Bulbar syndrome in newborns. Symptoms and treatment of bulbar syndrome. Difference from bulbar syndrome

Motor neuron diseases (amyotrophic lateral sclerosis, Fazio-Londe spinal amyotrophy, Kennedy bulbospinal amyotrophy).
Myopathies (oculopharyngeal, Kearns-Sayre syndrome).
Dystrophic myotonia.
Paroxysmal myoplegia.
Myasthenia.
Polyneuropathy (Guillain-Barré, post-vaccination, diphtheria, paraneoplastic, with hyperthyroidism, porphyria).
Polio.
Processes in the brain stem, posterior cranial fossa and craniospinal region (vascular, tumor, syringobulbia, meningitis, encephalitis, granulomatous diseases, bone anomalies).
Psychogenic dysphonia and dysphagia.

Motor neuron diseases

The end stage of all forms of amyotrophic lateral syndrome (ALS) or the onset of its bulbar form are typical examples of bulbar dysfunction. Usually the disease begins with bilateral damage to the nucleus of the XII nerve and its first manifestations are atrophy, fasciculations and paralysis of the tongue. In the first stages, dysarthria without dysphagia or dysphagia without dysarthria may occur, but quite quickly there is a progressive deterioration of all bulbar functions. At the onset of the disease, difficulty swallowing liquid food is observed more often than solid food, but as the disease progresses, dysphagia develops when eating solid food. In this case, the weakness of the tongue is accompanied by weakness of the masticatory and then facial muscles, the soft palate hangs down, the tongue in the oral cavity is motionless and atrophic. Fasciculations are visible in it. Anarthria. Constant drooling. Weakness of the respiratory muscles. In the same area or in other regions of the body, symptoms of upper motor neuron involvement are detected.

Criteria for the diagnosis of amyotrophic lateral sclerosis

the presence of signs of damage to the lower motor neuron (including EMG - confirmation of the anterior horn process in clinically intact muscles); clinical symptoms of upper motor neuron damage (pyramidal syndrome); progressive course.

“Progressive bulbar palsy” is today considered as one of the variants of the bulbar form of amyotrophic lateral sclerosis (just like “primary lateral sclerosis” as another type of amyotrophic lateral sclerosis, occurring without clinical signs of damage to the anterior horns spinal cord).

Increasing bulbar palsy may be a manifestation of progressive spinal amyotrophy, in particular - terminal stage amyotrophy Werdnig-Hoffmann, and in children - spinal amyotrophy Fazio-Londe. The latter refers to autosomal recessive spinal amyotrophies with onset in early childhood. In adults, X-linked bulbar spinal amyotrophy is known, beginning at the age of 40 years and older (Kennedy disease). Characterized by weakness and atrophy of the proximal muscles upper limbs, spontaneous fasciculations, limited volumes active movements in the arms, decreased tendon reflexes in the biceps and triceps brachii muscles. As the disease progresses, bulbar (usually mild) disorders develop: choking, tongue atrophy, dysarthria. The leg muscles are involved later. Characteristics: gynecomastia and pseudohypertrophy of the calf muscles.

With progressive spinal amyotrophies, the process is limited to damage to the cells of the anterior horns of the spinal cord. Unlike amyotrophic lateral sclerosis, the process here is always symmetrical, it is not accompanied by symptoms of upper motor neuron involvement and has a more favorable course.

Myopathies

Some forms of myopathies (oculopharyngeal, Kearns-Sayre syndrome) may manifest as impaired bulbar functions. Oculopharyngeal myopathy (dystrophy) is a hereditary (autosomal dominant) disease, characterized by a late onset (usually after 45 years) and muscle weakness, which is limited to the facial muscles (bilateral ptosis) and bulbar muscles (dysphagia). Ptosis, swallowing disorders and dysphonia progress slowly. The main maladaptive syndrome is dysphagia. The process extends to the limbs only in some patients and in late stages diseases.

One of the forms of mitochondrial encephalomyopathy, namely Kearns-Sayre syndrome (“ophthalmoplegia plus”), manifests itself, in addition to ptosis and ophthalmoplegia, with a myopathic symptom complex that develops later eye symptoms. Involvement of the bulbar muscles (larynx and pharynx) is usually not severe enough, but can lead to changes in phonation and articulation, and choking.

Obligate signs of Kearns-Sayre syndrome:

external ophthalmoplegia
pigmentary degeneration retina
cardiac conduction disturbances (bradycardia, atrioventricular block, syncope, possible sudden death)
increased protein levels in the cerebrospinal fluid

Dystrophic myotonia

Dystrophic myotonia (or Rossolimo-Kurshman-Steinert-Batten myotonic dystorophy) is inherited in an autosomal dominant manner and affects men 3 times more often than women. Her debut occurs at the age of 16-20 years. The clinical picture consists of myotonic, myopathic syndromes and extramuscular disorders ( dystrophic changes in the lens, testes and other endocrine glands, skin, esophagus, heart and sometimes in the brain). Myopathic syndrome is most pronounced in the muscles of the face (masticatory and temporal muscles, which leads to a characteristic facial expression), neck and, in some patients, in the limbs. Damage to the bulbar muscles leads to a nasal tone of voice, dysphagia and choking, and sometimes to respiratory disorders (including sleep apnea).

Paroxysmal myoplegia (periodic paralysis)

Paroxysmal myoplegia is a disease (hypokalemic, hyperkalemic and normokalemic forms), manifested by generalized or partial attacks of muscle weakness (without loss of consciousness) in the form of paresis or plegia (up to tetraplegia) with decreased tendon reflexes and muscle hypotonia. The duration of attacks varies from 30 minutes to several days. Provoking factors: rich food rich in carbohydrates, abuse of table salt, negative emotions, physical exercise, night sleep. Only in some attacks there is involvement of the cervical and cranial muscles. Rarely the respiratory muscles are involved in the process to one degree or another.

Differential diagnosis carried out with secondary forms of myoplegia, which occur in patients with thyrotoxicosis, with primary hyperaldosteronism, hypokalemia with some gastrointestinal diseases, kidney diseases. Iattrogenic variants of periodic paralysis have been described when prescribed drugs that promote the removal of potassium from the body (diuretics, laxatives, licorice).

Myasthenia gravis

Bulbar syndrome is one of the dangerous manifestations of myasthenia gravis. Myasthenia gravis is a disease leading clinical manifestation which is pathological muscle fatigue, decreasing until complete recovery after taking anticholinesterase drugs. The first symptoms are often dysfunction of the extraocular muscles (ptosis, diplopia and limited mobility eyeballs) and facial muscles, as well as muscles of the limbs. In approximately one third of patients, involvement of the masticatory muscles, muscles of the pharynx, larynx and tongue is observed. There are generalized and local (mainly ocular) forms.

Differential diagnosis myasthenia gravis is carried out with myasthenic syndromes (Lambert-Eaton syndrome, myasthenic syndrome with polyneuropathies, myasthenia-polymyositis complex, myasthenic syndrome with botulinum intoxication).

Polyneuropathy

Bulbar palsy in polyneuropathies is observed in the picture of a generalized polyneuropathic syndrome against the background of tetraparesis or tetraplegia with characteristic sensory disorders, which facilitates the diagnosis of the nature of bulbar disorders. The latter are characteristic of such forms as acute demyelinating polyneuropathy Guillain-Barré, post-infectious and post-vaccination polyneuropathy, diphtheria and paraneoplastic polyneuropathy, as well as polyneuropathy in hyperthyroidism and porphyria.

Polio

Acute poliomyelitis, as a cause of bulbar palsy, is recognized by the presence of general infectious (preparalytic) symptoms, the rapid development of paralysis (usually in the first 5 days of illness) with greater damage to the proximal parts than the distal ones. A period of reverse development of paralysis soon after its appearance is characteristic. There are spinal, bulbar and bulbospinal forms. More often affected lower limbs(in 80% of cases), but the development of hemitype or cross-type syndromes is possible. Paralysis is sluggish in nature with loss of tendon reflexes and rapid development of atrophy. Bulbar palsy can be observed in the bulbar form (10-15% of the entire paralytic form of the disease), in which the nuclei of not only the IX, X (less often XII) nerves, but also the facial nerve are affected. Damage to the anterior horns of segments IV-V can cause respiratory paralysis. In adults, the bulbospinal form more often develops. Involvement of the reticular formation of the brain stem can lead to cardiovascular (hypotension, hypertension, cardiac arrhythmias), respiratory (“atactic breathing”) disorders, swallowing disorders, and disturbances in the level of wakefulness.

Differential diagnosis carried out with others viral infections, capable of affecting the lower motor neuron: rabies and herpes zoster. Other diseases that often require a differential diagnosis from acute polio include Guillain-Barré syndrome, acute intermittent porphyria, botulism, toxic polyneuropathies, transverse myelitis, and acute spinal cord compression due to epidural abscess.

Bulbar syndrome (paralysis) occurs with peripheral paralysis of muscles innervated by the IX, X and XII pairs of cranial nerves in the case of their combined lesions. The clinical picture includes: dysphagia, dysphonia or aphonia, dysarthria or anarthria.

Pseudobulbar syndrome (paralysis) is a central paralysis of muscles innervated by the IX, X and XII pairs of cranial nerves. The clinical picture of pseudobulbar syndrome resembles that of bulbar syndrome (dysphagia, dysphonia, dysarthria), but it is much milder. By its nature, pseudobulbar palsy is a central paralysis and, accordingly, it has symptoms of spastic paralysis.

Often, despite the early use of modern drugs, complete recovery from bulbar and pseudobulbar syndromes (paralysis) does not occur, especially when months and years have passed since the injury.

However, very good results are achieved when using stem cells for bulbar and pseudobulbar syndromes (paralysis).

Stem cells introduced into the body of a patient with bulbar or pseudobulbar syndrome (paralysis) not only physically replace the defect in the myelin sheath, but also take on the function of damaged cells. Being integrated into the patient’s body, they restore the myelin sheath of the nerve, its conductivity, strengthen and stimulate it.

As a result of treatment, in patients with bulbar and pseudobulbar syndrome (paralysis), dysphagia, dysphonia, aphonia, dysarthria, anarthria disappear, brain functions are restored, and the person returns to normal functioning.

Pseudobulbar palsy

Pseudobulbar palsy (synonymous with false bulbar palsy) - clinical syndrome, characterized by disorders of chewing, swallowing, speech, and facial expressions. It occurs when the central pathways running from the motor centers of the cerebral cortex to the motor nuclei of the cranial nerves are interrupted medulla oblongata, in contrast to boulevard paralysis (see), in which the nuclei themselves or their roots are affected. Pseudobulbar palsy develops only with bilateral damage to the cerebral hemispheres, since interruption of the pathways to the nuclei of one hemisphere does not cause noticeable bulbar disorders. Reason pseudobulbar palsy Usually there is atherosclerosis of cerebral vessels with foci of softening in both hemispheres of the brain. However, pseudobulbar palsy can also be observed with the vascular form of cerebral syphilis, neuroinfections, tumors, and degenerative processes affecting both hemispheres of the brain.

One of the main symptoms of pseudobulbar palsy is impaired chewing and swallowing. Food gets stuck behind the teeth and on the gums, the patient chokes when eating, and liquid food flows out through the nose. The voice takes on a nasal tone, becomes hoarse, loses intonation, difficult consonants are completely dropped out, some patients cannot even speak in a whisper. Due to bilateral paresis of the facial muscles, the face becomes amicable, mask-like, and often has a crying expression. Characterized by attacks of violent convulsive crying and laughter, occurring without corresponding emotions. Some patients may not have this symptom. The tendon reflex of the lower jaw increases sharply. Symptoms of so-called oral automatism appear (see Reflexes). Often pseudobulbar syndrome occurs simultaneously with hemiparesis. Patients often have more or less pronounced hemiparesis or paresis of all extremities with pyramidal signs. In other patients, in the absence of paresis, a pronounced extrapyramidal syndrome appears (see Extrapyramidal system) in the form of slowness of movements, stiffness, increased muscle tone (muscle rigidity). The intellectual impairments observed in pseudobulbar syndrome are explained by multiple foci of softening in the brain.

The onset of the disease in most cases is acute, but sometimes it can develop gradually. In most patients, pseudobulbar palsy occurs as a result of two or more attacks of cerebrovascular accident. Death occurs from bronchopneumonia caused by food getting into Airways, associated infection, stroke, etc.

Treatment should be directed against the underlying disease. To improve the act of chewing, you need to prescribe prozerin 0.015 g 3 times a day with meals.

Pseudobulbar palsy (synonym: false bulbar palsy, supranuclear bulbar palsy, cerebrobulbar palsy) is a clinical syndrome characterized by disorders of swallowing, chewing, phonation and speech articulation, as well as amymia.

Pseudobulbar palsy, in contrast to boulevard paralysis (see), which depends on damage to the motor nuclei of the medulla oblongata, occurs as a result of a break in the paths running from the motor zone of the cerebral cortex to these nuclei. When supranuclear pathways are damaged in both hemispheres of the brain, voluntary innervation of the bulbar nuclei is lost and “false” bulbar palsy occurs, false because anatomically the medulla oblongata itself is not affected. Damage to the supranuclear tracts in one hemisphere of the brain does not produce noticeable bulbar disorders, since the nuclei of the glossopharyngeal and vagus nerves (as well as the trigeminal and upper branch facial nerve) have bilateral cortical innervation.

Pathological anatomy and pathogenesis. With pseudobulbar palsy, in most cases there is severe atheromatosis of the arteries of the base of the brain, affecting both hemispheres while sparing the medulla oblongata and pons. More often, pseudobulbar palsy occurs due to thrombosis of the cerebral arteries and is observed mainly in old age. In middle age, P. p. can be caused by syphilitic endarteritis. In childhood, P. p. is one of the symptoms of childhood cerebral palsy with bilateral damage to the corticobulbar conductors.

The clinical course and symptomatology of pseudobulbar palsy is characterized by bilateral central palsy, or paresis, of the trigeminal, facial, glossopharyngeal, vagus and hypoglossal cranial nerves in the absence of degenerative atrophy in the paralyzed muscles, preservation of reflexes and disorders of the pyramidal, extrapyramidal or cerebellar systems. Swallowing disorders with P. p. do not reach the degree of bulbar paralysis; due to weakness of the masticatory muscles, patients eat extremely slowly, food falls out of the mouth; patients choke. If food enters the respiratory tract, aspiration pneumonia can develop. The tongue is motionless or extends only to the teeth. Speech is insufficiently articulated, with a nasal tint; the voice is quiet, the words are pronounced with difficulty.

One of the main symptoms of pseudobulbar palsy is attacks of convulsive laughter and crying, which are of a violent nature; the facial muscles, which in such patients cannot contract voluntarily, become excessively contracted. Patients may begin to cry involuntarily when showing their teeth or stroking the upper lip with a piece of paper. The occurrence of this symptom is explained by a break in the inhibitory pathways heading to the bulbar centers, a violation of the integrity of subcortical formations (the optic thalamus, striatum, etc.).

The face acquires a mask-like character due to bilateral paresis of the facial muscles. During attacks of violent laughter or crying, the eyelids close well. If you ask the patient to open or close his eyes, he opens his mouth. This peculiar disorder of voluntary movements should also be classified as one of characteristic features pseudobulbar palsy.

There is also an increase in deep and superficial reflexes in the area of masticatory and facial muscles, as well as the emergence of reflexes of oral automatism. This should include Oppenheim's symptom (sucking and swallowing movements when touching the lips); lip reflex (contraction orbicularis muscle mouth when tapping in the area of this muscle); Bekhterev's oral reflex (lip movements when tapping with a hammer around the mouth); buccal Toulouse-Wurp phenomenon (movement of the cheeks and lips is caused by percussion on the side of the lip); Astvatsaturov's nasolabial reflex (proboscis-like closing of the lips when tapping on the root of the nose). When stroking the patient's lips, a rhythmic movement of the lips and lower jaw occurs - sucking movements, sometimes turning into violent crying.

There are pyramidal, extrapyramidal, mixed, cerebellar and infantile forms of pseudobulbar palsy, as well as spastic.

The pyramidal (paralytic) form of pseudobulbar palsy is characterized by more or less clearly expressed hemi- or tetraplegia or paresis with increased tendon reflexes and the appearance of pyramidal signs.

Extrapyramidal form: slowness of all movements, amymia, stiffness, increased muscle tone of the extrapyramidal type with a characteristic gait (small steps) come to the fore.

Mixed form: a combination of the above forms of P. p.

Cerebellar form: ataxic gait, coordination disorders, etc. come to the fore.

The children's form of P. p. is observed when spastic diplegia. The newborn sucks poorly, choke and choke. Subsequently, the child develops violent crying and laughter, and dysarthria is detected (see Infantile paralysis).

Weil (A. Weil) described the familial spastic form of P. p. With it, along with pronounced focal disorders inherent in P. p., noticeable intellectual retardation is noted. A similar form was also described by M. Klippel.

Since the symptom complex of pseudobulbar palsy is caused mostly by sclerotic lesions of the brain, patients with P. p. often exhibit corresponding mental symptoms: decreased

memory, difficulty thinking, increased efficiency, etc.

The course of the disease corresponds to the variety of causes causing pseudobulbar palsy and the prevalence of the pathological process. The progression of the disease is most often stroke-like with varying periods between strokes. If after a stroke (see) paretic phenomena in the extremities subside, then the bulbar phenomena remain for the most part persistent. More often, the patient’s condition worsens due to new strokes, especially with cerebral atherosclerosis. The duration of the disease varies. Death occurs from pneumonia, uremia, infectious diseases, new hemorrhage, nephritis, cardiac weakness, etc.

The diagnosis of pseudobulbar palsy is not difficult. Should be differentiated from various forms boulevard palsy, neuritis of the bulbar nerves, parkinsonism. The absence of atrophy and increased bulbar reflexes speak against apoplectic bulbar palsy. It is more difficult to distinguish P. p. from parkinson-like disease. It has a slow course, in the later stages apoplectic strokes occur. In these cases, attacks of violent crying are also observed, speech is upset, and patients cannot eat on their own. The diagnosis may be difficult only in distinguishing cerebral atherosclerosis from the pseudobulbar component; the latter is characterized by severe focal symptoms, strokes, etc. Pseudobulbar syndrome in these cases may appear as an integral part of the main suffering.

Bulbar and pseudobulbar syndromes

In the clinic, it is more often observed not isolated, but combined damage to the nerves of the bulbar group or their nuclei. The simitom complex of movement disorders that occurs when the nuclei or roots of the IX, X, XII pairs of cranial nerves at the base of the brain are damaged is called boulevard syndrome (or bulbar palsy). This name comes from Lat. bulbus bulb (the old name for the medulla oblongata, in which the nuclei of these nerves are located).

Bulbar syndrome can be unilateral or bilateral. With bulbar syndrome, peripheral paresis or paralysis of muscles occurs, which are innervated by the glossopharyngeal, vagus and hypoglossal nerves.

With this syndrome, swallowing disorders are primarily observed. Normally, when eating, food is directed towards the pharynx by the tongue. At the same time, the larynx rises upward, and the root of the tongue presses the epiglottis, covering the entrance to the larynx and opening the way for the bolus of food to the pharynx. The soft palate rises upward, preventing liquid food from entering the nose. With bulbar syndrome, paresis or paralysis of the muscles involved in the act of swallowing occurs, resulting in impaired swallowing - dysphagia. The patient chokes while eating, swallowing becomes difficult or even impossible (phagia). Liquid food goes into the nose, solid food can go into the larynx. Food entering the trachea and bronchi can cause aspiration pneumonia.

In the presence of bulbar syndrome, voice and speech articulation disorders also occur. The voice becomes hoarse (dysphonia) with a nasal tinge. Paresis of the tongue causes a violation of speech articulation (dysarthria), and its paralysis causes anarthria, when the patient, well understanding the speech addressed to him, cannot pronounce words himself. The tongue atrophies; with pathology of the nucleus of the XII pair, fibrillary muscle twitching is observed in the tongue. The pharyngeal and palatal reflexes decrease or disappear.

With bulbar syndrome, autonomic disorders (respiratory and cardiac problems) are possible, which in some cases cause an unfavorable prognosis. Bulbar syndrome is observed with tumors of the posterior cranial fossa, ischemic stroke in the medulla oblongata, syringobulbia, amyotrophic lateral sclerosis, tick-borne encephalitis, post-diphtheria polyneuropathy and some other diseases.

Central paresis of muscles innervated by the bulbar nerves is called pseudobulbar syndrome. It occurs only with bilateral damage to the corticonuclear pathways running from the motor cortical centers to the nuclei of the bulbar group nerves. Damage to the corticonuclear pathway in one hemisphere does not lead to such a combined pathology, since the muscles innervated by the bulbar nerves, in addition to the tongue, receive bilateral cortical innervation. Since pseudobulbar syndrome is a central paralysis of swallowing, phonation and speech articulation, it also causes dysphagia, dysphonia, and dysarthria, but unlike bulbar syndrome, there is no atrophy of the tongue muscles and fibrillary twitches, the pharyngeal and palatal reflexes are preserved, and the mandibular reflex increases. With pseudobulbar syndrome, patients develop reflexes of oral automatism (proboscis, nasolabial, palmomental, etc.), which is explained by disinhibition due to bilateral damage to the corticonuclear pathways of the subcortical and brainstem formations, at the level of which these reflexes are closed. For this reason, sometimes violent crying or laughing occurs. With pseudobulbar syndrome, movement disorders may be accompanied by a decrease in memory, attention, and intelligence. Pseudobulbar syndrome is most often observed in acute cerebrovascular accidents in both hemispheres of the brain, dyscirculatory encephalopathy, and amyotrophic lateral sclerosis. Despite the symmetry and severity of the lesion, pseudobulbar syndrome is less dangerous than bulbar syndrome, since it is not accompanied by a violation of vital functions. important functions.

With bulbar or pseudobulbar syndrome, it is important to carefully care for the oral cavity, monitor the patient while eating to prevent aspiration, and tube feeding for aphagia.

Such a problem as the development of pseudobulbar syndrome in a child is a real test for parents. The point 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, in contrast to the bulbar form, this syndrome eliminates the occurrence of cardiovascular disorders 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 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 proserine on cervical region 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 motor activity against the background of physical inactivity or an increase in muscle tone in 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, early recovery period begins within the first 2-3 weeks of a child’s life. When dealing with a problem such as pseudobulbar syndrome, treatment at week 4 and beyond includes recovery period 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, it is actually observed complete absence 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 is 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 is similar to bulbar syndrome clinical picture, however, has a number 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

Scroll possible reasons The origin of paralysis is quite wide, it includes genetic, vascular, degenerative and infectious factors. Thus, genetic causes include acute intermittent porphyria and bulbo spinal amyotrophy Kennedy, to degenerative ones - 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.

To others probable reasons Bulbar syndrome includes such diseases and processes in the posterior cranial fossa, brain and craniospinal region 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 particularly 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 in pathological process cardiac and respiratory systems 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 may be varying degrees expressiveness 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 urgent Care. The main goal of such assistance is to eliminate the threat to the patient's life before he is transported to medical institution, in which adequate treatment will then be selected and prescribed.

Doctor, depending on clinical symptoms and the nature of the pathology, can predict the outcome of the disease, as well as the effectiveness of the proposed treatment of 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;
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.

Bulbar syndrome refers to a pathology of cranial nerves, when not a single nerve is affected, but a whole group: glossopharyngeal, vagus and hypoglossal, the nuclei of which are located in the medulla oblongata.

Symptoms and causes of bulbar syndrome

Bulbar syndrome (otherwise known as bulbar palsy) can be unilateral or bilateral. Swallowing disorders most often occur against the background of pathology. This leads to the fact that it becomes difficult or impossible (aphagia). Also, usually as a result of the syndrome, speech and voice articulation disorders occur - it acquires a nasal and hoarse tone.

Disorders of the autonomic system may also develop, usually manifesting themselves in the form of disturbances in cardiac activity and breathing.

The most common signs of bulbar syndrome are:

Lack of facial expressions, inability to chew, swallow, or close your mouth independently;
Entry of liquid food into the nasopharynx;
Hanging of the soft palate and deviation of the uvula in the direction opposite to the paralysis;
Absence of palatal and pharyngeal reflexes;
Slurred, nasal speech;
Phonation disorders;
Cardiovascular disorders;
Arrhythmia.

Doctors include various diseases as the main causes of bulbar syndrome:

Myopathy – oculopharyngeal, Kearns-Sayre syndrome. Feature hereditary disease oculopharyngeal myopathy, as one of the causes of bulbar syndrome, is a late onset (most often after 45 years), as well as muscle weakness limited to the facial muscles and bulbar muscles. The main maladaptive syndrome is dysphagia;
Motor neuron diseases – Fazio-Londe spinal amyotrophy, amyotrophic lateral sclerosis, Kennedy bulbospinal amyotrophy. At the onset of the disease, there is difficulty swallowing liquid food more often than solid food. However, as the disease progresses, along with tongue weakness, weakness of the facial and masticatory muscles develops, and the tongue in the oral cavity becomes atrophic and immobile. Weakness of the respiratory muscles and constant drooling occur;
Dystrophic myotonia, which most often affects men. The disease begins at 16-20 years of age. Bulbar syndrome leads to dysphagia, nasal tone of voice, choking, and in some cases – to respiratory disorders;
Paroxysmal myoplegia, manifested by partial or generalized attacks of muscle weakness, which can last from half an hour to several days. The development of the disease is provoked by increased consumption of table salt, plentiful food rich in carbohydrates, stress and negative emotions, and increased physical activity. The respiratory muscles are rarely involved;
Myasthenia gravis, the main clinical manifestation of which is excessive muscle fatigue. Its first symptoms are usually dysfunction of the extraocular muscles and muscles of the face and limbs. Often, bulbar syndrome affects the muscles of mastication, as well as the muscles of the larynx, pharynx and tongue;
Polyneuropathy, in which bulbar palsy is observed against the background of tetraplegia or tetraparesis with quite characteristic disorders that make it easier to diagnose and treat bulbar syndrome;
Poliomyelitis, which is recognized by general infectious symptoms, rapid development of paralysis (usually in the first 5 days of illness) with significantly greater damage to the proximal parts than to the distal ones;
Processes occurring in the posterior fossa, brainstem and craniospinal region, including tumors, vascular diseases, granulomatous diseases and bone abnormalities. Typically these processes involve the two halves of the medulla oblongata;
Psychogenic dysphonia and dysphagia, which can accompany both psychotic disorders and conversion disorders.

Pseudobulbar syndrome

Pseudobulbar syndrome usually occurs only against the background of bilateral damage to the corticonuclear pathways that go to the nuclei of the bulbar nerves from the motor cortical centers.

This pathology is manifested by central paralysis of swallowing, disturbances in phonation and speech articulation. Also, against the background of pseudobulbar syndrome, the following may occur:

Dysphagia;
Dysarthria;
Dysphonia.

However, unlike bulbar syndrome, in these cases atrophy of the tongue muscles does not develop. The palatal and pharyngeal reflexes are also usually preserved.

Pseudobulbar syndrome is usually observed against the background of dyscirculatory encephalopathy, acute cerebrovascular accidents in both hemispheres of the brain, and amyotrophic lateral sclerosis. It is considered less dangerous because it does not lead to disruption of vital functions.

Diagnosis and treatment of bulbar syndrome

After consulting a therapist, to diagnose bulbar syndrome it is usually necessary to undergo a series of studies:

General urine analysis;
EMG of the muscles of the tongue, limbs and neck;
General and biochemical blood test;
CT or MRI of the brain;
Esophagoscopy;
Examination by an ophthalmologist;
Clinical and EMG tests for myasthenia gravis with pharmacological load;
Study of cerebrospinal fluid;

Depending on the clinical symptoms and the nature of the pathology, the doctor can predict the outcome and effectiveness of the proposed treatment for bulbar syndrome. As a rule, therapy is aimed at eliminating the causes of the underlying disease. At the same time, measures are taken to support and resuscitate impaired important body functions, namely:

Breathing – artificial ventilation;
Swallowing – drug therapy proserine, vitamins, ATP;
Drooling - prescription of atropine.

It is quite important against the background of pseudobulbar and bulbar syndrome to carry out careful care of the oral cavity, and also, if necessary, monitor patients while eating to prevent aspiration.