How to treat pseudobulbar syndrome (paralysis)? Pseudobulbar syndrome Bulbar pseudobulbar

With the defeat of some parts of the brain, serious pathological processes can appear that reduce the standard of living of a person, and in some cases, threaten with a fatal outcome.

Bulbar and pseudobulbar syndrome are disorders of the central nervous system, the symptoms of which are similar to each other, but their etiology is different.

Bulbar arises as a result of damage to the medulla oblongata - the nuclei of the glossopharyngeal, vagus and hypoglossal nerves that are in it.

Pseudobulbar syndrome (paralysis) is manifested due to impaired conduction of the cortical-nuclear pathways.

Clinical picture of bulbar syndrome

The main diseases during or after which bulbar palsy occurs:

• stroke affecting the medulla oblongata;
• infections (tick-borne borreliosis, acute polyradiculoneuritis);
• trunk glioma;
• botulism;
• displacement of brain structures with damage to the medulla oblongata;
• genetic disorders (porphyrin disease, Kennedy's bulbospinal amyotrophy);
• syringomyelia.

Porphyria is a genetic disorder that often causes bulbar palsy. The informal name - vampire disease - is given because of the person's fear of the sun and exposure to light on the skin, which begins to burst, become covered with ulcers and scars. Due to the involvement of the cartilage in the inflammatory process and the deformity of the nose, ears, as well as the exposure of the teeth, the patient becomes like a vampire. There is no specific treatment for this pathology.

Isolated bulbar palsies are rare due to the involvement of nearby structures of the medulla oblongata during the lesion of the nuclei.

The main symptoms that occur in the patient:

• speech disorders (dysarthria);
• swallowing disorders (dysphagia);
• voice changes (dysphonia).

Patients speak with difficulty, indistinctly, their voice becomes weak, to the point that it becomes impossible to pronounce the sound. The patient begins to make sounds in the nose, his speech is blurred, slowed down. Vowel sounds become indistinguishable from each other. There may be not only paresis of the muscles of the tongue, but their complete paralysis.

Patients choke on food, often unable to swallow it. Liquid food enters the nose, aphagia may occur (complete inability to make swallowing movements).

The neurologist diagnoses the disappearance of the soft palate and pharyngeal reflexes and notes the appearance of twitching of individual muscle fibers, muscle degeneration.

In severe lesions, when the cardiovascular and respiratory centers are involved in the medulla oblongata, disturbances in the rhythm of breathing and heart activity occur, which is fatal.

Manifestations and causes of pseudobulbar syndrome

Diseases, after or during which pseudobulbar palsy develops:

• vascular disorders affecting both hemispheres (vasculitis, atherosclerosis, hypertensive lacunar infarcts of the brain);
• traumatic brain injury;
• brain damage due to severe hypoxia;
• epileptoform syndrome in children (a single episode of paralysis may occur);
• demyelinating disorders;
• Pick's disease;
• bilateral perisylvian syndrome;
• multisystem atrophy;
• intrauterine pathology or birth trauma in newborns;
• genetic disorders (amyotrophic lateral sclerosis, olivopontocerebellar degeneration, Creutzfeldt-Jakob disease, familial spastic paraplegia, etc.);
• Parkinson's disease;
• glioma;
• neurological conditions after inflammation of the brain and its membranes.

Creutzfeldt-Jakob disease, in which not only pseudobulbar syndrome is observed, but also symptoms of rapidly progressing dementia, is a serious disease, the predisposition to which is laid genetically. It develops due to the ingestion of abnormal tertiary proteins, similar in their action to viruses. In most cases, death occurs within a year or two from the onset of the disease. There is no cure to eliminate the cause.

The symptoms that accompany pseudobulbar palsy, like bulbar palsy, are expressed in dysphonia, dysphagia and dysarthria (in a mild version). But these two lesions of the nervous system have differences.

If atrophy and degeneration of muscles occur with bulbar paralysis, then these phenomena are absent with pseudobulbar paralysis. There are also no defibrillary reflexes.

Pseudobulbar syndrome is characterized by uniform paresis of the facial muscles, which are spastic in nature: there are disorders of differentiated and voluntary movements.

Since disturbances in pseudobulbar palsy occur above the medulla oblongata, there is no life threat due to respiratory or cardiovascular system arrest.

The main symptoms that indicate that pseudobulbar palsy has developed, and not bulbar, are expressed in violent crying or laughter, as well as reflexes of oral automatism, which are normally characteristic of children, and in adults indicate the development of pathology. This may be, for example, a proboscis reflex, when the patient stretches his lips with a tube, if you make light taps near the mouth. The same action is performed by the patient if an object is brought to the lips. Contractions of the facial muscles can be induced by tapping the back of the nose or pressing the palm under the thumb.

Pseudobulbar paralysis leads to multiple softened foci of the substance of the brain, so the patient experiences a decrease in motor activity, disorders and weakening of memory and attention, a decrease in intelligence and the development of dementia.

Patients may develop hemiparesis - a condition in which the muscles of one side of the body become paralyzed. Paresis of all limbs may occur.

In severe brain lesions, pseudobulbar palsy may appear together with bulbar palsy.

Therapeutic effects

Since pseudobulbar syndrome and bulbar syndrome are secondary diseases, treatment should be directed to the causes of the underlying disease, if possible. With the weakening of the symptoms of the primary disease, signs of paralysis may be smoothed out.

The main goal pursued by the treatment of severe forms of bulbar palsy is to maintain the vital functions of the body. For this, appoint:

• artificial ventilation of the lungs;
• tube feeding;
• prozerin (with its help, the swallowing reflex is restored);
• atropine with profuse salivation.

After resuscitation, complex treatment should be prescribed, which can affect the primary and secondary diseases. Thanks to this, life is saved and its quality is improved, and the patient's condition is alleviated.

The question of the treatment of bulbar and pseudobulbar syndromes through the introduction of stem cells remains debatable: supporters believe that these cells can produce the effect of physical replacement of myelin and restore neuronal function, opponents point out that the effectiveness of stem cells has not been proven and, on the contrary, increases the risk of developing cancerous tumors.

Restoration of reflexes in a newborn begins to be carried out in the first 2 to 3 weeks of life. In addition to medical treatment, he undergoes massage and physiotherapy, which should have a tonic effect. Doctors give an uncertain prognosis, since complete recovery does not occur even with adequately chosen treatment, and the underlying disease may progress.

Bulbar and pseudobulbar syndrome are severe secondary lesions of the nervous system. Their treatment should be comprehensive and be sure to be directed to the underlying disease. In severe cases of bulbar paralysis, respiratory and cardiac arrest may occur. The prognosis is unclear and depends on the course of the underlying disease.

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, with an untimely reaction, are overcome for a long time.

What is pseudobulbar syndrome

The essence of this disease is reduced to the appearance of multiple large and small foci of hemorrhage, which lead to damage on both hemispheres of the fibers that connect the motor nuclei of the cerebral cortex with the brain stem.

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

With such a problem, as a rule, bulbar functions begin to suffer. These are swallowing, chewing, articulation and phonation. Violation of such functions leads to such pathologies as dysphagia, dysphonia, 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;

Nasolabial reflex of Astvatsaturov;

Distant-arial and other similar reflexes.

Pseudobulbar syndrome - causes

The development of this syndrome is a consequence of atherosclerosis of the cerebral vessels and the resulting softening foci, 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 that affect both hemispheres, can also have a negative impact.

In fact, pseudobulbar syndrome occurs when, against the background of any disease, the central pathways leading 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 is manifested by severe atheromatosis of the arteries of the base of the brain, which affects both hemispheres. In childhood, a bilateral lesion of the corticobulbar conductors is fixed, resulting in cerebral palsy.

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

Symptoms

One of the main symptoms of this disease is a violation of swallowing and chewing. In this state, 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 the meal. Moreover, there are changes in the voice - it acquires a new shade. The sound becomes hoarse, consonants fall 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 manifest attacks of violent convulsive laughter or crying. But these symptoms are not always present.

It is worth mentioning the tendon reflex of the lower jaw, which in the process of developing the syndrome can increase dramatically.

Often pseudobulbar syndrome is fixed in parallel with a disease such as hemiparesis. Possible manifestation of extrapyramidal syndrome, which leads to stiffness, increased muscle tone and slowness of movement. Intellectual impairments are not excluded, which can be explained by the presence of multiple foci of softening in the brain.

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

The syndrome itself can have both a gradual onset and an acute development. But if we consider the most common indicators, then 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 the 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's palsy. Such a disease proceeds 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 should determine the patient's condition.

The development of the syndrome in children

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

When examining a baby with pseudobulbar syndrome, fibrillation and atrophy are not detected, but a reflex of oral automatism is recorded. Also, a similar syndrome can lead to the appearance of 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 system of vertebrobasilar arteries, inoperable malignant tumors of the trunk or demyelinating processes.

Treatment of the syndrome

In order to influence the pseudobulbar syndrome in children, one must initially take into account the stage of its course. 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, agents are usually used that are focused on normalizing lipid metabolism, coagulation processes and lowering cholesterol in the blood. Useful will be drugs that improve microcirculation, bioenergetics of neurons and metabolic processes in the brain.

A similar effect is exerted by drugs such as Encephabol, Aminalon, Cerebrolysin, etc. In some cases, doctors may prescribe drugs that have anticholinesterase effects (Prozerin, Oksazil).

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 it may not be possible to completely neutralize the disease. This means that the child will suffer from swallowing disorders for the rest of his life, and not only.

But if you respond in a timely manner, then 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, restore the functions of cells that have been damaged. Such a restorative effect is able to return the patient to a full life.

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 circular muscle of the mouth, feeding through a probe and electrophoresis with prozerin on the cervical spine.

Speaking about the first signs of recovery, it is worth noting that they include the appearance of newborn reflexes, which were absent before, 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 hypodynamia 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, unless you have to deal with untreated severe lesions, the 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 a recovery period therapy.

At the same time, for children who had to undergo convulsions, drugs are selected more carefully. Cortexin is often used, the course of which is 10 injections. In addition to these measures, Pantogam and Nootropil are orally administered 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 held 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. In this case, this method of treatment is combined with the adoption of "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 that are the result of pseudobulbar syndrome. Its essence is reduced to a violation of the pathways connecting the nuclei of the bulbar group with the cerebral cortex.

This disease can have three degrees:

- Light. Violations are minor and manifest themselves in the fact that children do not pronounce growling and hissing sounds well. While writing the text, the child sometimes confuses the letters.

- Medium. Occurs more often than others. In this case, there is actually a complete absence of mimic movements. Children have difficulty chewing and swallowing food. At the same time, the tongue also moves poorly. In this state, the child cannot speak clearly.

- Severe degree (anarthria). Mimic movements are completely absent, as well as the mobility of the muscles of the speech apparatus. In such children, the lower jaw sags, while the tongue remains motionless.

With this disease, medical treatment methods, massage and reflexology are used.

It is easy to conclude that this syndrome is a fairly serious threat to the health of the child, so the disease requires parents to quickly respond to symptoms and patience in the treatment process.

Bulbar and pseudobulbar palsy

Bulbar and pseudobulbar paralysis - these diseases are most often the result of vascular damage to the brain and with atherosclerosis.

The brain is divided into three large parts: the cerebral hemispheres, the cerebellum and the brainstem (the part of the brain that connects the brain and spinal cord). The brain stem consists of an accumulation of cells (nuclei) through which the large brain controls the muscles of the face and eyes, receives information from receptors and sensory organs (hearing, taste, balance, vision). The part of the trunk in which the nerves that control swallowing, the vocal cords, and the neck muscles that tilt the head are located is called the bulbar.

The control of the nuclei in each half of the brain stem is carried out from two hemispheres simultaneously, therefore, with a stroke in one of them, the patient's swallowing and voice are not upset - the healthy hemisphere successfully replaces the patient's functions. When cells in the brainstem are damaged, functions are disrupted, since there is no one to transmit commands to the cells of the large brain.

Such paralysis is called bulbar. However, sometimes the disease affects both cerebral hemispheres (two strokes, brain atrophy in old age, etc.), and then, with the preservation of motor cells in the trunk, the patient develops disorders similar to bulbar palsy, or pseudobulbar palsy.

Symptoms and course:

Pseudobulbar paralysis proceeds according to the type of central (lack of atrophy, fibrillar twitching and degeneration reaction). Symptoms with it always appear symmetrically, in contrast to bulbar palsy, which can be symmetrical and asymmetrical, depending on the degree of suffering of the parties.

As with bulbar palsy, patients complain of swallowing and articulation disorders. However, the latter are usually less pronounced than with bulbar palsy, and are not accompanied by respiratory and cardiac disorders. Having the character of central paralysis, pseudobulbar paralysis, depending on the level of damage to the supranuclear pathways, is accompanied by a number of pathological reflexes, known as reflexes of oral automatism.

These include: a) sucking reflex (dashed irritation of the lips causes contraction of m. orbicularis oris and sucking movement of the lips), b) proboscis reflex (with a light blow on the lips with a hammer, they are pulled forward). The afferent part of these reflexes is the trigeminal nerve, the efferent part is the facial nerve. The group of reflexes of oral automatism also includes the palmar-chin reflex (Marinesko-Radovichi). With dashed irritation of the skin of the palm, mainly the thenar region, the chin muscle contracts on the side of the same name. The normal mandibular reflex also increases (raised lower jaw when struck with a hammer on the lateral edges of the chin). The afferent and efferent parts of this reflex pass through the trigeminal nerve. With pseudobulbar paralysis, often (with high lesions close to the cortex) involuntary laughter and crying occur, due to the involvement of cortical-subcortical, in particular cortical-thalamic connections in the process. The defeat of these connections causes the disinhibition of the subcortical mechanisms that implement the emotional and expressive movements of laughter and crying.
In general: in patients with bulbar paralysis, swallowing, nasal speech, and hoarseness are upset. Patients with pseudobulbar paralysis have similar complaints, but they are always combined with disorders such as "forced" crying or laughter: a person cries very easily (laughs) and cannot stop in any way. With pseudobulbar paralysis, intellectual disorders are always expressed.

Recognition:

In case of acute swallowing disorders (choking), voice change, nasal voice, it is urgent to call an ambulance. If such disorders occur in older people and develop slowly, then this does not require emergency care, but a brain tumor can also occur under the guise of a disease, so it will not hurt to show a neurologist.

Treatment:

The same as in cerebral atherosclerosis or stroke (see).

When a pseudobulbar syndrome is detected, it is necessary first of all to treat the underlying disease. If it is hypertension, prescribe antihypertensive and vascular therapy. With specific vasculitis (syphilitic, tuberculous), antimicrobial agents and antibiotics must be used. In this case, the treatment can be carried out in conjunction with narrow specialists - a dermatovenereologist or a phthisiatrician.

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

There is no universal remedy for the treatment of pseudobulbar syndrome. The doctor selects a complex therapy scheme, taking into account all existing disorders. At the same time, in addition to taking medications, special exercises for the affected muscles, breathing exercises according to Strelnikova, and physiotherapy can be used. In case of violation of speech in children, classes with a speech therapist are necessarily assigned, this will significantly improve the adaptation of the child in society.

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

The prognosis for pseudobulbar paralysis is serious, with bulbar paralysis it depends on the severity and cause of the development of paralysis.

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

One of the main symptoms of pseudobulbar palsy is a violation of chewing and swallowing. Food gets stuck behind the teeth and on the gums, the patient chokes when eating, liquid food flows out through the nose. The voice acquires a nasal tone, becomes hoarse, loses intonation, difficult consonants fall out completely, some patients cannot even speak in a whisper. Due to bilateral paresis of the facial muscles, the face becomes amimic, mask-like, and often has a weeping expression. Attacks of violent convulsive crying and laughter are characteristic, occurring without corresponding ones. Some patients may not have this symptom. The tendon reflex of the lower jaw increases sharply. Symptoms of the so-called oral automatism appear (see). Often, pseudobulbar syndrome occurs simultaneously with hemiparesis. Patients often have more or less pronounced hemiparesis or paresis of all limbs with pyramidal signs. In other patients, in the absence of paresis, a pronounced extrapyramidal syndrome appears (see) in the form of slowness of movement, stiffness, muscle increase (muscle rigidity). The intellectual impairment observed in pseudobulbar syndrome is 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 entering the respiratory tract, an associated infection, stroke, etc.

Treatment should be directed against the underlying disease. To improve the act of chewing, you need to appoint 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 articulation of speech, as well as amimia.

Pseudobulbar paralysis, in contrast to the bulbar paralysis (see), which depends on the defeat of the motor nuclei of the medulla oblongata, occurs as a result of a break in the paths going from the motor zone of the cerebral cortex to these nuclei. With the defeat of the supranuclear pathways in both hemispheres of the brain, the voluntary innervation of the bulbar nuclei falls out and “false” bulbar palsy occurs, false because the anatomically the medulla oblongata itself does not suffer. The defeat of the supranuclear pathways in one hemisphere of the brain does not give noticeable bulbar disorders, since the nuclei of the glossopharyngeal and vagus nerves (as well as the trigeminal and upper branches of the facial nerve) have bilateral cortical innervation.

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

The clinical course and symptomatology of pseudobulbar palsy are characterized by bilateral central paralysis, or paresis, of the trigeminal, facial, glossopharyngeal, vagus, and hypoglossal cranial nerves in the absence of degenerative atrophy in paralyzed muscles, preservation of reflexes, and disorders of the pyramidal, extrapyramidal, or cerebellar systems. Swallowing disorders in pseudobulbar paralysis 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 may develop. The tongue is immobile or protrudes only to the teeth. Speech is insufficiently articulated, with a nasal tinge; the voice is quiet, the words are difficult to pronounce.

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 voluntarily contract, come into excessive contraction. Patients may begin to cry involuntarily when showing their teeth, stroking a piece of paper on their upper lip. The occurrence of this symptom is explained by a break in the inhibitory pathways leading to the bulbar centers, a violation of the integrity of the subcortical formations (the visual tubercle, the 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 attributed to one of the characteristic signs of pseudobulbar paralysis.

There is also an increase in deep and superficial reflexes in the area of ​​chewing 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); labial reflex (contraction of the circular muscle of the mouth when tapping in the region of this muscle); Bechterew's oral reflex (lip movements when tapping with a hammer in the circumference of the mouth); Toulouse-Wurp buccal phenomenon (movement of the cheeks and lips is caused by percussion along the lateral part 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 childhood forms of pseudobulbar palsy, as well as spastic.

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

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

Mixed form: a combination of the above forms of pseudobulbar paralysis.

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

The children's form of pseudobulbar paralysis is observed with spastic diplegia. At the same time, the newborn sucks poorly, chokes and chokes. In the future, violent crying and laughter appear in the child, and dysarthria is found (see Infantile paralysis).

Weil (A. Weil) describes a family spastic form of pseudobulbar palsy. With it, along with pronounced focal disorders inherent in pseudobulbar palsy, a noticeable intellectual retardation is noted. A similar form was also described by M. Klippel.

Since the symptom complex of pseudobulbar palsy is mostly due to sclerotic lesions of the brain, patients with pseudobulbar palsy often have corresponding mental symptoms: memory loss, difficulty thinking, increased efficiency, etc.

The course of the disease corresponds to the variety of causes that cause pseudobulbar palsy and the prevalence of the pathological process. The development of the disease is most often stroke-like with different periods between strokes. If after a stroke (see) the paretic phenomena in extremities decrease, then the bulbar phenomena remain mostly persistent. More often, the patient's condition worsens due to new strokes, especially with atherosclerosis of the brain. The duration of the disease is varied. Death occurs from pneumonia, uremia, infectious diseases, new hemorrhage, nephritis, heart weakness, etc.

The diagnosis of pseudobulbar palsy is not difficult. It should be differentiated from various forms of bulbar paralysis, neuritis of the bulbar nerves, parkinsonism. Against apoplectic bulbar palsy speaks the absence of atrophy and increased bulbar reflexes. It is more difficult to distinguish between pseudobulbar palsy and Parkinson's 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, patients cannot eat on their own. The diagnosis can present difficulties only in delimitation of an atherosclerosis of a brain from a pseudobulbar component; the latter is characterized by gross focal symptoms, strokes, etc. Pseudobulbar syndrome in these cases may appear as an integral part of the underlying suffering.

The main causes of pseudobulbar palsy:

1. Vascular diseases affecting both hemispheres (lacunar condition in hypertension, atherosclerosis; vasculitis).
2. Perinatal pathology and birth trauma including.
3. Congenital bilateral periaqueductal syndrome.
4. Traumatic brain injury.
5. Episodic pseudobulbar paralysis in children with epileptiform opercular syndrome.
6. Degenerative diseases affecting the pyramidal and extrapyramidal systems: ALS, primary lateral sclerosis, familial spastic paraplegia (rarely), OPCA, Pick's disease, Creutzfeldt-Jakob disease, progressive supranuclear palsy, Parkinson's disease, multiple system atrophy, other extrapyramidal diseases.
7. Demyelinating diseases.
8. Consequences of encephalitis or meningitis.
9. Multiple or diffuse (glioma) neoplasm.
10. Hypoxic (anoxic) encephalopathy (“a disease of the revitalized brain”).
11. Other reasons.

Vascular diseases
Vascular diseases affecting both hemispheres are the most common cause of pseudobulbar palsy. Repeated ischemic disorders of cerebral circulation, usually in people over 50 years of age, with hypertension, atherosclerosis, vasculitis, systemic diseases, heart and blood diseases, multiple lacunar cerebral infarcts, etc., as a rule, lead to a picture of pseudobulbar paralysis. The latter can sometimes develop with a single stroke, apparently due to decompensation of latent cerebrovascular insufficiency in the other hemisphere. In vascular pseudobulbar palsy, the latter may be accompanied by hemiparesis, tetraparesis, or bilateral pyramidal insufficiency without paresis. A vascular disease of the brain is detected, usually confirmed by an MRI picture.

Perinatal pathology and birth trauma
Due to perinatal hypoxia or asphyxia, as well as birth trauma, various forms of cerebral palsy (ICP) can develop with the development of spastic-paretic (diplegic, hemiplegic, tetraplegic), dyskinetic (mainly dystonic), atactic and mixed syndromes, including with pseudobulbar palsy. In addition to periventricular leukomalacia, these children often have unilateral hemorrhagic infarction. More than half of these children show symptoms of mental retardation; about one-third develop epileptic seizures. In the anamnesis, there are usually indications of perinatal pathology, psychomotor retardation, and the neurological status reveals residual symptoms of perinatal encephalopathy.
The differential diagnosis of cerebral palsy includes some degenerative and hereditary metabolic disorders (type I glutaric aciduria; arginase deficiency; dopa-responsive dystonia; hyperekplexia (with rigidity); Lesch-Nyhan's disease), as well as progressive hydrocephalus, subdural hematoma. MRI detects certain disorders in the brain in almost 93% of patients with cerebral palsy.

Congenital bilateral aqueduct syndrome
This defect occurs in pediatric neurological practice. It leads (as well as congenital bilateral sclerosis of the hippocampus) to a pronounced violation of speech development, which sometimes even imitates childhood autism and a picture of pseudobulbar paralysis (mainly with speech disorders and dysphagia). Mental retardation and epileptic seizures are observed in approximately 85% of cases. MRI reveals a malformation of the peri-Sylvian gyri.
Severe traumatic brain injury (TBI)
Severe traumatic brain injury in adults and children often leads to various types of pyramidal syndrome (spastic mono-, hemi-, tri- and tetraparesis or plegia) and pseudobulbar disorders with severe speech and swallowing disorders. The connection with the trauma in the anamnesis leaves no reason for diagnostic doubts.

Epilepsy
Described episodic pseudobulbar palsy with epileptiform opercular syndrome in children (paroxysmal oral apraxia, dysarthria and salivation), observed in the slow phase of nocturnal sleep. The diagnosis is confirmed by epileptic discharges in the EEG during a nocturnal seizure.

Degenerative diseases
Many degenerative diseases involving the pyramidal and extrapyramidal systems may be accompanied by pseudobulbar syndrome. Such diseases include amyotrophic lateral sclerosis, progressive supranuclear palsy (these forms as the cause of pseudobulbar syndrome are more common than others), primary lateral sclerosis, familial spastic paraplegia (rarely leads to severe pseudobulbar syndrome), Pick's disease, Creutzfeldt-Jakob disease, Parkinson's disease, secondary parkinsonism, multiple system atrophy, less often - other extrapyramidal diseases.

Demyelinating diseases
Demyelinating diseases often involve the corticobulbar pathways from both sides, leading to pseudobulbar syndrome (multiple sclerosis, post-infectious and post-vaccination encephalomyelitis, progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis, AIDS-dementia complex, adrenoleukodystrophy).
In the same group ("myelin diseases") can be attributed myelin metabolic diseases(Pelicius-Merzbacher disease, Alexander disease, metachromatic leukodystrophy, globoid leukodystrophy).

Consequences of encephalitis and meningitis
Encephalitis, meningitis and meningoencephalitis, along with other neurological syndromes, may include pseudobulbar syndrome in their manifestations. Symptoms of the main infectious lesion of the brain are always detected.

Multiple or diffuse glioma
Some variants of brainstem glioma manifest variable clinical symptoms depending on its localization within the caudal, middle (pons varolii) or oral parts of the brainstem. Most often, this tumor begins in childhood (in 80% of cases before the age of 21) with symptoms of involvement of one or more cranial nerves (usually VI and VII on the one hand), progressive hemiparesis or paraparesis, ataxia. Sometimes conduction symptoms precede the defeat of the cranial nerves. Headaches, vomiting, edema in the fundus join. Pseudobulbar syndrome develops.
Differential diagnosis with pontine form of multiple sclerosis, vascular malformation (usually cavernous hemangioma) and stem encephalitis. MRI is of great help in the differential diagnosis. It is important to distinguish between focal and diffuse forms of glioma (astrocytoma).

Hypoxic (anoxic) encephalopathy
Hypoxic encephalopathy with serious neurological complications is typical for patients who survived resuscitation after asphyxia, clinical death, prolonged coma, etc. The consequences of severe hypoxia, in addition to prolonged coma in the acute period, include several clinical variants, including dementia with (or without) extrapyramidal syndromes, cerebellar ataxia, myoclonic syndromes, Korsakov's amnestic syndrome. Delayed post-anoxic encephalopathy with poor outcome is considered separately.
Sometimes there are patients with hypoxic encephalopathy, in which persistent residual effects consist in predominant hypokinesia of bulbar functions (hypokinetic dysarthria and dysphagia) against the background of minimally expressed or completely regressing general hypokinesia and hypomia (this variant of pseudobulbar disorders is called "extrapyramidal pseudobulbar syndrome" or "pseudopseudobulbar syndrome "). These patients do not have any disorders in the limbs and trunk, but are disabled due to the above manifestations of a kind of pseudobulbar syndrome.

Other causes of pseudobulbar syndrome
Sometimes pseudobulbar syndrome manifests itself as an integral part of more extensive neurological syndromes. For example, pseudobulbar syndrome in the picture of the central myelinolysis of the bridge (malignant neoplasm, liver failure, sepsis, alcoholism, chronic renal failure, lymphoma, cachexia, severe dehydration and electrolyte disturbances, hemorrhagic pancreatitis, pellagra) and overlapping with it locked-in syndrome(occlusion of the basilar artery, traumatic brain injury, viral encephalitis, post-vaccination encephalitis, tumor, hemorrhage, central pontine myelinolysis).
Central myelinolysis of the pons is a rare and potentially lethal syndrome that presents with rapid onset of tetraplegia (against a background of somatic disease or Wernicke's encephalopathy) and pseudobulbar palsy due to demyelination of the central pons, which is visible on MRI and can in turn lead to "locked-in" syndrome. person." Locked-in syndrome (isolation syndrome, de-efferentation syndrome) is a condition in which selective supranuclear motor de-energization leads to paralysis of all four limbs and caudal parts of the craniocerebral innervation without impaired consciousness. The syndrome is manifested by tetraplegia, mutism (aphonia and anarthria of pseudobulbar origin) and the inability to swallow with intact consciousness; while the possibility of communication is limited only by vertical movements of the eyes and eyelids. CT or MRI shows destruction of the medioventral pons.