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.
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  .
Entire Body System
High palate Multiple mitochondrial DNA deletions Increased serum lactate Progressive muscle weakness Hearing impairment Proximal muscle weakness Flexion contracture Progressive proximal muscle weakness Increased connective tissue Respiratory insufficiency [mendelian.co]
The diplopia remained unchanged and an insidious facial weakness developed. [jnnp.bmj.com]
It is characterized by early onset of symptoms, mild proximal muscle weakness, hyporeflexia or areflexia, normal serum creatine kinase (CK) levels and myopathic electromyography finding, uniform type 1 fibers, and nonprogression. [ncbi.nlm.nih.gov]
Ophthalmoplegia or eye muscle weakness is a condition that is characterized by either paralysis or weakness of the eye muscles. [belmarrahealth.com]
Some affected individuals also have general weakness of the skeletal muscles (myopathy), which may be especially noticeable during exercise. Muscle weakness may also cause difficulty swallowing (dysphagia). [diseaseinfosearch.org]
Acta Medica Croatica 61(4): 395-398, 2007 Tolosa hunt syndrome or painful ophthalmoplegia. Bollettino di Oculistica 59(1-2): 43-54, 1980 Painful ophthalmoplegia the tolosa hunt syndrome. [eurekamag.com]
‘The patient was observed until approximately 20 months later, when he developed new complaints of a 4.5-kg weight loss, bone and joint pain, and ophthalmoplegia.’ [en.oxforddictionaries.com]
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]
There was no associated pain, watering, or redness of eye. Over the next 2 days, patient complained of vision loss in left eye; which was painless and progressive. [annalsofian.org]
- Short Stature
Other findings in the syndrome may include muscle weakness, short stature, hearing loss, and the loss of ability to coordinate voluntary movements (ataxia) due to problems in the part of the brain called the cerebellum. [medicinenet.com]
DDx: Other mitochondrial disorders (Leigh’s disease) and leukodystrophies, the distinguishing feature being involvement of the subcortical white matter in KSS as opposed to the deep white matter in leukodystrophies An 11-year-old girl with short stature [ajnr.org]
Related symptoms: Short stature Muscle weakness Ptosis Flexion contracture High palate SOURCES: OMIM MENDELIAN More info about MYASTHENIC SYNDROME, CONGENITAL, 2A, SLOW-CHANNEL; CMS2A Low match NEMALINE MYOPATHY 10; NEM10 Nemaline myopathy-10 is an autosomal [mendelian.co]
The patient's personal history also included thrombocytopaenia secondary to bone marrow hypoplasia, hypoparathyroidism, short stature, and primary adrenal insufficiency. [elsevier.es]
Other findings may include muscle weakness, short stature, hearing loss, and/or the loss of ability to coordinate voluntary movements (ataxia) due to problems affecting part of the brain (cerebellum). [rarediseases.org]
Some forms of ophthalmoplegia are congenital and fall into the category of Congenital Cranial Dysinnervation Disorders (CCDDs). These disorders arise from a primary defect of cranial nucleus/nerve development or guidance. [tandfonline.com]
The main symptoms are as follows: the heightened tone of the neck muscles, as a rule combined with other indications of Parkinsonism and an inclination to fall down. To varying degree there are also bulbar signs and subcortical dementia. [medigoo.com]
[…] by, last update March 20, 2019 aka Neurological Mind-boggler 008 An elderly lady presented to the emergency department following a fall. Apart from a few minor scrapes she came through the ordeal relatively unscathed. [lifeinthefastlane.com]
Head trauma is a rare cause (46), but increasingly recognized, especially in males: about half are bilateral INO and half unilateral, and most are due to falls, blunt trauma or falls (27, 47). [neuroophthalmology.ca]
Case 3 An 18-month-old male child, a product of non- consanguineous marriage, presented with chief complaints of delayed developmental milestones and frequent falls during walking. [jneuro.com]
- Surgical Procedure
Comparison between reverse INO and typical INO (adapted from  ) Diagnostic procedures A brain MRI may be indicated to further elucidate the etiology. [eyewiki.aao.org]
Surgical procedures can lift drooping eyelids or a patch over one eye can be used to relieve double vision. Because there is no blink response, a surgically lifted eyelid exposes the cornea of the eye so that it may become dry or be scratched. [encyclopedia.com]
Further questioning reveals a 1-day history of coughing, sneezing, and watery eye discharge occurring 1 week earlier that seemingly improved after antihistamine use. [pedsinreview.aappublications.org]
She coughed with suctioning. Her limbs were flaccid. There was finger flexion on the left in response to deep noxious stimuli. Her reflexes were symmetrically brisk. There was no response to plantar stimulation. [stroke.ahajournals.org]
Case Report In the second half of January 2015, a previously healthy 41-year-old male IT specialist developed a viral infection that was manifested by cough and throat pain. [omicsonline.org]
He reported feeling "drowsiness" and blurred vision that progressed to ptosis; he later developed dysphagia and dysarthria. [ncbi.nlm.nih.gov]
Besides ptosis and ophthalmoplegia there were pigmental degenerations of retina, cataracts, cardiac conduction defects, sensoryneural abnormalities, depressed ventilatorydrive, dysphagia and dysphasia. [worldcat.org]
[…] external ophthalmoplegia without diplopia, cerebellar atrophy, proximal skeletal muscle weakness with generalized muscle wasting, profound emaciation, respiratory failure, spinal deformity and facial muscle weakness (manifesting with ptosis, dysphonia, dysphagia [orpha.net]
- Vascular Disease
Vascular disease is implicated in over a third of cases. References Wu YT, Cafiero-Chin M, Marques C. Wall-eyed bilateral internuclear ophthalmoplegia: review of pathogenesis, diagnosis, prognosis and management. Clin Exp Optom 2015; 98 (1):25–30. [ejcrim.com]
Página 249 - Omenn GS, Motulsky AG: A quantitative assessment of plasma homocysteine as a risk factor for vascular disease. [books.google.es]
Genetic and mitochondrial diseases, a history of vascular disease or stroke, or genetic syndromes can vary your outlook drastically. It’s important to discover, address, and treat the cause of your ophthalmoplegia for the best outcome. [healthline.com]
Vol. 53: Vascular diseases. Part I. Vinken P.J., Bruyn G.W., Klawans H.L., Editors, Tool J.F. Co-Editor. Elsevier Science Publishers, pp. 390–393, Amsterdam, 1988. [link.springer.com]
The fibers responsible for facial sweating (except those that travel to the medial forehead) branch off at the superior cervical ganglion and travel with the external carotid artery; therefore, postganglionic lesions affecting the ICA or cavernous sinus [pubs.rsna.org]
[…] limited to the medial forehead, whereas first- and second-order Horner syndrome will cause anhidrosis of the entire face ( 22 ). [pubs.rsna.org]
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]
Congenital fibrosis of the extraocular muscles type 2, an inherited exotropic strabismus fixus, maps to distal 11q13. Am J Hum Genet. 1998 Aug;63(2):517-25. [disorders.eyes.arizona.edu]
The degree of anisocoria, if present, was recorded. Anisocoria, if present, was again measured under dim light conditions to rule out simple (physiological) anisocoria. [ijo.in]
Anisocoria, Physiologic (Anisocoria) Anisometropia Anisometropic Amblyopia (Amblyopia) Anophthalmia (Anophthalmos) Anophthalmos Anterior Ischemic Optic Neuropathy (Optic Neuropathy, Ischemic) Anterior Uveitides (Uveitis, Anterior) Anton Syndrome (Blindness [provisu.ch]
- Visual Impairment
A rating scale of visual function is thus required as visual acuity alone may not reflect levels of visual handicap. Our data show that the majority of CPEO patients are visually impaired. [nature.com]
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. [symptoma.com]
Conclusions : CPEO is a frequent cause of significant visual impairment among patients with confirmed mitochondrial disease and it is invariably associated with the development of debilitating neurological complications. [iovs.arvojournals.org]
According to the World Health Organization about 285 million people over the world are suffering from visual impairment, of which 45 million people are affected by blindness and 246 million have lowered vision. [scienceforum.ru]
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]
The location of a visual pathway lesion will determine the extent of visual loss, with optic nerve disease leading to monocular visual problems, chiasmatic disease leading to bitemporal hemianopsia, and retrochiasmatic disease causing loss of all or part [pubs.rsna.org]
CASE REPORT Two cases of VBD are presented, both with acute bilateral ophthalmoplegia and cranial nerve palsies, and vertebrobasilar arterial thrombosis that resulted in ischemic stroke. [ncbi.nlm.nih.gov]
Certain parts of the cerebral cortex (including the frontal eye fields ), as in stroke. Toxic envenomation by mambas, taipans, and kraits. [en.wikipedia.org]
Common causes are multiple sclerosis in young people (often bilateral) and stroke in the elderly (typically unilateral). [msdmanuals.com]
Other common causes of ophthalmoplegia include: thyroid disease, brain injury, migraines, stroke, infection and brain tumor. [epainassist.com]
Clinical presentation of a 45-year-old African American woman with human immunodeficiency virus who presented to a medical center with complaint of new-onset headache on the right side. [jaoa.org]
A 9 year old boy presented with headache for 1 week and restricted movements and drooping in both eyes for last 3 days. On examination he had bilateral ophthalmoplegia and ptosis. [ncbi.nlm.nih.gov]
Sir, The headache, neurologic deficits, and cerebrospinal fluid (CSF) lymphocytosis (HaNDL) syndrome is characterized by a temporary neurological deficit followed by a severe headache with persistent CSF lymphocytosis. [nature.com]
Headache 1987;27:573-4. 7. Kidron D, Pomeranz S. Malignant pseudotumor cerebri. Report of two cases. J Neurosurg 1989;71:443-5. [Figure 1], [Figure 2], [Figure 3], [Figure 4] [annalsofian.org]
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]
Eighteen months since the attack, he has improved completely with mild asymmetric mydriasis persisting. This may represent first attack of RPON in a child with migraine. [ncbi.nlm.nih.gov]
Typical symptoms are double vision, ptosis and mydriasis. 2 See also Eye muscles [flexikon.doccheck.com]
Figure 1: Mydriasis on both eyes with divergent strabismus a mild upper lid ptosis right. Figure 2: Upward look with mydriasis more on the left eye, restriction of upward movement with manifestation of divergency of eyeballs. [omicsonline.org]
At full internal O. it is noted a mydriasis, lack of its photoharmose and convergence, paralysis of accommodation (see. [bigmed.info]
The diagnosis of ophthalmoplegia is made on the basis of clinical findings as well as pharmacologic testing using topical pilocarpine, which will result in the resolution of mydriasis within 30min. [onlinelibrary.wiley.com]
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]
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 . 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 . 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.
Treatment Treatment Options: No effective treatment is known. [disorders.eyes.arizona.edu]
We review the causes of ophtalmoparesis and the treatment with RTX in GPA. [ncbi.nlm.nih.gov]
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]
With treatment (eg, radiation therapy for a tumor, treatment of multiple sclerosis), improvement may occur but is often limited after infarction. [msdmanuals.com]
Till now no specific treatment has emerged for these patients; for patients with severe ptosis the eyelid surgeryis possible method of choice. [worldcat.org]
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]
Treatment and prognosis (whether the disorder abates or eventually resolves) depends on the cause of impaired horizontal eye movements. [msdmanuals.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]
For symptomatic diplopia, treatment options include fogging one glasses lens, patching one eye, a Fresnel prism or ground-in prism glasses, or strabismus surgery.  Prognosis Little is documented regarding the prognosis of reverse INO, due to it's [eyewiki.aao.org]
Prognosis The prognosis essentially depends on the number of organs involved and, in each of them, on the proportion of the abnormal mtDNA. In the majority of cases, life expectancy can be normal with appropriate support. [orpha.net]
In older people, stroke is a more common etiology. [eyewiki.aao.org]
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]
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]
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]
In this article, potential pathophysiological mechanisms are discussed. [ncbi.nlm.nih.gov]
Pathophysiology Lutz first described a case of INO of abduction in 1923.  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]
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]
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]
Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. [books.google.es]
Corneal Ulcer Causes You can prevent many causes of corneal ulcers. Use the correct protective eyewear when doing any work or play that can lead to eye injury. [aao.org]
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 , 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 , infection or stroke. Intrinsic muscle dysfunction is less common but may be observed in cases of multiple sclerosis , myasthenia gravis, and diabetes mellitus , among others. These are systemic disorders not usually restricted to ocular muscles. Congenital OP may indicate developmental defects  or hereditary disorders such as congenital muscle dystrophy . Additionally, mutations of mitochondrial DNA are commonly associated with myopathy, including but not limited to OP .
- 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.