Pseudobulbar syndrome in children and adults. Causes of the syndrome

Such a problem as the development of pseudo bulbar syndrome a child is a real test for parents. The point is that the symptoms 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 hemorrhages that lead to damage on both hemispheres of the fibers that connect the motor nuclei 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. It's about about 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. Own Negative influence capable of providing vascular form syphilis of the brain, as well as neuroinfections, degenerative processes, infections and tumors affecting both hemispheres.

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

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. 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, in contrast to the bulbar form, this syndrome excludes the occurrence of cardiovascular disorders 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 cervical region 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 at successful treatment there should be a raise 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. Wherein this way 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 complete absence 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 muscle mobility 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 not difficult to conclude that this syndrome is enough serious threat the health of the child, so the disease requires parents to quickly respond to symptoms and patience in the treatment process.

Pseudobulbar syndrome or pseudobulbar palsy is pathological condition, in which damage to the cranial nerves develops, which leads to paralysis of the facial muscles, the muscles involved in speaking, chewing and swallowing. The disease is similar in symptoms to bulbar paralysis, however, proceeds more easily. leads to atrophy of muscle fibers, and this is not observed with pseudobulbar syndrome.

The development of the syndrome is associated with brain damage (in particular, its frontal lobes) with vascular disorders or as a result of trauma, inflammatory or degenerative process. Characteristic signs of pathology: violations of the processes of swallowing, changes in voice and articulation, spontaneous crying and laughter, disruption of the facial muscles. Most often, this syndrome develops in combination with other neurological disorders.

Since the cause of the disease is brain damage and vascular disorders, it is recommended to use drugs for treatment that improve cerebral circulation and metabolic processes in nervous tissue. Effectively apply folk remedies nootropic action based on medicinal plants.

How does the disease develop?

In the brain, the cortex and subcortical structures are distinguished. The cortex appeared evolutionarily at a later stage, and it is responsible for higher nervous activity. Subcortical structures, in particular, the medulla oblongata, exist for a longer time. They can work autonomously, without the participation of the cerebral cortex. This structure provides the basic processes of life: breathing, heartbeat, the centers of which are located in the medulla oblongata. Normally, all parts of the brain are interconnected, and there is a clear regulation of human life. However, if these connections are broken, the subcortical structures continue to function autonomously.

The development of pseudobulbar syndrome is just the same caused by a violation of the connection between the cortex and the nuclei motor neurons pyramidal centers of the medulla oblongata, from which the cranial nerves depart. Violation of this connection is not dangerous to human life, since the medulla oblongata itself was not affected in this case, but causes symptoms associated with a violation of the normal functioning of the cranial nerves: facial paralysis, speech disorder and others.

Pathology develops with damage to the frontal lobes. In order for a pseudobulbar syndrome to arise, a bilateral lesion of the frontal lobes is necessary, since bilateral connections are formed in the brain: between the nuclei of motor neurons and the right and left hemispheres of the brain.

Causes of paralysis

Bulbar and pseudobulbar paralysis have similar manifestations: in both cases, there is a violation of the innervation of the facial, chewing, swallowing muscles, structures responsible for speech and breathing. With bulbar palsy, the cranial nerves themselves or the structures of the medulla oblongata are damaged, and such damage leads to muscle atrophy and can be life-threatening for the patient. With pseudobulbar paralysis, there is a violation of intracerebral regulation. In this case, the nuclei of the medulla oblongata do not receive signals from other parts of the brain. In this case, however, there is no damage to the nervous tissue itself and there is no danger to human life.

The development of pseudobulbar palsy can lead to various reasons:

  1. Pathology of cerebral vessels. This reason is the most common. Ischemic or hemorrhagic stroke, vasculitis, atherosclerosis and other vascular pathologies lead to pseudobulbar paralysis. This disorder is more common in the elderly.
  2. Embryonic development disorders and congenital brain injuries. Hypoxia or birth trauma can lead to the development of cerebral palsy in an infant, one of the manifestations of which may be pseudobulbar syndrome. Also, such paralysis can develop with congenital plumbing syndrome. Manifestations of pseudobulbar syndrome in this case are observed already in childhood. The child suffers not only from bulbar disorders, but also from a number of other neurological pathologies.
  3. Traumatic brain injury.
  4. Epilepsy with damage to the corresponding structures.
  5. Degenerative and demyelinating processes in the nervous tissue.
  6. brain inflammation or meninges.
  7. benign or malignant tumor in particular glioma. The manifestations of the disorder depend on the location of the tumor. If the growth of the neoplasm affects the regulation of the pyramidal structures of the medulla oblongata, the patient will develop a pseudobulbar syndrome.
  8. Brain damage due to hypoxia. Lack of oxygen has a complex negative effect. Brain tissues are extremely sensitive to oxygen starvation and are the first to suffer from hypoxia. Damage in this case is often complex and includes, among other things, pseudobulbar syndrome.

Symptoms of pathology

Manifestations of pseudobulbar syndrome are complex. The patient has violations of the processes of chewing, swallowing, speech. Also, the patient may experience spontaneous laughter or crying. Violations are less pronounced than with bulbar paralysis. Also in this case there is no muscular atrophy.

Pseudobulbar paralysis leads to impaired speech. It becomes fuzzy, articulation is disturbed. Also, the patient's voice becomes more deaf. These symptoms are associated with paralysis or, on the contrary, spasm of the muscles responsible for articulation.

One of the most key symptoms pseudobulbar syndrome is oral automatism. These are reflexes that are characteristic only of infants, but never occur in healthy adults.

A common symptom of this disease is spontaneous laughter or crying. This condition occurs due to uncontrolled contraction of facial muscles. A person is not able to control these reactions. You also need to understand that they cannot be provoked by anything. In addition to the emergence involuntary movements for such people, violations of the voluntary regulation of the facial muscles are characteristic. For example, when intending to close their eyes, a person may open their mouth instead.

The development of pseudobulbar palsy is associated with damage to the tissue of the cerebral cortex. In most cases, such damage is complex and is manifested not only by dysregulation of the nuclei of motor neurons in the medulla oblongata, but also by other neurological disorders.

Treatment of the disease

Treatment of the disease should be primarily aimed at eliminating the cause of the pathology. The most common cause of paralysis is vascular disease, so therapy is aimed at improving cerebral circulation. In therapy, nootropics are also used that improve metabolic processes in the brain.

It is also useful to do physical therapy and fulfill breathing exercises. It is important to knead the muscles of the neck 2-3 times a day: tilt the head forward - back and to the sides, in circular motions. After warming up with your hands, you need to rub the muscles of the neck and massage the scalp with your fingertips. This will help relieve the symptom. oxygen starvation and improve brain nutrition. If speech is impaired, articulatory gymnastics should be performed. If the symptoms of pseudobulbar palsy appear in childhood, it is necessary to conduct classes with a speech therapist, as well as independently develop speech in the child.

Help in the treatment and folk remedies that have a nootropic effect. Many commercial nootropics are based on herbal ingredients. folk remedies have a similar but milder effect and do not cause negative side effects. Medicinal drugs should be taken in courses. The duration of the course is 2-4 weeks, after which you need to take a break. It is also recommended to alternate medicines so that there is no addiction and the healing effect is not lost.

Paralysis)

dysfunction of the muscles (paralysis) innervated by the IX, X and XII pairs of cranial nerves as a result of bilateral damage to the central motor neurons and cortical nuclear pathways going to the nuclei of these nerves.

Pseudobulbar paralysis often develops with severe atherosclerosis of cerebral vessels. Bilateral neurological disorders occur after repeated ischemic or hemorrhagic strokes, which result in the formation of multiple small lesions in the cerebral hemispheres. Cortical-nuclear conductors (see. Pyramidal system) at this may damage various levels, more often in the internal capsule, the bridge of the brain. It is possible to develop P. p. and with a unilateral shutdown of blood flow in a large cerebral artery, due to which the opposite hemisphere also decreases (the so-called stealing), and develops a chronic brain. The cause of P. p. can also be diffuse cerebral vessels in vasculitis, for example, syphilitic, tuberculous, rheumatic, periarteritis nodosa, systemic lupus erythematosus, Degos disease, etc. In addition, P. p. of nuclear tracts in hereditary degenerative diseases, Pick's disease, Creutzfeldt-Jakob disease, post-resuscitation complications in persons who have undergone cerebral hypoxia. IN acute period cerebral hypoxia P. p. may develop due to diffuse lesions cerebral cortex.

It is clinically characterized by a swallowing disorder - dysphagia (dysphagia), articulation disorders - dysarthria (dysarthria) or anarthria, a change in phonation - dysphonia (hoarseness). tongue muscles, soft palate and pharynx is not accompanied by atrophy and is much less pronounced than with bulbar paralysis (bulbar paralysis). Caused by oral automatism (see Reflexes), which are associated with a concomitant dysfunction of the central motor neurons and cortical-nuclear pathways to the nuclei of the facial and trigeminal nerves. Patients are forced to eat slowly, choke when swallowing due to the ingress of liquid food into (soft palate); salivation is noted. Often P. p. is accompanied by bouts of violent laughter or crying, which are not associated with emotions and arise as a result of spastic contraction of facial muscles. At the same time, weak-heartedness, impaired attention, memory, followed by a decrease in intelligence can be observed.

By the nature of the accompanying symptoms, pyramidal, extrapyramidal, pontine, as well as hereditary and children's forms of P. p. are distinguished. In the pyramidal form, in addition to the manifestations of P. p., diparesis of the extremities is observed, deep reflexes almost always increase and pathological hand and foot reflexes are caused. In the extrapyramidal form, the signs of P. p. are combined with the akineticorid syndrome (Akinetic-rigid syndrome). The pontine form is characterized by a combination of signs of P. p. with central paralysis of the muscles innervated by the V, VII and VI pairs of cranial nerves. The hereditary form of P. p. is one of the components of the complex of neurological manifestations caused by genetic disorder brain metabolism with degeneration of pyramidal neurons. The children's form P. p. develops as a result of the birth brain or transferred intrauterine encephalitis and is characterized by a combination of P. p. with spastic diparesis, choreic, athetoid or torsion hyperkinesis (Hyperkinesis).

Diagnosis in typical cases is not difficult. P. p. should be differentiated from bulbar palsy. With the latter, there are no reflexes of oral automatism, the pharyngeal, reflex from the soft palate is not called, there are also fascicular twitches of the muscles of the tongue, nasality is more pronounced. Treatment depends on the form of P. p. its stage of the underlying disease. With the progression of P. p., drugs are used that normalize blood coagulation processes that reduce the content of cholesterol in the blood. Shown drugs that improve microcirculation in the brain, metabolic processes and bioenergetics of neurons (aminalon, encephabol, cerebrolysin, etc.), anticholinesterase agents (prozerin, oxazil, etc.). At acute development P. p. necessary in a hospital, probe. With severe swallowing disorders, food masses with obturation are possible respiratory tract, which requires resuscitation (see Asphyxia).

Bibliographer.: Gusev E.I., Grechko V.E. and Burd G.S. Nervous diseases, M., 1988; Collins R.Ts. Diagnosis of nervous diseases. from English, M., 1986.


1. Small medical encyclopedia. - M.: Medical Encyclopedia. 1991-96 2. First aid. - M.: Great Russian Encyclopedia. 1994 3. encyclopedic Dictionary medical terms. - M.: Soviet Encyclopedia. - 1982-1984.

See what "Pseudobulbar palsy" is in other dictionaries:

    PSEUDOBULBAR PARALYSIS- (false bulbar paralysis), loss of function of the lingual, chewing, facial, pharyngeal and laryngeal muscles, and sometimes the corresponding eye movements due to damage to the central, cortical nuclear conductors motor nuclei… … Big Medical Encyclopedia

    PSEUDOBULBAR PARALYSIS- [from Greek. pseudos lie lat. bulbus bulb, the shape of which resembles the medulla oblongata and Greek. paralysis relaxation] central paralysis facial expressions of the articulatory muscles, muscles of the tongue, pharynx, soft palate and larynx, due to ... ...

    ICD 10 G12.212.2 ICD 9 335.23335.23 DiseasesDB ... Wikipedia

    Loss or impairment of movement in one or more parts of the body. Paralysis is a symptom of many organic diseases nervous system. The condition in which voluntary movements are not completely lost is called paresis. Causes. Paralysis is not... ... Collier Encyclopedia

    PSEUDOBULBAR PARALYSIS- [from Greek. pseudos lie and lat. bulbus bulb, the shape of which resembles the medulla oblongata] paralysis of mimic articulatory muscles innervated by motor cranial cerebral nerves due to bilateral damage to the cortical stem ... ... Psychomotor: Dictionary Reference

    - (paralysis pseudobulbaris; synonym: false bulbar palsy, bulbar supranuclear palsy) paresis of muscles innervated by motor cranial nerves, caused by bilateral lesions of cortical nuclear fibers in the hemispheres or in the trunk ... ... Big Medical Dictionary

    Paralysis pseudobulbar- Syn.: Pseudobulbar syndrome. Combined dysfunction of the caudal group of cranial nerves, due to bilateral damage to the corticonuclear pathways leading to their nuclei. In this case, the clinical picture resembles the manifestations of bulbar ... ... Encyclopedic Dictionary of Psychology and Pedagogy Great Psychological Encyclopedia

Bulbar and pseudobulbar paralysis.

Bulbar paralysis.

This is a symptom complex resulting from damage to the motor nuclei, roots, or the 9th, 10th, 12th pairs of cranial nerves themselves, with a clinic of flaccid atrophic (peripheral) paresis, paralysis of the muscles innervated by these nerves. Especially pronounced in bilateral lesions.

Bulbar palsy is characteristic of amyotrophic lateral sclerosis, progressive Duchenne bulbar palsy, poliomyelitis, poliomyelitis-like diseases, tick-borne encephalitis, tumors of the tegmentum oblongata and cerebellum, syringobulbia.

Etiology: with tumors and arachnoiditis in the posterior cranial fossa, carcinomatosis, sarcomatosis, granulomatous processes, meningitis with predominant localization in the posterior cranial fossa, with diphtheria polyneuritis, infectious-allergic polyradiculoneuritis.

Clinic: There is a violation of articulation (dysarthria, anartria), swallowing (dysphagia, aphagia), phonation (dysphonia, aphonia), a nasal tone of speech (nasolalia) is noted. There is a descent of the soft palate, its immobility when pronouncing sounds, sometimes a deviation of the palatine uvula. The defeat of the 10th nerve is manifested by a disorder of breathing and cardiovascular activity. Signs come to light peripheral paralysis(atrophy of the muscles of the tongue, a decrease in its volume, folding of the mucous membrane of the tongue). The defeat of the nuclei is characterized by fasciculation of the tongue. Palatal, pharyngeal, cough, gag reflexes reduced or absent, paresis of the circular muscle of the mouth is often noted.

Diagnosis is based on the clinic. Differential diagnosis is carried out with pseudobulbar paralysis. Treatment consists in the treatment of the underlying disease. With bilateral damage to the 10th pair, the outcome is fatal.

Pseudobulbar paralysis.

This is a symptom complex that occurs with bilateral interruption of the corticonuclear pathways 9, 10, 12 cranial nerves, with clinical picture central paresis or paralysis of the muscles innervated by these cranial nerves.

Most often accompanied by pseudobulbar palsy the following diseases Key words: multiple sclerosis, gliomas of the base and other tumors of the base of the brain bridge, circulatory disorders in the vertebrobasilar basin, central pontine myelinolysis. Lesions of corticonuclear fibers in the region of the legs of the brain are more often associated with impaired cerebral circulation and tumors. More orally located bilateral lesions of the corticonuclear pathways are usually observed in diffuse or multifocal processes in both hemispheres - cerebrovascular diseases, demyelinating diseases, encephalitis, intoxication, brain trauma and their consequences.

The defeat of the cortical-nuclear tracts of 9, 10, 12 pairs of cranial nerves leads to the appearance of a picture of central paralysis.

Clinic: Manifested by disorders of swallowing (dysphagia), phonation (dysphonia), articulation of speech (dysarthria). There is a tendency to violent laughter and crying, which is due to bilateral interruption of descending cortical fibers that conduct inhibitory impulses.

In contrast to bulbar paralysis, in pseudobulbar syndrome, paralyzed muscles do not atrophy and there is no degeneration reaction. At the same time, the reflexes associated with the brain stem are not only preserved, but also pathologically increased - palatine, pharyngeal, cough, vomiting. The presence of symptoms of oral automatism is characteristic.

Diagnosis is based on the clinic. Differential diagnosis with bulbar paralysis. Treatment and prognosis depend on the nature and severity of the disease that caused this pathological condition.

16. Clinical manifestations damage to the frontal lobe of the brain .

frontal lobe syndrome common name a set of syndromes of various, functionally significant structures of the frontal lobe of the cerebral hemispheres. Each of these syndromes can be presented in 2 variants - irritative and prolapsed. In addition, the characterization of the frontal lobe syndrome is influenced by the law of functional asymmetry cerebral hemispheres, syndromes of the left and right hemispheres have significant differences. In accordance with this law, the left hemisphere in right-handers is responsible for verbal functions(for left-handers - on the contrary), the right one - for non-verbal, gnostic-practical functions (for left-handers - on the contrary).

Syndrome posterior divisions frontal lobe. It occurs when the following Brodmann fields are affected: 6 (agraphia), 8 (friendly turn of the eyes and head in the opposite direction and "cortical" gaze paralysis to the side, aspantality, astasia-abasia), 44 (motor aphasia).

Syndrome of the middle section. Occurs with the defeat of 9, 45, 46, 47 Brodmann fields. The main symptoms of this syndrome are mental disorders, which can be represented by 2 main syndromes:

· Apatico-abulic syndrome - lack of initiative to any movement.

· Disinhibited-euphoric syndrome - the opposite of the first syndrome. Those. infantilism, foolishness, euphoria.

Other symptoms of damage to the middle part of the frontal lobe may include:

Symptom of "mimic facialis" (Vincent's symptom) - the presence of insufficiency of lower mimic innervation during crying, laughing, smiling.

Grasping phenomena - Yanishevsky's reflex (at the slightest touch to the palmar surface, the hand reflexively clenches into a fist), Robinson's reflex (the phenomenon of automatic obsessive grasping and pursuit);

· Typical changes in posture (reminds the posture of Parkinson's);

Syndrome anterior section(poles). Occurs with the defeat of the 10th and 11th fields according to Brodman. The leading symptoms of this syndrome are disorders of statics and coordination, referred to as frontal ataxia (deviation of the body at rest to the side, overshooting), adiadochokinesis and impaired coordination on the side opposite to the focus, less often on the side of the focus. Sometimes these symptoms are referred to as pseudocerebellar. They differ from true cerebellar disorders in lesser intensity of disorders, the absence of hypotension of the muscles of the extremities and their combination with changes in muscle tone according to the extrapyramidal type (rigor, the phenomena of “gear wheel” and “contracontinence”). The described symptoms are caused by damage to the fronto-bridge and ponto-cerebellar tracts, starting mainly in the poles of the frontal lobes.

Under surface syndrome. It is similar to the syndromes of the middle part of the frontal lobe and the frontal pole, differing in the presence of an obligatory lesion of the olfactory nerve. The leading symptom, in addition to mental disorders (apatic-abulic or disinhibited-euphoric syndromes), is hyp- or anosmia (decrease or absence of smell) on the side of the focus. With the spread of the pathological focus posteriorly, Foster-Kennedy syndrome may appear (primary atrophy of the disc optic nerve, on the side of the focus, as a result of pressure on the optic nerve) and the presence of a congestive optic disc. There may also be pain on percussion of the zygomatic process or the frontal region of the head and the presence of exophthalmos homolateral to the focus, indicating the proximity pathological process to the base of the skull and to the orbit.



Syndrome of the precentral region. Occurs when the precentral gyrus is damaged (field 4 and partially 6 according to Brodmann), which is the motor area of ​​the cortex big brain. Here are the centers of elementary motor functions - flexion, extension, adduction, abduction, pronation, supination, etc. The syndrome is known in 2 versions:

Option of irritation (irritation). Gives a syndrome of partial (focal) epilepsy. These seizures (Jackson's, Kozhevnikov's) are expressed by clonic or tonic-clonic convulsions, stereotypically starting from a certain part of the body, according to the somatotopic projection: irritation of the lower parts of the precentral gyrus gives partial seizures that begin in the muscles of the pharynx, tongue and lower mimic group (opercular syndrome - attacks of chewing or swallowing movements, lip licking, smacking). Unilateral irritation of the cerebral cortex in the region of the middle part of the precentral gyrus gives clonic-tonic paroxysms - an irritative syndrome of the anterior central gyrus, - clonic or clonic-tonic convulsions in the arm contralateral to the focus, starting from the distal sections (hand, fingers) of the latter. Irritation of the cerebral cortex in the region of the paracentral lobule causes the appearance of clonic or clonic-tonic convulsions, starting from the muscles of the foot of the opposite part of the body.

· Cerebral prolapse variant. It is manifested by a violation of arbitrary motor function - central paresis (paralysis). A characteristic sign of cortical disorders of motor function is the monoplegic type of paralysis or paresis, a combination of monoparesis of the arm or leg with central paresis of the muscles innervated by the facial and hypoglossal nerves- a syndrome of loss of functions of the anterior central gyrus, caused by a unilateral lesion of a certain area of ​​the cerebral cortex in the region of the anterior central gyrus.

Neurologists identify the following main causes of pseudobulbar palsy:

  1. Vascular diseases affecting both hemispheres (lacunar condition with hypertension, atherosclerosis; vasculitis).
  2. Perinatal pathology and birth trauma including.
  3. Congenital bilateral periaqueductal syndrome.
  4. Traumatic brain injury.
  5. Episodic pseudobulbar palsy in epileptiform opercular syndrome in children.
  6. Degenerative diseases affecting the pyramidal and extrapyramidal systems: ALS, primary lateral sclerosis, familial spastic paraplegia (rare), 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 pseudobulbar paralysis. 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 the latent vascular brain failure in the other hemisphere. In vascular pseudobulbar palsy, the latter may be accompanied by hemiparesis, tetraparesis, or bilateral pyramidal insufficiency without paresis. Revealed vascular disease brain, usually confirmed by MRI.

Perinatal pathology and birth trauma

Due to perinatal hypoxia or asphyxia, as well as birth injury may develop a variety of forms of childhood cerebral palsy(CP) with the development of spastic-paretic (diplegic, hemiplegic, tetraplegic), dyskinetic (mainly dystonic), atactic and mixed syndromes, including with a picture of 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. The history usually includes indications of perinatal pathology, delayed psychomotor development neurological status residual symptoms of perinatal encephalopathy are revealed.

Differential Diagnosis cerebral palsy includes some degenerative and hereditary metabolic disorders (type I glutaric aciduria; arginase deficiency; dopa-responsive dystonia; hyperekplexia (with rigidity); Lesch-Nyhan 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 children neurological practice. It leads (like congenital bilateral sclerosis of the hippocampus) to a pronounced violation speech development, which sometimes even mimics childhood autism and a picture of pseudobulbar palsy (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

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

Degenerative diseases

Many degenerative diseases occurring with the involvement of the pyramidal and extrapyramidal systems, may be accompanied by pseudobulbar syndrome. These diseases include lateral amyotrophic 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 metabolic diseases myelin (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 present with variable clinical symptoms depending on its localization within the caudal, middle (pons varolii) or oral parts of the brain stem. 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 shape multiple sclerosis, vascular malformation (usually cavernous hemangioma) and stem encephalitis. IN differential diagnosis MRI is of great help. It is important to distinguish between focal and diffuse forms of glioma (astrocytoma).

Hypoxic (anoxic) encephalopathy

Hypoxic encephalopathy with severe neurological complications characteristic of patients who survived resuscitation after asphyxia, clinical death, long coma and so on. The consequences of severe hypoxia, in addition to prolonged coma in the acute period, include several clinical options, including dementia with (or without) extrapyramidal syndromes, cerebellar ataxia, myoclonic syndromes, Korsakov's amnestic syndrome. Separately considered delayed postanoxic encephalopathy with bad outcome.

Sometimes there are patients with hypoxic encephalopathy who have 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 component more extensive neurological syndromes. For example, pseudobulbar syndrome in the picture of central pontine myelinolysis ( malignant neoplasm, liver failure, sepsis, alcoholism, chronic kidney failure, lymphoma, cachexia, severe dehydration and electrolyte disturbances, hemorrhagic pancreatitis, pellagra) and the "locked-in person" syndrome overlapping with it (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 manifests itself in the rapid development of tetraplegia (against the background of a somatic disease or Wernicke's encephalopathy), and pseudobulbar palsy due to demyelination central departments pons, which is visible on an MRI and can in turn lead to locked-in syndrome. 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.

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