Ophthalmoplegia refers to abnormal eye movements and possibly disturbed adaptation and accommodation resulting from paralysis of the extraocular muscles involved in these processes. Ophthalmoplegia may indicate cranial nerve palsy, but may also be related to a variety of other diseases.
Presentation
Any combination of abnormal eye movements may be presented:
- Oculomotor nerve palsy is generally associated with downwards and outwards positioning of the ipsilateral eye. Because this cranial nerve also innervates the pupillary sphincter and levator palpebrae superioris, this form of OP is accompanied by mydriasis and ptosis.
- Isolated trochlear nerve palsy is related to preponderance of inferior oblique muscle function, resulting in upwards movements of the ipsilateral eye while gazing downwards and upon adduction.
- Functional impairment of the abducens nerve results in lateral rectus muscle paralysis and consequently disturbed outwards movements of the affected eye.
- Multiple nerve palsies give rise to more complex anomalies corresponding to the actions of maintained eye muscle function.
OP patients often claim visual impairment, mainly diplopia. The degree of diplopia is dependent on the current direction of gaze. Patients may "learn" to compensate for OP by adopting abnormal head positions. This condition is commonly referred to as ocular torticollis [8] [9].
Eyes
- Strabismus
In all cases, a rectus muscle snapped under minimal tension while held on a strabismus hook during strabismus surgery. [ncbi.nlm.nih.gov]
[…] movements, V pattern deviation Eye movements, X pattern deviation Ocular paresis Paresis of extraocular muscles Restrictive strabismus Strabismus Strabismus (eye condition Strabismus (eye condition) Strabismus, A pattern deviation Strabismus, V pattern [icd9data.com]
- Esotropia
Ophthalmologic examination is significant for left esotropia and mild bilateral hypertropia. Extraocular movements are painless, with incomplete abduction of both eyes, worse on the left. [pedsinreview.aappublications.org]
The clinical arguments are unconvincing, since mild VI nerve palsies may not create a primary position esotropia, and dissociated adduction nystagmus can be associated with VI nerve palsies (60) as the result of central adaptation to paresis or limitation [neuroophthalmology.ca]
[…] patient presented with vertical diplopia, which is commonly accompanied by compensatory contra lateral head tilt, while abducens (VI) nerve palsy was considered if the ophthalmologist indicated that the patient had binocular horizontal diplopia and esotropia [bmcophthalmol.biomedcentral.com]
Face, Head & Neck
- Myopathic Facies
We report a 2-year-old boy who presented with congenital hypotonia, breathing and feeding difficulty, myopathic facies, proximal muscle weakness, ptosis, total external ophthalmoplegia and delayed motor developmental milestones. [ncbi.nlm.nih.gov]
Neurologic
- Dizziness
He denies recent trauma, dizziness, weakness, headaches, nausea, vomiting, diarrhea, fever, and travel. The patient denies recent ingestion of alcohol, illicit drugs, medications, or toxins. [pedsinreview.aappublications.org]
If you experience blurred vision, double vision, “floaters” in your vision, a sudden escalating headache, or dizziness, contact your doctor. Ophthalmoplegia is most often a symptom of another syndrome or disease. [healthline.com]
- Vertigo
To prevent permanent vestibuloauditory damage, symptoms of hearing loss, tinnitus, or vertigo require urgent referral to an otolaryngologist. Sometimes topical corticosteroids Keratitis may resolve with treatment of the underlying condition. [msdmanuals.com]
Vertigo and facial nerve palsy were associated in three cases and one case, respectively. [ci.nii.ac.jp]
Notably, the absence of concomitant neurological signs, such as vertigo, ataxia, sensory symptoms, dysarthria, facial palsy, or pyramidal tract dysfunction, has been shown to be significantly correlated with a more rapid recovery [1, 5]. [webeye.ophth.uiowa.edu]
Medication consumption decreased from 39% at 8 weeks to 88% after 8 weeks. https://healthscrutiny.com/vertigo-and-dizziness-program-review/ [pigglesbear.co.uk]
- Spastic Quadriplegia
She had bilateral upper motor neuron facial weakness, dysarthria, and a spastic quadriplegia, yet she could move all limbs on command. Her reflexes were brisk and there were bilateral extensor plantar responses. [stroke.ahajournals.org]
- Receptive Aphasia
Aboulia parietal lobe: Receptive aphasia Hemispatial neglect Gerstmann syndrome Astereognosis occipital lobe: Bálint's syndrome Cortical blindness Pure alexia temporal lobe: Cortical deafness Prosopagnosia Thalamus Thalamic syndrome Other Subclavian [en.wikipedia.org]
- Scanning Speech
Diagnosis[edit] Can be seen in multiple sclerosis, stroke, and other pathologies.[4] Accompanying symptoms include scanning speech, intention tremor, incontinence, and nystagmus. [en.wikipedia.org]
Workup
OP is diagnosed clinically and has to be associated with an underlying disorder. Demographic parameters, symptom onset and disease progression, the presence of unilateral or bilateral complaints and concomitant pathologies should be considered when establishing a list of differential diagnoses. With regards to cranial nerve palsies, extensive knowledge regarding the course of cranial nerves coordinating eye muscle contractions is required to deduce the site of injury from clinical symptoms [1]. Brain imaging may be indicated to visualize lesions of the central nervous system and in this context, magnetic resonance imaging is generally the most sensitive technique. However, computed tomography is usually preferred for an evaluation of orbital tissues [10]. Images obtained by means of computed tomography may, for instance, depict spindle-shaped, enlarged extraocular muscles, as is characteristic of Graves disease. Other entities may be associated with abnormally thin, atrophic ocular musculature. Laboratory analyses of blood samples are usually carried out to assess the patient's general condition and to reveal the underlying pathology. Cerebrospinal fluid specimens may be obtained for similar reasons.
Serum
- Hypercapnia
Equipment and dive profile analysis led to the conclusion of hypercapnia and arterial gas embolism as the probable causes of the diver's symptoms. This is a unique case of isolated bilateral ophthalmoplegia presenting in a diving injury. [ncbi.nlm.nih.gov]
Treatment
Treatment [ edit ] Treatment and prognosis depend on the underlying condition. For example, in thiamine deficiency, treatment would be the immediate administration of vitamin B1. See also [ edit ] Paresis References [ edit ] External links [ edit ] [en.wikipedia.org]
We review the causes of ophtalmoparesis and the treatment with RTX in GPA. [ncbi.nlm.nih.gov]
Treatment Treatment Options: No effective treatment is known. [disorders.eyes.arizona.edu]
With treatment (eg, radiation therapy for a tumor, treatment of multiple sclerosis), improvement may occur but is often limited after infarction. [msdmanuals.com]
Treatment: Treatment and prognosis depend on the underlying causes. Treatment during the symptomatic interval is directed at alleviating symptoms. For more appropriate treatment options consult your ophthalmologist. [medigoo.com]
Prognosis
Prognosis & Life Expectancy of Ophthalmoplegia Patients with isolated ophthalmoplegia will have a normal life expectancy with a good overall prognosis. The prognosis of ophthalmoplegia also depends on the underlying cause of this condition. [epainassist.com]
The patient also had upper and lower respiratory tract involvement and kidney manifestations with good prognosis. We review the causes of ophtalmoparesis and the treatment with RTX in GPA. [ncbi.nlm.nih.gov]
Treatment and prognosis (whether the disorder abates or eventually resolves) depends on the cause of impaired horizontal eye movements. [msdmanuals.com]
Treatment [ edit ] Treatment and prognosis depend on the underlying condition. For example, in thiamine deficiency, treatment would be the immediate administration of vitamin B1. See also [ edit ] Paresis References [ edit ] External links [ edit ] [en.wikipedia.org]
For symptomatic diplopia, treatment options include fogging one glasses lens, patching one eye, a Fresnel prism or ground-in prism glasses, or strabismus surgery. [13] Prognosis Little is documented regarding the prognosis of reverse INO, due to it's [eyewiki.aao.org]
Etiology
Highly variable and depend on the etiology, but the lesion will be affecting the medial midbrain or pons of the brainstem 3,5,8. Treatment and prognosis Highly variable and depend on the etiology 2. Promoted articles (advertising) [radiopaedia.org]
In older people, stroke is a more common etiology. [eyewiki.aao.org]
Our case demonstrates the importance of considering isolated neurosarcoidosis as a potential underlying etiology of painful ophthalmoplegia, even without systemic manifestation of the disease. [ncbi.nlm.nih.gov]
At age 3 days she suffered left-sided posterior intracerebral and subdural hemorrhages of unknown etiology. [journals.lww.com]
Epidemiology
April 2014 Volume 55, Issue 13 Free ARVO Annual Meeting Abstract | April 2014 A national epidemiological study of chronic progressive external ophthalmoplegia in the United Kingdom - molecular genetic features and neurological burden Author Affiliations [iovs.arvojournals.org]
Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis Dec. 1997; 176 Suppl 2:92-8. Burina A, Sinanović O, Smajlović D, Vidović M. Bilateral oculomotor nerve palsy in Guillain-Barre syndrome. Med Arh. 2008; 62(2):119-20. Lo YL. [journals.sbmu.ac.ir]
Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol 1993;50:78-80. 3. Radhakrishnan K, Thacker AK, Bohlaga NH, Maloo JC, Gerryo SE. Epidemiology of idiopathic intracranial hypertension: A prospective and case - control study. [annalsofian.org]
CONGENITAL & HEREDITARY OPHTHALMOPLEGIAS Oculopharyngeal Muscular Dystrophy 5 ● Polyadenylate-binding protein, Nuclear, 1 (PABPN1; PABN1; PABP2) ; Chromosome 14q11.2; Dominant or Recessive ● Second locus in non-French-Canadian (Italian & Norman) families Epidemiology [neuromuscular.wustl.edu]
Pathophysiology
In this article, potential pathophysiological mechanisms are discussed. [ncbi.nlm.nih.gov]
Internuclear ophthalmoplegia: pathophysiology and diagnosis. Bailliéres Clin Neurol. 1992 Aug;1(2):455-70. 3. Liu, GT, et al. Neuro-Ophthalmology Diagnosis and Management. W.B. Saunders Company; 2001. 4. Keane, JR. [reviewofoptometry.com]
Pathophysiology Lutz first described a case of INO of abduction in 1923. [2] Although his hypothesis that supranuclear fibers in the pons divide to innervate the ipsilateral medial rectus and the contralateral lateral rectus is now known to be untrue, [eyewiki.aao.org]
John Leigh for discussions regarding the pathophysiologic mechanism for the ophthalmoplegia. Disclosures None. Footnotes Correspondence to Dr Matthew J. [stroke.ahajournals.org]
Prevention
We also review the related literature and highlight the mechanism and preventive measures. [ncbi.nlm.nih.gov]
Preventing viral outbreaks Some forms of viral keratitis can't be completely eliminated. [mayoclinic.org]
One example is the proptosis (bulging eyes) caused by Grave’s disease, which can also prevent normal eye movement. Rarely, ophthalmoplegia can also be brought on by a deficiency in vitamin B-1, also known as thiamine. [eyehealthweb.com]
Prevention There is no way to prevent ophthalmoplegia. Resources ORGANIZATIONS American Academy of Neurology. 1080 Montreal Ave., St. Paul, MN 55116. (612) 695-1940. 〈 http://www.aan.com 〉. [encyclopedia.com]
Summary
Ophthalmoplegia (OP) generally refers to the presence of eye movement alterations that are caused by paralysis of the extraocular musculature. Because cranial nerve palsies account for considerable shares of OP cases [1], extraocular muscles shall be enumerated according to their respective innervation:
- The oculomotor nerve (III) accounts for the coordination of superior and inferior rectus muscles, medial rectus muscles, and inferior oblique muscles.
- The trochlear nerve (IV) is required for superior oblique muscle function.
- The lateral rectus muscles are innervated by the abducens nerve (VI).
In the broader sense, OP may also describe clinical symptoms arising from internal muscle paralysis. In detail, pupillary sphincter and dilator and/or the ciliary muscle may be affected. These muscles mediate narrowing and widening of the pupil, a means of adaptation to light intensity, and changes of lens shape, thus adapting the eye to short range focus. The pupillary sphincter and ciliary muscle are innervated by the oculomotor nerve.
Cranial nerve palsies may be caused by space-occupying lesions such as a cerebral aneurysm or brain tumors or may be triggered by an increased intracranial pressure, inflammation [2], infection or stroke. Intrinsic muscle dysfunction is less common but may be observed in cases of multiple sclerosis [3], myasthenia gravis, and diabetes mellitus [4], among others. These are systemic disorders not usually restricted to ocular muscles. Congenital OP may indicate developmental defects [5] or hereditary disorders such as congenital muscle dystrophy [6]. Additionally, mutations of mitochondrial DNA are commonly associated with myopathy, including but not limited to OP [7].
References
- Stalcup ST, Tuan AS, Hesselink JR. Intracranial causes of ophthalmoplegia: the visual reflex pathways. Radiographics. 2013;33(5):E153-169.
- Zurawski J, Akhondi H. Tolosa-Hunt syndrome-a rare cause of headache and ophthalmoplegia. Lancet. 2013;382(9895):912.
- Hassen GW, Bhardwaj N. Images in clinical medicine. Bilateral internuclear ophthalmoplegia in multiple sclerosis. N Engl J Med. 2013;368(3):e3.
- Greco D, Gambina F, Maggio F. Ophthalmoplegia in diabetes mellitus: a retrospective study. Acta Diabetol. 2009;46(1):23-26.
- Krasny A, Lutz S, Gramsch C, Diepenbruck S, Schlamann M. Accessory eye muscle in a young boy with external ophthalmoplegia. Clin Anat. 2011;24(8):948-949.
- Voit T, Parano E, Straub V, et al. Congenital muscular dystrophy with adducted thumbs, ptosis, external ophthalmoplegia, mental retardation and cerebellar hypoplasia: a novel form of CMD. Neuromuscul Disord. 2002;12(7-8):623-630.
- McClelland C, Manousakis G, Lee MS. Progressive External Ophthalmoplegia. Curr Neurol Neurosci Rep. 2016;16(6):53.
- Mitchell PR. Ocular torticollis. Trans Am Ophthalmol Soc. 1999; 97:697-769.
- Boricean ID, Barar A. Understanding ocular torticollis in children. Oftalmologia. 2011;55(1):10-26.
- LeBedis CA, Sakai O. Nontraumatic orbital conditions: diagnosis with CT and MR imaging in the emergent setting. Radiographics. 2008;28(6):1741-1753.