Bulbar and pseudobulbar palsy. Bulbar and pseudobulbar palsies Pseudobulbar palsies

Pseudobulbar palsy occurs in vascular diseases of the brain with bilateral supranuclear damage to the motor conductors, i.e. in the presence of multifocal lesions localized in both hemispheres of the brain. Small softenings and cysts are often found. When pseudo bulbar palsy a violation occurs motor functions limbs, tongue, larynx, chewing, pharyngeal and facial as a result of loss of central supranuclear innervation (corticonuclear and corticospinal conductors).

The symptoms of pseudobulbar palsy are varied.

1. Usually there are disorders - impaired articulation (dysarthria, anarthria), phonation (nasal tone of speech, which can be slurred, silent and quiet), sometimes impaired coordination (chanted speech).

2. Swallowing disorders - dysphagia, manifested in choking when particles enter the respiratory tract, leakage of liquid into the nasopharyngeal space, drooling as a result of insufficient swallowing of saliva.

3. Chewing disorder, causing retention in the mouth due to pareticity of the chewing teeth and tongue. Disorder of the function of facial muscles (mask-like appearance due to weakness of the facial muscles); symptoms of oral automatism:

a) proboscis reflex (protrusion of the lips with a “proboscis” when they are percussed);

b) lip reflex (protrusion of the lips forward when tapping on the upper one and bringing the lips closer together when irritating them with strokes);

c) sucking reflex (sucking movements when touching the lips);

d) Astvatsaturov’s nasolabial reflex (proboscis-like protrusion of the lips when tapping on the root of the nose);

e) ankylosing spondylitis (chin contraction when the chin is tapped);

f) palmar-chin reflex Marinesko-Radovici (contraction of the chin upon stroke stimulation of the palm);

g) buccal-labial reflex (raising the mouth or baring the mouth when the cheek is irritated by strokes).

4. Friendly movements on - moving the chin to the side is friendly with turning the eyes, baring the teeth on the side to which the eyeballs are voluntarily retracted; involuntary opening of the mouth when abducted upward; friendly extension of the head when opening, abduction of the protruding tongue to the side is friendly to the rotation of the eyes; friendly turn of the head towards abduction eyeballs.

5. Increased masseter reflex.

6. Changes in gait - gait with small steps, insufficient balancing or lack of friendly balancing of the arms when walking (acheirokinesis), stooping and stiffness.

7. The presence of pyramidal-extrapyramidal tetraparesis (sometimes asymmetrical), more pronounced on one side with increased tone, increased tendon and periosteal reflexes, decreased or absent abdominal reflexes and the presence of pathological reflexes (Babinsky, Rossolimo symptoms, etc.).

8. Sometimes the presence of constant or paroxysmal hyperkinesis in the presence of tetraparesis.

9. Involuntary appearance of emotional-facial discharges, i.e. forced crying, laughter, as a result of disinhibition of thalamostriatal-brain-stem automatisms during a bilateral process in the brain. Violent laughter (also laughter) manifests itself in fits and starts.

Sometimes the patient suddenly begins to cry for no apparent reason, but it occurs when trying to start a conversation or during speech, during various emotional experiences. It often occurs in conjunction with various motor manifestations: when actively opening the eyeballs, when moving the eyeballs to the side, when closing the eyes. Hyperkinetic discharges are observed, occurring paroxysmally during emotional discharges during violent crying. Involuntary movements manifest themselves in different ways: in some cases they consist of intermittent waving of the hand, in others - the raised hand jerkily approaches the head. In rare cases, a hyperkinetic discharge consists of a cycle of movements: for example, extending the arm, swinging the hand, then rhythmically patting the chest, and finally rotating the torso to the side.

Based on his own material (over 100 cases of pseudobulbar palsy, varied in clinical picture, localization of vascular foci of the etiology of the vascular process), N. K. Bogolepov developed symptoms and described new symptoms of pseudobulbar palsy.

Pseudobulbar palsy occurs after repeated strokes. In cases, the first stroke passes unnoticed, leaving no trace, and after a second stroke, bilateral motor disorders develop: on the side opposite to the lesion, phenomena of central paralysis occur, on the side of the same name as the lesion, plastic hypertension and hyperkinesis appear in the hand; At the same time, disturbances in speech, phonation, facial expressions and sometimes swallowing develop.

Clinical and anatomical analysis of such cases reveals the presence of foci in both hemispheres of the brain: an old focus of softening, remaining after the first stroke, manifested by no symptoms until the second stroke, and a fresh focus of softening, which caused the development of not only motor disorders in the limbs opposite to the lesion, which contributed to the appearance of extrapyramidal symptoms on the side of the same name. Apparently, the compensation of motor functions that was present after the first stroke is disrupted with a second stroke, and a picture of pseudobulbar palsy appears.

Anatomical control of cases of pseudobulbar palsy reveals multiple small foci of softening; sometimes large white softening, - red softening combined with small foci; in cases of large softening, it is combined with a cyst after softening in the other hemisphere of the brain. Etiological factor vascular disease brain is arteriosclerosis, less often syphilitic endarteritis. There are cases where pseudobulbar palsy develops as a result of repeated embolisms.

M.I. Astvatsaturov points out that pseudobulbar palsy may be based on small cavities in the area of the subcortical nodes and the internal bursa. Symptomatology in such cases may be due to damage not only to the corticobulbar conductors and the striatum. Between the striatal (akinetic) and corticobulbar (paralytic) varieties of pseudobulbar paralysis, according to M.I. Astvatsaturov, there is a difference in that in the first case there is a lack of motor initiative in the corresponding muscles without the phenomena of real paresis or paralysis, the automatic ease of performing swallowing is lost and phonation movements. In the corticobulbar form of pseudobulbar palsy, on the contrary, there is central paralysis caused by damage to the corticospinal tracts, and elementary motor functions are lost. L. M. Shenderovich, in a work devoted to pseudobulbar palsy, identified four forms:

paralysis, depending on bilateral damage to the pathways from the pons to the nuclei and medulla oblongata(cortical origin);
paralysis caused by symmetrical damage to the striatal bodies (striatal origin);
paralysis that occurs when a combination of cortical lesions (including the corticobulbar tract) of one hemisphere and the striatal system of the other hemisphere;
special children's uniform.

The first group of pseudobulbar palsies can include cases when multiple foci of softening are localized in both hemispheres of the brain - the cortical form of pseudobulbar palsy. As one example of the form of pseudobulbar palsy, we can point to the case described by V. M. Bekhterev in the city. At the autopsy, atrophy of the brain convolutions was found in the right hemisphere in the region of the middle and upper part of the frontal and upper part central gyri, mainly in the upper part of the sulci praecentralis (the first and third frontal gyri, respectively) and in the left hemisphere of the brain - in the upper part of the first frontal gyrus, respectively, the upper part of the sulcus Rolandi and in the posterior segment of the third frontal gyrus. Along with brain atrophy, there was an accumulation serous fluid in the subarachnoid space. Along with the anomaly of the cerebral vessels was discovered: the left posterior communicating artery was absent, the left posterior cerebral artery departed from the main artery, and the right one from the communicating artery, the left anterior cerebral artery was much thinner than the right one.

This bilateral brain damage caused bilateral motor impairment, speech disorder, seizures with the head abducted and in the direction opposite to the lesion.

In the cortical form of pseudobulbar palsy, the psyche is most clearly disturbed, there are pronounced speech disorders, epileptic seizures, violent crying, dysfunction of the pelvic organs.

The second group of pseudobulbar palsies is the extrapyramidal form of pseudobulbar palsy. Symptoms of the pseudo form bulbar disorders It can be varied depending on whether pallidal, striatal or thalamic formations are affected in bilateral localization of foci. Motor disturbances are represented by paresis, which is sometimes deep and more pronounced in the lower extremities. Motor disturbances are of an extrapyramidal nature: the torso and head are bent, half-bent, amicable; there is inactivity, stiffness, akinesis, freezing of the limbs in their assigned position, plastic hypertension, increased postural reflexes, slow gait with small steps. Speech, phonation, swallowing and chewing are impaired primarily due to the inability of the boulevard muscles to perform movements quickly and clearly, and therefore dysarthria, aphonia, and dysphagia develop. Damage to the striatum affects the functions of speech, swallowing and chewing, since in the subcortical nodes and in the visual thalamus there is a somatotopic distribution according to functional sign(has to do with swallowing functions anterior section striatal system).

Among pseudobulbar palsies caused by focal lesions in the subcortical nodes, several options can be distinguished: a) Pseudobulbar parkinsonism - a syndrome of pseudobulbar palsy with a predominance of akinetic-rigid disorders, expressed in four limbs, caused by small foci (lacunae or small cysts) localized in pallidal system. The course of pseudobulbar parkinsonism is progressive: akinesis and rigidity gradually lead to the forced position of the patient in bed, to the development of flexion contracture of both. Along with extrapyramidal disorders, pseudobulbar reflexes are expressed, possibly depending on the presence of other small focal lesions in the brain, causing disinhibition of oral automatisms, b) Pseudobulbar striatal syndrome - a syndrome of pseudobulbar palsy with motor extrapyramidal-pyramidal paresis of four limbs (unevenly expressed on both sides), with impaired articulation, phonation and swallowing and with the presence of various hyperkinesis. c) Pseudobulbar thalamo-striatal syndrome - pseudobulbar paralysis as a result of multiple foci involving the area of the thalamus and striatum, accompanied by paroxysmal seizures and hyperkinetic discharges associated with emotional arousals. The importance of the visual thalamus in pseudobulbar palsies was pointed out by V. M. Bekhterev. The occurrence of crying and laughter, which are an expression of emotional experience, must be associated with the function of the visual thalamus, which plays in the implementation of emotional arousals. The appearance of violent crying or laughter in pseudobulbar palsy indicates disinhibition of thalamo-striatal automatisms and occurs with bilateral brain damage. Damage to the thalamus opticum in pseudobulbar palsy can sometimes be indirect (for example, disinhibition of the thalamus opticum due to damage to the frontothalamic connections); in other cases, direct damage to the thalamus occurs by one of the many foci that causes the development of pseudobulbar palsy.

The third group of pseudobulbar palsies consists of the most common cases when there is combined lesion one hemisphere and the subcortical nodes of the other hemisphere. Symptoms vary significantly depending on the size and location of the lesion in the brain, the extent of the lesion in the subcortical white matter and the region of the subcortical nodes. What matters is the nature of the vascular process: with the syphilitic process, phenomena of brain irritation often occur along with symptoms of prolapse, and in such cases, paralysis of the limbs is combined with epileptic seizures. A peculiar variant of pseudobulbar paralysis of cortical-subcortical localization is represented by cases when not epileptic seizures are observed, as indicated above, but subcortical seizures that occur during emotional discharges.

The fourth group consists of cases of pseudobulbar palsy that occur when vascular lesions are localized in the pons. This form of pseudobulbar palsy was first described by I. N. Filimonov in the city. Based on a clinical and anatomical study, I. N. Filimonov concluded that with bilateral localization of the lesion at the base of the middle third of the pons, paralysis of four limbs and the trunk occurs (with preservation of tendon reflexes and the appearance of pathological reflexes) and paralysis of the trigeminal, facial, vagus and hypoglossal nerves characteristic of supranuclear lesions develops (with preservation of automatic and reflex functions), pronounced bulbar reflexes and violent crying appear. In the case of S. N. Davidenkov, pseudobulbar palsy developed in connection with syphilitic endarteritis of the paramedian arteries emerging from the trunk of the basilar artery and supplying the ventromedial section of the pons. S. N. Davidenkov established a number of patterns characteristic of the pontine form of pseudobulbar palsy, and emphasized the signs that make it possible to distinguish pseudobulbar palsy when the process is localized in the pons from pseudobulbar palsy of the cortical-subcortical

of different origin. He proposed to call the described pseudobulbar palsy of pontine localization Filimonov's syndrome.

Pseudobulbar palsy of pontine localization, described by I. N. Filimonov N. Davidenkov, is characterized by the following signs: 1. With the development of pseudobulbar palsy of pontine localization, absolute immobility of the patient occurs, caused by deep paralysis of the limbs; consciousness remains intact. The picture of pseudobulbar paralysis reveals (in the case of I.N. Filimonov) a peculiar dissociation of motor disorders. Paralysis of four limbs with anarthria, dysphagia, paralysis of the tongue, lips and lower jaw combined with the preservation of the oculomotor system and partial preservation of the function of the muscles that rotate the head and the muscles innervated by the superior branch of the facial nerve; severe impairment of the motor functions of the lips and tongue). 3. Cervical tonic reflexes (in the case of S.N. Davidenkov) can be expressed in the first days of the development of pseudobulbar palsy and, with passive rotation of the head, manifest themselves in automatic extension and after a few seconds in the protective flexion reflex of the same name (without the participation of the opposite limbs). 4. Pseudobulbar palsy in the case of I. N. Filimonov was flaccid; in the case of S.N. Davidenkov, the phenomena of early contracture with tonic tensions were noted, leading to tonic changes in the position of the limbs in spontaneous movements with protective reflexes in both arms and legs (clearly expressed in the first time of development of pseudobulbar paralysis). the period of recovery of motor functions in pseudobulbar palsy of pontine localization revealed a significant similarity in the form of active movements with involuntary reflex movements, the absence of friendly global movements characteristic of capsular hemiplegia and the appearance of imitative kinesia, i.e. symmetrical flexion or extension synergies of the forearm with active movements of the opposite ( active movements lower limbs were not accompanied by friendly movements).

Cerebellar abnormalities were noted in cases of pseudobulb palsy of pontine origin. With pronounced pseudobulbar palsy, a combination of various pseudobulbar symptoms is observed.

Based on a clinical and anatomical study of a case of pseudobulbar palsy, I. N. Filimonov proved the separate course of the conduction pathways for the extremities (at the base of the pons) and for the cervical and ocular (in the tegmentum of the pons), causing the dissociation of paralysis during obliteration of the paramedian arteries.

Pseudobulbar pontine syndrome should always be differentiated from apoplectic bulbar palsy, when there is paralysis of the limbs with partial damage to the cranial nerves.

The presence of amyotrophies in pseudobulbar palsies is not a reason to distinguish special group. We have repeatedly observed trophic disorders in pseudobulbar paralysis, which in some cases manifested themselves in excessively pronounced progressive general emaciation (lack of subcutaneous fat, diffuse muscle atrophy, thinning and atrophy of the skin) or partial atrophy, developing in paralyzed limbs, in the proximal part. In the origin of general exhaustion, damage to the subcortical formations (especially the putamen) and the hypothalamic region undoubtedly plays a role; development

partial atrophy of paralyzed limbs in pseudobulbar palsies may be associated with cortical damage.

Sometimes pseudobulbar syndrome due to arteriosclerosis manifests itself as short-term crises and micro-strokes. Patients experience memory loss, choking on food, difficulty writing, speaking, insomnia, decreased intelligence, mental disorders and mild pseudobulbar symptoms.

The clinical manifestations of Pseudobulbar palsy were first described by Magnus in 1837, and in 1877 R. Lepine gave the name to this syndrome. In 1886, G. Oppenheim and E. Siemerling showed that Pseudobulbar palsy is observed with severe atherosclerosis of the cerebral vessels with the formation of multiple cysts in both hemispheres of the brain. In this case, the cortical-nuclear pathways (see the full body of knowledge: Pyramidal system) are affected on both sides at different levels, most often in the internal capsule, pons, and also in the cerebral cortex.

Pseudobulbar palsy is often observed with repeated ischemic disorders of cerebral circulation in both hemispheres. But it is also possible to develop the so-called single-stroke Pseudobulbar palsy, in which cerebral blood flow apparently decreases or a hidden regional deficiency in the other hemisphere of the brain is decompensated (see full body of knowledge: Stroke).

Pseudobulbar palsy is observed with diffuse vascular processes in the brain (for example, syphilitic endarteritis, rheumatic vasculitis, systemic lupus erythematosus), as well as with perinatal brain damage, hereditary changes in the corticonuclear tracts, Pick's disease (see full body of knowledge: Pick's disease) , Creutzfeldt-Jakob disease (see full body of knowledge: Creutzfeldt-Jakob disease), post-resuscitation complications (see full body of knowledge: Resuscitation) in persons who have suffered cerebral hypoxia (see full body of knowledge: Hypoxia). IN acute period cerebral hypoxia Pseudobulbar palsy can be observed as a consequence of diffuse damage to the cerebral cortex.

It is more often observed in people over 50 years of age suffering from cerebrovascular pathology.

Clinical, picture Pseudobulbar paralysis is characterized by a swallowing disorder - dysphagia (see full body of knowledge), a disorder of chewing, articulation - dysarthria or anarthria (see full body of knowledge: Dysarthria.). Paralysis of the muscles of the lips, tongue, soft palate, muscles involved in the act of swallowing, chewing, phonation, are not atrophic in nature and are much less pronounced than with bulbar palsy (see full body of knowledge). Reflexes of oral automatism are evoked (see full body of knowledge: Pathological reflexes). Patients are forced to eat extremely slowly due to weakness of the masticatory muscles, choking when swallowing; liquid food pours out through the nose while eating; salivation is observed. The reflex from the soft palate is usually increased, in some cases it is not evoked or is sharply reduced even with preserved motor function of the palatine muscles; the mandibular reflex is increased; Paresis of the tongue muscles is often observed, and patients cannot hold their tongue out of their mouth for a long time.

Articulatory disorders in pseudobulbar palsy manifest themselves differently depending on the damage to individual or all muscle groups of the larynx, vocal cords, pharynx, and respiratory muscles.

Due to bilateral paresis of the facial muscles, hypomimia is observed with limited voluntary wrinkling of the forehead, squinting of the eyes, and baring of the teeth. Often Pseudobulbar palsy is accompanied by attacks of violent crying (less often laughter) due to spastic contraction of the facial muscles in a grimace of suffering without tearing and adequate emotions.

Sometimes disturbances in the voluntary movements of the eyeballs are detected while their reflex movements are preserved, and an increase in deep reflexes from the masticatory muscles that are in a spastic state. Pseudobulbar paralysis can be combined with hemiparesis or tetraparesis (see full body of knowledge: Paralysis, paresis) of varying severity, urinary disorders in the form of urgency or urinary incontinence.

When some areas of the brain are damaged, serious pathological processes can appear that reduce a person’s standard of living, and in some cases, threaten death.

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 occurs due to damage to the medulla oblongata - the nuclei of the glossopharyngeal, vagus and hypoglossal nerves that are located in it.

Pseudobulbar syndrome (paralysis) manifests itself due to disruption of the conductivity of the corticonuclear 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, bulbospinal amyotrophy Kennedy);
Syringomyelia.

Porphyria – genetic disorder, in which bulbar palsy often appears. The unofficial name - vampire disease - is given because of a person’s fear of the sun and the effect of light on the skin, which begins to burst, become covered with ulcers and scars. Due to involvement in inflammatory process cartilage and deformation of the nose, ears, as well as exposure of teeth, the patient becomes like a vampire. Specific treatment this pathology is absent.

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

The main symptoms that occur in the patient:

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

Patients have difficulty speaking indistinctly, their voice becomes weak, to the point that it becomes impossible to make a sound. The patient begins to pronounce sounds through the nose, his speech is blurred and slow. Vowel sounds become indistinguishable from each other. Not only paresis of the tongue muscles, but their complete paralysis may occur.

Patients choke on food and often cannot swallow it. Liquid food gets into the nose, aphagia may occur ( complete absence opportunities to do 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 case of severe damage, when the cardiovascular and respiratory centers are involved in the medulla oblongata, disturbances in the rhythm of breathing and heart activity occur, which can be fatal.

Manifestations and causes of pseudobulbar syndrome

Diseases after or during which pseudobulbar palsy develops:

vascular disorders affecting both hemispheres (vasculitis, atherosclerosis, hypertensive lacunar cerebral infarctions);
traumatic brain injuries;
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;
multiple system atrophy;
intrauterine pathology or birth trauma in newborns;
genetic disorders (amyotrophic lateral sclerosis, olivopontocerebellar degenerations, 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 genetically determined. It develops due to the entry into the body 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 treatment to eliminate the cause.

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

If with bulbar palsy atrophy and degeneration of muscles occurs, then these phenomena are absent with pseudobulbar palsy. Defibrillar reflexes also do not occur.

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

Since disturbances in pseudobulbar palsy occur above the medulla oblongata, life is threatened due to respiratory or cardiovascular systems does not arise .

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 could be, for example, a proboscis reflex, when the patient stretches out his lips with a tube if light tapping is done near the mouth. The same action is performed by the patient if he brings any object to his lips. Contractions of the facial muscles can be caused by tapping the bridge of the nose or pressing the palm under thumb hands.

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

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

With severe brain damage, pseudobulbar palsy can appear together with bulbar palsy.

Therapeutic effects

Since pseudobulbar syndrome and bulbar syndrome are secondary diseases, treatment should be aimed at the causes of the underlying disease, if possible. When symptoms subside primary disease, signs of paralysis may be smoothed out.

The main goal of treatment is severe forms Bulbar palsy is to maintain vital body functions. For this purpose they prescribe:

artificial ventilation;
tube feeding;
prozerin (it is used to restore the swallowing reflex);
atropine for excessive salivation.

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

The issue of treating bulbar and pseudobulbar syndromes through the introduction of stem cells remains controversial: supporters believe that these cells can produce the effect of physical replacement of myelin and restore the functions of neurons, opponents point out that the effectiveness of using stem cells has not been proven and, on the contrary, increases the risk of developing cancerous tumors.

Restoration of reflexes in a newborn begins in the first 2 to 3 weeks of life. Besides drug 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 must be comprehensive and must be aimed at the underlying disease. In severe cases of bulbar palsy, respiratory and cardiac arrest may occur. The prognosis is unclear and depends on the course of the underlying disease.

Bulbar syndrome(paralysis) occurs with peripheral paralysis of muscles innervated by pairs IX, X and XII cranial nerves in case of their combined defeat. 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 are interrupted, going from the motor centers of the cerebral cortex to the motor nuclei of the cranial nerves of the 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. The cause of pseudobulbar palsy is usually atherosclerosis of cerebral vessels with areas 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, liquid food flows out through the nose. The voice takes on a nasal tint, 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 entering the respiratory tract, 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 nerve(like the trigeminal and superior branches of the 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 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; facial muscles, which in such patients cannot contract voluntarily, become excessively contracted. Patients may begin to cry involuntarily when showing their teeth, stroking a piece of paper upper lip. 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); labial reflex (contraction of the orbicularis oris muscle 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-shaped 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 extrapyramidal type with a characteristic gait (small steps).

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

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

The childhood form of P. p. is observed with 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. It should be differentiated from various forms of bulbar 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 flow, late 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 component basic 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 possible autonomic disorders(breathing disorders, cardiac activity), causing in some cases poor prognosis. Bulbar syndrome is observed in 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 with acute disorders cerebral circulation in both hemispheres of the brain, dyscirculatory encephalopathy, 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 impairment of vital 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.

PSEUDOBULBAR PARALYSIS(Greek pseudes false + lat. bulbus bulb; Greek, paralysis relaxation; syn.: false bulbar palsy, supranuclear bulbar palsy) - a syndrome characterized by paralysis of muscles innervated by the V, VII, IX, X, XII cranial nerves, as a result of bilateral damage to the corticonuclear pathways to the nuclei of these nerves.

The wedge and manifestations of P. p. were first described by Magnus in 1837, and in 1877 by R. Lepine who gave the name to this syndrome. In 1886, G. Oppenheim and E. Siemerling showed that P. p. is observed with severe atherosclerosis of cerebral vessels with the formation of multiple cysts in both hemispheres of the brain. In this case, the cortical-nuclear tracts (see Pyramidal system) are affected on both sides at different levels, most often in the internal capsule, pons, and also in the cerebral cortex.

P. p. is often observed with repeated ischemic disorders of cerebral circulation in both hemispheres. But it is possible to develop the so-called single-stroke P. p., with which, apparently, cerebral blood flow decreases or hidden regional failure in the other hemisphere of the brain is decompensated (see Stroke).

P. p. is observed in diffuse vascular processes in the brain (for example, syphilitic endarteritis, rheumatic vasculitis, systemic lupus erythematosus), as well as in perinatal brain damage, hereditary changes in the corticonuclear tracts, Pick's disease (see Pick's disease) , Creutzfeldt-Jakob disease (see Creutzfeldt-Jakob disease), post-resuscitation complications (see Resuscitation) in persons who have suffered cerebral hypoxia (see Hypoxia). In the acute period of cerebral hypoxia, P. p. can be observed as a consequence of diffuse damage to the cerebral cortex.

It is more often observed in people over 50 years of age suffering from cerebrovascular pathology.

Clinical picture P. p. is characterized by a swallowing disorder - dysphagia (see), a disorder of chewing, articulation - dysarthria or anarthria (see Dysarthria). Paralysis of the muscles of the lips, tongue, soft palate, muscles involved in the act of swallowing, chewing, phonation, are not atrophic in nature and are much less pronounced than with bulbar palsy (see). Reflexes of oral automatism are evoked (see Pathological reflexes). Patients are forced to eat extremely slowly due to weakness of the masticatory muscles, choking when swallowing; liquid food pours out through the nose while eating; salivation is observed. The reflex from the soft palate is usually increased, in some cases it is not evoked or is sharply reduced even with preserved motor function of the palatine muscles; the mandibular reflex is increased; Paresis of the tongue muscles is often observed, and patients cannot hold their tongue out of their mouth for a long time.

Articulatory disorders with P. p. manifest themselves differently depending on the damage to individual or all muscle groups of the larynx, vocal cords, pharynx, and respiratory muscles.

Due to bilateral paresis of the facial muscles, hypomimia is observed with limited voluntary wrinkling of the forehead. closing eyes, baring teeth. Often II. etc. is accompanied by bouts of forced crying (less often laughter) due to spastic contraction of the facial muscles in a grimace of suffering without tearing and adequate emotions.

Sometimes disturbances in the voluntary movements of the eyeballs are detected while their reflex movements are preserved, and an increase in deep reflexes from the masticatory muscles that are in a spastic state. P. p. can be combined with hemiparesis or tetraparesis (see Paralysis, paresis) of varying severity, urinary disorders in the form of urgency or urinary incontinence.

Motor disturbances characteristic of P. p. may be accompanied by weakness, impaired attention with a subsequent decrease in intelligence, depending on the severity of the cerebral pathol, the process.

By associated symptoms, often accompanying P. p., there are pyramidal, extrapyramidal, pontine and mixed forms, as well as hereditary and childhood forms.

With a pyramidal shape Hemi- and tetraparalysis or paresis are observed. In the absence of noticeable paresis of the limbs, there is often an increase in tendon reflexes in combination with pyramidal patol, carpal and foot reflexes (see Pathological reflexes).

With extrapyramidal form signs of P. p. are combined with akinetic-rigid syndrome (see Amyostatic symptom complex), when mixed - with symptoms of pyramidal and extrapyramidal insufficiency (see Pyramidal system, Extrapyramidal system).

The pontine form of P. p., first described by I. N. Filimonov in 1923, is caused by an isolated lesion of the corticonuclear fibers at the level of the pons. It is characterized by flaccid tetraplegia or tetraparesis with preservation of deep reflexes, central paralysis of the muscles innervated by the V, VII, X, XII pairs of cranial nerves, while maintaining the functions of the oculomotor muscles and muscles that provide head movements (Filimonov syndrome).

The hereditary form of P. p. is based on hereditarily determined insufficiency of the cortical-nuclear pathways with their subsequent degeneration and sclerosis.

Children's uniform P. p. develops as a result of birth trauma or intrauterine encephalitis and is characterized by more diffuse damage to the motor sphere than in adults - spastic paresis, choreotic, athetoid, torsion hyperkinesis (see Infantile paralysis, Hyperkinesis).

Recognition of P. p. in typical cases does not present any difficulties.

P. p. follows differentiate with bulbar palsy (see). Unlike P. p., with bulbar paralysis there are no reflexes of oral automatism, a pharyngeal reflex, a reflex from the soft palate, atrophies, fibrillary, fascicular twitching in the muscles of the tongue are observed. There is a certain difficulty differential diagnosis with atherosclerotic parkinsonism (see), in which P. p. joins the pronounced akinetic-rigid syndrome in the progressive stage.

During treatment, the use of medicines, normalizing lipid metabolism, cholesterol levels in the blood, blood clotting processes, rheological properties blood; drugs that normalize microcirculation in the cerebral vessels, metabolic processes and bioenergetics in the brain (Cerebrolysin, nootropil, encephabol, etc.), as well as drugs with anticholinesterase action (proserin, oxazil, etc.).

Forecast P. item depends on the nature of the underlying disease and the prevalence of patol, the process. P. p. does not undergo noticeable reverse development even with regression of paresis of the limbs. In severe swallowing disorders, obstruction is possible respiratory tract food, requiring emergency resuscitation measures (see Asphyxia).

Prevention P. of vascular origin includes the identification and treatment of atherosclerosis of cerebral vessels (see Atherosclerosis), the prevention of cerebral strokes (see). It is necessary to observe a work and rest regime, eat with a limited total calorie content, reduce animal proteins and foods containing cholesterol.

Bibliography: Krol M. B. and Fedorova E. A. Basic neuropathological syndromes, M., 1966; Lugovsky B.K. and Kuznetsov M.T. Pseudobulbar palsy in Pick's disease, Zdravookhr. Belarus, No. 8, p. 84, 1968; S e r e b r o v A. M. To assess the reflex from the nasal mucosa in patients with pseudobulbar syndrome of vascular origin, Zhurn. neuropath, and psychiat., t. 71, no. 1, p. 55, 1971, bibliogr.; Triumphov A.V. Topical diagnosis of diseases of the nervous system, JI., 1974; F i l i m o n o v I. N. On the characteristics of pseudobulbar palsies of pontine localization in connection with the question of the course of supranuclear conductors of cranial motor nerves, Nevrol, notes, vol. 2, p. 16, 1923; Ch u-gunov S.A. Hereditary form of pseudobulbar palsy, Med. zhurn., No. 4, p. 44, 1922; Shenderovich JI. M. Forms of pseudobulbar palsy, Sovrem. psychoneurol., vol. 5, no. 12, p. 469, 1927, bibliogr.; Buge A.e.a. Syndrome pseudobulbaire aigu par infarctus bilatéral limité du territoire des artères chorioidiennes antérieures, Rev. neurol., t. 135, p. 313.1979; Lepine R. Note sur la paralysie glosso-labiée cérébrale à forme pseudo-bulbaire, Rev. Med. Chir., p. 909, 1877; Russell R. W. Supranuclear palsy of eyelid closure, Brain, v. 103, p. 71, 1980.

L. G. Erokhina, N. N. Leskova.