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Ophthalmoplegia (ophthalmoparesis)

, medical expert
Last reviewed: 04.07.2025
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Each eye is moved (rotated) by six muscles: four rectus and two oblique. Eye movement disorders can be caused by damage at different levels: hemisphere, brainstem, cranial nerves and, finally, muscles. Symptoms of eye movement disorders depend on the location, size, severity and nature of the damage.

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Causes of ophthalmoplegia (ophthalmoparesis)

  1. Myasthenia gravis.
  2. Aneurysms of the vessels of the circle of Willis.
  3. Spontaneous or traumatic carotid-cavernous fistula.
  4. Diabetic ophthalmoplegia.
  5. Dysthyroid ophthalmopathy.
  6. Tolosa-Hant syndrome.
  7. Tumor and pseudotumor of the orbit.
  8. Temporal arteritis.
  9. Ischemia in the brainstem region.
  10. Parasellar tumor.
  11. Metastases to the brain stem.
  12. Meningitis (tuberculous, carcinomatous, fungal, sarcoidosis, etc.).
  13. Multiple sclerosis.
  14. Wernicke's encephalopathy.
  15. Migraine with aura (ophthalmoplegic).
  16. Encephalitis.
  17. Orbital trauma.
  18. Cavernous sinus thrombosis.
  19. Cranial neuropathies and polyneuropathies.
  20. Miller Fisher syndrome.
  21. Pregnancy.
  22. Psychogenic oculomotor disorders.

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Myasthenia gravis

Ptosis and diplopia may be the first clinical sign of myasthenia. At the same time, the characteristic fatigue in response to physical activity in the arms may be absent or remain unnoticed by the patient. The patient may not pay attention to the fact that these symptoms are less pronounced in the morning and increase during the day. By offering the patient a long test of opening and closing the eyes, pathological fatigue can be confirmed. The test with prozerin under EMG control is the most reliable way to detect myasthenia.

Aneurysms of the vessels of the circle of Willis

Congenital aneurysms are localized mainly in the anterior parts of the Willis circle. The most common neurological sign of an aneurysm is unilateral paralysis of the external eye muscles. The third cranial nerve is usually affected. Sometimes the aneurysm is visualized on MRI.

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Spontaneous or traumatic carotid-cavernous fistula

Since all nerves supplying extraocular muscles pass through the cavernous sinus, pathological processes in this localization can lead to paralysis of the external eye muscles with double vision. Of great importance is the fistula between the internal carotid artery and the cavernous sinus. Such a fistula can be the result of a craniocerebral injury. It can also occur spontaneously, probably due to the rupture of a small arteriosclerotic aneurysm. In most cases, the first branch (ophthalmic) of the trigeminal nerve suffers at the same time and the patient complains of pain in the area of its innervation (forehead, eye).

The diagnosis is facilitated if the patient complains of rhythmic noise, synchronous with the work of the heart and decreasing when the carotid artery on the same side is compressed. Angiography confirms the diagnosis.

Diabetic ophthalmoplegia

Diabetic ophthalmoplegia in most cases begins acutely and is manifested by incomplete paralysis of the oculomotor nerve and unilateral pain in the front of the head. An important feature of this neuropathy is the preservation of the vegetative fibers to the pupil and therefore the pupil is not dilated (in contrast to paralysis of the third nerve in aneurysm, in which the vegetative fibers are also affected). As with all diabetic neuropathies, the patient is not necessarily aware of diabetes.

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Dysthyroid ophthalmopathy

Dysthyroid ophthalmopathy (orbitopathy) is characterized by an increase in the volume (edema) of the external eye muscles in the orbit, which manifests itself as ophthalmoparesis and double vision. Ultrasound examination of the orbit helps to recognize the disease, which can manifest itself in both hyper- and hypothyroidism.

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Tolosa-Hunt syndrome (painful ophthalmoplegia)

This eponym denotes a nonspecific granulomatous inflammation in the wall of the cavernous sinus at the bifurcation of the carotid artery, which manifests itself with characteristic periorbital or retroorbital pain, involvement of the third, fourth, sixth cranial nerves and the first branch of the trigeminal nerve, good responsiveness to corticosteroids and the absence of neurological symptoms of involvement of the nervous system beyond the cavernous sinus. Tolosa-Hunt syndrome of painful ophthalmoplegia should be a "diagnosis of exclusion"; it is made only when other possible causes of "steroid-responsive" ophthalmoparesis (space-occupying processes, systemic lupus erythematosus, Crohn's disease) have been excluded.

Pseudotumor of the orbit

The term pseudotumor is used to describe enlarged extraocular muscles (due to inflammation) and sometimes other orbital contents (lacrimal gland, fatty tissue). Orbital pseudotumor is accompanied by conjunctival injection and mild exophthalmos, retro-orbital pain, which can sometimes simulate migraine or cluster headache. Orbital ultrasound or CT reveals enlarged orbital contents, mainly muscles, similar to that seen in dysthyroid ophthalmopathy. Both Tolosa-Hunt syndrome and orbital pseudotumor respond to corticosteroid treatment.

In addition to the above symptoms, an orbital tumor is also accompanied by compression of the second pair and, consequently, a decrease in visual acuity (Bonnet syndrome).

Temporal arteritis

Giant cell (temporal) arteritis is typical for mature and elderly people and affects mainly the branches of the external carotid artery, mainly the temporal artery. High ESR is typical. Polymyalgic syndrome may be observed. Occlusion of the branches of the ophthalmic artery in 25% of patients leads to blindness in one or both eyes. Ischemic neuropathy of the optic nerve may develop. Damage to the arteries that feed the oculomotor nerves can lead to their ischemic damage and the development of ophthalmoplegia. Strokes may occur.

Ischemic lesions of the brainstem

Cerebral circulation disorders in the area of penetrating branches of the basilar artery lead to damage to the nuclei of the III, IV or VI cranial nerves, which is usually accompanied by alternating syndromes with contralateral hemiplegia (hemiparesis) and conductive sensory disorders. There is a picture of acute cerebral catastrophe in a patient of mature or elderly age suffering from vascular disease.

The diagnosis is confirmed by neuroimaging and ultrasound examination.

Parasellar tumor

Tumors of the pituitary-hypothalamic region and craniopharyngiomas are manifested by changes in the sella turcica and visual fields (chiasmal syndrome), as well as specific endocrine disorders characteristic of a particular type of tumor. Cases of tumor growth directly and outward are rare. The syndrome that occurs in this case is characterized by the involvement of the III, IV and VI nerves and dilation of the homolateral pupil as a result of irritation of the plexus of the internal carotid artery. Due to the slow growth of pituitary tumors, an increase in intracranial pressure is not very typical.

Metastases to the brain stem

Metastases to the brainstem, affecting the territory of the nuclei of certain oculomotor nuclei, lead to slowly progressing oculomotor disorders in the picture of alternating syndromes against the background of increased intracranial pressure and neuroimaging signs of a volumetric process. Gaze paralysis is possible. Defects of horizontal gaze are more typical for damage to the pons; disorders of vertical gaze are more common with damage to the mesencephalon or diencephalon.

Meningitis

Any meningitis (tuberculous, carcinomatous, fungal, sarcoid, lymphomatous, etc.), developing mainly on the basal surface of the brain, usually involves the cranial nerves and most often the oculomotor nerves. Many of the listed types of meningitis can often occur without headache. Cytological examination of cerebrospinal fluid (microscopy), the use of CT MRI and radionuclide scanning are important.

Multiple sclerosis

Brainstem lesions in multiple sclerosis often lead to diplopia and oculomotor disorders. Internuclear ophthalmoplegia or damage to individual oculomotor nerves is not uncommon. It is important to identify at least two lesions, confirm a recurrent course, and obtain relevant evoked potential and MRI data.

Wernicke's encephalopathy

Wernicke's encephalopathy is caused by vitamin B12 deficiency in patients with alcoholism due to malabsorption or malnutrition and is manifested by acute or subacute development of brainstem damage: damage to the third nerve, various types of gaze disorders, internuclear ophthalmoplegia, nystagmus, cerebellar ataxia and other symptoms (confusion, memory disorders, polyneuropathy, etc.). The dramatic therapeutic effect of vitamin B1 is characteristic.

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Migraine with aura (ophthalmoplegic)

This form of migraine is extremely rare (according to one headache clinic - 8 cases per 5000 patients with headache) most often in children under 12 years of age. Headache is observed on the side of ophthalmoplegia and usually precedes it by several days. Migraine episodes are noted weekly or less often. Ophthalmoplegia is usually complete, but can also be partial (one or more of the three oculomotor nerves). Patients over 10 years of age require angiography to exclude aneurysm.

Differential diagnosis includes glaucoma, Tolosa-Hunt syndrome, parasellar tumor, pituitary apoplexy. Diabetic neuropathy, Wegener's granulomatosis, and orbital pseudotumor must also be excluded.

Encephalitis

Encephalitis with damage to the oral parts of the brainstem, for example, Bickerstaff encephalitis or other brainstem forms of encephalitis, may be accompanied by ophthalmoparesis against the background of other symptoms of damage to the brainstem.

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Ophthalmic herpes

Ophthalmic herpes accounts for 10 to 15% of all cases of herpes zoster and presents with pain and rash in the area of innervation of the first branch of the trigeminal nerve (often involving the cornea and conjunctiva). Extraocular muscle paralysis, ptosis, and mydriasis often accompany this form, indicating involvement of the third, fourth, and sixth cranial nerves in addition to damage to the Gasserian ganglion.

Orbital trauma

Mechanical damage to the eye socket with hemorrhage into its cavity can lead to various oculomotor disorders due to damage to the corresponding nerves or muscles.

Cavernous sinus thrombosis

Sinus thrombosis is manifested by headache, fever, impaired consciousness, chemosis, exophthalmos, and edema in the eyeball area. Edema is observed in the fundus, and visual acuity may decrease. Involvement of the III, IV, VI cranial nerves and the first branch of the trigeminal nerve is characteristic. After a few days, the process passes through the circular sinus to the opposite cavernous sinus and bilateral symptoms appear. Cerebrospinal fluid is usually normal, despite concomitant meningitis or subdural empyema.

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Cranial neuropathies and polyneuropathies

Cranial neuropathies with paresis of the eyeball muscles are observed in alcoholic syndromes of damage to the nervous system, beriberi, polyneuropathy in hyperthyroidism, idiopathic cranial polyneuropathy, hereditary amyloid polyneuropathy (Finnish type) and other forms.

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Miller Fisher syndrome

Fisher syndrome is characterized by ophthalmoplegia (but no ptosis), cerebellar ataxia (without scanned speech) and areflexia. In addition to these obligatory symptoms, the VII, IX and X nerves are often involved (dysphagia without dysarthria). Rare symptoms: nystagmus, Bell's phenomenon, decreased consciousness, flaccid tetraparesis, pyramidal signs, tremor and some others. Protein-cell dissociation in the cerebrospinal fluid is often detected. The course is characterized by an acute onset with a subsequent "plateau" of symptoms and subsequent recovery. The syndrome is a kind of intermediate form between Bickerstaff encephalitis and Guillain-Barré polyneuropathy.

Pregnancy

Pregnancy is accompanied by an increased risk of developing oculomotor disorders of various origins.

Psychogenic oculomotor disorders

Psychogenic oculomotor disorders are more often manifested by gaze disorders (convergence spasm or "pseudo-abducens", gaze spasms in the form of different types of eye deviation) and are always observed in the context of other characteristic motor (multiple motor disorders), sensory, emotional-personal and vegetative manifestations of polysyndromic hysteria. Positive diagnostics of psychogenic disorders and clinical and paraclinical exclusion of the current organic disease of the nervous system are mandatory.

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