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Optic neuropathies

INTRODUCTION

The optic nerve carries the greater than one million axons that derive from the retinal ganglion cells and project to eight visual nuclei. Actually an extension or tract of the central nervous system, not a true peripheral nerve, the conventional designation of the optic nerve applies to that portion of this tract that extends from the eye to the optic chiasm.

This topic will review functional anatomy of the optic nerve and the differential diagnosis of optic nerve pathologies. Specific disorders are discussed in detail separately. (See "Optic neuritis: Pathophysiology, clinical features, and diagnosis" and "Nonarteritic anterior ischemic optic neuropathy: Clinical features and diagnosis".)

ANATOMY

The optic nerve has four anatomic segments: the intraocular, intraorbital, intracanalicular, and intracranial sections [1,2].

The intraocular portion, or the optic disc, consists of the unmyelinated retinal ganglion cell axons and astrocytes. As the nerve exits the globe, crossing the collagenous lamina cribrosa, it increases in diameter as it becomes myelinated by oligodendrocytes.

The intraorbital segment of the optic nerve is approximately 25 to 30 mm. Within the orbital apex, the optic nerve is surrounded by dura, arachnoid, and pia mater. The subarachnoid space contains cerebrospinal fluid continuous with that of the brain and spinal cord. The dural covering fuses with the sclera around the optic nerve and more posteriorly with the periosteum of the optic canal. The nerve courses through the annulus of Zinn, which contains the connective tissue origins of the superior, medial, lateral, and inferior rectus muscles.

                          

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Literature review current through: Oct 2014. | This topic last updated: Oct 14, 2014.
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References
Top
  1. Glaser JS. Topical diagnosis: Prechiasmal visual pathways. In: Neuro-ophthalmology, Glaser JS (Ed), JB Lippincott, Philadelphia 1990. p.83.
  2. Sadun AA. The afferent visual system: Anatomy and Physiology. In: Ophthalmology, 2nd, Yanoff M, Duker JS (Eds), Mosby, St. Louis 2004. p.186.
  3. Hayreh SS. Posterior ischaemic optic neuropathy: clinical features, pathogenesis, and management. Eye (Lond) 2004; 18:1188.
  4. Sadda SR, Nee M, Miller NR, et al. Clinical spectrum of posterior ischemic optic neuropathy. Am J Ophthalmol 2001; 132:743.
  5. Buono LM, Foroozan R. Perioperative posterior ischemic optic neuropathy: review of the literature. Surv Ophthalmol 2005; 50:15.
  6. Dunker S, Hsu HY, Sebag J, Sadun AA. Perioperative risk factors for posterior ischemic optic neuropathy. J Am Coll Surg 2002; 194:705.
  7. Swartz NG, Beck RW, Savino PJ, et al. Pain in anterior ischemic optic neuropathy. J Neuroophthalmol 1995; 15:9.
  8. Liu GT. Visual loss: optic neuropathies. In: Neuro-Ophthalmology: Diagnosis and Management, Liu GT, Volpe NJ, Galetta SL (Eds), WB Saunders, Philadelphia 2001.
  9. Arnold AC. Ischemic optic neuropathies. Ophthalmol Clin North Am 2001; 14:83.
  10. Gass A, Moseley IF. The contribution of magnetic resonance imaging in the differential diagnosis of optic nerve damage. J Neurol Sci 2000; 172 Suppl 1:S17.
  11. Optic nerve decompression surgery for nonarteritic anterior ischemic optic neuropathy (NAION) is not effective and may be harmful. The Ischemic Optic Neuropathy Decompression Trial Research Group. JAMA 1995; 273:625.
  12. Dickersin K, Manheimer E, Li T. Surgery for nonarteritic anterior ischemic optic neuropathy. Cochrane Database Syst Rev 2006; :CD001538.
  13. Newman NJ, Scherer R, Langenberg P, et al. The fellow eye in NAION: report from the ischemic optic neuropathy decompression trial follow-up study. Am J Ophthalmol 2002; 134:317.
  14. Liozon E, Herrmann F, Ly K, et al. Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients. Am J Med 2001; 111:211.
  15. Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med 2006; 354:1273.
  16. Foroozan R, Buono LM, Savino PJ, Sergott RC. Acute demyelinating optic neuritis. Curr Opin Ophthalmol 2002; 13:375.
  17. Frohman EM, Frohman TC, Zee DS, et al. The neuro-ophthalmology of multiple sclerosis. Lancet Neurol 2005; 4:111.
  18. The clinical profile of optic neuritis. Experience of the Optic Neuritis Treatment Trial. Optic Neuritis Study Group. Arch Ophthalmol 1991; 109:1673.
  19. Rizzo JF 3rd, Andreoli CM, Rabinov JD. Use of magnetic resonance imaging to differentiate optic neuritis and nonarteritic anterior ischemic optic neuropathy. Ophthalmology 2002; 109:1679.
  20. Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med 1992; 326:581.
  21. Lee AG, Brazis PW. Systemic infections of neuro-ophthalmic significance. Ophthalmol Clin North Am 2004; 17:397.
  22. Anninger WV, Lomeo MD, Dingle J, et al. West Nile virus-associated optic neuritis and chorioretinitis. Am J Ophthalmol 2003; 136:1183.
  23. Chan CK, Limstrom SA, Tarasewicz DG, Lin SG. Ocular features of west nile virus infection in North America: a study of 14 eyes. Ophthalmology 2006; 113:1539.
  24. Golnik KC. Infectious optic neuropathy. Semin Ophthalmol 2002; 17:11.
  25. Venkatesh P, Garg SP, Verma L, et al. Combined optic neuropathy and central retinal artery occlusion in miliary tuberculosis. Retina 2001; 21:375.
  26. Rex JH, Larsen RA, Dismukes WE, et al. Catastrophic visual loss due to Cryptococcus neoformans meningitis. Medicine (Baltimore) 1993; 72:207.
  27. Fukushima A, Yasuoka M, Tsukahara M, Ueno H. A case of cat scratch disease neuroretinitis confirmed by polymerase chain reaction. Jpn J Ophthalmol 2003; 47:405.
  28. Gilden DH, Mahalingam R, Cohrs RJ, Tyler KL. Herpesvirus infections of the nervous system. Nat Clin Pract Neurol 2007; 3:82.
  29. Küçükerdönmez C, Akova YA, Yilmaz G. Ocular toxoplasmosis presenting as neuroretinitis: report of two cases. Ocul Immunol Inflamm 2002; 10:229.
  30. Benz MS, Glaser JS, Davis JL. Progressive outer retinal necrosis in immunocompetent patients treated initially for optic neuropathy with systemic corticosteroids. Am J Ophthalmol 2003; 135:551.
  31. Ray S, Gragoudas E. Neuroretinitis. Int Ophthalmol Clin 2001; 41:83.
  32. Smith GT, Goldmeier D, Migdal C. Neurosyphilis with optic neuritis: an update. Postgrad Med J 2006; 82:36.
  33. Sibony P, Halperin J, Coyle PK, Patel K. Reactive Lyme serology in optic neuritis. J Neuroophthalmol 2005; 25:71.
  34. Krim E, Guehl D, Burbaud P, Lagueny A. Retrobulbar optic neuritis: a complication of Lyme disease? J Neurol Neurosurg Psychiatry 2007; 78:1409.
  35. Farris BK, Pickard DJ. Bilateral postinfectious optic neuritis and intravenous steroid therapy in children. Ophthalmology 1990; 97:339.
  36. DeStefano F, Verstraeten T, Jackson LA, et al. Vaccinations and risk of central nervous system demyelinating diseases in adults. Arch Neurol 2003; 60:504.
  37. Evliyaoglu F, Karadag R, Burakgazi AZ. Ocular neuropathy in peripheral neuropathies. Muscle Nerve 2012; 46:681.
  38. Ginestal RC, Plaza JF, Callejo JM, et al. Bilateral optic neuritis and Guillain-Barré syndrome following an acute Mycoplasma pneumoniae infection. J Neurol 2004; 251:767.
  39. Pfausler B, Engelhardt K, Kampfl A, et al. Post-infectious central and peripheral nervous system diseases complicating Mycoplasma pneumoniae infection. Report of three cases and review of the literature. Eur J Neurol 2002; 9:93.
  40. Pawate S, Moses H, Sriram S. Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM 2009; 102:449.
  41. Joseph FG, Scolding NJ. Neurosarcoidosis: a study of 30 new cases. J Neurol Neurosurg Psychiatry 2009; 80:297.
  42. Kidd D, Burton B, Plant GT, Graham EM. Chronic relapsing inflammatory optic neuropathy (CRION). Brain 2003; 126:276.
  43. Petzold A, Plant GT. Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported. J Neurol 2014; 261:17.
  44. Theodoridou A, Settas L. Demyelination in rheumatic diseases. J Neurol Neurosurg Psychiatry 2006; 77:290.
  45. Jabs DA, Miller NR, Newman SA, et al. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol 1986; 104:564.
  46. Giorgi D, Balacco Gabrieli C. Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis. Clin Rheumatol 1999; 18:124.
  47. Rabadi MH, Kundi S, Brett D, Padmanabhan R. Neurological pictures. Primary Sjögren syndrome presenting as neuromyelitis optica. J Neurol Neurosurg Psychiatry 2010; 81:213.
  48. Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83:280.
  49. Monteiro ML, Borges WI, do Val Ferreira Ramos C, et al. Bilateral optic neuritis in wegener granulomatosis. J Neuroophthalmol 2005; 25:25.
  50. Kansu T, Kirkali P, Kansu E, Zileli T. Optic neuropathy in Behçet's disease. J Clin Neuroophthalmol 1989; 9:277.
  51. Gupta G, Gelfand JM, Lewis JD. Increased risk for demyelinating diseases in patients with inflammatory bowel disease. Gastroenterology 2005; 129:819.
  52. Ernst BB, Lowder CY, Meisler DM, Gutman FA. Posterior segment manifestations of inflammatory bowel disease. Ophthalmology 1991; 98:1272.
  53. Pillay N, Gilbert JJ, Ebers GC, Brown JD. Internuclear ophthalmoplegia and "optic neuritis": paraneoplastic effects of bronchial carcinoma. Neurology 1984; 34:788.
  54. Yu Z, Kryzer TJ, Griesmann GE, et al. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol 2001; 49:146.
  55. Cross SA, Salomao DR, Parisi JE, et al. Paraneoplastic autoimmune optic neuritis with retinitis defined by CRMP-5-IgG. Ann Neurol 2003; 54:38.
  56. Lee AG, Tang RA, Roberts D, et al. Primary central nervous system lymphoma involving the optic chiasm in AIDS. J Neuroophthalmol 2001; 21:95.
  57. Grimm SA, McCannel CA, Omuro AM, et al. Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report. Neurology 2008; 71:1355.
  58. Howell N. LHON and other optic nerve atrophies: the mitochondrial connection. Dev Ophthalmol 2003; 37:94.
  59. Miller NR, Newman NJ, Biousse V, Kerrison JB. Walsh and Hoyt's Clinical Neuro-ophthalmology, Lippincott, Williams and Wilkins, Philadelphia 2005.
  60. Smith JL, Hoyt WF, Susac JO. Ocular fundus in acute Leber optic neuropathy. Arch Ophthalmol 1973; 90:349.
  61. Kermode AG, Moseley IF, Kendall BE, et al. Magnetic resonance imaging in Leber's optic neuropathy. J Neurol Neurosurg Psychiatry 1989; 52:671.
  62. Lamirel C, Cassereau J, Cochereau I, et al. Papilloedema and MRI enhancement of the prechiasmal optic nerve at the acute stage of Leber hereditary optic neuropathy. J Neurol Neurosurg Psychiatry 2010; 81:578.
  63. Votruba M, Thiselton D, Bhattacharya SS. Optic disc morphology of patients with OPA1 autosomal dominant optic atrophy. Br J Ophthalmol 2003; 87:48.
  64. Alexander C, Votruba M, Pesch UE, et al. OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28. Nat Genet 2000; 26:211.
  65. Kerrison JB. Optic neuropathies caused by toxins and adverse drug reactions. Ophthalmol Clin North Am 2004; 17:481.
  66. McKinley SH, Foroozan R. Optic neuropathy associated with linezolid treatment. J Neuroophthalmol 2005; 25:18.
  67. Melamud A, Kosmorsky GS, Lee MS. Ocular ethambutol toxicity. Mayo Clin Proc 2003; 78:1409.
  68. ten Tusscher MP, Jacobs PJ, Busch MJ, et al. Bilateral anterior toxic optic neuropathy and the use of infliximab. BMJ 2003; 326:579.
  69. Foroozan R, Buono LM, Sergott RC, Savino PJ. Retrobulbar optic neuritis associated with infliximab. Arch Ophthalmol 2002; 120:985.
  70. Sherman JH, Aregawi DG, Lai A, et al. Optic neuropathy in patients with glioblastoma receiving bevacizumab. Neurology 2009; 73:1924.
  71. Hayreh SS. Erectile dysfunction drugs and non-arteritic anterior ischemic optic neuropathy: is there a cause and effect relationship? J Neuroophthalmol 2005; 25:295.
  72. Pomeranz HD, Bhavsar AR. Nonarteritic ischemic optic neuropathy developing soon after use of sildenafil (viagra): a report of seven new cases. J Neuroophthalmol 2005; 25:9.
  73. Chen D, Hedges TR. Amiodarone optic neuropathy--review. Semin Ophthalmol 2003; 18:169.
  74. Nagra PK, Foroozan R, Savino PJ, et al. Amiodarone induced optic neuropathy. Br J Ophthalmol 2003; 87:420.
  75. Epidemic optic neuropathy in Cuba--clinical characterization and risk factors. The Cuba Neuropathy Field Investigation Team. N Engl J Med 1995; 333:1176.
  76. Newman NJ. Optic neuropathy. Neurology 1996; 46:315.
  77. Sadun AA. Metabolic optic neuropathies. Semin Ophthalmol 2002; 17:29.
  78. Kisimbi J, Shalchi Z, Mahroo OA, et al. Macular spectral domain optical coherence tomography findings in Tanzanian endemic optic neuropathy. Brain 2013; 136:3418.
  79. Plant GT, Mtanda AT, Arden GB, Johnson GJ. An epidemic of optic neuropathy in Tanzania: characterization of the visual disorder and associated peripheral neuropathy. J Neurol Sci 1997; 145:127.
  80. Bowman RJ, Wedner S, Bowman RF, et al. Optic neuropathy endemic in secondary school children in Dar es Salaam, Tanzania. Br J Ophthalmol 2010; 94:146.
  81. Dolin PJ, Mohamed AA, Plant GT. Epidemic of bilateral optic neuropathy in Dar es Salaam, Tanzania. N Engl J Med 1998; 338:1547.
  82. Solberg Y, Rosner M, Belkin M. The association between cigarette smoking and ocular diseases. Surv Ophthalmol 1998; 42:535.
  83. Kermode AG, Plant GT, Miller DH, et al. Tobacco-alcohol amblyopia: magnetic resonance imaging findings. J Neurol Neurosurg Psychiatry 1989; 52:1447.
  84. Jiang GL, Tucker SL, Guttenberger R, et al. Radiation-induced injury to the visual pathway. Radiother Oncol 1994; 30:17.
  85. Simsek T, Simsek E, Ilhan B, et al. Traumatic optic nerve avulsion. J Pediatr Ophthalmol Strabismus 2006; 43:367.
  86. Sarkies N. Traumatic optic neuropathy. Eye (Lond) 2004; 18:1122.
  87. Levin LA, Beck RW, Joseph MP, et al. The treatment of traumatic optic neuropathy: the International Optic Nerve Trauma Study. Ophthalmology 1999; 106:1268.
  88. Steinsapir KD. Treatment of traumatic optic neuropathy with high-dose corticosteroid. J Neuroophthalmol 2006; 26:65.
  89. Yu-Wai-Man P, Griffiths PG. Steroids for traumatic optic neuropathy. Cochrane Database Syst Rev 2013; 6:CD006032.