The presentation of the arteritic and nonarteritic forms is similar. They are characterized by a rapid and painless loss of vision that can occur over minutes, hours or days [5]. Diminished visual acuity and impaired pupillary response also develop. Additionally, the optic disk and the nerve fibers are swollen. This swelling overshadows the vessels supplying the optic nerve. 

In patients with giant cell arteritis, the clinical features include malaise, myalgia, headaches, jaw claudication, and temporal artery tenderness [2].

Ophthalmic exam

The optic disk may appear hyperemic in nonarteritic cases while it is likely to be pale colored in the arteritic form. Additionally, findings include hemorrhages around the disk. Finally, testing of the visual fields reveals inferior and central deficits. 

• Pallor

  Temporal pallor Temporal pallor may be observed in traumatic or nutritional optic neuropathy, and it is most commonly seen in patients with multiple sclerosis, particularly in those with a history of optic neuritis. [emedicine.medscape.com]

  (a, b) Disc pallor in a 44-year-old female with ethambutol toxicity. She was treated with ethambutol for 2 months for tuberculoma brain. (c, d) Goldman visual fields of the same patient showing bilateral centrocecal scotomas. [epomedicine.com]

  Two months later, VA was 20/20 OD and hand motions OS, with relative afferent pupillary defect and a pallor of the optic disc in OS. [ncbi.nlm.nih.gov]

   ○ Temporal disc pallor may be seen in the fellow eye, indicative of previous optic neuritis. [slideshare.net]

  PION shows a normal fundoscopy and fluorescein angiography, but pallor of the disc develops within about 8 weeks. Central field defects usually occur with or without other defects. [news-medical.net]

• Fever

  Typical symptoms of an acute human immunodeficiency virus (HIV) infections like fever and rash are not specific and can be caused by a multitude of other pathogens, such as Zika or rickettsiosis. [ncbi.nlm.nih.gov]

  […] aggressively treated systemic hypertension Arteritic ischemic optic neuropathy in giant cell arteritis, but that is usually accompanied by headache, jaw pain provoked by chewing ("jaw claudication"), limb girdle joint pain, fatigue, malaise, low-grade fever [kellogg.umich.edu]

  […] will feel unwell for some time with any of the following: Pain in the temples Pain when chewing Scalp pain or tingling Neck pain Muscle aches and pains, particularly in the upper legs or arms General fatigue Loss of appetite Unexplained loss of weight Fever [my.clevelandclinic.org]

  Other symptoms include high fever, nausea, vomiting, pain with urination, or pain from straining with a bowel movement, groin pain, pain with intercourse, and simply feeling ill. [webmd.com]

  These are cases with pain that's hard to control with vomiting, high fever and if you are not getting better with antibiotics given by mouth. Occasionally, for bad cases, narcotics are needed for a few days. [urologyhealth.org]

• Weight Loss

  Pseudotumor cerebri, for example, often affects overweight women, so weight loss may be a good treatment option. [sightmd.com]

  They may also feel tired and run a fever, combined with recent weight loss. How is Ischemic Optic Neuropathy diagnosed? Your ophthalmologist will perform a complete eye examination, including pupil dilation. [rvscny.com]

  ON secondary to PAN should be suspected in male patients around their fourth decade who present with sudden visual loss, nonspecific systemic affection, such as weight loss and fatigue, and who lack inciting factors involved in acute loss of vision. [frontiersin.org]

  In a young patient, previous history of eye pain, paraesthesiae, ataxia or weakness suggest demyelination In an older patient, previous history of transient visual loss, diplopia, temporal pain, jaw claudication, fatigue, weight loss and myalgia suggests [patient.info]

• Disability

  INTRODUCTION: Non-arteritic anterior ischemic optic neuropathy (NAION) is one of the most widespread visually disabling diseases in the middle-aged and elderly population. [ncbi.nlm.nih.gov]

  Please enable JS and disable any ad blocker [practicalcardiology.modernmedicine.com]

  […] moderately disabled, needing some help, but can walk unaided; 4. moderate to severe disability, unable to walk, needing help with activities of daily living; and 5. severely disabled, bed ridden, requiring constant care. [onlinelibrary.wiley.com]

  The dysthyroid optic neuropathy Dysthyroid optic neuropathy (DON) is the most feared complication of thyroid eye disease, and it constitutes an important factor of permanent or temporary disability. [intechopen.com]

• Short Stature

  Berk–Tabatznik syndrome is a condition that shows symptoms of short stature, congenital optic atrophy and brachytelephalangy. [en.wikipedia.org]

• Hypotension

  Optic nerve enhancement in hypotensive ischemic optic neuropathy. J Neuroophthalmol. 2004;24:235-236. Rizzo JF 3rd, Andreoli CM, Rabinov JD. [appliedradiology.com]

  Non-arteritic anterior ischaemic optic neuropathy (NAION) may develop due to severe anaemia and hypotension which is seen in acute blood loss. The devastating visual loss is often irreversible. [ncbi.nlm.nih.gov]

  It is due to too long a period of hypotension, complicated by hemodilution, and orbital and eye swelling including chemosis. [news-medical.net]

• Neck Pain

  About 80% of those affected will feel unwell for some time with any of the following: Pain in the temples Pain when chewing Scalp pain or tingling Neck pain Muscle aches and pains, particularly in the upper legs or arms General fatigue Loss of appetite [my.clevelandclinic.org]

  This condition may cause symptoms before vision loss, such as overall fatigue, fever, pain in the temples, neck pain, pain when chewing, and scalp pain. [provisu.ch]

  Common signs and symp tom s may include general fatigue, weight loss, fever, temple pain, neck pain, pain on chewing, scalp tenderness when combing their hair, headache, anemia and achy joints. [eyeassociates.com]

  Common signs and symptoms may include general fatigue, weight loss, fever, temple pain, neck pain, pain on chewing, scalp tenderness when combing their hair, headache, anemia and achy joints. [lowvision.org]

• Macula

  The optic nerve is a cable of retinal ganglion cells that enters the eye approximately 4 mm nasally and 0.8 mm superiorly from the center of the macula at the posterior pole. [emedicine.medscape.com]

  The retina in both disks was flat, and the macula and vessels seemed to be normal. Computed tomography of the head showed no evidence of acute intracranial hemorrhage or infarction. [anesthesiology.pubs.asahq.org]

  The macula was flat with even pigment and showed a positive foveal reflex OU. [practicalcardiology.com]

  Leber idiopathic stellate neuroretinitis is an acute sporadic inflammation of the optic nerve and macula, characterized by both disc and macular edema, the resolution of the latter leading to a macular "star," hence the name. [medlink.com]

  (A) Right eye with normal appearance of the optic nerve and macula. (B) Left eye with optic nerve hyperemia, swollen, retinal folds, dilated veins, and arteries. (C) Right eye with normal appearance of the optic nerve and macula. [frontiersin.org]

• Acne Vulgaris

  Isotretinoin Isotretinoin (Accutane), used in the treatment of severe acne vulgaris, has been described as rarely causing toxic optic neuropathy, presenting as decreased night vision and loss of color vision. [emedicine.medscape.com]

  • Used in the treatment of severe acne vulgaris, • Rarely causing toxic optic neuropathy, presenting as decreased night vision and loss of color vision. [slideshare.net]

• Pupillary Abnormality

  There were no pupillary abnormalities noted. Ophthalmoscopy revealed variable, and often times subtle, temporal pallor of the optic nerve. OCT imaging showed RNFL thinning in all patients. [iovs.arvojournals.org]

  Both types of disorders can result from tumors, inflammation, trauma, systemic disorders, and degenerative or other processes, causing such symptoms as vision loss, diplopia, ptosis, pupillary abnormalities, periocular pain, facial pain, or headache. [merckmanuals.com]

  Pupillary response to light is compromised and, subsequently, is lost. Definitive diagnosis of methanol toxicity requires a confirmed increase in the serum methanol level with gas chromatography (>20 mg/dl). [ijo.in]

  Pupillary abnormalities can be subtle, and visual evoked potential may be needed to confirm the diagnosis. The diagnosis is often made late when optic nerve pallor has already set in and vision is severely affected. [ncbi.nlm.nih.gov]

• Blurred Vision

  He was admitted to the hospital with blurred vision after the consumption of alcohol (600-700 ml of cologne). He was treated with intravenous ethanol, NaHCO3 and hemodialysis. On admission, a brain and orbital MRI was performed. [ncbi.nlm.nih.gov]

  Toxic Optic Neuropathies: Tamoxifen 20. • A drug for epilepsy • Causes acute angle-closure glaucoma (acute eye pain, redness, blurred vision, haloes). • Treatment of this type of acute angle-closure glaucoma is by cycloplegia and topical steroids (rather [slideshare.net]

  Overall, one out of four patients on linezolid had at least one episode of ocular complaints, usually blurred vision. [journals.plos.org]

  Blurred vision, changes in colour perception, decreased visual acuity and eye pain are noted to occur in <1% of cases (Samarakoon et al. 2007). [onlinelibrary.wiley.com]

• Visual Acuity Decreased

  There are altitudinal visual field defects sometimes combined with decreased visual acuity. [healthcentral.com]

  Visual acuity: Decrease in visual acuity is insidious and bilaterally symmetrical. 3. Visual field: Typically show central scotomas or bitemporal defects and, less commonly, peripheral constriction. 4. [epomedicine.com]

  Like other optic neuropathies, patients with TON may have decreased central visual acuity, decreased color vision, an afferent pupillary defect and/or visual field deficits. [eyewiki.aao.org]

  Within 1 week, the patient's visual acuity decreased to less than 20/200 OU and results of a visual field examination found markedly depressed vision bilaterally. [jamanetwork.com]

• Marcus-Gunn Pupil

  Clinical features include retro-orbital pain that is aggravated by eye movement, loss of color vision, and contrast sensitivity that may progress to severe visual loss, an afferent pupillary defect (Marcus-Gunn pupil), and in some instances optic disc [fpnotebook.com]

  The swinging light test (or Marcus Gunn test) classically reveals a Marcus Gunn pupil, and fundoscopy may demonstrate diffuse optic disk swelling indicative of papillitis (will not be present if inflammation is solely retrobulbar) 1. [radiopaedia.org]

  If a relative afferent defect (deafferented pupil, afferent pupillary defect, or Marcus Gunn pupil) is present, the pupil paradoxically dilates when the flashlight swings to the side of the defect. [merckmanuals.com]

• Prolapse

  They found that patients with Graves’ orbitopathy who have severe optic nerve crowding, intracranial fat prolapse and/or muscle index greater than 50% present on orbital CT scans are more likely to have coexisting optic neuropathy. [intechopen.com]

• Confusion

  Yet it is a highly controversial and confusing subject because of the general lack of in-depth scientific understanding of the subject. [books.google.ro]

  Because of this possibility for confusion, it is inadequate to designate a patient with Leber hereditary optic neuropathy a "case of Leber's." The content you are trying to view is available only to logged in, current MedLink Neurology subscribers. [medlink.com]

  Subsequently, the patient experiences headache, shortness of breath, abdominal pain, confusion, and visual loss of any degree. Coma and death may ensue. In patients with severe visual impairment the pupils may be dilated and non-reactive to light. [statpearls.com]

  The optic neuropathy from amiodarone, as discussed in this article, should not be confused with acute nonarteritic ischemic optic neuropathy – like picture (NAION). [emedicine.medscape.com]

  Symptoms of WE & KP WERNICKE KORSAKOFF Confusion Anterograde amnesia Ataxia Retrograde amnesia Ophthalmoplegia/Nystagmus Confabulation Metabolic ophthalmoplegia: WE 49. [slideshare.net]

• Neurologic Manifestation

  Lyme disease optic neuritis increased intracranial pressure pseudotumor cerebri meningitis neurologic manifestations children blindness Received August 7, 2000. Accepted January 29, 2001. [pediatrics.aappublications.org]

  Other neurological manifestations may occur if there is concurrent acute disseminated encephalomyelitis (Bangsgaard et al 2006). [ncbi.nlm.nih.gov]

• Cerebellar Ataxia

  – Mitochondrial diseases are characterized by association of optical neuropathy alongwith cerebellar ataxia as a result of a missense variation in the mitochondrial DNA (valine replaced by alanine). 11 In older individuals – Ø Loss of vision – Autoimmune [biotecharticles.com]

  Neurological examination showed cerebellar ataxia and pyramidal weakness. [hindawi.com]

• Paresis

  However, such examination may miss mild paresis of ocular movement sufficient to cause diplopia. [merckmanuals.com]

Diagnosis is established through a history, ophthalmic exam, and laboratory studies. The history should include a thorough assessment of risk factors such as hypertension, diabetes, atherosclerosis, and obstructive sleep apnea. Also, the ophthalmic exam consists of a detailed evaluation of the eye including structures such as the optic disk, small nerves, and vessels as well as papilledema and hemorrhage.

Laboratory tests

The erythrocyte sedimentation rate (ESR) is helpful. In the arteritic type, ESR is increased in approximately 90% of cases. In the nonarteritic variant, ESR is typically normal unless other diseases coexist. Furthermore, the Westergren ESR is preferred over the Wintrobe ESR. Additionally, the C-reactive protein (CRP) is helpful in the diagnosis of giant cell arteritis. The abnormal levels of ESR and CRP are suggestive of inflammation. Finally, a complete blood count (CBC) may reveal anemia. 

Biopsy

Biopsy of the temporal artery confirms the diagnosis of giant cell arteritis. 

Imaging studies

While ultrasonography has been utilized in the assessment of temporal arteries, this modality has not been used routinely in the diagnosis of anterior ischemic optic neuropathy. Another technique, ocular plethysmography, reveals abnormal results in the arteritic variant. MRI and CT scan are not beneficial in older individuals with either form of neuropathy. A possible advantageous test, fluorescein angiography, may differentiate between the two types of neuropathy [6]. Cerebral angiography identifies stenosis or vessel occlusion in giant cell arteritis, however, this procedure is used less commonly now. Optical coherence tomography (OCT) may be used in individuals with anterior ischemic optic neuropathy [7].

Therapy consists of medical treatment and surgical intervention. Also, giant cell arteritis is treated by an internist in conjunction with a rheumatologist. 

Steroid use in giant cell arteritis

In giant cell arteritis, patients are managed with a steroid course and scheduled tapering. Specifically, the initial dose ranges from 40 to 60 milligrams of prednisone per day, reflecting the patient's weight and level of severity. These patients are monitored with monthly ESR and/or CRP.  In cases with recurrent episodes, the tapering is delayed while flare-ups may require small doses. Other regimens are being explored as well. For example, a trial at Mayo Clinic is investigating the intravenous administration of steroids and whether it reduces the need for chronic use of steroids. In cases where steroids are becoming intolerable due to side effects, methotrexate or cyclosporin may be used concurrently to decrease the steroid dosage. Of important note, periodic monitoring of complete blood count and liver function tests are required. 

Steroid use in nonarteritic anterior ischemic optic neuropathy 

Steroid therapy in these patients is debatable and usually not helpful. The use of this drug may not be justified especially in the context of long-term side effects.

Surgical therapy

One procedure, optic nerve fenestration, was studied in the Ischemic Optic Neuropathy Decompression Trial (IONDT) [8] but found to be ineffective [9]. 

Other

While management of hypertension, diabetes mellitus, atherosclerosis and obstructive sleep apnea is beneficial for the overall health of the individual, this does not recover loss of vision. 

Spontaneous recovery is observed in approximately 40% of patients with nonarteritic ischemic optic neuropathy. Recurrence in the same eye is very rare while the other eye may develop neuropathy in 20% of cases within the following 5 years. 

In patients with the arteritic form, the visual impairment is not recovered. For those with untreated giant cell arteritis, the other eye loses vision in 25% to 50% of cases.

There are two variants of ischemic optic neuropathy, namely nonarteritic and arteritic. These are attributed to impaired blood supply to the optic nerve. This leads to vision loss. 

Nonarteritic ischemic optic neuropathy

This condition presents more commonly in individuals aged 50 years or above. There are predisposing risk factors such as hypertension, tobacco smoking, atherosclerosis, diabetes, and obstructive sleep apnea. Individuals prone to thrombosis formation and nocturnal hypertension are also susceptible to this type of neuropathy [1]. Finally, use of certain medications such as amiodarone is another risk factor [1].

Arteritic ischemic optic neuropathy

This variant is observed in the elderly population. It results from inflammation in the arteries such as giant cell arteritis, in which inflammation obstructs the perfusion of the optic nerve. 

With regard to patient demographics, there is a race preference for the different types. For example, nonarteritic ischemic optic neuropathy is more common in whites than in black people. As for arteritic anterior ischemic optic neuropathy, it exhibits the greatest incidence in the Scandinavian countries. 

In the United States, the incidence of nonarteritic anterior ischemic optic neuropathy is 2.3 to 10.3 per population of 100,000 and increases significantly with age as does giant cell arteritis [2]. Furthermore, the arteritic group demonstrates an incidence of 0.36 per 100,000. With regard to age, both types of anterior ischemic optic neuropathy present in patients above 50 years of age [3]. As for gender, the arteritis form is more predominant in women. 

The pathogenesis of nonarteritic anterior ischemic optic neuropathy is secondary to the diminished perfusion in the posterior circulation. Furthermore, these blood vessels, the short posterior ciliary arteries, perfuse the optic disk and optic nerve as it exits. The results of the ischemia include visual loss [4], impaired inferior visual field, swollen optic disk, and hemorrhage. The loss of vision may occur at rapid onset or take a few days. 

Arteritic anterior optic neuropathy is observed less often and typically develops subsequently to inflammation. 

Posterior ischemic optic neuropathy occurs when the abnormality occurs between the optic chiasm and the globe. This is not common and is usually a disease of exclusion. It features altitudinal visual field abnormalities and visual impairment. Possible causes include diabetes mellitus, connective tissue disease, and trauma.  

Individuals with small disks may have an anatomical predisposition for nonarteritic anterior ischemic optic neuropathy. Hence, a reduced blood supply is enhanced by the swelling causing more damage. As for the arteritic form, the arterial supply to the eye and orbit is jeopardized. 

If the patient presents with anterior ischemic optic neuropathy, there are no preventative measures to protect the other eye. The use of aspirin has been advocated by clinicians even though its efficacy is unknown. 

As for nonarteritic anterior ischemic optic neuropathy, levodopa may be beneficial in improving visual function [10].

Optic neuropathy refers to the loss of vision secondary to injury of the optic nerve. Causes include ischemia, toxins, hypertension, diabetes mellitus and compression in the orbit. Ischemic optic neuropathy is classified in accordance with the site of the ischemic injury. The anterior type is more common and usually results from an insufficient blood supply to the optic disk while the posterior form is typically diagnosed when other causes are ruled out.  

There are the arteric and nonarteritic variants of ischemic optic neuropathy. The former is less common and is due to an inflammatory process in the arteries. Nonarteritic ischemic optic neuropathy is thought to occur with predisposing factors such as hypertension, diabetes, atherosclerosis, and other conditions. 

Generally, optic neuropathy presents with rapid onset vision loss as well as deficits in the visual fields. Additionally, there may be changes in the optic disk and other findings such as swelling and hemorrhage.

Diagnosis is determined through history, ophthalmic exam and laboratory studies. Also, certain imaging modalities may be useful. As for management, the treatment depends on the type of optic neuropathy. While corticosteroids are useful in the treatment of giant cell arteritis, they are not effective in other types of optic neuropathy. 

Patients with optic neuropathy experience a sudden loss of central and peripheral vision. This occurs due to an obstruction of blood flow to the optic nerve. There are two types known as the arteritic and nonarteritic ischemic optic neuropathy.

The arteritic type results from inflammation in the arteries supplying the eye. An example of this is known as giant cell or temporal arteritis. This type is found in people 70 years old or above. It is most common in Scandinavian countries followed by Germany.

The nonarteritic type occurs in individuals with predispositions such as high blood pressure, smoking, diabetes mellitus, atherosclerosis, obstructive sleep apnea, low nighttime blood pressure and others. This type is observed in people 50 years old or above. It is more common in white people than in blacks.

Symptoms include visual loss that occurs over minutes, hours or even days. This disease is not associated with pain. One or both eyes may be affected depending on the cause of the neuropathy. Also, patients develop visual field defects. Individuals with giant cell arteritis experience pain when chewing and combing the hair. Also, they have headaches, muscle aches, and other pains as well. 

The diagnosis of the disease includes a thorough history and detailed assessment of the risk factors. Furthermore, is important to perform an ophthalmic exam to evaluate the eye structure such as the optic disk, and findings such as swelling and hemorrhage. There are helpful tests to guide the diagnosis of giant cell arteritis. A biopsy of the temporal artery will confirm the diagnosis. Also, blood tests such as erythrocyte sedimentation rate (ESR) and the C-reactive protein level are abnormal in this condition suggesting inflammation. 

The treatment of giant cell arteritis includes corticosteroids based on a tapering schedule. In certain severe cases, intravenous corticosteroids may be necessary. As for the nonarteritic form, corticosteroids are not as effective. Also, treatment of the risk factors such as high blood pressure, diabetes, atherosclerosis and obstructive sleep apnea are important for the overall health of the patient. 

1. Uhtoff W. Zu den entzundlichen sehnerven: Affectionen bei arteriosklerose. Ber Dtsch Ophthalmol Gesampte. 1924; 44:196-198.
2. Pula JH, Macdonald CJ. Current options for the treatment of optic neuritis. Clinical Ophthalmology. 2012;6: 1211-23.
3. Hayreh SS, Podhajsky PA, Raman R, et al. Giant cell arteritis: validity and reliability of various diagnostic criteria. American journal of ophthalmology. 1997; 123(3): 285-296.
4. Hayreh SS, Podhajsky PA, Zimmerman B. Occult giant cell arteritis: ocular manifestations. American journal of ophthalmology. 1998; 125(4): 521-526.
5. The Postoperative Visual Loss Study Group. Risk Factors Associated with Ischemic Optic Neuropathy after Spinal Fusion Surgery. Anesthesiology. 2012; 116(1):15-24.
6. Hayreh SS. Optic disc edema in raised intracranial pressure. V. Pathogenesis. Archives of ophthalmology. 1977; 95(9): 1553-1565.
7. Subei AM, Eggenberger ER. Optical coherence tomography: another useful tool in a neuro-ophthalmologist's armamentarium. Curr Opin Ophthalmol. 2009; 20(6):462-6.
8. The Ischemic Optic Neuropathy Decompression Trial Research Group. 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. Journal of American Medical Association. 1995; 273(8):625-32.
9. Atkins EJ, Bruce BB, Newman NJ, et al. Treatment of nonarteritic anterior ischemic optic neuropathy. Survey of Ophthalmology. 2010; 55(1):47-63.
10. Hayreh SS. Does Levodopa improve visual function in NAION? Ophthalmology. 2000; 107(8): 1434-1438