Facial hyperkinesis

[1], [2], [3], [4]

Organic Hyperkinesis

[5], [6], [7], [8]

Hyperkinetic syndromes with primary involvement of facial muscles

[9], [10], [11]

Facial paraspasm

The following forms of blepharospasm are distinguished:

• primary: blepharo-spasm oromandibular dystonia syndrome (facial paraspasm, Mezha syndrome, Bruegel syndrome);
• secondary - with organic brain diseases (Parkinson's disease, progressive supranuclear palsy, multiple systemic atrophy, multiple sclerosis, dystonia plus syndromes, vascular, inflammatory, metabolic and toxic (including neuroleptic) lesions of the nervous system;
• due to ophthalmological reasons;
• other forms (facial hemispasm, facial synkinesis, painful tic and other "peripheral" forms).

Primary (dystonic) blepharospasm is observed in the facial paraspasm picture. Facial paraspasm is a peculiar form of idiopathic (primary) dystonia, described in the literature under various names: Mesa paraspasm, Bruegel syndrome, blepharospasm syndrome - oromandibular dystonia, cranial dystonia. Women fall 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 muscles of the mouth joins. The latter is called oromandibular dystonia, and the whole syndrome is referred to 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), therefore many patients simply do not live to a generalized stage of paraspasm. In this regard, this blepharospasm syndrome can legitimately be considered both as a stage and as a form of facial paraspasm. An isolated blepharospasm is sometimes called essential blepharospasm.

Much less often, the disease begins in the lower half of the face (“lower Bruegel syndrome”). As a rule, with this variant of the debut of Bruegel's syndrome, there is no further generalization of dystonia in the face, that is, blepharospasm does not join oromandibular dystonia and at all subsequent stages of the disease this syndrome remains focal.

Facial paraspasm occurs most often at 5-6 decade of life. Extremely rarely, the disease develops in childhood. In typical cases, the disease begins with a somewhat increased blinking, which gradually becomes more frequent with the subsequent appearance of tonic spasms of the circular muscles of the eye with a squinting motion (blepharospasm). At the onset of the disease in approximately 20% of cases, blepharospasm is unilateral or clearly asymmetrical. Extremely rarely, blepharospasm remains stably one-sided with many years of observation. In the latter case, the differential diagnosis of Bruegel syndrome and facial hemispasm becomes relevant. The motor pattern of the blepharospasm itself in these diseases is different, but a more reliable and simple technique in differential diagnosis is the analysis of the dynamics of hyperkinesis.

Starting gradually, facial paraspasm progresses very slowly, over a period of 2–3 years, after which it acquires a steady flow. Occasionally, in about 10% of patients, not very long remissions are possible.

Severe blepharospasm is manifested by an extremely intense screw-up and may be accompanied by facial flushing, 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, characterized by great diversity. More often, blepharospasm stops during any oral activity (smoking, sucking candy, eating seeds, expressive speech, etc.), emotional activation (for example, during a doctor's visit), after a night's sleep, drinking alcohol, in the dark, with closing one eye and, especially, when closing both eyes.

Blepharospasm has a pronounced stress effect and, as the disease progresses, causes serious maladjustment due to the inability to use your eyesight in everyday life. This is accompanied by noticeable emotional-personal and dissomnic disorders. Two thirds of patients with severe blepharospasm become “functionally blind” because they cannot use the function of vision, which is in itself intact.

Like all other dystonic hyperkinesis, blepharospasm depends on the peculiarities of postural innervation: it is almost always possible to find eyeball positions in which blepharospasm stops. It usually diminishes or disappears completely with extreme abductions of the eyeballs during follow-up movements. Patients celebrate relief with half-eyed ages (writing, washing, knitting, talking and moving with half-eyed eyes). Hyperkinesis often decreases in a sitting position and, as a rule, subsides in a prone position, which is typical in varying degrees for all forms of dystonia. The greatest provocative effect on blepharospasm has a natural sunlight outside.

The described phenomena are the strongholds of the clinical diagnosis of dystonic hyperkinesis. Their value increases with the identification of several characteristic symptoms mentioned above in a patient.

Differential diagnosis of blepharospasm should be carried out in the circle of the above primary and secondary forms of blepharospasm. This list should only be supplemented with eyelid apraxia syndrome, with which it is sometimes necessary to differentiate blepharospasm. We must not forget, however, that apraxia of eyelid opening and blepharospasm can often coexist in the same patient.

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

Blepharospasm due to ophthalmologic causes rarely causes diagnostic difficulties. These diseases of the eye (conjunctivitis, keratitis) are usually accompanied by pain, and such patients immediately come to the eye of the ophthalmologist. Blepharospasm itself does not possess any of the above listed properties of dystonic blepharospasm. This also applies to other "peripheral" forms of blepharospasm (for example, with hemispasm).

[12], [13], [14], [15], [16]

Oral hyperkinesis

The following forms of oral hyperkinesis are distinguished:

• tardive dyskinesia
• other drug-induced oral hyperkinesis (cerrucal, oral contraceptives, other drugs),
• spontaneous orofacial dyskinesia of the elderly,
• other forms (“lower” Bruegel's syndrome, “galloping” tongue syndrome, “rabbit” syndrome, bruxism, “lingual” epilepsy, myokimii language, and others).

Late (tardive) dyskinesia is an iatrogenic, poorly curable, quite common disease, which is a direct consequence of the wide spread of neuroleptics in the medical practice of doctors of different specialties. Violent movements in late dyskinesia usually begin in the muscles of the face and tongue. The triad of pathological movements is most characteristic: the so-called cheek-tongue-chewing (bukko-lingvo-masticator) syndrome.

Less commonly, the muscles of the trunk and extremities are involved in hyperkinesis.

Typically inconspicuous onset in the form of subtle movements of the tongue and motor restlessness in the perioral region. 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 automatism of licking, sucking, chewing with chomping, smacking, chewing and lap movements, sometimes with labial splashing sounds, breathing, groaning, panting, groaning and other unarticularized vocalization. Characterized by rolling and protruding tongue, as well as more complex grimaces mainly in the lower half of the face. These dyskinesias can usually be arbitrarily suppressed for a short time. For example, oral hyperkinesia stops when the patient brings food to the mouth while he chews, swallows, or talks. Sometimes on the background of oral hyperkinesis light hypomimia is detected. In the extremities, dyskinesia preferentially affects the distal regions (“fingers playing the piano”) and can sometimes be observed from only one side.

The differential diagnosis of tardive dyskinesia requires, above all, the exclusion of the so-called spontaneous orofacial dyskinesia of the elderly, stereotypies, and oral hyperkinesis in neurological and somatic diseases. The clinical manifestations of spontaneous orofacial dyskinesia are completely identical to those in late dyskinesia, which undoubtedly indicates the commonality of their pathogenetic mechanisms. In this case, neuroleptic drugs play the role of the most significant risk factor, which allows to identify susceptibility to dyskinesias at any age.

The criteria for the diagnosis of tardive dyskinesia are the following features:

1. its symptoms become noticeable after reducing the dose of neuroleptics or cancel them;
2. the same symptoms diminish or disappear with the resumption of neuroleptic treatment or an increase in the dose of the latter;
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 in the clinical manifestations of tardive dyskinesia the language takes an active part: rhythmic or constant protrusion, forced ejection of it from the mouth; patients are usually not able to keep the tongue protruding from the mouth for 30 seconds.

Cancellation of antipsychotic drugs can lead to aggravation of the patient's condition and the emergence of new dyskinetic symptoms. In some cases, their cancellation leads to a decrease or disappearance of dyskinesia (sometimes after a period of temporary amplification of hyperkinesis). 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 question of the withdrawal of neuroleptics should be decided strictly individually because of the danger of a relapse of psychosis. A number of risk factors that predispose to the development of tardive dyskinesia have been identified: the duration of treatment with neuroleptics, older age, gender (women are more often ill), prolonged use of anticholinergics, prior organic brain damage, and a certain genetic predisposition is also assumed.

Although tardive dyskinesia often develops in mature 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 the relationship between the appearance of dyskinesia and the use of antipsychotics. Spontaneous orofacial dyskinesia of the elderly (oral masticatory syndrome of the elderly, spontaneous orofacial dyskinesia) appears only in the elderly (usually in those over 70 years old) who did not receive neuroleptics. It has been observed that spontaneous oral dyskinesia in the elderly in a high percentage of cases (up to 50% and above) is combined with essential tremor.

The differential diagnosis of tardive dyskinesia should also be carried out with another neuroleptic phenomenon in the oral area — rabbit syndrome. The latter is manifested by the rhythmic tremor of perioral muscles, mainly the upper lip, sometimes involving the masticatory muscles (tremor of the mandible), with a frequency of about 5 per second. Language is usually not involved in hyperkinesis. Externally, violent movements are similar to those of a rabbit's mouth. This syndrome also develops on the background of long-term neuroleptic treatment, but unlike late dyskinesia, it responds to anticholinergic treatment.

In the debut of the disease, late dyskinesia and spontaneous oral dyskinesia in the elderly sometimes have to be differentiated from the beginning of Huntington's chorea.

In severe cases, tardive dyskinesia is manifested by generalized choreic movements, less commonly by ballistic throws, dystonic spasms and postures. These cases require a differential diagnosis with a wider range of diseases (Huntington's chorea, neuroacantocytosis, 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 are features of dystonic hyperkinesis, but are associated with the intake of the above substances and are often paroxysmal (transient) in nature.

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

Oromandibular dystonia (or “lower Bruegel's syndrome”) is difficult to diagnose in cases where it is the first and main manifestation of Bruegel's syndrome. If it is combined with blepharospasm, the diagnosis does not usually cause difficulties. Oromandibular dystonia is characterized by involvement in hyperkinesis of not only the muscles of the oral pole, but also the muscles of the tongue, the diaphragm of the mouth, cheeks, chewing, neck and even the respiratory muscles. The involvement of the neck 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 arbitrary 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 options:

1. muscle spasm covering the mouth and clenching the jaws (dystonic trismus);
2. a spasm of the muscles that open the mouth (the classic version depicted in the famous Bruegel picture) and
3. persistent trismus with lateral jerky movements of the mandible, bruxism and even hypertrophy of the masticatory muscles.

The lower version of Bruegel's syndrome is often accompanied by difficulty in swallowing, chewing and articulation (spastic dysphonia and dysphagia).

Diagnostics 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 connection of its manifestations with postural loads, time of day, the effect of alcohol, corrective gestures and paradoxical kinesies, etc.), identifying other dystonic syndromes, which in Bruegel's syndrome occur in other parts of the body (outside the face) in 30–80% of patients.

Often there is a situation when poorly fitting dentures lead to the occurrence of excessive motor activity in the oral area. This syndrome is more common in women aged 40-50 years who are prone to neurotic reactions.

Episodic repetitive movements of the tongue ("lingual epilepsy") are described in children with epilepsy (including during sleep; in patients after craniocerebral injury (without any changes in the EEG) in the form of untreated (3 per second) retraction and protrusions at the root tongue (“galloping tongue syndrome”), or rhythmic ejection of it from the mouth (a type of myoclonus) with a favorable course and outcome.

Lingual dystonia syndrome after electrical injury is described; myokimii language after radiation therapy.

Bruxism is another commonly occurring hyperkinesis in the oral area. It is manifested by periodic stereotypical movements of the lower jaw with clenching and characteristic grinding of teeth during sleep.. Bruxism is observed in healthy people (from 6 to 20% of the entire population) and is often combined with such phenomena as periodic movements of the limbs during sleep, sleep apnea, epilepsy, tardive dyskinesia, schizophrenia, mental retardation, post-traumatic stress disorder. An outwardly similar phenomenon during wakefulness is usually described as trismism.

[17], [18]

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 with a convoluted artery, less often with a tumor, even less often with other causes).

Hyperkinesis in facial hemispasm is paroxysmal. Paroxysm is composed of a series of short quick twitches, most noticeable in the circular muscle of the eye, which, overlapping each other, turn into tonic spasm, giving the patient a characteristic facial expression that cannot be confused with anything. At the same time, squinting or squinting of the eye, pulling up the cheeks and the corner of the mouth upwards, sometimes (with pronounced spasm) deviation of the tip of the nose toward the spasm, often a contraction of the chin muscles and platysms are noted. Upon careful examination during paroxysm, large fasciculations and myoclonias with a noticeable tonic component are seen. In the interictal period, microsymptoms of increased muscle tone in the affected half of the face are revealed: a relief and in-depth nasolabial fold, often a slight shortening of the muscles of the lips, nose and chin on the ipsilateral sides of the face. Paradoxically, at the same time, subclinical signs of facial nerve insufficiency on the same side are revealed (a smaller delay in the angle of the mouth with a grin, a symptom of “eyelashes” with an arbitrary closing of the eyes). Paroxysms usually last from a few seconds to 1-3 minutes. During the day there are hundreds of attacks. It is important to note that, unlike other facial hyperkinesis (tics, facial paraspism), patients with facial hemispasm can never demonstrate their hyperkinesis. It is not amenable to volitional control, is not accompanied by corrective gestures and paradoxical kinesies. There is less dependence than in many other forms, the dependence of hyperkinesis on the functional state of the brain. Arbitrary squinting sometimes provokes hyperkinesis. The most significant is the state of emotional stress, leading to an increase in 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 is preserved, but it occurs much less frequently, which is objectified in a night-time polygraphic study.

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

On the side of hemispasm, as a rule, subclinical, but rather obvious persistent (background) symptoms of light nerve VII nerve insufficiency are detected.

Emotional disorders of a predominantly anxious and anxious-depressive nature tend to aggravate 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 need to be carefully examined to rule out symptomatic forms of hemispasm (compression lesions of the facial nerve upon exiting the brain stem). Differential diagnosis of facial hemispasm with another unilateral face hyperkinesis - post-paralytic 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 nevertheless is accompanied by non-robust, clinical signs of a lesion of the facial nerve, as compared with hyperkinesis. This form is characterized by pathological synkinesis in the face typical of postparalitic contractures.

In the debut of facial hemispasm, it may be necessary to differentiate with facial myokinia. This is more often a unilateral syndrome, manifested by small worm-shaped contractions of the muscles of perioral or periorbital localization. Paroxysmalism is not very typical for it, its manifestations are practically independent of the functional state of the brain and the presence of this syndrome always indicates the current organic lesion of the brainstem (most often it is multiple sclerosis or a tumor of the pons).

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

Differential diagnosis in such difficult cases is also recommended with the pathology of the temporomandibular joint, tetanus, partial epilepsy, tonic spasms in multiple sclerosis, hemimastikatora spasm, tetany, facial myokiemii, lip-spasm with hysteria.

Sometimes it is necessary to differentiate with tics or psychogenic (“hysterical” according to the old terminology) hyperkinesis in the face, proceeding according to the type of facial hemispasm. Among other things, it is useful to remember that only those muscles that are innervated by the facial nerve take part in the formation of facial hemispasm.

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

[19], [20], [21]

Facial hyperkinesis combined or occurring against the background of more common hyperkinesis and other neurological syndromes

• Idiopathic tics and Tourette syndrome.
• Generalized drug dyskinesia (1-dopa, antidepressants and other drugs).
• Choreic hyperkinesis in the face (chorea of Huntington, Sydengam, benign hereditary chorea, etc.).
• Facial myokimii (brain stem tumors, multiple sclerosis, etc.).
• Facial crumpy.
• Facial hyperkinesis of epileptic nature.

It is necessary to emphasize once again that in a number of diseases, facial hyperkinesis can only be a stage or a component of a generalized hyperkinetic syndrome of the most diverse origin. So idiopathic tics, Tourette's disease, Huntington's chorea, or Sidegenam's chorea, common crampy, many drug dyskinesias (for example, associated with the treatment of dopascic preparations), etc. At first they can only appear as facial dyskinesias. At the same time, a wide range of diseases is known in which facial hyperkinesis is immediately detected in the picture of the generalized hyperkinetic syndrome (myoclonic, choreic, dystonic or ticotic). Many of these diseases are accompanied by characteristic neurological and / or somatic manifestations, which greatly facilitate the diagnosis.

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

[22], [23]

Hyperkinetic syndromes in the face, not associated with the participation of facial muscles

1. Okohlirny dystonia (dystonic deviation of a look).
2. Syndromes of excessive rhythmic activity in the eye muscles:
   • opsoklonus,
   • "Nystagmus" century
   • bobbing syndrome
   • Dipping syndrome, e) Ping-Pong gaze syndrome,
   • periodic alternating deviation of gaze with dissociated head movements,
   • periodic alternating nystagmus,
   • cyclic oculomotor paralysis with spasms,
   • periodic alternating asymmetric deviation,
   • myocomia syndrome of the superior oblique muscle of the eye,
   • Duane's syndrome.
3. Masticator spasm (trizm). Hemimastikatorny 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 practitioner. (In addition, some of these hyperkinesis are often combined with facial dyskinesia)

Oculogeal dystonia (dystonic gaze deviation) is a characteristic symptom of post-encephalitic parkinsonism and one of their early and characteristic signs of neuroleptic side effects (acute dystonia). Oculogeal crises can be an isolated dystonic phenomenon or be combined with other dystonic syndromes (tongue protrusion, blepharospasm, etc.). Attacks of upward deviation of gaze (less often - downward, even less often - lateral deviation or oblique deviation of gaze) 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: there are different-frequency, different-amplitude and different-vector movements of the eyeballs ("dancing eyes syndrome"). This is a rare syndrome indicating an organic lesion of stem-cerebellar connections of different etiology. Most cases of opsoclonus described in the literature relate 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 century" - a rare phenomenon, manifested by a series of fast, rhythmic, upward jerky movements of the upper eyelid. It is described in many diseases (multiple sclerosis, tumors, craniocerebral injury, Miller Fisher syndrome, alcoholic encephalopathy, etc.) and is caused by eye movements such as convergence or when moving the eye. "Nystagmus" is considered to be a sign of damage to the midbrain tire.

The ocular bobbing is manifested by characteristic vertical eye movements, sometimes referred to as “float movements”: with a frequency of 3-5 per minute, in most cases, bilateral friendly, rapid deflection of the eyeballs are observed with a subsequent return to their original position, but at a slower pace than the downward movement. This ocular "swing" is observed with open eyes and is usually absent if the eyes are closed. At the same time there is bilateral paralysis of the horizontal gaze. The syndrome is characteristic of bilateral damage to the pons (hemorrhage in the bridge, glioma, traumatic damage to the bridge; often observed in the syndrome of "locked man" or comatose state). Atypical bobbing (with intact horizontal eye movements) has been described for obstructive hydrocephalus, metabolic encephalopathy and compression of the cerebellar hematoma.

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

Glaring ping-pong syndrome (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 lesions (heart attacks) with relative intact brain stem.

Periodic alternating deviation of gaze with dissociated movements of the head is a unique rare syndrome of cyclic disturbances of eye movements, combined with contraversive movements of the head. Each cycle includes three phases: 1) friendly deviation of the eyes to the side while simultaneously turning the head in the opposite direction, lasting for 1-2 minutes; 2) a period of “switching” with a duration of 10 to 15 seconds, during which the head and eyes regain their original normal position and 3) friendly deviation of the eyes to the other side with compensatory contralateral face rotation, lasting also 1-2 minutes. Then the cycle is constantly repeated again, stopping only in a dream. During the cycle, gaze paralysis is observed in the direction opposite to the direction of the ocular deviation. In most of the cases described, non-specific involvement of the structures of the posterior cranial fossa is postulated.

Periodic alternating nystagmus can be congenital or acquired and also manifests itself in three phases. In the first phase, repeating for 90-100 seconds are observed. Horizontal tremors of nystagmus, in which the eyes “beat” in one direction; the second phase of 5-10 second "neutrality", during which the nystagmus may be absent or there is a pendulum-like nystagmus or nystagmus down and the third phase, which also lasts 90-100 seconds, during which the eyes "hit" in the opposite direction. If the patient tries to look towards the fast phase, the nystagmus becomes rougher. The syndrome is presumably based on bilateral damage to the paramedian reticular formation at the pontomesencephalic level.

Alternating skew dewiation. Oblique deviation or Hertwig-Magendiesche syndrome (Hertwig-Magendiesche) is manifested by vertical divergence of the eye of supranuclear origin. The degree of divergence may remain constant or depend on the direction of gaze. Syndrome is usually caused by acute lesions in the brainstem. Sometimes this sign can be intermittent and then there is a periodic alternation of the side of the higher eye. The syndrome is associated with bilateral damage at the pretextual level (acute hydrocephalus, tumor, stroke, and multiple sclerosis are the most common causes).

Cyclic oculomotor paralysis (the phenomenon of cyclic oculomotor spasm and relaxation) is a rare syndrome in which the third (oculomotor) nerve is characterized by alternating the phase of its paralysis and the phase of enhancing its functions. This syndrome is congenital or acquired in early childhood (in most, but not all cases). In the first phase, a picture of complete or almost complete paralysis of the oculomotor (III) nerve with ptosis develops. Then within 1 minute it decreases and then another phase develops, in which the upper eyelid contracts (eyelid retraction), the eye converges slightly, the pupil narrows, and accommodation spasm can increase the refraction by several diopters (up to 10 diopters). Cycles are observed at variable intervals of a few minutes. These two phases make up a cycle that periodically repeats both during sleep and in wakefulness. Arbitrary eyes have no effect on them. The alleged cause is aberrant regeneration after injury to the 3rd nerve (birth injury, aneurysm).

The syndrome of superior oblique myokymia is characterized by rapid rotator vibrations of one eyeball with monocular oscillopsy (“objects jump up and down”, “tele-screen flashes”, “eye fluttering”) and torsional diplopia. These sensations are especially unpleasant when reading, watching television, work that requires accurate observation. Reveals hyperactivity of the superior oblique muscle of the eye. The etiology is unknown. Often a good therapeutic effect has carbamazepine.

Duan syndrome is a hereditary weakness of the lateral rectus muscle of the eye with narrowing of the palpebral fissure. The abduction ability of the eye is reduced or absent; coercion and convergence are limited. Bringing the eyeball is accompanied by its retraction and narrowing of the palpebral fissure; during abduction, the palpebral fissure widens. Syndrome often one-sided.

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

Separately worth hemimastikatorny spasm. This is a rare syndrome characterized by a unilateral strong contraction of one or several masseter muscles. Most patients with hemimastikatory spasm have hemiatrophy of the face. The presumed cause of hemismasticatory spasm in facial hemiatrophy is associated with compression neuropathy of the motor portion of the trigeminal nerve due to changes in the deep tissues in facial hemiatrophy. Clinically hemimetomy spasm manifests itself with short twitches (resembling facial hemispasm) or prolonged spasms (from a few seconds to several minutes as with cramping). Cramps are painful; during the spasm, the bite of the tongue, the dislocation of the temporomandibular joint and even the breaking of teeth are described. Involuntary movements are provoked by chewing, talking, closing the mouth and other voluntary movements.

One-sided spasm of chewing muscles is possible in the picture of epileptic seizure, diseases of the temporomandibular joint, tonic spasm in multiple sclerosis and unilateral dystonia of the mandible.

[24], [25], [26],

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

The following forms are distinguished:

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

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

[27], [28]

Laryngospasm, pharyngospasm, esophagospam

As organic causes of these syndromes, dystonia (usually acute dystonic reactions), tetanus, tetany, some muscle diseases (poliomyositis), diseases with local mucous membrane irritation can be mentioned. Manifestations of extrapyramidal (and pyramidal) hypertonus can lead to these syndromes, but usually in the context of more or less generalized disorders of muscle tone.

Myoclonus of the soft palate and myorrhythmia

Cycle palatine myoclonus (soft palate nystagmus, soft palate tremor, myorrhythmia) can be observed either in isolation as rhythmic (2-3 per second) soft palate contractions (sometimes with a characteristic clicking sound), or in combination with a coarse rhythmic myoclonus of the mandible, tongue, larynx, platysma, diaphragm and distal arms. This distribution is very typical for myorrhythmia. 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, a vertical ocular myoclonus (“swing”) synchronous with a bicycle-palatine myoclonus (oculo-palatine myoclonus) can join. Isolated myoclonus of the soft palate can be either idiopathic or symptomatic (tumors of the pons and medulla, encephalomyelitis, craniocerebral injury). It is noticed that the idiopathic miclonus often disappears during sleep (as well as during anesthesia and in a comatose state), while symptomatic myoclonus is more stable in these conditions.

Generalized miorhythmia without involving the soft palate is rare. Its most frequent etiology is considered to be a vascular lesion of the brain stem and cerebellar degeneration associated with alcoholism, other diseases occurring with malabsorption, celiac disease.

[29], [30], [31],

Psychogenic hyperkinesis in the face

1. Spasm of convergence.
2. Spongy spasm.
3. Pseudoblepharospasm.
4. Deviations (including "geotropic") gaze.
5. Other forms.

Psychogenic hyperkinesis is diagnosed by 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).

Criteria for the clinical diagnosis of psychogenic tremor, psychogenic myoclonus, psychogenic dystonia and psychogenic parkinsonism are currently developed. Here we will mention only specific (almost exclusively in conversion disorders) facial hyperkinesis. These include such phenomena as convergence spasm (unlike organic convergence spasm, which is very rare, convergence psychogenic spasm is accompanied by spasm of accommodation with constriction of the pupils), Brissot's tongue spasm (although a dystonic phenomenon completely reproducing this syndrome; despite external identity, they are completely different in their dynamism), pseudo-blepharospasm (a rare syndrome observed in the picture of pronounced others, including mimic, demon active manifestations), various deviations of a look (rolling of eyes, deviation of a look aside, “geotropic deviation of a look when the patient at any change of position of the head tends to look down (" to the earth "); often the direction of a deviation changes in the course of one survey of the patient. Other (“other”) forms of psychogenic facial hyperkinesis, which, as is well known, are distinguished by the extreme diversity of their manifestations.

[32], [33], [34], [35], [36]

Facial stereotypes in mental illness

Stereotypes in mental illness 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, lips, tongue, “schizophrenic smile,” etc.). The syndrome is described as a behavior disorder in schizophrenia, autism, mental retardation and in the picture of the neuroleptic syndrome. In the latter case, it is often combined with other neuroleptic syndromes and is called hard stereotypy. Rarely, stereotypes develop as a complication of therapy with dofas-containing drugs in the treatment of Parkinson's disease.

[37], [38], [39], [40]

Pathological laughter and crying

With known reservations, this well-known clinical phenomenon can be viewed as a variant of the specific “hyperkinesis” or rhythmic activity of certain functionally related muscles.

The following forms are distinguished:

1. Pseudobulbar palsy.
2. Attacks of laughter with hysteria.
3. Pathological laughter in mental illness.
4. Attacks of epileptic laughter.

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

Attacks of laughter with hysteria are now less common. They are not always motivated, or provoked by anxiety or conflict, sometimes “contagious” (even “epidemics” of laughter are described), observed in people with certain personality disorders and cannot be explained by any organic reasons.

Pathological laughter in mental illness often looks like a compulsive phenomenon that occurs without external provocation and fits into the picture of obvious psychotic behavioral disorders that are often seen “with the naked eye” (inadequate and strange behavior)

[41], [42], [43], [44], [45], [46]

Seizures of epileptic nature

Epileptic laughter attacks (helolepsy) are described in the frontal and temporal localization of epileptic foci (with the involvement of the sapplementary, limbic cortex and also some subcortical structures), they can be accompanied by other very diverse automatisms and epileptic discharges on the EEG. The attack begins absolutely suddenly and also suddenly ends. Awareness and memory of an attack can sometimes be intact. The laughter itself looks outwardly normal, or resembles a caricature of laughter and can sometimes alternate with crying, accompanied by sexual arousal. Described helolepsy in combination with premature puberty; there are observations on helolepsy in patients with hypothalamic tumors. Such patients need to be carefully examined to confirm the epileptic nature of laughter attacks and to identify the underlying disease.

The usual dystonic hyperkinesis in the face of the transient character is described as a complication of chicken pox (upward deviation of the gaze, tongue protrusion, muscle spasm opening the mouth with inability to speak). The attacks were repeated for several days, followed by recovery.

Rare forms of hyperkinesis include spasmus nutans (pendulum-like nystagmus, tortikollis and titubation) in children between the ages of 6-12 months. Up to 2-5 years. It refers to benign (transient) disorders.

[47], [48], [49]