Summary: Paralysis of one or more of the ocular muscles due to disorders of the eye muscles, neuromuscular junction, supporting soft tissue, tendons, or innervation to the muscles.

Top Publications

  1. Crevits L, Verschelde H, Casselman J. Ophthalmoplegic migraine: an unresolved problem. Cephalalgia. 2006;26:1255-9 pubmed
  2. Im M, Kim B, Seo Y, Park J, Lee J. Complete ophthalmoplegia after herpes zoster. Clin Exp Dermatol. 2007;32:162-4 pubmed
    ..We report a case of complete ophthalmoplegia resulting from ophthalmic herpes zoster...
  3. Jindal G, Parmar V, Gupta V. Isolated ptosis as acute ophthalmoplegia without ataxia, positive for anti-GQ1b immunoglobulin G. Pediatr Neurol. 2009;41:451-2 pubmed publisher
    Anti-GQ1b IgG antibody syndrome comprises a wide range of diseases presenting with ophthalmoplegia and ataxia...
  4. Ashker L, Weinstein J, Dias M, Kanev P, Nguyen D, Bonsall D. Arachnoid cyst causing third cranial nerve palsy manifesting as isolated internal ophthalmoplegia and iris cholinergic supersensitivity. J Neuroophthalmol. 2008;28:192-7 pubmed publisher
    ..The initial diagnosis was postganglionic internal ophthalmoplegia attributed to a viral ciliary ganglionopathy...
  5. Giraud P, Valade D, Lanteri Minet M, Donnet A, Geraud G, Guegan Massardier E. Is migraine with cranial nerve palsy an ophthalmoplegic migraine?. J Headache Pain. 2007;8:119-22 pubmed
    ..A biological blood test and an MRI are systematically required to help clinicians in their diagnosis and to exclude alternative aetiology of headache with palsy. ..
  6. Bladen J, Moosajee M, Angunawela R, Roberts C. Transient internal ophthalmoplegia after inferior oblique myectomy. J AAPOS. 2009;13:596-7 pubmed publisher
    Internal ophthalmoplegia causing pupillary dilatation and loss of accommodation following damage to the ciliary ganglion is a rare complication of strabismus surgery...
  7. Ferraris S, Clark S, Garelli E, Davidzon G, Moore S, Kardon R, et al. Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1. Arch Neurol. 2008;65:125-31 pubmed publisher
    ..A 42-year-old man experienced hearing loss, progressive external ophthalmoplegia (PEO), loss of central vision, macrocytic anemia, and hypogonadism...
  8. Asok T, Aziz S, Faisal H, Tan A, Mallika P. Central retinal artery occlusion and ophthalmoplegia following spinal surgery in the prone position. Med J Malaysia. 2009;64:323-4 pubmed
    ..In the immediate postoperative period, he developed right proptosis, periorbital swelling, chemosis and total ophthalmoplegia. The vision in his right eye was only counting fingers and the intraocular pressure was 68 mmHg...
  9. Sanjay S, Chan E, Gopal L, Hegde S, Chang B. Complete unilateral ophthalmoplegia in herpes zoster ophthalmicus. J Neuroophthalmol. 2009;29:325-37 pubmed publisher
    ..between 1968 and 2008, herpes zoster ophthalmicus (HZO) may rarely be associated with complete unilateral ophthalmoplegia, defined here as impaired ocular ductions in all 4 directions within 3 months of onset of manifestations of ..

More Information


  1. Laugel V, This Bernd V, Cormier Daire V, Speeg Schatz C, de Saint Martin A, Fischbach M. Early-onset ophthalmoplegia in Leigh-like syndrome due to NDUFV1 mutations. Pediatr Neurol. 2007;36:54-7 pubmed
    ..biochemical, and molecular data of a 7-year-old male who presented at the age of 7 months with progressive ophthalmoplegia and later developed cerebellar ataxia, spasticity, and dystonia...
  2. Posada I, Gallardo M, Domínguez C, Rivera H, Cabello A, Arenas J, et al. [Mitochondrial DNA depletion and POLG mutations in a patient with sensory ataxia, dysarthria and ophthalmoplegia]. Med Clin (Barc). 2010;135:452-5 pubmed publisher
    ..The majority of disorders characterized by multiple mtDNA deletions present with progressive external ophthalmoplegia, though this feature is not usually found in syndromes caused by mtDNA depletion...
  3. Tajsharghi H, Hilton Jones D, Raheem O, Saukkonen A, Oldfors A, Udd B. Human disease caused by loss of fast IIa myosin heavy chain due to recessive MYH2 mutations. Brain. 2010;133:1451-9 pubmed publisher
    ..The relatively mild phenotype is interesting in relation to the more severe phenotypes generally seen in relation to recessive null mutations in sarcomeric proteins...
  4. Delengocky T, Bui C. Complete ophthalmoplegia with pupillary involvement as an initial clinical presentation of herpes zoster ophthalmicus. J Am Osteopath Assoc. 2008;108:615-21 pubmed
    ..The present case may be the first to identify a patient with complete ophthalmoplegia with pupil involvement as a pre-eruptive manifestation of herpes zoster...
  5. Bharucha D, Campbell T, Valencia I, Hardison H, Kothare S. MRI findings in pediatric ophthalmoplegic migraine: a case report and literature review. Pediatr Neurol. 2007;37:59-63 pubmed
    ..recognized as a cranial neuralgia) is a form of migraine characterized by recurrent episodes of headache with ophthalmoplegia related to paresis of cranial nerves III, IV, or VI, with onset typically in childhood...
  6. Zhang X, Peng J, Tang Z, Xu C, Zhou X, Gong S, et al. Mutation p.Arg954Trp of KIF21A causes congenital fibrosis of the extraocular muscles in a Chinese family. Yi Chuan Xue Bao. 2006;33:685-91 pubmed
    ..Our results indicate that mutation p.Arg954Trp of the KIF21A is the genetic basis of the Chinese family with CFEOM1. ..
  7. Vasconcelos L, Stancioli F, Leal J, da Silva A, Gomez R, Teixeira A. Ophthalmoplegic migraine: a case with recurrent palsy of the abducens nerve. Headache. 2008;48:961-4 pubmed publisher
    ..In this article, we report a patient with OM that presented recurrent palsy of the abducens nerve and other atypical features. Case reports of OM with abducens nerve palsy were also reviewed. ..
  8. De Benedetti G, Sepe M, Verallo O, Caselli M. Complete traumatic unilateral ophthalmoplegia with intact far vision and complete recovery after 2 months-case report. Orbit. 2008;27:187-9 pubmed publisher
  9. Dinges W, Witherspoon S, Itani K, Garg A, Peterson D. Blepharoptosis and external ophthalmoplegia associated with long-term antiretroviral therapy. Clin Infect Dis. 2008;47:845-52 pubmed publisher
    ..without prior ART association, is usually caused by age-associated involutional ptosis, but it is also seen in mitochondrial myopathies with external ophthalmoplegia, cardiac conduction disturbances, and neurological impairments.
  10. Obuchowska I, Mariak Z. [Internuclear ophthalmoplegia--causes, symptoms and management]. Klin Oczna. 2009;111:165-7 pubmed
    Internuclear ophthalmoplegia (INO) is a disorder of eye movements caused by a lesion in an area of the brain called the medial longitudinal fasciculus (MLF). The most common causes of INO are multiple sclerosis and brainstem infarction...
  11. Ward M, Faris C, Upile T, Patel N. Ophthalmoplegia secondary to raised intracranial pressure after unilateral neck dissection with internal jugular vein sacrifice. Head Neck. 2011;33:587-90 pubmed publisher
    ..Head and neck surgeons must be aware of this and have a low threshold for investigation of the signs and symptoms of intracranial hypertension in the postoperative period. © 2010 Wiley Periodicals, Inc. Head Neck, 2011. ..
  12. Acaroglu G, Akinci A, Zilelioglu O. Retinopathy in patients with diabetic ophthalmoplegia. Ophthalmologica. 2008;222:225-8 pubmed publisher
    ..028 and p = 0.018, respectively). Presence and level of diabetic retinopathy are significantly lower in diabetics with cranial nerve palsy than in the age-, sex- and disease-duration-matched controls. ..
  13. Khan A, Khalil D, Al Tassan N. Congential fibrosis of the extraocular muscles type I (CFEOM1) on the Arabian Peninsula. Ophthalmic Genet. 2008;29:25-8 pubmed publisher
    ..To assess for KIF21A mutation in the first two reported Saudi Arabian families with the classic phenotype of congenital fibrosis of the extraocular muscles type I (CFEOM1)...
  14. McMillan H, Keene D, Jacob P, Humphreys P. Ophthalmoplegic migraine: inflammatory neuropathy with secondary migraine?. Can J Neurol Sci. 2007;34:349-55 pubmed
    ..A detailed understanding of the natural history of OM is essential for the clinical. This review provides support that OM may result from cranial nerve inflammation with headache a secondary and later feature of this condition. ..
  15. Nieri A, Bazan R, Almeida L, Rocha F, Raffin C, Bigal M, et al. Bilateral painful idiopathic ophthalmoplegia: a case report. Headache. 2007;47:848-51 pubmed
    Around 3% of the individuals with painful ophthalmoplegia have bilateral complaints. In the vast majority of these cases, appropriate investigation demonstrates a secondary etiology, and we are not aware of idiopathic cases reported...
  16. Weiss M, Saneto R. Sensory ataxic neuropathy with dysarthria and ophthalmoparesis (SANDO) in late life due to compound heterozygous POLG mutations. Muscle Nerve. 2010;41:882-5 pubmed publisher
    ..cause a number of phenotypically heterogeneous mitochondrial diseases, most commonly progressive external ophthalmoplegia, and are characterized by the accumulation of multiple, large-scale deletions of mitochondrial DNA...
  17. Wang H, Shih Y, Chen C, Chao H, Lee M, Hsueh Y. Valosin-containing protein and neurofibromin interact to regulate dendritic spine density. J Clin Invest. 2011;121:4820-37 pubmed publisher
    ..The data presented here demonstrate that there is a link between IBMPFD and NF1 and indicate a role for VCP in synapse formation...
  18. Weihl C. Another VCP interactor: NF is enough. J Clin Invest. 2011;121:4627-30 pubmed publisher
    ..Thus, aberrant interactions between VCP and NF1 may explain the dementia phenotype and cognitive delay observed in patients with IBMPFD and neurofibromatosis type 1...
  19. Forer B, Hui N, Sethi D. Unilateral ophthalmoplegia secondary to anterior clinoid process mucocele. J Neuroophthalmol. 2010;30:321-4 pubmed publisher
    A 50-year-old Chinese man presented with rapidly progressive unilateral ophthalmoplegia and then an ipsilateral afferent pupil defect...
  20. Lal V, Sahota P, Singh P, Gupta A, Prabhakar S. Ophthalmoplegia with migraine in adults: is it ophthalmoplegic migraine?. Headache. 2009;49:838-50 pubmed publisher
    ..We report 62 adults, seen consecutively, who developed acute ophthalmoplegia with severe attacks of migraine over a 10-year (1996-2005) period...
  21. Koc Z, Koc F, Yerdelen D, Ozdogu H. Rhino-orbital-cerebral mucormycosis with different cerebral involvements: infarct, hemorrhage, and ophthalmoplegia. Int J Neurosci. 2007;117:1677-90 pubmed
    ..Headache, blurred vision, fever, painful ophthalmoplegia, and cranial nerve involvement were among the clinical findings...
  22. Greco D, Gambina F, Maggio F. Ophthalmoplegia in diabetes mellitus: a retrospective study. Acta Diabetol. 2009;46:23-6 pubmed publisher
    b>Ophthalmoplegia, despite being a rare entity in diabetes mellitus, is associated with great anxiety for the patients and often appears to be a serious problem from a diagnostic and therapeutic point of view...
  23. García Santana S, Rodriguez J, Serrano L, Hedges T. Esthesioneuroblastoma presenting with complete external ophthalmoplegia. Semin Ophthalmol. 2009;24:245-6 pubmed publisher
    ..Neuroimaging revealed a mass involving the medial wall of the left cavernous sinus, without orbital involvement. Biopsy of the mass provided a pathologic diagnosis of esthesioneuroblastoma...
  24. Fraunfelder F, Richards A. Diplopia, blepharoptosis, and ophthalmoplegia and 3-hydroxy-3-methyl-glutaryl-CoA reductase inhibitor use. Ophthalmology. 2008;115:2282-5 pubmed publisher
    To report the association between 3-hydroxy-3-methyl-glutaryl-CoA (HMG-CoA) reductase inhibitors (statins) and diplopia, blepharoptosis (ptosis), and ophthalmoplegia.
  25. Darin N, Kyllerman M, Wahlstrom J, Martinsson T, Oldfors A. Autosomal dominant myopathy with congenital joint contractures, ophthalmoplegia, and rimmed vacuoles. Ann Neurol. 1998;44:242-8 pubmed
    ..clinical features were congenital joint contractures, which normalized during early childhood, external ophthalmoplegia, and proximal muscle weakness. Muscle atrophy was most prominent in the pectoralis and quadriceps muscles...
  26. Traboulsi E, Engle E. Mutations in KIF21A are responsible for CFEOM1 worldwide. Ophthalmic Genet. 2004;25:237-9 pubmed
  27. Kohno T, Oohira A, Hori S. Near reflex substituting for acquired horizontal gaze palsy: a case report. Jpn J Ophthalmol. 2004;48:584-6 pubmed
    ..The existence of convergence substituting for adduction in this patient with horizontal gaze palsy was confirmed by refraction change in addition to pupillary change. ..
  28. Beals R, Weleber R. Distal arthrogryposis 5: a dominant syndrome of peripheral contractures and ophthalmoplegia. Am J Med Genet A. 2004;131:67-70 pubmed
    ..All affected members had limitations of ocular motility and some had ptosis. Restrictive lung disease is a feature in most affected patients in this family. It is possible that this syndrome may be due to a muscle abnormality...
  29. Takeda S, Yamada M, Kawasaki K, Oyanagi K, Ikuta F, Arai M, et al. Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction. Acta Neuropathol. 1994;88:193-200 pubmed
    ..Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS. ..
  30. Lessell S, Collins T. Ophthalmoplegia in Powassan encephalitis. Neurology. 2003;60:1726-7 pubmed
  31. Kim J, Hwang J. Adduction on attempted abduction: the opposite of synergistic divergence. Arch Ophthalmol. 2006;124:918-20 pubmed
  32. Yamada K, Chan W, Andrews C, Bosley T, Sener E, Zwaan J, et al. Identification of KIF21A mutations as a rare cause of congenital fibrosis of the extraocular muscles type 3 (CFEOM3). Invest Ophthalmol Vis Sci. 2004;45:2218-23 pubmed
    ..Each represents a specific form of paralytic strabismus characterized by congenital restrictive ophthalmoplegia, often with accompanying ptosis...
  33. Yamada K, Andrews C, Chan W, McKeown C, Magli A, de Berardinis T, et al. Heterozygous mutations of the kinesin KIF21A in congenital fibrosis of the extraocular muscles type 1 (CFEOM1). Nat Genet. 2003;35:318-21 pubmed
    ..We identified six different mutations in 44 of 45 probands. The primary mutational hotspots are in the stalk domain, highlighting an important new role for KIF21A and its stalk in the formation of the oculomotor axis. ..
  34. Demer J, Clark R, Engle E. Magnetic resonance imaging evidence for widespread orbital dysinnervation in congenital fibrosis of extraocular muscles due to mutations in KIF21A. Invest Ophthalmol Vis Sci. 2005;46:530-9 pubmed
  35. Voit T, Parano E, Straub V, Schroder J, Schaper J, Pavone P, et al. Congenital muscular dystrophy with adducted thumbs, ptosis, external ophthalmoplegia, mental retardation and cerebellar hypoplasia: a novel form of CMD. Neuromuscul Disord. 2002;12:623-30 pubmed
    ..In addition, both showed ptosis, external ophthalmoplegia, mild mental retardation, and mild cerebellar hypoplasia on MRI...
  36. Venkatesh C, Pillai V, Raghunath A, Prakash V, Vathsala R, Pericak Vance M, et al. Clinical phenotype and linkage analysis of the congenital fibrosis of the extraocular muscles in an Indian family. Mol Vis. 2002;8:294-7 pubmed
    ..The present data showed a classic CFEOM phenotype in an Indian family. The family's phenotype is consistent with linkage to CFEOM1 locus on chromosome 12p11.2-q12. ..
  37. Hanisch F, Bau V, Zierz S. Congenital fibrosis of extraocular muscles type 1 with progression of ophthalmoplegia. Eur J Med Res. 2005;10:366-8 pubmed
    ..However, regarding the underlying known molecular basis of CFEOM1, a continuous disease progression due to the kinesin dysfunction cannot be excluded. ..
  38. Lance J, Zagami A. Ophthalmoplegic migraine: a recurrent demyelinating neuropathy?. Cephalalgia. 2001;21:84-9 pubmed
    ..Recurrent painful ophthalmoplegia started in infancy in two cases, childhood in two instances and adult life in one...
  39. Engle E, Kunkel L, Specht L, Beggs A. Mapping a gene for congenital fibrosis of the extraocular muscles to the centromeric region of chromosome 12. Nat Genet. 1994;7:69-73 pubmed
    ..dominant, ocular disorder characterized by congenital, nonprogressive, bilateral ptosis and external ophthalmoplegia. The pathophysiology of this disorder is unknown and it is unclear if it has a primary neurogenic or ..
  40. Payne M, Yang Z, Katz B, Warner J, Weight C, Zhao Y, et al. Dominant optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia: a syndrome caused by a missense mutation in OPA1. Am J Ophthalmol. 2004;138:749-55 pubmed
    ..family segregating a dominantly inherited syndrome of optic atrophy, sensorineural hearing loss, ptosis, and ophthalmoplegia. Observational case series...
  41. Stommel E, Ward T, Harris R. MRI findings in a case of ophthalmoplegic migraine. Headache. 1993;33:234-7 pubmed
    ..There is little published information on MRI of cranial nerves in patients with migraine headaches with ophthalmoplegia. We present a case of ophthalmoplegic migraine with a cranial nerve abnormality which was subsequently shown ..
  42. Lamantea E, Tiranti V, Bordoni A, Toscano A, Bono F, Servidei S, et al. Mutations of mitochondrial DNA polymerase gammaA are a frequent cause of autosomal dominant or recessive progressive external ophthalmoplegia. Ann Neurol. 2002;52:211-9 pubmed
    One form of familial progressive external ophthalmoplegia with multiple mitochondrial DNA deletions recently has been associated with mutations in POLG1, the gene encoding pol gammaA, the catalytic subunit of mitochondrial DNA polymerase...
  43. Jungbluth H, Zhou H, Hartley L, Halliger Keller B, Messina S, Longman C, et al. Minicore myopathy with ophthalmoplegia caused by mutations in the ryanodine receptor type 1 gene. Neurology. 2005;65:1930-5 pubmed
    ..External ophthalmoplegia is an additional finding in a subset of patients with MmD...
  44. Doherty E, Macy M, Wang S, Dykeman C, Melanson M, Engle E. CFEOM3: a new extraocular congenital fibrosis syndrome that maps to 16q24.2-q24.3. Invest Ophthalmol Vis Sci. 1999;40:1687-94 pubmed
    ..To define the clinical characteristics and determine the gene localization for a previously undescribed form of congenital fibrosis of the extraocular muscles (CFEOM), referred to as CFEOM type 3 (CFEOM3)...
  45. van der Dussen D, Bloem B, Liauw L, Ferrari M. Ophthalmoplegic migraine: migrainous or inflammatory?. Cephalalgia. 2004;24:312-5 pubmed
  46. Bosley T, Oystreck D, Robertson R, al Awad A, Abu Amero K, Engle E. Neurological features of congenital fibrosis of the extraocular muscles type 2 with mutations in PHOX2A. Brain. 2006;129:2363-74 pubmed
    ..Clinical presentation, neuroimaging and Phox2a-/- animal models all support the concept that CFEOM2 is a primary neurogenic abnormality with secondary myopathic changes. ..
  47. Shin D, Kim J, Kang S. Ophthalmoplegic migraine with reversible thalamic ischemia shown by brain SPECT. Headache. 2002;42:132-5 pubmed
    ..In both patients, SPECT during an attack of ophthalmoplegia and headache demonstrated significantly decreased regional cerebral blood flow (rCBF) in the thalamus on the ..
  48. Filosto M, Mancuso M, Nishigaki Y, Pancrudo J, Harati Y, Gooch C, et al. Clinical and genetic heterogeneity in progressive external ophthalmoplegia due to mutations in polymerase gamma. Arch Neurol. 2003;60:1279-84 pubmed
    The mendelian forms of progressive external ophthalmoplegia (PEO) associated with multiple mitochondrial DNA deletions are clinically heterogeneous disorders transmitted as dominant or recessive traits...
  49. Krasnianski M, Eger K, Neudecker S, Jakubiczka S, Zierz S. Atypical phenotypes in patients with facioscapulohumeral muscular dystrophy 4q35 deletion. Arch Neurol. 2003;60:1421-5 pubmed
    ..Recent studies have shown that this deletion is found in patients with other phenotypes in addition to those with the classic Landouzy-Dejerine FSHD phenotype...
  50. Martinsson T, Darin N, Kyllerman M, Oldfors A, Hallberg B, Wahlstrom J. Dominant hereditary inclusion-body myopathy gene (IBM3) maps to chromosome region 17p13.1. Am J Hum Genet. 1999;64:1420-6 pubmed
    ..an autosomal dominant inclusion-body myopathy characterized by congenital joint contractures, external ophthalmoplegia, and predominantly proximal muscle weakness...
  51. Ali M, Venkatesh C, Ragunath A, Kumar A. Mutation analysis of the KIF21A gene in an Indian family with CFEOM1: implication of CpG methylation for most frequent mutations. Ophthalmic Genet. 2004;25:247-55 pubmed
    ..Previous work has demonstrated that this CpG dinucleotide is a mutational hotspot in the KIF21A gene, and our finding suggests that its high mutability may result, in part, from its methylated state. ..
  52. Halfon M, Bonardo P, Valiensi S, Zaffaroni M, Fernandez Pardal M, Ribero Ayerza D, et al. Central retinal artery occlusion and ophthalmoplegia following spinal surgery. Br J Ophthalmol. 2004;88:1350-2 pubmed
  53. Siraj C, Krishnan J, Nair R, Girija A. Invasive aspergillosis producing painful ophthalmoplegia. J Assoc Physicians India. 2005;53:901-2 pubmed
    Painful ophthalmoplegia is caused by the lesions of orbital apex and anterior cavernous sinus. Cavernous sinus syndrome can be produced by intracranial invasive aspergillosis...