Facial hyperkinesias

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Hyperkinesis of organic origin

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Hyperkinetic syndromes with predominant involvement of facial muscles

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Facial paraspasm

The following forms of blepharospasm are distinguished:

• primary: blepharospasm-oromandibular dystonia syndrome (facial paraspasm, Mezha syndrome, Bruegel syndrome);
• secondary - in organic diseases of the brain (Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, multiple sclerosis, "dystonia plus" syndromes, vascular, inflammatory, metabolic and toxic (including neuroleptic) lesions of the nervous system;
• caused by ophthalmological reasons;
• other forms (facial hemispasm, facial synkinesis, painful tics and other “peripheral” forms).

Primary (dystonic) blepharospasm is observed in the picture of facial paraspasm. Facial paraspasm is a specific form of idiopathic (primary) dystonia, described in the literature under different names: Mezh paraspasm, Bruegel syndrome, blepharospasm-oromandibular dystonia syndrome, cranial dystonia. Women are affected three times more often than men.

As a rule, the disease begins with blepharospasm, and in such cases we are talking about focal dystonia with blepharospasm syndrome. Usually, after a few years, dystonia of the oral muscles joins in. The latter is called oromandibular dystonia, and the entire syndrome is designated as segmental dystonia with blepharospasm and oromandibular dystonia. However, the time interval between the appearance of blepharospasm and the onset of oromandibular dystonia sometimes spans many years (up to 20 years or more), so many patients simply do not live to see the generalized stage of paraspasm. In this regard, this blepharospasm syndrome can be legitimately considered both as a stage and as a form of facial paraspasm. In this case, isolated blepharospasm is sometimes called essential blepharospasm.

Much less often, the disease begins with the lower half of the face ("lower Bruegel syndrome"). As a rule, with this type of debut of Bruegel syndrome, dystonia does not subsequently generalize across the face, that is, blepharospasm does not join oromandibular dystonia and at all subsequent stages of the disease, this syndrome remains focal.

Facial paraspasm most often occurs in the 5th-6th decade of life. The disease develops in childhood extremely rarely. In typical cases, the disease begins with slightly increased blinking, which gradually increases in frequency followed by the appearance of tonic spasms of the orbicularis oculi muscle with squinting (blepharospasm). At the onset of the disease, blepharospasm is unilateral or clearly asymmetrical in approximately 20% of cases. It is extremely rare for blepharospasm to remain persistently unilateral after long-term observation. In the latter case, differential diagnosis of Bruegel's syndrome and facial hemispasm becomes relevant. The motor pattern of blepharospasm itself in these diseases is different, but a more reliable and simple method in differential diagnosis is the analysis of the dynamics of hyperkinesis.

Having begun gradually, facial paraspasm then very slowly, over 2-3 years, progresses, after which it becomes stationary. Rarely, in about 10% of patients, short-term remissions are possible.

Severe blepharospasm is manifested by extremely intense blinking and may be accompanied by facial hyperemia, dyspnea, straining and hand movements, indicating unsuccessful attempts by the patient to overcome blepharospasm. Blepharospasm is characterized by corrective gestures (especially in the early stages of the disease) and paradoxical kinesias, which are distinguished by a great variety. Most often, blepharospasm ceases during any oral activity (smoking, sucking candy, eating sunflower seeds, expressive speech, etc.), emotional activation (for example, during a visit to the doctor), after a night's sleep, drinking alcohol, in the dark, when closing one eye and, especially, when closing both eyes.

Blepharospasm has a pronounced stress-producing effect and, as the disease progresses, causes serious maladaptation due to the inability to use one's vision in everyday life. This is accompanied by noticeable emotional-personal and dissomnic disorders. Two-thirds of patients with severe blepharospasm become "functionally blind", since they cannot use the function of vision, which is preserved in itself.

Like all other dystonic hyperkinesis, blepharospasm depends on the characteristics of postural innervation: it is almost always possible to find such positions of the eyeballs in which blepharospasm ceases. It usually decreases or completely disappears with extreme abduction of the eyeballs during tracking movements. Patients note relief with half-lowered eyelids (writing, washing, knitting, communicating and moving with half-lowered eyes). Hyperkinesis often decreases in a sitting position and, as a rule, subsides in a lying position, which is typical to one degree or another for all forms of dystonia. The greatest provoking effect on blepharospasm is natural sunlight outdoors.

The described phenomena are the mainstays of clinical diagnostics of dystonic hyperkinesis. Their value increases when several of the above-mentioned characteristic symptoms are detected in the patient.

Differential diagnosis of blepharospasm should be made within the scope of the above-mentioned primary and secondary forms of blepharospasm. This list should only be supplemented by the syndrome of apraxia of opening of the eyelids, with which blepharospasm sometimes has to be differentiated. It should not be forgotten, however, that apraxia of opening of the eyelids and blepharospasm can often coexist in the same patient.

Secondary forms of dystonic blepharospasm, observed in the picture of various organic diseases of the brain (Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, multiple sclerosis, "dystonia plus" syndromes, vascular, inflammatory, metabolic and toxic, including neuroleptic, lesions of the nervous system) carry all the clinical features of dystonic blepharospasm and are recognized, firstly, due to typical dynamic characteristics (corrective gestures and paradoxical kinesia, effects of night sleep, alcohol, changes in visual afferentation, etc.) and, secondly, by the accompanying neurological symptoms that manifest the diseases listed above.

Blepharospasm caused by ophthalmological reasons rarely causes diagnostic difficulties. These eye diseases (conjunctivitis, keratitis) are usually accompanied by pain and such patients immediately come to the attention of an ophthalmologist. Blepharospasm itself does not have any of the above-mentioned properties of dystonic blepharospasm. The same applies to other "peripheral" forms of blepharospasm (for example, with hemispasm).

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Oral hyperkinesis

The following forms of oral hyperkinesis are distinguished:

• tardive dyskinesia,
• other drug-induced oral hyperkinesias (cerucal, oral contraceptives, other drugs),
• spontaneous orofacial dyskinesia of the elderly,
• other forms (lower Bruegel syndrome, galloping tongue syndrome, rabbit syndrome, bruxism, lingual epilepsy, myokymia of the tongue and others).

Late (tardive) dyskinesia is an iatrogenic, poorly treatable, fairly common disease, which is a direct consequence of the widespread use of neuroleptics in the medical practice of doctors of various specialties. Violent movements in late dyskinesia usually begin in the muscles of the face and tongue. The most characteristic triad of pathological movements is the so-called buccal-lingual-masticatory (bucco-lingual-masticatory) syndrome.

Less often, the muscles of the trunk and limbs are involved in hyperkinesis.

Typically, the onset is subtle, with barely perceptible tongue movements and motor restlessness in the perioral area. In more severe cases, irregular but almost constant movements of the tongue, lips, and lower jaw are clearly visible. These movements often take the form of motor automatisms of licking, sucking, chewing with smacking, smacking, chewing, and lapping movements, sometimes with lip smacking sounds, breathing, grunting, puffing, groaning, and other inarticulate vocalizations. Rolling and protruding of the tongue are characteristic, as are more complex grimaces, primarily in the lower half of the face. These dyskinesias can usually be voluntarily suppressed for a short period of time. For example, oral hyperkinesis ceases when the patient brings food to the mouth while chewing, swallowing, or talking. Mild hypomimia is sometimes detected against the background of oral hyperkinesis. In the extremities, dyskinesia predominantly affects the distal parts (“piano fingers”) and can sometimes be observed on only one side.

Differential diagnosis of tardive dyskinesia requires, first of all, exclusion of the so-called spontaneous orofacial dyskinesia of the elderly, stereotypy, oral hyperkinesias in neurological and somatic diseases. Clinical manifestations of spontaneous orofacial dyskinesia are completely identical to those in tardive dyskinesia, which undoubtedly indicates the commonality of their pathogenetic mechanisms. In this case, neuroleptic drugs are assigned the role of the most significant risk factor, allowing to identify a predisposition to dyskinesia at any age.

The diagnostic criteria for tardive dyskinesia are the following features:

1. its symptoms become noticeable after the dose of neuroleptics is reduced or discontinued;
2. the same symptoms are reduced or disappear when treatment with neuroleptics is resumed or the dose of the latter is increased;
3. Anticholinergic drugs, as a rule, do not help such patients and often worsen the manifestations of tardive dyskinesia.

At all stages of the disease, the tongue plays a very active role in the clinical manifestations of tardive dyskinesia: rhythmic or constant protrusion, forced extrusion of the tongue from the mouth; patients are usually unable to keep the tongue out of the mouth for 30 seconds.

Discontinuation of antipsychotic drugs may lead to a worsening of the patient's condition and the appearance of new dyskinetic symptoms. In some cases, their withdrawal leads to a decrease or disappearance of dyskinesia (sometimes after a period of temporary increase in hyperkinesia). In this regard, tardive dyskinesia is divided into reversible and irreversible or persistent. It is believed that the presence of symptoms of tardive dyskinesia 3 months after the withdrawal of neuroleptics can be considered as a criterion for persistent dyskinesia. The issue of discontinuing neuroleptics should be decided strictly individually due to the risk of relapse of psychosis. A number of risk factors have been identified that predispose to the development of tardive dyskinesia: duration of treatment with neuroleptics, older age, gender (women are more often affected), long-term use of anticholinergics, previous organic brain damage, a certain role of genetic predisposition is also assumed.

Although tardive dyskinesia most often develops in adulthood and old age, it can appear in young and even childhood. In addition to the clinical picture, an important diagnostic factor is the identification of a connection between the occurrence of dyskinesia and the use of a neuroleptic. Spontaneous orofacial dyskinesia of the elderly (oral masticatory syndrome of the elderly, spontaneous orofacial dyskinesia) appears only in the elderly (usually in people over 70 years old) who have not received neuroleptics. It has been noted that spontaneous oral dyskinesia in the elderly in a high percentage of cases (up to 50% and above) is combined with essential tremor.

Differential diagnosis of tardive dyskinesia should also be made with another neuroleptic phenomenon in the oral area - the "rabbit" syndrome. The latter is manifested by rhythmic tremor of the perioral muscles, mainly the upper lip, sometimes with the involvement of the masticatory muscles (tremor of the lower jaw), with a frequency of about 5 per second. The tongue is usually not involved in hyperkinesis. Externally, the violent movements are similar to the movements of the mouth of a rabbit. This syndrome also develops against the background of long-term treatment with neuroleptics, but, unlike tardive dyskinesia, it responds to treatment with anticholinergics.

At the onset of the disease, tardive dyskinesia and spontaneous oral dyskinesia in the elderly sometimes have to be differentiated from the onset of Huntington's chorea.

In severe cases, tardive dyskinesia manifests itself in generalized choreic movements, less frequently in ballistic throws, dystonic spasms and postures. These cases require differential diagnosis with a wider range of diseases (Huntington's chorea, neuroacanthocytosis, hyperthyroidism, systemic lupus erythematosus, other causes of chorea).

There are also other, drug-induced or toxic forms of oral hyperkinesis (especially when using cerucal, oral contraceptives, alcohol), which in their clinical manifestations have features of dystonic hyperkinesis, but are associated with the use of the above substances and are often paroxysmal (transient) in nature.

Other forms of oral hyperkinesis include rather rare syndromes: “lower” Bruegel syndrome (oromandibular dystonia), “galloping” tongue syndrome, the already mentioned “rabbit” syndrome, bruxism, etc.

Oromandibular dystonia (or "lower Bruegel syndrome") is difficult to diagnose in cases where it is the first and main manifestation of Bruegel syndrome. If it is combined with blepharospasm, the diagnosis is usually not difficult. Oromandibular dystonia is characterized by the involvement of not only the muscles of the oral pole in hyperkinesis, but also the muscles of the tongue, diaphragm, cheeks, masticatory, cervical and even respiratory muscles. Involvement of the cervical muscles may be accompanied by manifestations of torticollis. In addition, a number of movements in the face and even in the trunk and limbs in such patients are not pathological; they are completely voluntary and reflect the patient's active attempts to counteract muscle spasms.

Oromandibular dystonia is characterized by a variety of its manifestations. In typical cases, it takes the form of one of three well-known variants:

1. spasm of the muscles that close the mouth and squeeze the jaws (dystonic trismus);
2. spasm of the muscles that open the mouth (the classic version, depicted in the famous painting by Bruegel) and
3. constant trismus with lateral jerking movements of the lower jaw, bruxism and even hypertrophy of the masticatory muscles.

The lower variant of Bruegel's syndrome is often accompanied by difficulties with swallowing, chewing and articulation (spastic dysphonia and dysphagia).

The diagnosis of oromandibular dystonia is based on the same principles as the diagnosis of any other dystonic syndrome: mainly on the analysis of the dynamics of hyperkinesis (the relationship of its manifestations with postural loads, time of day, the effect of alcohol, corrective gestures and paradoxical kinesias, etc.), the identification of other dystonic syndromes, which in Bruegel's syndrome occur in other parts of the body (outside the face) in 30 - 80% of patients.

It is not uncommon for ill-fitting dentures to cause excessive motor activity in the oral area. This syndrome is more common in women aged 40-50, prone to neurotic reactions.

Episodic repetitive movements of the tongue ("lingual epilepsy") have been described in children with epilepsy (including during sleep; in patients after traumatic brain injury (without any changes in the EEG) in the form of undulating (3 per second) depressions and protrusions at the root of the tongue ("galloping tongue syndrome"), or rhythmic pushing it out of the mouth (a type of myoclonus) with a favorable course and outcome.

The syndrome of lingual dystonia after electrical trauma and myokymia of the tongue after radiation therapy is described.

Bruxism is another common oral hyperkinesis. It manifests itself as periodic, stereotypical movements of the lower jaw with clenching and characteristic grinding of the teeth during sleep. Bruxism is observed in healthy individuals (6 to 20% of the entire population) and is often associated with such phenomena as periodic limb movements during sleep, sleep apnea, epilepsy, tardive dyskinesia, schizophrenia, mental retardation, and post-traumatic stress disorder. An externally similar phenomenon during wakefulness is usually described as trismus.

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Facial hemispasm

Facial hemispasm is characterized by stereotypical clinical manifestations, which facilitates its diagnosis.

The following forms of facial hemispasm are distinguished:

• idiopathic (primary);
• secondary (compression of the facial nerve by a tortuous artery, less often by a tumor, and even less often by other causes).

Hyperkinesis in facial hemispasm is paroxysmal. The paroxysm consists of a series of short, rapid twitches, most noticeable in the orbicularis oculi muscle, which, superimposed on each other, turn into a tonic spasm, giving the patient a characteristic facial expression that cannot be confused with anything else. In this case, there is squinting or squinting of the eye, pulling the cheek and corner of the mouth up, sometimes (with a pronounced spasm) deviation of the tip of the nose in the direction of the spasm, often contraction of the muscles of the chin and platysma. Upon careful examination during the paroxysm, large fasciculations and myoclonus with a noticeable tonic component are visible. In the interictal period, microsymptoms of increased muscle tone are revealed in the affected half of the face: a prominent and deepened nasolabial fold, often a slight shortening of the muscles of the lips, nose and chin on the ipsilateral side of the face. Paradoxically, subclinical signs of facial nerve insufficiency on the same side are simultaneously revealed (lesser retraction of the corner of the mouth when grinning, the "eyelash" symptom when squinting voluntarily). Paroxysms usually last from several seconds to 1-3 minutes. Hundreds of attacks are observed during the day. It is important to note that, unlike other facial hyperkinesis (tics, facial paraspasm), patients with facial hemispasm can never demonstrate their hyperkinesis. It is not subject to volitional control, is not accompanied by corrective gestures and paradoxical kinesias. There is a smaller dependence of the severity of hyperkinesis on the functional state of the brain than in many other forms. Voluntary squinting sometimes provokes hyperkinesis. The most significant is the state of emotional stress, leading to an increase in the frequency of motor paroxysms, while at rest it disappears, although not for long. Periods free from hyperkinesis usually last no more than a few minutes. During sleep, hyperkinesis persists, but occurs much less frequently, which is objectified by a night polygraphic study.

In more than 90% of patients, hyperkinesis begins in the orbicularis oculi muscle, and in the vast majority of cases, in the muscles of the lower eyelid. Over the next few months or years (usually 1-3 years), other muscles innervated by the facial nerve are involved (up to m. stapedius, which leads to a characteristic sound that the patient feels in the ear during spasm), which are synchronously involved in a motor paroxysm. Subsequently, a certain stabilization of the hyperkinetic syndrome is observed. Spontaneous recovery does not occur. An integral part of the clinical picture of facial hemispasm is a characteristic syndromic environment, occurring in 70-90% of cases: arterial hypertension (usually easily tolerated by the patient), insomnia disorders, emotional disorders, moderate cephalgic syndrome of a mixed nature (tension headaches, vascular and cervicogenic headaches). A rare but clinically significant syndrome is trigeminal neuralgia, which, according to the literature, occurs in approximately 5% of patients with facial hemispasm. Rare cases of bilateral facial hemispasm have been described. The second side of the face is usually involved after several months or years (up to 15 years), and in this case, attacks of hyperkinesis on the left and right halves of the face are never synchronous.

On the side of hemispasm, as a rule, subclinical, but quite obvious constant (background) symptoms of mild insufficiency of the VII nerve are detected.

Emotional disorders, predominantly of an anxious and anxious-depressive nature, tend to worsen with the development in some cases of maladaptive psychopathological disorders, up to severe depression with suicidal thoughts and actions.

Although most cases of facial hemispasm are idiopathic, these patients require careful examination to exclude symptomatic forms of hemispasm (compression lesions of the facial nerve at the exit from the brainstem). Differential diagnosis of facial hemispasm with another unilateral hyperkinesis of the face - postparalytic contracture - does not cause any particular difficulties, since the latter develops after neuropathy of the facial nerve. But it should be remembered that there is a so-called primary facial contracture, which is not preceded by paralysis, but which is nevertheless accompanied by mild, compared to the hyperkinesis itself, clinical signs of facial nerve damage. This form is characterized by pathological synkinesis in the face typical of postparalytic contractures.

At the onset of facial hemispasm, differentiation from facial myokymia may be necessary. This is most often a unilateral syndrome, manifested by small vermiform contractions of the muscles of the perioral or periorbital localization. Paroxysmality is not very characteristic of it, its manifestations practically do not depend on the functional state of the brain, and the presence of this syndrome always indicates a current organic lesion of the brainstem (most often multiple sclerosis or a tumor of the pons).

Rare cases of facial paraspasm manifest themselves in atypical forms such as unilateral blepharospasm and even unilateral Bruegel's syndrome on the upper and lower half of the face. Formally, such hyperkinesis looks like hemispasm, since it involves one half of the face, but in the first case, hyperkinesis has clinical and dynamic signs characteristic of dystonia, in the second - of facial hemispasm.

In such difficult cases, differential diagnosis is also recommended to include pathology of the temporomandibular joint, tetanus, partial epilepsy, tonic spasms in multiple sclerosis, hemimasticatory spasm, tetany, facial myokymia, and labiolingual spasm in hysteria.

Sometimes it is necessary to differentiate from tics or psychogenic ("hysterical" in the old terminology) hyperkinesis in the face, occurring as a type of facial hemispasm. Among other things, it is useful to remember that only those muscles that are innervated by the facial nerve participate in the formation of facial hemispasm.

In case of significant diagnostic difficulties, night polygraphy can play a decisive role. According to our data, in 100% of cases of facial hemispasm, night polygraphy reveals a pathognomonic EMG phenomenon for this disease in the form of paroxysmal, high-amplitude (over 200 μV) fasciculations occurring in the superficial stages of night sleep, grouped into bursts of irregular duration and frequency. The paroxysm begins suddenly with maximum amplitudes and ends just as abruptly. It is an EMG correlate of hyperkinesis and is specific for facial hemispasm.

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Facial hyperkinesis, combined with or arising against the background of more widespread hyperkinesis and other neurological syndromes

• Idiopathic tics and Tourette syndrome.
• Generalized drug-induced dyskinesia (1-dopa, antidepressants and other drugs).
• Choreic hyperkinesis of the face (Huntington's chorea, Sydenham's chorea, benign hereditary chorea, etc.).
• Facial myokymia (brain stem tumors, multiple sclerosis, etc.).
• Facial crumples.
• Facial hyperkinesis of epileptic nature.

It is necessary to emphasize once again that in a number of diseases facial hyperkinesis may be only a stage or a component of a generalized hyperkinetic syndrome of various origins. Thus, idiopathic tics, Tourette's disease, Huntington's chorea or Sydenham's chorea, widespread cramps, many drug-induced dyskinesias (for example, associated with treatment with dopa-containing drugs), etc. may initially manifest only as facial dyskinesias. At the same time, a wide range of diseases is known in which facial hyperkinesis is immediately revealed in the picture of a generalized hyperkinetic syndrome (myoclonic, choreic, dystonic or tic). Many of these diseases are accompanied by characteristic neurological and (or) somatic manifestations, which significantly facilitate diagnosis.

This group also includes facial hyperkinesis of epileptic nature (opercular syndrome, facial spasms, gaze deviations, "lingual" epilepsy, etc.). In this case, differential diagnosis should be carried out in the context of all clinical and paraclinical manifestations of the disease.

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Hyperkinetic syndromes in the facial area not associated with the participation of facial muscles

1. Oculogyric dystonia (dystonic gaze deviation).
2. Syndromes of excessive rhythmic activity in the oculomotor muscles:
   • opsoclonus,
   • "nystagmus" of the eyelids,
   • bobbing syndrome,
   • dipping syndrome, d) gaze "ping-pong" syndrome,
   • periodic alternating gaze deviation with dissociated head movements,
   • periodic alternating nystagmus,
   • cyclic oculomotor paralysis with spasms,
   • periodic alternating asymmetric deviation,
   • superior oblique myokymia syndrome,
   • Duan syndrome.
3. Masticatory spasm (trismus). Hemimasticatory spasm.

Clinicians consider it appropriate to include in this section the following (IV) group of hyperkinetic syndromes in the head and neck area of non-facial localization due to the importance of this problem for the practicing physician. (In addition, some of these hyperkinesias are often combined with facial localization of dyskinesias)

Oculogyric dystonia (dystonic gaze deviation) is a characteristic symptom of postencephalitic parkinsonism and one of the early and characteristic signs of neuroleptic side effects (acute dystonia). Oculogyric crises can be an isolated dystonic phenomenon or combined with other dystonic syndromes (tongue protrusion, blepharospasm, etc.). Attacks of upward gaze deviation (less often downward, even less often lateral deviation or oblique gaze deviation) last from several minutes to several hours.

Syndromes of excessive rhythmic activity of the oculomotor muscles. They combine several characteristic phenomena. Opsoclonus - constant or periodic chaotic, irregular saccades in all directions: movements of the eyeballs of different frequencies, different amplitudes and different vectors are observed ("dancing eyes syndrome"). This is a rare syndrome indicating an organic lesion of the brainstem-cerebellar connections of various etiologies. Most cases of opsoclonus described in the literature are related to viral encephalitis. Other causes: tumors or vascular diseases of the cerebellum, multiple sclerosis, paraneoplastic syndrome. In children, 50% of all cases are associated with neuroblastoma.

"Nystagmus of the eyelids" is a rare phenomenon manifested by a series of rapid, rhythmic, upward jerky movements of the upper eyelid. It is described in many diseases (multiple sclerosis, tumors, craniocerebral trauma, Miller Fisher syndrome, alcoholic encephalopathy, etc.) and is caused by such eye movements as convergence or when shifting the gaze. "Nystagmus of the eyelids" is considered a sign of damage to the tegmentum of the midbrain.

Ocular bobbing syndrome is characterized by characteristic vertical eye movements, sometimes called "floating movements": with a frequency of 3-5 per minute, in most cases, bilateral concomitant, rapid downward deviations of the eyeballs are observed, followed by their return to the original position, but at a slower pace than the downward movements. This ocular "swinging" is observed with the eyes open and is usually absent if the eyes are closed. Bilateral horizontal gaze palsy is noted. The syndrome is characteristic of bilateral pons injuries (hemorrhage into the pons, glioma, traumatic injury to the pons; often observed in locked-in syndrome or coma). Atypical bobbing (with preserved horizontal eye movements) has been described in obstructive hydrocephalus, metabolic encephalopathy, and compression of the pons by a cerebellar hematoma.

Ocular dipping syndrome is the opposite of bobbing syndrome. The phenomenon also manifests itself with characteristic vertical eye movements, but in the opposite rhythm: slow downward eye movements are observed, followed by a pause in the extreme lower position and then a rapid return to the middle position. Such cycles of ocular movements are observed several times per minute. The final phase of lifting the eyeballs is sometimes accompanied by wandering eye movements in the horizontal direction. This syndrome has no topical significance and often develops in hypoxia (respiratory disorders, carbon monoxide poisoning, hanging, epileptic status).

The gaze syndrome "ping-pong" (periodic alternating gaze) is observed in patients in a comatose state and is manifested by slow wandering movements of the eyeballs from one extreme position to another. Such repetitive rhythmic horizontal friendly eye movements are associated with bilateral hemispheric damage (infarctions) with relative intactness of the brainstem.

Periodic alternating gaze deviation with dissociated head movements is a unique rare syndrome of cyclic eye movement disorders combined with contraversive head movements. Each cycle includes three phases: 1) concomitant deviation of the eyes to the side with simultaneous turn of the head in the opposite direction lasting 1-2 min; 2) a “switching” period lasting 10 to 15 seconds, during which the head and eyes again acquire the initial normal position and 3) concomitant deviation of the eyes to the other side with compensatory contralateral turn of the face, also lasting 1-2 min. Then the cycle is constantly repeated again, stopping only during sleep. During the cycle, paralysis of gaze in the direction opposite to the direction of ocular deviation is observed. In most of the described cases, nonspecific involvement of the posterior cranial fossa structures is postulated.

Periodic alternating nystagmus may be congenital or acquired and also manifests itself in three phases. The first phase is characterized by horizontal nystagmus impulses that repeat for 90-100 seconds, in which the eyes "beat" in one direction; the second phase is a 5-10 second "neutrality" phase, during which nystagmus may be absent or pendulum-like nystagmus or downward nystagmus may occur, and the third phase, also lasting 90-100 seconds, during which the eyes "beat" in the opposite direction. If the patient tries to look in the direction of the fast phase, the nystagmus becomes more severe. The syndrome is presumably based on bilateral damage to the paramedian reticular formation at the pontomesencephalic level.

Alternating skew deviation. Skew deviation or Hertwig-Magendiesche syndrome is characterized by vertical divergence of the eyes of supranuclear origin. The degree of divergence may remain constant or depend on the direction of gaze. The syndrome is usually caused by acute damage to the brainstem. Sometimes this sign may be intermittent and then periodic alternation of the side of the higher eye is observed. The syndrome is associated with bilateral damage at the pretectal level (acute hydrocephalus, tumor, stroke and multiple sclerosis are the most common causes).

Cyclic oculomotor palsy (phenomenon of cyclic oculomotor spasm and relaxation) is a rare syndrome in which the third (oculomotor) nerve is characterized by alternating phases of its paralysis and phases of increased function. This syndrome can be congenital or acquired in early childhood (in most, but not all cases). The first phase is characterized by complete or almost complete paralysis of the oculomotor (III) nerve with ptosis. It then decreases within 1 minute and then another phase develops, in which the upper eyelid contracts (eyelid retraction), the eye slightly converges, the pupil narrows, and accommodation spasm can increase refraction by several diopters (up to 10 diopters). Cycles are observed at variable intervals within a few minutes. The two phases constitute a cycle that is periodically repeated both during sleep and wakefulness. Voluntary gaze has no effect on them. The probable cause is aberrant regeneration after damage to the third nerve (birth injury, aneurysm).

Superior oblique myokymia syndrome is characterized by rapid rotational oscillations of one eyeball with monocular oscillopsia ("objects jump up and down", "TV screen flickers", "eye swaying") and torsional diplopia. The above-mentioned sensations are especially unpleasant when reading, watching TV, or doing work that requires precise observation. Hyperactivity of the superior oblique muscle of the eye is revealed. The etiology is unknown. Carbamazepine often has a good therapeutic effect.

Duane's syndrome is a hereditary weakness of the lateral rectus muscle of the eye with narrowing of the palpebral fissure. The eye's ability to abduct is reduced or absent; adduction and convergence are limited. Adduction of the eyeball is accompanied by its retraction and narrowing of the palpebral fissure; during abduction, the palpebral fissure widens. The syndrome is usually unilateral.

Masticatory spasm is observed not only in tetanus, but also in some hyperkinetic, in particular dystonic, syndromes. A variant of the "lower" Bruegel syndrome is known, in which dystonic spasm of the muscles that close the mouth develops. Sometimes the degree of trismus is such that problems with feeding the patient arise. Transient trismus is possible in the picture of acute dystonic reactions of neuroleptic origin. Dystonic trismus sometimes has to be differentiated from trismus in polymyositis, in which the involvement of the masticatory muscles is sometimes observed in the early stages of the disease. Mild trismus is observed in the picture of dysfunction of the temporomandibular joint. Trismus is typical for an epileptic seizure, as well as extensor seizures in a patient in a coma.

Hemimasticatory spasm stands apart. This is a rare syndrome characterized by unilateral strong contraction of one or more masticatory muscles. Most patients with hemimasticatory spasm have facial hemiatrophy. The presumed cause of hemimasticatory spasm in facial hemiatrophy is associated with compression neuropathy of the motor portion of the trigeminal nerve due to changes in deep tissues in facial hemiatrophy. Clinically, hemimasticatory spasm manifests itself as short twitches (resembling facial hemispasm) or prolonged spasms (from a few seconds to several minutes, as in cramps). Spasms are painful; tongue biting, dislocation of the temporomandibular joint, and even tooth breakage have been described during spasm. Involuntary movements are provoked by chewing, talking, closing the mouth, and other voluntary movements.

Unilateral spasm of the masticatory muscles is possible in the picture of an epileptic seizure, diseases of the temporomandibular joint, tonic spasms in multiple sclerosis and unilateral dystonia of the lower jaw.

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Hyperkinetic syndromes in the head and neck area of non-facial localization

The following forms are distinguished:

1. Tremor, tics, chorea, myoclonus, dystonia.
2. Laryngospasm, pharyngospasm, esophagospasm.
3. Myoclonus of the soft palate. Myorhythmia.

Tremor, tics, myoclonus and dystonia most often involve the muscles of the head and neck, mainly non-facial. However, there are exceptions: isolated tremor of the lower jaw or isolated "smile tremor" (as well as "voice tremor") as variants of essential tremor. Single or multiple tics limited to the face area are known. Myoclonus can sometimes be limited to individual muscles of the face or neck (including epileptic myoclonus with nodding movements of the head). Unusual and rare dystonic syndromes are unilateral dystonic blepharospasm, dystonic spasms on one half of the face (imitating facial hemispasm), unilateral dystonia of the lower jaw (a rare variant of Bruegel syndrome) or "dystonic smile". Stereotypes are sometimes manifested by nodding and other movements in the head and neck area.

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Laryngospasm, pharyngospasm, esophagospasm

Organic causes of the above syndromes may include dystonia (usually acute dystonic reactions), tetanus, tetany, some muscle diseases (poliomyositis), and diseases that occur with local irritation of the mucous membrane. Manifestations of extrapyramidal (and pyramidal) hypertonia may lead to these syndromes, but usually in the context of more or less generalized disorders of muscle tone.

Soft palate myoclonus and myorhythmia

Velo-palatine myoclonus (nystagmus of the soft palate, tremor of the soft palate, myorhythmia) can be observed either in isolation as rhythmic (2-3 per second) contractions of the soft palate (sometimes with a characteristic clicking sound), or in combination with coarse rhythmic myoclonus of the muscles of the lower jaw, tongue, larynx, platysma, diaphragm and distal parts of the hands. Such a distribution is very typical for myorhythmia. This myoclonus is indistinguishable from tremor, but is characterized by an unusually low frequency (from 50 to 240 oscillations per minute), which distinguishes it even from parkinsonian tremor. Sometimes vertical ocular myoclonus ("swinging") synchronous with velo-palatine myoclonus (oculopalatine myoclonus) can join. Isolated myoclonus of the soft palate can be either idiopathic or symptomatic (pontine and medulla tumors, encephalomyelitis, traumatic brain injury). It has been noted that idiopathic myoclonus often disappears during sleep (as well as during anesthesia and in a comatose state), while symptomatic myoclonus is more persistent in these states.

Generalized myorhythmia without soft palate involvement is rare. Its most common etiology is considered to be vascular damage to the brainstem and cerebellar degeneration associated with alcoholism, other diseases occurring with malabsorption, celiac disease.

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Psychogenic hyperkinesis in the facial area

1. Convergence spasm.
2. Labiolingual spasm.
3. Pseudoblepharospasm.
4. Deviations (including “geotropic”) of gaze.
5. Other forms.

Psychogenic hyperkinesis is diagnosed according to the same criteria as psychogenic hyperkinesis of non-facial localization (they differ from organic hyperkinesis by an unusual motor pattern, unusual dynamics of hyperkinesis, features of the syndromic environment and course).

Currently, criteria for clinical diagnostics of psychogenic tremor, psychogenic myoclonus, psychogenic dystonia and psychogenic parkinsonism have been developed. Here we will mention only specific (occurring almost exclusively in conversion disorders) facial hyperkinesis. These include such phenomena as convergence spasm (unlike organic convergence spasm, which is very rare, psychogenic convergence spasm is accompanied by accommodation spasm with constriction of the pupils), Brissot's labiolingual spasm (although a dystonic phenomenon has recently been described that completely reproduces this syndrome; despite their external identity, they are completely different in their dynamism), pseudoblepharospasm (a rare syndrome observed in the picture of pronounced other manifestations, including facial, demonstrative ones), various gaze deviations (eye rolling, gaze deviation to the side, "geotropic gaze deviation, when the patient tends to look down ("at the ground" with any change in head position); the direction of deviation often changes during one examination of the patient. Other ("other") forms of psychogenic facial hyperkinesis are also possible, which, as is known, are distinguished by an extreme variety of their manifestations.

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Facial Stereotypes in Mental Illnesses

Stereotypes in mental illnesses or as a complication of neuroleptic therapy are manifested by constant repetition of meaningless actions or elementary movements, including in the facial area (raising eyebrows, movements of the lips, tongue, "schizophrenic smile", etc.). The syndrome is described as a behavioral disorder in schizophrenia, autism, delayed mental maturation and in the picture of neuroleptic syndrome. In the latter case, it is often combined with other neuroleptic syndromes and is called tardive stereotypies. Stereotypes rarely develop as a complication of therapy with dopa-containing drugs in the treatment of Parkinson's disease.

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Pathological laughter and crying

This well-known clinical phenomenon, with certain reservations, can be considered as a variant of specific “hyperkinesis” or rhythmic activity of certain functionally related muscles.

The following forms are distinguished:

1. Pseudobulbar palsy.
2. Laughing fits during hysteria.
3. Pathological laughter in mental illnesses.
4. Epileptic fits of laughter.

Pathological laughter and crying in the picture of pseudobulbar paralysis usually does not cause diagnostic difficulties, since it is accompanied by characteristic neurological symptoms and disorders of bulbar functions (swallowing, phonation, articulation, chewing and sometimes breathing).

Laughing fits in hysteria are less common nowadays. They are not always motivated, or are provoked by anxiety or conflicts, are sometimes “contagious” (even “epidemics” of laughter have been described), are observed in people with certain personality disorders and cannot be explained by any organic causes.

Pathological laughter in mental illnesses often appears as a compulsive phenomenon that occurs without external provocation and fits into the picture of obvious psychotic behavioral disorders that are often visible to the “naked eye” (inadequate and strange behavior)

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Laughing fits of epileptic nature

Epileptic fits of laughter (gelolepsy) are described with frontal and temporal localization of epileptic foci (with involvement of the supplementary, limbic cortex and some subcortical structures), they can be accompanied by other most diverse automatisms and epileptic discharges on the EEG. The attack begins absolutely suddenly and ends just as suddenly. Awareness and memory of the attack can sometimes be preserved. The laughter itself looks normal or resembles a caricature of laughter and can sometimes alternate with crying, accompanied by sexual arousal. Helolepsy is described in combination with premature puberty; there are observations of gelolepsy in patients with a hypothalamic tumor. Such patients need a thorough examination to confirm the epileptic nature of the laughing fits and identify the underlying disease.

Eusual dystonic hyperkinesis in the person of a transient nature is described as a complication of chickenpox (deviation of upward gaze, protrusion of the tongue, spasm of the muscles opening the mouth with the inability to speak). The attacks were repeated for several days with subsequent recovery.

Rare forms of hyperkinesis include spasmus nutans (pendulum-shaped nystagmus, torticollis and titubation) in children aged 6-12 months to 2-5 years. It is classified as a benign (transient) disorder.

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