Forced head position and "hanging head" syndrome

The head is steadily turned or tilted to one side or the other. The presented list of diseases is not complete. Here, violations of the head pose are not analyzed in patients in a coma or a serious condition due to extensive damage to the cerebral hemispheres and (or) the brain stem.

I. The main causes of the forced position of the head:

1. Unilateral paralysis of the nerve block (IV nerve, n. Trochlearis).
2. One-sided paralysis of the nerve (VI nerve, n. Abducens).
3. Complete homonymous hemianopsia.
4. Paralysis of the horizontal gaze.
5. Evasion of the eyes to the side.
6. Tumor of the posterior cranial fossa.
7. Paralysis of the accessory nerve (XI nerve, n. Accessorius).
8. Retroflexion of the head with ocular myopathy.
9. Spasmodic torticollis.
10. Vertebrogenic torticollis (including in the picture of neurological complications of cervical osteochondrosis).
11. Myogenic torticollis (myofascial pain syndrome, tumors, injuries, congenital retractions of the nodding muscle, etc.).
12. Grisel syndrome.
13. Meningeal syndrome.
14. Tremor of the head.
15. Nystagmus.
16. Sandifer's syndrome.
17. Benign corticollis in infants.
18. Progressive supranuclear palsy.
19. Psychogenic torticollis.
20. Combination (mix) of psychogenic and organic hyperkinesis in the muscles of the neck.
21. Periodic alternating gaze deviation with secondary dissociated head turns.

II. The main causes of the syndrome of the "drooping head":

1. Tick-borne encephalitis.
2. Amyotrophic lateral sclerosis.
3. Poliomyositis.
4. Dermatomyositis.
5. Myasthenia gravis.
6. Myopathy.
7. Insufficiency of carnitine.
8. Endocrine myopathy.
9. HVDP.
10. Parkinsonism.
11. Bechterew's disease.
12. Hypokalemia.

I. Forced head position

Unilateral paralysis of the nerve block (IV cranial nerve, n. Trochlearis).

It can be difficult to identify the vertical discrepancy of the eyes that occurs when the nerve block is paralyzed. Often patients can not clearly describe double vision when looking down, for example, when they go down the stairs. Most of them, however, keep their head turned or deflected to the unaffected (healthy) side to compensate for the impaired function of the superior oblique muscle. If the head and gaze are held straight, you can notice an easy removal of the affected eye to the top, which is intensified when the eye is withdrawn, because in this position the upper oblique muscle should move the eye downward. Vertical discrepancy of the eyes becomes most obvious when the head deviates to the affected side, since in this position the action of the upper rectus muscle is not balanced completely by the upper oblique muscle - the Bielschowski symptom.

One-sided paralysis of the abducens nerve (VI cranial nerve, n. Abducens).

Many, but not all patients with nerve palsy, try to avoid doubling by turning their head to the affected side, compensating for the paralyzed external eye-out. In the starting position (when looking ahead), there can be a converging strabismus, which is amplified by moving the eyes to the affected side, as well as doubling. Isolated paresis of the nerve VI in adults is described in diabetes mellitus, aneurysms, sarcoidosis, metastases, pituitary adenoma, giant cell arteritis, multiple sclerosis, syphilis, meningioma, glioma, trauma and other lesions.

A rather difficult problem is the syndrome of isolated chronic defeat of the abductor (VI) of the cranial nerve. As a criterion for chronic course, a period of 6 months is suggested. The causes of chronic paresis of the VI nerve are conventionally divided into primary and secondary. Primary paralysis has no obvious cause. It is extremely important to pay attention to their steady or progressive course. Secondary paresis of the nerve VI includes known causes (eg, after myelogoraphy or after lumbar puncture, with increased intracranial pressure, against a background of brain injury, a tumor and other brain diseases).

In children and adults, isolated chronic paralysis of the sixth nerve often have a different origin. In children, paresis of the sixth nerve in about 30% of cases may be the first manifestation of a tumor. Most of these children develop other neurological signs within a few weeks.

In adults with isolated paralysis of the VI nerve and the corresponding diplopia, diabetes often shows up in the examination, or arterial hypertension. In these diseases, the paralysis of the sixth nerve usually has a benign course and is largely reversed within 3 months. However, even if a patient with diabetes has a nerve palsy that persists for more than 3 months without significant recovery, it is necessary to consider any alternative cause of this nerve palsy.

It must also be remembered that there are syndromes of "pseudo-abrasence" or "pseudo-abutence" syndromes: a distideoid orbitopathy, a spasm of convergence may give rise to the illusion of bilateral paralysis of the abducent nerve, congenital Down syndrome, myasthenia gravis and other causes.

Complete homonymous hemianopsia.

Damage to the visual pathways after their partial intersection in chiasm (often vascular or tumor origin) leads to a complete homonymous hemianopsia. Patients are "blind" in the field of vision, the contralateral side of the lesion.

Some of them instinctively compensate for the absence of one of the fields of vision by turning the head to the "blind" side. There is no inclination of the head. Eye movements are not violated if hemianopsia is not accompanied by a horizontal eye paralysis or contralateral inattention (neglect). In both cases, the patient is unable or at least reluctant enough to move his eyes towards the hemiaptic field. Sometimes it is very difficult to differentiate the paralysis of the eyes from the inattention syndrome. The hemianopsia is revealed through the so-called confrontation method. The patient is asked to look at the examiner, who holds his arms outstretched in both directions at the level of his head. The patient should see the movements of the examiner's fingers - on one or the other hand, or simultaneously from both sides.

Paralysis of the horizontal gaze.

The defeat of the frontal lobe of the brain or brainstem can lead to paralysis of the horizontal gaze. As a rule, at the same time, the preserved cerebral oculomotor centers "push" the gaze toward the contralateral side. If the hemispheres are damaged, the eyes will be rejected to the non-paralyzed limbs (the patient "looks at the hearth"). Damage of the conductive pathways in the brainstem is observed deviation of the eyes to the affected side (the patient "looks at the paralysis"). In contrast to hemianopsia, patients do not compensate for the paralysis of the eye by turning the head toward the paralyzed side, that is, away from damage. On the contrary, often enough, not only the eyes, but also the head are turned to the affected side. The hemispheric paralysis of the gaze is usually transient, the sternal paralysis is also, but persists for a longer time.

Eye evasion (Ocular tilt).

A rare condition involving the ipsilateral lateral inclination of the head, a friendly turn of the eyes in the same direction and a slow deviation with the ipsilateral eye withdrawn downwards (one eye is located below the other). The syndrome points to ipsilateral brainstem damage at the level of the midbrain. In rare cases, the syndrome occurs when the antechamber of the cochlea, part of the peripheral vestibular organ (labyrinth), which participates in the regulation of body posture, is damaged. The reaction of eye evasion can be tonic (persistent) and phasic.

Causes: damage to the vestibular nerve, barotrauma, lateral stroke of the trunk (Wallenberg-Zakharchenko syndrome), lateral medullary compression, pantomedullar ischemia and mesodiensephalic lesions.

Tumor of the posterior cranial fossa.

In the case of a tumor in the region of the posterior cranial fossa, a forced position of the head may be observed in the form of an easy tilt or rotation toward the lesion, which is not accompanied by obvious oculomotor disturbances or any visual field defects. In the old literature, the phenomenon was called the "vestibular inclination". Headache, neck stiffness and edema of the optic nerve disk are sufficient for the diagnosis, which is easily confirmed by neuroimaging.

Paralysis of the accessory nerve.

Both the nipple muscles and the upper part of the trapezius muscles are innervated by the additional nerve (XI cranial nerve). As the nodding muscles turn their heads in the opposite direction, the paralysis of one of them interferes with the physiological balance between them. The result is the position of the head with a slight turn toward the paralyzed muscle and an elevated chin in the same direction; while the shoulder on the side of the lesion is slightly omitted.

Causes: Isolated paresis of XI pair is observed with low (subnuclear) injuries in the neck region and occurs as a complication of surgical intervention in the internal jugular vein, after carotid endarteriectomy, trauma in the neck and shoulder, radiation therapy.

Retroflexion of the head in the eye forms of neuromuscular diseases.

Any form of ocular myopathy, in which the lifting of the eyelids and / or eyes is weakened, lead to compensatory retroflection of the head. In this case, several diagnoses are considered. Myasthenia gravis is characterized by weakness in performing repeated movements, which decreases with subcutaneous or other injection of cholinesterase inhibitors. Distiroidal orbitopathy is not always diagnosed by pathological changes in laboratory data. Quite often neuroimaging imaging of orbits reveals characteristic changes in extraocular muscles, which makes it possible to establish an accurate diagnosis. In some cases, there are variants of muscular dystrophy, in others - the weakness of the muscles of the eye is neurogenic, which is combined with disorders in other parts of the central and peripheral nervous system ("ophthalmoplegia plus" or Kirns-Seir syndrome, a variant of mitochondrial cytopathy).

Spasmodic torticollis.

Spastic torticollis (torticollis, retrocollis, antero-collis, laterocollis, "torticollum without torticollis") is not always accompanied by a hyperkinetic component, which greatly facilitates the diagnosis. There are purely tonic forms ("locked head", "nailed head").

The diagnosis is proved when there are such phenomena as corrective gestures, paradoxical kinesias, variability of torticollis in different phases of the diurnal cycle, lying down, with alcohol load, rotation inversion phenomenon, dystonic syndromes in other parts of the body.

Vertebrogenic torticollis.

This form of torticollis develops due to mechanical limitation of mobility of the cervical spine (Bechterew's disease, other spondylitis and spondylopathy, including compression radiculopathy and muscular-tonic reflex manifestations of osteochondrosis, spondylosis and other age-related changes in the cervical spine). There is pain syndrome, muscular tension in the neck, neurological (motor, reflex and sensitive) and neurovisualizing signs of spine disease. In contrast to spasmodic torticollis, there is no characteristic for the dystonia of dynamism of symptoms.

Myogenic torticollis.

Myogenic torticollis is characteristic of congenital retractions of the nodal and other neck muscles, traumatic, tumor, inflammatory and other diseases of individual neck muscles.

Griselle syndrome.

Grisel's syndrome (Grisel) develops in the inflammatory process in the area of the atlanto-epistropheal articulation (torticollis atlanto-epistrophealis), often in girls with asthenic physique. There is a painful torticollis against tonsillectomy, tonsillitis, inflammation of the paranasal sinuses. The torticollis develops as a result of relaxation of the joint capsule or rupture of the transverse ligament.

The diagnosis is mainly provided by X-ray examination of the craniovertebral area.

Meningeal syndrome.

A pronounced meningeal syndrome is sometimes manifested by retroflexion of the head and even a change in the whole body posture. Characteristic symptoms of shell irritation (Kernig, Brudzinsky, etc.) and liquor syndrome.

Causes: subarachnoid hemorrhage, meningitis, edema of the brain and others.

Tremor of the head, nystagmus.

The forced position of the head is sometimes formed as a compensatory, arbitrary reaction in the head tremor (especially with asymmetric head oscillations with a large displacement in one direction - a tremor with a rotator component), with some forms of nystagmus (spasmus nutans). The patient arbitrarily changes the position of the head so that he can use his vision without hindrance.

Sandifer's syndrome.

With a hernia of the esophageal opening of the diaphragm and gastroesophageal reflux, children sometimes develop "dystonic" postures (several cases have been described only with corticollis). Children take the most incredible poses (twisting the body, tilting the head, etc.) so that food without delay passes from the esophagus to the stomach. Esophagoscopy confirms the diagnosis. Often children with this disorder are mistakenly treated as patients with a primary neurological disease.

Benign corticollis in infants.

The disease is manifested by torticollis attacks lasting from several minutes to several hours, which usually develop in the first year of life and spontaneously terminate at 2-5 years of life. Some of these children develop a migraine, which usually reveals a genetic predisposition in these families.

Psychogenic torticollis.

Clinical features of psychogenic dystonia, including psychogenic torticollis: a sudden (often emotionally) beginning with a dystonia of rest; often a fixed spasm of muscles without the dynamism that is characteristic of organic dystonia (there are no corrective gestures, paradoxical kinesia, night sleep (morning) effect, clinical manifestations do not depend on the position of the body). These patients often reveal selective failure of the affected muscles (patients refuse to perform some actions, referring to their inability to perform, and at the same time easily perform other actions involving the same muscles with distraction of attention, for such patients is characterized by the presence of other paroxysmal conditions , as well as multiple motor disorders (in the status at the time of the examination or in the anamnesis) in the form of pseudo-cuts, pseudo-hypoxia, pseudo-seizures, etc. Patients often respond to a placebo. Sihogennaya dystonia, usually accompanied by pain in the affected area with passive movements. Patients differ multiple somatization (the set of physical symptoms in the absence of visceral pathologies detected objectively).

Isolated (monosymptomatic) psychogenic torticollis is now extremely rare.

The combination of psychogenic and organic hyperkinesis.

It is also possible to combine psychogenic and organic hyperkinesis (mixed) in one and the same patient (including in the neck muscles). For these, diagnostically very simple cases, the most characteristic features of the clinical picture of such a combination of syndromes are formulated in the literature.

Periodic alternating gaze deviation with secondary dissociated head turns.

This is a rare and unique syndrome in its clinical manifestations, which can not be confused with other neurological syndromes.

II. Hanging Head Syndrome

In neurological literature, the syndrome of the "hanging head" is often identified as an independent symptom complex, in which the weakness of the extensor muscles of the neck with a characteristic "lowered" head (floppy head syndrome, dropped head syndrome) is the leading clinical manifestation.

Main reasons:

Tick-borne encephalitis.

In tick-borne encephalitis, the disease begins with general infectious symptoms (fever, general weakness, headache, inflammatory changes in the blood), followed by the addition of meningeal syndrome and atrophic paralysis in the muscles of the neck, shoulder girdle and proximal parts of the hands. The "hanging", impotently falling head is one of the most characteristic signs of the acute period of tick-borne encephalitis. Sometimes bulbar musculature is involved. Symptoms of pyramid pathways may be detected on the legs.

Differential diagnosis in an acute period is carried out with epidemic meningitis, acute poliomyelitis. The diagnosis takes into account such factors as tick bite, epidemic situation, serological studies.

Amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis affects predominantly the extensor muscles and, with its rare debulking from the neck muscles, the patient begins to have difficulty keeping the head in the usual upright position; in the end he begins to support his head, leaning his chin on his wrist or fist. Characteristic atrophies with fasciculations and EMG signs of lesion of the anterior horns, including in clinically preserved muscles, are revealed. Early symptoms of the defeat of the upper motoneuron ("atrophy with hyperreflexia") are early revealed, there is a steadily progressing course with disruption of bulbar functions.

Poliomyositis and dermatomyositis.

Polymyositis and dermatomyositis often lead to a picture of a "drooping head", which is accompanied by myalgic syndrome, muscle tightening, proximal muscle weakness, elevated blood plasma levels, characteristic changes in EMG (fibrillation potentials, positive waves, reduction in the duration of action potentials of motor units) and in biopsyte of muscle tissue.

Myasthenia gravis.

Myasthenia gravis may manifest weakness of the extensor muscles of the neck, which is restored in response to the introduction of anticholinesterase drugs; The diagnosis is confirmed by EMG-study and a probe with proserin.

Myopathy.

Some forms of myopathy are manifested by persistent syndrome of the "hanging head". As a rule, other muscles of the trunk and extremities are involved. A myopathy of unknown etiology with isolated weakness of the extensor muscles of the neck and a pronounced hanging head ("head on chest") is described.

Other reasons.

Severe forms of chronic inflammatory demyelinating polyneuropathy (CVD) can also (rarely) lead to paralysis of the neck muscles, which is observed in the picture of generalized motor defect and severe myelinopathy in EMG studies.

With different forms of Parkinsonism and Bekhterev's disease, the flexion not only of the head but also of the spine (the posture of the "flexors", the posture of the "petitioner") is observed against the background of other neurological (parkinsonism) and radiographic (Bekhterev's disease) manifestations of these diseases.

Relatively sharp development of the syndrome of the hanging head is also described in case of severe hypokalemia due to diarrhea in connection with chemotherapy.

Among other reasons for the syndrome of the hanging head, which are described in the literature, other forms of polyneuropathy, hyperparathyroidism, partial forms of cataplexy are rarely mentioned.