and pseudobulbar, there are several differences between these ailments.

Thus, with the pseudobulbar form of the disease, it is not the medulla oblongata that is affected, but its other parts, which affects the symptoms. Based on this, the patient does not experience such a manifestation as respiratory arrest or cardiac arrhythmia, but uncontrollable crying or laughter occurs.

Causes of the disease

There are several causes of bulbar palsy, in particular:

Genetic.
Degenerative.
Infectious.

As a rule, Bulbar palsy and pseudobulbar palsy develop as a result of one or another disease, and depending on this, the above division occurs. For example, Kennedy amyatrophy is a genetic disease that results in this disease.

What does a twisted tongue look like during paralysis?

In turn, diseases such as Guyet Barre syndrome, Lyme disease or polio act as degenerative causes.

In addition, bulbar paresis and paralysis can develop as a result of amyotrophic lateral sclerosis, botulism, syringobulbia, meningitis and encephalitis.

Important! People who have had to deal with such a phenomenon as ischemic stroke (transient disturbance of cerebral circulation) are at risk of acquiring infectious bulbar paralysis.

Symptoms

The symptoms of bulbar palsy are quite specific, and unlike the pseudobulbar type, the disease can lead to cardiac or respiratory arrest.

The main symptom of this disease is difficulty in the swallowing reflex, which can lead to increased uncontrolled salivation in the patient.

In addition, the disease is characterized by:

violation of speech functions;
difficulty chewing food;
twitching of the tongue, in case of paralysis;
protruding tongue located in the throat in the direction opposite to paralysis;
sagging upper palate;
phonation disorder;

Phonation – using the larynx to produce sounds

arrhythmia;
violation of the respiratory and cardiac functions of the body.

Infectious bulbar palsy is similar to pseudobulbar palsy in many symptoms, however, with the second type of disease there is no arrest of cardiac and respiratory activity. Also, no tongue twitching was noted during tongue paralysis.

Illness in childhood

If with adults everything is more or less clear, then with young children not everything is as clear as it seems.

Thus, in young children it is difficult to diagnose the disease based on the main symptom - increased salivation as a result of a difficult swallowing reflex. Every 3 children experience such a symptom, not because there is pathology, but because of the characteristics of the child’s body.

How to recognize an illness in a child? To do this, you need to examine the baby’s oral cavity and pay attention to the position of the tongue. If it is unnaturally shifted to the side or there is twitching, it makes sense to contact a specialist for a thorough analysis.

In addition, such children have problems swallowing food; it can fall out of the mouth or get into the nasopharynx if it is a liquid.

In addition, a sick child may experience partial facial paralysis, which manifests itself in the absence of changes in the baby’s facial expressions.

How to diagnose the disease?

Diagnosis of this disease does not include a huge number of tests and instrumental treatment methods. The basis is an external examination of the patient by a specialist, as well as a procedure such as electromyography.

Electromyography is the study of bioelectric potentials that arise in human muscles when muscle fibers are excited.

Based on the data obtained, the doctor makes a conclusion and prescribes treatment.

Treatment

As a rule, treatment of bulbar palsy is carried out in a hospital. However, it does not occur to a person to make an appointment with a surgeon in advance, and going to a medical institution with symptoms characteristic of this disease occurs already in the later stages of the disease, which entails a threat to the patient’s life.

For this reason, it is important to eliminate the threat to human life. In particular:

The patient may need to remove mucus from the throat to perform artificial ventilation. Only after the patient’s life is not in danger is it possible to transport him to a medical facility.

The hospital organizes treatment aimed at eliminating symptoms and directly eliminating, in particular:

To restore the swallowing reflex, a person is prescribed Proserin, Adenosine triphosphate and a vitamin complex

To eliminate uncontrollable drooling, the patient is prescribed Atropine

To eliminate symptomatic manifestations, medications specific to a particular symptom are prescribed (for each person individually)

Although the basis of treatment is aimed at eliminating the cause, this syndrome is difficult to treat, and since the nerve roots deep in the brain are usually affected, surgery to eliminate the problem is unlikely.

Nevertheless, thanks to modern treatment methods, bulbar palsy is not a death sentence, and it is possible to live with it normally, subject to supportive therapy. Therefore, if primary symptoms occur, it is better to contact a specialist once again and get good news than to delay it and hear an unpleasant diagnosis. Take care of yourself and your loved ones and do not self-medicate.

Bulbar syndrome in medicine is the so-called peripheral paralysis of the muscles of the soft palate, lips, tongue, vocal cords, which, in turn, occurs due to damage to 9, 10 and 12 pairs of the cranial nerves themselves or their nuclei. At the moment, experts have identified a great variety of reasons leading to this kind of illness. These include inflammatory processes in the brain, the appearance of tumors in it, and disruption of trophism. However, on the other hand, the concepts of bulbar and pseudobulbar syndrome should also be distinguished.

Primary symptoms of the disease

When a disease such as bulbar syndrome occurs, patients usually complain of the following symptoms:

liquid food getting into the nose;
dysphonia;
disturbances in respiratory processes (arrhythmia, Cheyne-Stokes breathing);
drop in cardiac activity;
almost complete disappearance of the mandibular reflex;
regular muscle twitching of the tongue.

Diagnosis

Experts' forecasts and possible complications

After diagnosis, a specialist is most often able to give an accurate prognosis of the course of the disease. Thus, in advanced stages and damage to the medulla oblongata, there is a high probability of death for the patient. The thing is that in this kind of situation, disturbances occur in the primary functions of the respiratory processes, as well as in the entire cardiovascular activity of the patient’s body as a whole.

Bulbar syndrome. Treatment

Therapy, according to modern medicine, should primarily be aimed at treating the underlying disease and simultaneously compensating for already lost vital primary functions of the body. So, to improve swallowing, glutamic acid, proserine, various vitamins, nootropic drugs and ATP are prescribed. In case of increased salivation, atropine is used. Absolutely all patients are fed through a special tube. At more advanced stages of the disease, when respiratory disturbances are observed, artificial ventilation is recommended. As for the issue of resuscitation measures, they, in turn, are carried out solely according to indications.

Conclusion

In conclusion, we note that modern medicine has literally taken a hundred steps forward in this matter over the past few years. Today, the number of deaths due to this diagnosis has decreased significantly.

Bulbar syndrome (or bulbar palsy) is a complex lesion of the IX, X and XII cranial nerves (vagus, glossopharyngeal and hypoglossal nerves), whose nuclei are located in the medulla oblongata. They innervate the muscles of the lips, soft palate, tongue, pharynx, larynx, as well as the vocal cords and epiglottic cartilage.

Symptoms

Bulbar palsy is a triad of three leading symptoms: dysphagia(swallowing disorder), dysarthria(violation of the correct pronunciation of articulate speech sounds) and aphonia(violation of speech sonority). A patient suffering from this paralysis cannot swallow solid food, and liquid food will enter the nose due to paresis of the soft palate. The patient’s speech will be incomprehensible with a hint of nasalism (nasalism), this disturbance is especially noticeable when the patient pronounces words containing such complex sounds as “l” and “r”.

To make a diagnosis, the doctor must conduct a study of the functions of the IX, X and XII pairs of cranial nerves. Diagnosis begins with finding out whether the patient has problems swallowing solid and liquid food, or whether he chokes on it. During the answer, the patient’s speech is carefully listened to, and the disturbances characteristic of paralysis noted above are noted. Then the doctor examines the oral cavity and performs laryngoscopy (a method of examining the larynx). With unilateral bulbar syndrome, the tip of the tongue will be directed towards the lesion, or completely motionless with bilateral one. The mucous membrane of the tongue will be thinned and folded - atrophic.

Examination of the soft palate will reveal its lag in pronunciation, as well as deviation of the uvula in the healthy direction. Using a special spatula, the doctor checks the palatine and pharyngeal reflexes, irritating the mucous membrane of the soft palate and the back wall of the pharynx. The absence of gagging and coughing movements indicates damage to the vagus and glossopharyngeal nerves. The study ends with laryngoscopy, which will help confirm paralysis of the true vocal cords.

The danger of bulbar syndrome lies in damage to the vagus nerve. Insufficient function of this nerve will cause abnormal heart rhythm and breathing problems, which can immediately lead to death.

Etiology

Depending on what disease causes bulbar palsy, there are two types: acute and progressive. Acute most often occurs due to acute circulatory disturbance in the medulla oblongata (infarction) due to thrombosis, vascular embolism, and also when the brain is wedged into the foramen magnum. Severe damage to the medulla oblongata leads to disruption of the vital functions of the body and subsequent death of the patient.

Progressive bulbar palsy develops in amyotrophic lateral sclerosis. This rare disease is a degenerative change in the central nervous system that causes damage to motor neurons, causing muscle atrophy and paralysis. ALS is characterized by all the symptoms of bulbar palsy: dysphagia when taking liquid and solid food, glossoplegia and tongue atrophy, sagging of the soft palate. Unfortunately, treatment for amyotrophic sclerosis has not been developed. Paralysis of the respiratory muscles causes the death of the patient due to the development of suffocation.

Bulbar palsy often accompanies a disease such as myasthenia gravis. It is not for nothing that the second name of the disease is asthenic bulbar palsy. The pathogenesis consists of an autoimmune lesion of the body, causing pathological muscle fatigue.

In addition to bulbar lesions, the symptoms include muscle fatigue after physical activity, which disappears after rest. Treatment of such patients consists of the doctor prescribing anticholinesterase drugs, most often Kalimin. Prescribing Proserin is not advisable due to its short-term effect and a large number of side effects.

Differential diagnosis

It is necessary to correctly differentiate bulbar syndrome from pseudobulbar palsy. Their manifestations are very similar, however, there is a significant difference. Pseudobulbar palsy is characterized by reflexes of oral automatism (proboscis reflex, distance-oral and palmar-plantar reflex), the occurrence of which is associated with damage to the pyramidal tracts.

The proboscis reflex is detected by carefully tapping the upper and lower lips with a neurological hammer - the patient pulls them out. The same reaction can be observed when the hammer approaches the lips - the distance-oral reflex. Streak irritation of the skin of the palm above the eminence of the thumb will be accompanied by contraction of the mentalis muscle, causing the skin to be pulled up on the chin - the palmomental reflex.

Treatment and prevention

First of all, treatment of bulbar syndrome is aimed at eliminating the cause that caused it. Symptomatic therapy consists of eliminating respiratory failure using a ventilator. To restore swallowing, a cholinesterase inhibitor is prescribed. It blocks cholyesterase, as a result of which the action of acetylcholine is enhanced, leading to the restoration of conduction along the neuromuscular fiber.

M-anticholinergic Atropine blocks M-cholinergic receptors, thereby eliminating increased salivation. Patients are fed through a tube. All other treatment measures will depend on the specific disease.

There is no specific prevention for this syndrome. To prevent the development of bulbar palsy, it is necessary to promptly treat diseases that can cause it.

Video about how exercise therapy is performed for bulbar syndrome:

One of the most dangerous neurological disorders is bulbar syndrome. This symptom complex occurs with combined peripheral damage to several pairs of cranial nerves of the caudal group. Bulbar syndrome that quickly appears and increases in severity is potentially life-threatening. In this case, emergency hospitalization of the patient is necessary to monitor his condition and provide intensive care.

Pathogenesis

Depending on the etiology of the underlying disease, there is a disruption of synaptic transmission with relative structural preservation of the main formations, destruction of nuclei or pronounced compression of nerve structures. In this case, there is no disruption of the conduction of impulses along the cortical-nuclear pathways and damage to central motor neurons in the frontal region of the brain, which distinguishes bulbar syndrome from. This is important not only for diagnosing the level of damage in the central nervous system and determining the cause of the formation of the main symptoms, but also for assessing the prognosis of the disease.

Bulbar syndrome develops with simultaneous damage to the IX, X and XII pairs of cranial nerves. The pathological process can involve their motor nuclei in the medulla oblongata (previously called the bulb), roots extending to the base of the brain, or already formed nerves. Damage to the nuclei is usually bilateral or bilateral; mosaicism is not typical for this syndrome.

Paralysis of the muscles of the tongue, soft palate, pharynx, epiglottis and larynx that develops with bulbar syndrome is classified as peripheral. Therefore, they are accompanied by a decrease or loss of the palatine and pharyngeal reflex, hypotonicity and subsequent atrophy of paralyzed muscles. It is also possible that fasciculations may appear when examining the tongue. And the subsequent involvement of neurons of the respiratory and vasomotor centers in the medulla oblongata in the pathological process, and a violation of parasympathetic regulation become the cause of the development of life-threatening conditions.

Main reasons

The cause of bulbar syndrome can be:

vascular accidents in the vertebrobasilar region, leading to ischemic or hemorrhagic damage to the craniospinal region;
primary and metastatic tumors of the brainstem and medulla oblongata, sarcomatosis, granulomatosis of various etiologies;
conditions that give a positive mass effect without a clearly defined formation in the posterior cranial fossa and threaten wedging of the brain into the foramen magnum (hemorrhages, swelling of the nervous tissue in neighboring areas or acute diffuse brain damage);
leading to compression of the medulla oblongata;
fracture of the base of the skull;
and various etiologies;
polyneuropathy (paraneoplastic, diphtheria, Guillain-Barre, post-vaccination, endocrine), ;
, as well as genetically determined spinal-bulbar amyotrophy Kennedy, and bulbospinal amyotrophy of childhood (Fazio-Londe disease);
suppression of brain motor neuron activity by botulinum toxin.

Many authors also classify changes in the muscles of the soft palate, pharynx and larynx as bulbar syndrome. In this case, their cause is a violation of neuromuscular transmission or primary damage to muscle tissue in myopathies, or dystrophic myotonia. The medulla oblongata (bulb) remains intact in myopathic diseases, so they speak of a special form of bulbar palsy.

Clinical picture

A characteristic sign of bulbar syndrome is deviation of the tongue towards the affected side.

Combined peripheral damage to the glossopharyngeal, vagus and hypoglossal nerves leads to paresis of the muscles of the palate, pharynx, larynx, and tongue. Characteristic is the combination of the triad “dysphonia-dysarthria-dysphagia” with paresis of half the tongue, sagging of the palatal curtain and the disappearance of the pharyngeal and palatal reflexes. Changes visible in the oropharynx are most often asymmetrical; the appearance of bilateral bulbar symptoms is a prognostically unfavorable sign.

Upon examination, deviation (deviation) of the tongue towards the lesion is revealed. Its paralyzed half becomes hypotonic and inactive, and fasciculations may appear in it. With bilateral bulbar palsy, there is almost complete immobility of the entire tongue, or glossoplegia. Due to the increasing atrophy of the paretic muscles, the affected half of the tongue gradually becomes thinner and becomes pathologically folded.

Paresis of the muscles of the soft palate leads to immobility of the palatine arches, sagging and hypotension of the palatine curtain with deviation of the uvula to the healthy side. Together with the loss of the pharyngeal reflex, disruption of the functioning of the muscles of the pharynx and epiglottis, this becomes the cause of dysphagia. There are difficulties when swallowing, choking, reflux of food and liquids into the nasal cavity and respiratory tract. Therefore, patients with bulbar syndrome have a high risk of developing aspiration pneumonia and bronchitis.

When the parasympathetic portion of the hypoglossal nerve is damaged, the autonomic innervation of the salivary glands is disrupted. The resulting increase in saliva production, together with impaired swallowing, causes drooling. Sometimes it is so pronounced that patients are forced to constantly use a scarf.

Dysphonia in bulbar syndrome is manifested by nasality, deafness and hoarseness of the voice due to paralysis of the vocal cords and paresis of the soft palate. The nasal tone of speech is called nasolalia; such nasalization of sound pronunciation can appear in the absence of obvious disorders of swallowing and choking. Dysphonia is combined with dysarthria, when slurred speech occurs due to impaired mobility of the tongue and other muscles involved in articulation. Damage to the medulla oblongata often leads to a combination of bulbar syndrome with facial nerve paresis, which also affects speech intelligibility.

With severe paralysis of the muscles of the palate, pharynx and larynx, asphyxia may occur due to mechanical blocking of the lumen of the respiratory tract. With bilateral damage to the vagus nerve (or its nuclei in the medulla oblongata), the work of the heart and respiratory system is inhibited, which is due to a violation of their parasympathetic regulation.

Treatment

It is not the bulbar syndrome itself that requires treatment, but the underlying disease and the resulting life-threatening conditions. If the severity of symptoms increases and signs of cardiac and respiratory failure appear, the patient requires transfer to the intensive care unit. According to indications, mechanical ventilation is performed and a nasogastric tube is installed.

To correct disorders, in addition to etiotropic therapy, drugs of various groups with neurotrophic, neuroprotective, metabolic, and vascular effects are prescribed. Hypersalivation can be reduced with atropine. During the recovery period or for chronic diseases, massage, classes with a speech therapist, and kinesiotherapy are prescribed to improve speech and swallowing.

Bulbar syndrome is a serious sign of damage to the medulla oblongata. Its appearance requires a mandatory visit to a doctor to clarify the etiology and decide on the need for hospitalization.

Petrov K. B., MD, professor, brings to your attention a slide show about clinical variants of bulbar syndrome and non-drug methods of treating this condition: