Forced head posture and hanging head syndrome

The head is persistently turned or tilted to one side or the other. The list of diseases presented is not complete. It does not analyze head posture disorders in patients in a coma or in a serious condition due to extensive damage to the cerebral hemispheres and (or) the brain stem.

I. The main reasons for the forced position of the head:

1. Unilateral paralysis of the trochlear nerve (IV nerve, n. trochlearis).
2. Unilateral paralysis of the abducens nerve (VI nerve, n. abducens).
3. Complete homonymous hemianopsia.
4. Horizontal gaze paralysis.
5. Eyes shifting 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 in 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 sternocleidomastoid muscle, etc.).
12. Grisel syndrome.
13. Meningeal syndrome.
14. Head tremor.
15. Nystagmus.
16. Sandifer syndrome.
17. Benign torticollis in infants.
18. Progressive supranuclear palsy.
19. Psychogenic torticollis.
20. Combination (mix) of psychogenic and organic hyperkinesis in the neck muscles.
21. Periodic alternating gaze deviation with secondary dissociated head turns.

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

1. Tick-borne encephalitis.
2. Amyotrophic lateral sclerosis.
3. Polymyositis.
4. Dermatomyositis.
5. Myasthenia gravis.
6. Myopathy.
7. Carnitine deficiency.
8. Endocrine myopathy.
9. HFDP.
10. Parkinsonism.
11. Bechterew's disease.
12. Hypokalemia.

I. Forced head position

Unilateral paralysis of the trochlear nerve (IV cranial nerve, n. trochlearis).

The vertical divergence of the eyes that occurs with trochlear nerve palsy can be difficult to detect. Often patients cannot clearly describe the double vision when looking downward, for example when going down stairs. Most, however, keep their head turned or tilted toward the unaffected (healthy) side to compensate for the impaired function of the superior oblique muscle. If the head and gaze are held straight, a slight upward deflection of the affected eye can be noticed, which increases with abduction because in this position the superior oblique muscle must move the eye downward. The vertical divergence of the eyes becomes most obvious when the head is tilted toward the affected side because in this position the action of the superior rectus muscle is not fully balanced by the superior oblique muscle - Bielschowsky's sign.

Unilateral paralysis of the abducens nerve (VI cranial nerve, n. abducens).

Many, but not all, patients with abducens nerve palsy attempt to avoid double vision by turning the head to the affected side, compensating for the paralyzed external abduction of the eye. In the initial position (looking forward), convergent strabismus may be observed, which increases with eye movement to the affected side, as well as double vision. Isolated VIth nerve palsy in adults has been 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 damage to the abducens (VI) cranial nerve. A period of 6 months has been proposed as a criterion for chronic course. 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 its stationary or progressive course. Secondary paresis of the VI nerve includes known causes (for example, after myelography or after lumbar puncture, with increased intracranial pressure, against the background of craniocerebral trauma, tumor and other brain diseases).

In children and adults, isolated chronic VI nerve palsies often have different origins. In children, VI nerve palsy may be the first manifestation of a tumor in about 30% of cases. In most of these children, other neurological signs develop within a few weeks.

In adults with isolated VI nerve palsy and corresponding diplopia, diabetes mellitus or arterial hypertension are often detected during examination. In these diseases, VI nerve palsy usually has a benign course and is largely subject to regression within 3 months. However, even if VI nerve palsy persists for more than 3 months without significant recovery in a diabetic patient, some alternative cause of this VI nerve palsy must be considered.

It is also necessary to remember that there are syndromes of "pseudo-abducens paralysis" or "pseudo-abducens" syndromes: dysthyroid orbitopathy, convergence spasm can create the illusion of bilateral paralysis of the abducens nerve, congenital Down syndrome, myasthenia and other causes.

Complete homonymous hemianopsia.

Damage to the visual pathways after their partial crossing at the chiasm (usually of vascular or tumor origin) leads to complete homonymous hemianopsia. Patients are "blind" in the visual field contralateral to the affected side.

Some of them instinctively compensate for the absence of one of the visual fields by turning the head to the "blind" side. There is no head tilt. Eye movements are not impaired unless hemianopsia is accompanied by horizontal gaze palsy or contralateral neglect. In both cases, the patient is unable or at least reluctant to move the eyes toward the hemianoptic field. Sometimes it is very difficult to differentiate gaze palsy from neglect syndrome. Hemianopsia is detected using the so-called confrontation method. The patient is asked to look at the examiner, who holds his arms outstretched to both sides at the level of his head. The patient should see the examiner's fingers moving - on one or the other hand, or simultaneously on both sides.

Horizontal gaze paralysis.

Damage to the frontal lobe of the brain or the brainstem can lead to paralysis of horizontal gaze. As a rule, the intact cerebral oculomotor centers "push" the gaze to the contralateral side. If the hemispheres are damaged, the eyes will be deviated to the unparalyzed limbs (the patient "looks at the lesion"). Damage to the conduction pathways in the brainstem causes the eyes to deviate to the affected side (the patient "looks at the paralysis"). In contrast to hemianopsia, patients do not compensate for the gaze paralysis by turning their heads to the paralyzed side, i.e. away from the injury. Quite often, not only the eyes, but also the head are turned to the affected side. Hemispheric gaze palsy is usually transient, brainstem - also, but lasts longer.

Ocular tilt.

A rare condition involving ipsilateral lateral head tilt, consensual eye rotation to the same side, and slow downward deviation of the ipsilateral eye (one eye is lower than the other). The syndrome indicates ipsilateral damage to the brainstem at the level of the midbrain tegmentum. Rarely, the syndrome occurs with damage to the vestibule of the cochlea, part of the peripheral vestibular organ (labyrinth) that is involved in postural control. The eye deviation response may be tonic (persistent) or phasic.

Causes: vestibular nerve injury, barotrauma, lateral brainstem stroke (Wallenberg-Zakharchenko syndrome), lateral medullary compression, pontomedullary ischemia and mesodiencephalic lesions.

Tumor of the posterior cranial fossa.

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

Accessory nerve palsy.

Both sternocleidomastoid muscles and the upper part of the trapezius muscles are innervated by the accessory nerve (XI cranial nerve). Since the sternocleidomastoid muscles turn the head in the opposite direction, paralysis of one of them interferes with the physiological balance between them. The result is a head position with a slight rotation toward the side of the paralyzed muscle and a raised chin in the same direction; the shoulder on the affected side is slightly lowered.

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

Retroflexion of the head in ocular forms of neuromuscular diseases.

Any form of ocular myopathy that weakens the lid and/or eyelid lift results in compensatory head retroflexion. Several diagnoses are considered in this case. Myasthenia gravis is characterized by weakness in performing repetitive movements, which is relieved by subcutaneous or other administration of cholinesterase inhibitors. Dysthyroid orbitopathy is not always diagnosed based on pathological changes in laboratory data. Quite often, neuroimaging of the orbits reveals characteristic changes in the extraocular muscles, which allows an accurate diagnosis to be made. In some cases, there are variants of muscular dystrophy, in others, the weakness of the eye muscles is neurogenic, which is combined with disorders in other parts of the central and peripheral nervous system ("ophthalmoplegia plus" or Kearns-Sayre syndrome; a variant of mitochondrial cytopathy).

Spasmodic torticollis.

Spasmodic torticollis (torticollis, retrocollis, anterocollis, laterocollis, "torticollis without torticollis") is not always accompanied by a hyperkinetic component, which significantly facilitates the diagnosis. There are purely tonic forms ("locked head", "driven-in head").

The diagnosis is proven by the presence in the anamnesis of such phenomena as corrective gestures, paradoxical kinesia, variability of torticollis in different phases of the daily cycle, in the lying position, under alcohol load, the phenomenon of rotation inversion, 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 muscle-tonic reflex manifestations of osteochondrosis, spondylosis and other age-related changes in the cervical spine). There is a pain syndrome, muscle tension in the neck, neurological (motor, reflex and sensory) and neuroimaging signs of spinal disease. Unlike spasmodic torticollis, there is no dynamism of symptoms characteristic of dystonia.

Myogenic torticollis.

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

Grisel syndrome.

Grisel syndrome develops with an inflammatory process in the area of the atlanto-epistropheal joint (torticollis atlanto-epistrophealis), often in girls with asthenic body type. Painful torticollis occurs against the background of tonsillectomy, angina, inflammation of the paranasal sinuses. Torticollis develops due to relaxation of the joint capsule or rupture of the transverse ligament.

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

Meningeal syndrome.

Expressed meningeal syndrome sometimes manifests itself by retroflexion of the head and even changes in the posture of the whole body. Symptoms of irritation of the membranes (Kernig, Brudzinsky, etc.) and cerebrospinal fluid syndrome are characteristic.

Causes: subarachnoid hemorrhage, meningitis, cerebral edema and others.

Head tremor, nystagmus.

Forced head position is sometimes formed as a compensatory voluntary reaction in case of head tremor (especially in case of asymmetrical head oscillations with a large displacement to one side - tremor with a rotatory component), in some forms of nystagmus (spasmus nutans). The patient voluntarily changes the position of the head in order to use his vision without interference.

Sandifer syndrome.

In cases of esophageal hernia and gastroesophageal reflux, children sometimes develop "dystonic" postures (a few cases have been described with torticollis alone). Children adopt the most incredible postures (twisting their bodies, throwing their heads back, etc.) so that food passes from the esophagus to the stomach without delay. Esophagoscopy confirms the diagnosis. Children with this disorder are often mistakenly considered to have a primary neurological disorder.

Benign torticollis in infants.

The disease manifests itself in attacks of torticollis lasting from several minutes to several hours, which usually develop in the first year of life and spontaneously cease at 2-5 years of age. Some of these children later develop migraine, for which a genetic predisposition is usually revealed in these families.

Psychogenic torticollis.

Clinical features of psychogenic dystonia, including psychogenic torticollis: sudden (often emotiogenic) onset with rest dystonia; often fixed muscle spasm without the dynamism that is characteristic of organic dystonia (no corrective gestures, paradoxical kinesias, nocturnal (morning) sleep effect; clinical manifestations do not depend on body position). These patients often show selective insufficiency of the affected muscles (patients refuse to perform certain actions, citing the impossibility of performing them, and at the same time easily perform other actions involving the same muscles when distracted); such patients are characterized by the presence of other paroxysmal conditions, as well as multiple movement disorders (in the status at the time of examination or in the anamnesis) in the form of pseudoparesis, pseudostuttering, pseudoseizures, etc. Patients often respond to placebo. Psychogenic dystonia is usually accompanied by pain in the affected area during passive movements. Patients are characterized by multiple somatization (multiple somatic complaints in the absence of objectively identified visceral pathology).

Isolated (monosymptomatic) psychogenic torticollis is currently extremely rare.

Combination of psychogenic and organic hyperkinesis.

A combination of psychogenic and organic hyperkinesis (mixed) in the same patient is also possible (including in the neck muscles). For these diagnostically very difficult 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 syndrome, unique in its clinical manifestations, which cannot be confused with other neurological syndromes.

II. Drooping head syndrome

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

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 meningeal syndrome and atrophic paralysis in the muscles of the neck, shoulder girdle and proximal parts of the arms. A "drooping", powerlessly falling head is one of the most characteristic signs of the acute period of tick-borne encephalitis. Sometimes the bulbar muscles are involved. Symptoms of pyramidal tract involvement may be detected on the legs.

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

Amyotrophic lateral sclerosis.

Lateral amyotrophic sclerosis affects mainly the extensor muscles and, when it rarely begins in the neck muscles, the patient begins to experience difficulties in holding the head in the usual vertical position; eventually, he begins to support the head by resting the chin on his hand or fist. Characteristic atrophies with fasciculations and EMG signs of damage to the anterior horns are revealed, including in clinically intact muscles. Symptoms of damage to the upper motor neuron ("atrophy with hyperreflexia") are detected early, and there is a steadily progressive course with a violation of bulbar functions.

Polymyositis and dermatomyositis.

Polymyositis and dermatomyositis often result in a "drooping head" pattern, which is accompanied by myalgia syndrome, muscle tightness, proximal muscle weakness, increased blood CPK levels, characteristic changes in EMG (fibrillation potentials, positive waves, decreased motor unit action potential duration) and in muscle biopsy.

Myasthenia gravis.

Myasthenia may present with weakness of the neck extensor muscles, which is restored in response to the administration of anticholinesterase drugs; the diagnosis is confirmed by EMG and a proserin test.

Myopathies.

Some forms of myopathy present with persistent "drooping head" syndrome. As a rule, other muscles of the trunk and limbs are also involved. Myopathy of unknown etiology with isolated weakness of the neck extensor muscles and pronounced drooping head ("head on the chest") has been described.

Other reasons.

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

In various forms of Parkinsonism and Bechterew's disease, there is a flexion of not only the head, but also the spine (the "flexor" pose, the "supplicant" pose) against the background of other neurological (Parkinsonism) and radiological (Bechterew's disease) manifestations of these diseases.

A relatively acute development of the hanging head syndrome has also been described in severe hypokalemia caused by diarrhea associated with chemotherapy.

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