A case of presumed radiation optic neuropathy

International Nuclear Information System (INIS)

Atsumi, Osamu; Sakuraba, Tomoki; Kimura, Satoru; Narita, Kiyoharu; Maeda, Syuji

1991-01-01

A case of a 37-year-old woman with radiation optic neuropathy was reported. She had undergone subtotal removal of the right orbital tumor (adenoid cystic carcinoma) by frontal craniotomy, followed by radiation therapy (64 Gy). She had been quite well until she noticed a gradual loss of vision in her right eye 18 months later. Her visual acuity was 0.2 in the right eye and 1.5 in the left eye with right relative afferent pupillary defect and dense central scotoma. Funduscopy revealed optic disc swelling with surrounding retinal edema and small hemorrhage in the right eye. Fluorescein angiography revealed a hypoperfusion area and obstruction of the small retinal vessels in the posterior pole, but this was not large enough to explain the dense central scotoma. Although prednisolone therapy gave temporary improvement, the visual function gradually deteriorated. (author)

Leber's hereditary optic neuropathy and vitamin B12 deficiency

NARCIS (Netherlands)

Pott, Jan Willem R.; Wong, Kwok H.

2006-01-01

Background: Leber's hereditary optic neuropathy (LHON) is a maternally inherited optic neuropathy caused by mutations in mitochondrial DNA (mtDNA). It is also believed that several epigenetic factors have an influence on the development of LHON. Methods: A case series was observed. Results: Three

Diffusion-weighted MRI in acute posterior ischemic optic neuropathy

International Nuclear Information System (INIS)

Srinivasan, Sivasubramanian; Moorthy, Srikant; Sreekumar, KP; Kulkarni, Chinmay

2012-01-01

Blindness following surgery, especially cardiac surgery, has been reported sporadically, the most common cause being ischemic optic neuropathy. The role of MRI in the diagnosis of this condition is not well established. We present a case of postoperative posterior ischemic optic neuropathy that was diagnosed on diffusion-weighted MRI

Erythropoietin in Treatment of Methanol Optic Neuropathy.

Science.gov (United States)

Pakdel, Farzad; Sanjari, Mostafa S; Naderi, Asieh; Pirmarzdashti, Niloofar; Haghighi, Anousheh; Kashkouli, Mohsen B

2018-06-01

Methanol poisoning can cause an optic neuropathy that is usually severe and irreversible and often occurs after ingestion of illicit or homemade alcoholic beverages. In this study, we evaluated the potential neuroprotective effect of erythropoietin (EPO) on visual acuity (VA) in patients with methanol optic neuropathy. In a prospective, noncomparative interventional case series, consecutive patients with methanol optic neuropathy after alcoholic beverage ingestion were included. All patients initially received systemic therapy including metabolic stabilization and detoxification. Treatment with intravenous recombinant human EPO consisted of 20,000 units/day for 3 successive days. Depending on clinical response, some patients received a second course of EPO. VA, funduscopy, and spectral domain optical coherence tomography were assessed during the study. Main outcome measure was VA. Thirty-two eyes of 16 patients with methanol optic neuropathy were included. Mean age was 34.2 years (±13.3 years). The mean time interval between methanol ingestion and treatment with intravenous EPO was 9.1 days (±5.56 days). Mean follow-up after treatment was 7.5 months (±5.88 months). Median VA in the better eye of each patient before treatment was light perception (range: 3.90-0.60 logMAR). Median last acuity after treatment in the best eye was 1.00 logMAR (range: 3.90-0.00 logMAR). VA significantly increased in the last follow-up examination (P optic neuropathy and may represent a promising treatment for this disorder.

Early bilateral radiation-induced optic neuropathy with follow-up MRI

International Nuclear Information System (INIS)

McClellan, R.L.; El Gammal, T.; Kline, L.B.

1995-01-01

Most documented cases of radiation-induced optic neuropathy are unilateral and occur more than 1 year after radiotherapy to the sellar region. We describe a patient with bilateral radiation optic neuropathy 3 months following the completion of radiotherapy. MRI 13 months after the onset of visual failure showed bilateral optic atrophy with residual gadolinium enhancement. (orig.)

Early bilateral radiation-induced optic neuropathy with follow-up MRI

Energy Technology Data Exchange (ETDEWEB)

McClellan, R.L. [Alabama Univ., Birmingham (United States). Dept. of Radiology; El Gammal, T. [Alabama Univ., Birmingham (United States). Dept. of Radiology; Kline, L.B. [Alabama Univ., Birmingham (United States). Dept. of Radiology

1995-02-01

Most documented cases of radiation-induced optic neuropathy are unilateral and occur more than 1 year after radiotherapy to the sellar region. We describe a patient with bilateral radiation optic neuropathy 3 months following the completion of radiotherapy. MRI 13 months after the onset of visual failure showed bilateral optic atrophy with residual gadolinium enhancement. (orig.)

Postirradiation optic neuropathy in antral carcinoma

International Nuclear Information System (INIS)

Singh, J.; Vashist, S.

1984-01-01

A case is described of a patient who developed radiation-induced optic neuropathy 18 months following cobalt-60 irradiation for carcinoma of the left maxillary antrum and ethmoid sinus. This case is unusual because of the early onset of the optic nerve damage following radiation therapy and the ultimate emergence of the eye involved by tumor compression as the better eye in terms of visual acuity

Genetically determined optic neuropathies

DEFF Research Database (Denmark)

Milea, Dan; Amati-Bonneau, Patrizia; Reynier, Pascal

2010-01-01

The present review focuses on recent advances in the knowledge of hereditary optic neuropathies resulting from retinal ganglion cell degeneration, mostly due to mitochondrial dysfunctions.......The present review focuses on recent advances in the knowledge of hereditary optic neuropathies resulting from retinal ganglion cell degeneration, mostly due to mitochondrial dysfunctions....

Bilateral Non-arteritic Anterior Ischaemic Optic Neuropathy as the Presentation of Systemic Amyloidosis.

Science.gov (United States)

Kanaan, M Z; Lorenzi, A R; Thampy, N; Pandit, R; Dayan, Margaret

2017-12-01

A 75-year-old hypertensive female with stable idiopathic intermediate uveitis presented with bilateral sequential optic neuropathy with optic disc swelling. The optic neuropathy in the first affected eye (right) was thought to be due to non-arteritic anterior ischaemic optic neuropathy (NAION). Asymptomatic left optic disc swelling was found at routine review 2 months later, and a diagnosis of giant cell arteritis (GCA) was sought. Temporal artery duplex ultrasound showed the "halo sign," but a subsequent temporal artery biopsy showed light-chain (AL) amyloidosis with no signs of giant cell arteritis. In this case, bilateral sequential ischaemic optic neuropathy mimicking non-arteritic anterior ischaemic optic neuropathy was the presenting sign of systemic amyloidosis involving the temporal arteries.

Optic Neuropathy Secondary to Polyarteritis Nodosa, Case Report, and Diagnostic Challenges.

Science.gov (United States)

Vazquez-Romo, Kristian A; Rodriguez-Hernandez, Adrian; Paczka, Jose A; Nuño-Suarez, Moises A; Rocha-Muñoz, Alberto D; Zavala-Cerna, Maria G

2017-01-01

To describe a case of optic neuropathy as a primary manifestation of polyarteritis nodosa (PAN) and discuss diagnostic challenges. Case report. A 41-year-old Hispanic man presented with a 2-day history of reduced visual acuity in his left eye. Physical examination revealed a complete visual field loss in the affected eye. Best-corrected visual acuity (BCVA) in the left eye was hand motion, and fundus examination revealed a hyperemic optic disk with blurred margins, swelling, retinal folds, dilated veins, and normal size arteries. BCVA in the right eye was 20/20; no anomalies were seen during examination of the fundus. The patient was started on oral corticosteroids and once the diagnosis of PAN was made, cyclophosphamide was added to the treatment regimen. Six months later, the patient recovered his BCVA to 20/20 in his left eye. Rarely does optic neuropathy present as a primary manifestation of PAN; nevertheless, it represents an ophthalmologic emergency that requires expeditious anti-inflammatory and immunosuppressive treatment to decrease the probability of permanent visual damage. Unfortunately, diagnosing PAN is challenging as it necessitates a high index of suspicion. In young male patients who present for the first time with diminished visual acuity, ophthalmologists become cornerstones in the suspicion of this diagnosis and should be responsible for continuing the study until a diagnosis is reached to ensure rapid commencement of immunosuppressive treatment.

A case of anterior ischemic optic neuropathy associated with uveitis

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Sugahara M

2013-05-01

Full Text Available Michitaka Sugahara, Takayuki Fujimoto, Kyoko Shidara, Kenji Inoue, Masato Wakakura Inouye Eye Hospital, Tokyo, Japan Introduction: Here, we describe a patient who presented with anterior ischemic optic neuropathy (AION and subsequently developed uveitis. Case: A 69-year-old man was referred to our hospital and initially presented with best-corrected visual acuities (BCVA of 20/40 (right eye and 20/1000 (left eye and relative afferent pupillary defect. Slit-lamp examination revealed no signs of ocular inflammation in either eye. Fundus examination revealed left-eye swelling and a pale superior optic disc, and Goldmann perimetry revealed left-eye inferior hemianopia. The patient was diagnosed with nonarteritic AION in the left eye. One week later, the patient returned to the hospital because of vision loss. The BCVA of the left eye was so poor that the patient could only count fingers. Slit-lamp examination revealed 1+ cells in the anterior chamber and the anterior vitreous in both eyes. Funduscopic examination revealed vasculitis and exudates in both eyes. The patient was diagnosed with bilateral panuveitis, and treatment with topical betamethasone was started. No other physical findings resulting from other autoimmune or infectious diseases were found. No additional treatments were administered, and optic disc edema in the left eye improved, and the retinal exudates disappeared in 3 months. The patient's BCVA improved after cataract surgery was performed. Conclusion: Panuveitis most likely manifests after the development of AION. Keywords: anterior ischemic optic neuropathy, uveitis

Radiation optic neuropathy

International Nuclear Information System (INIS)

Kline, L.B.; Kim, J.Y.; Ceballos, R.

1985-01-01

Following surgery for pituitary adenoma, radiation therapy is an accepted treatment in reducing tumor recurrence. However, a potential therapeutic complication is delayed radionecrosis of perisellar neural structures, including the optic nerves and chiasm. This particular cause of visual loss, radiation optic neuropathy (RON), has not been emphasized in the ophthalmologic literature. Four cases of RON seen in the past five years are reported. Diagnostic criteria include: (1) acute visual loss (monocular or binocular), (2) visual field defects indicating optic nerve or chiasmal dysfunction, (3) absence of optic disc edema, (4) onset usually within three years of therapy (peak: 1-1 1/2 years), and (5) no computed tomographic evidence of visual pathway compression. Pathologic findings, differential diagnosis and therapy will be discussed in outlining the clinical profile of RON

Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China.

Science.gov (United States)

Gan, Dekang; Li, Mengwei; Wu, Jihong; Sun, Xinghuai; Tian, Guohong

2017-01-01

To evaluate the clinical classification and characteristics of hereditary optic neuropathy patients in a single center in China. Retrospective case study. Patients diagnosed with hereditary optic neuropathy between January 2014 and December 2015 in the neuro-ophthalmology division in Shanghai Eye and ENT Hospital of Fudan University were recruited. Clinical features as well as visual field, brain/orbital MRI, and spectrum domain optical coherence tomography (SD-OCT) were analyzed. Eighty-two patients diagnosed by gene test were evaluated, including 66 males and 16 females. The mean age of the patients was 19.4 years (range, 5-46 years). A total of 158 eyes were analyzed, including 6 unilateral, 61 bilateral, and 15 sequential. The median duration of the disease was 0.5 year (range, 0.1-20 years). Genetic test identified 68 patients with Leber hereditary optic neuropathy, 9 with dominant optic neuropathy, and 2 with a Wolfram gene mutation. There was also one case of hereditary spastic paraplegia, spinocerebellar ataxia, and polymicrogyria with optic nerve atrophy, respectively. Leber hereditary optic neuropathy is the most common detected type of hereditary optic neuropathy in Shanghai, China. The detection of other autosomal mutations in hereditary optic neuropathy is limited by the currently available technique.

Comparison of optic disc morphology of optic nerve atrophy between compressive optic neuropathy and glaucomatous optic neuropathy.

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Masayuki Hata

Full Text Available To compare the optic nerve head (ONH structure between compressive optic neuropathy (CON and glaucomatous optic neuropathy (GON, and to determine whether selected ONH quantitative parameters effectively discriminate between GON and CON, especially CON cases presenting with a glaucoma-like disc.We prospectively assessed 34 patients with CON, 34 age-matched patients with moderate or severe GON, and 34 age-matched healthy control subjects. The quantitative parameters of ONH structure were compared using the Heidelberg Retina Tomograph 2 (HRT2 and Spectralis optical coherence tomography with an enhanced depth imaging method.The mean and maximum cup depths of CON were significantly smaller than those with GON (P < 0.001 and P < 0.001, respectively. The distance between Bruch's membrane opening and anterior surface of the lamina cribrosa (BMO-anterior LC of CON was also significantly smaller than that of glaucoma but was similar to that of the healthy group (P < 0.001 and P = 0.47, respectively. Based on Moorfields regression analysis of the glaucoma classification of HRT2, 15 eyes with CON were classified with a glaucoma-like disc. The cup/disc area ratio did not differ between cases of CON with a glaucoma-like disc and cases of GON (P = 0.16, but the BMO-anterior LC and mean and maximum cup depths of CON cases with a glaucoma-like disc were smaller than those in GON (P = 0.005, P = 0.003, and P = 0.001, respectively.Measurements of the cup depths and the LC depth had good ability to differentiate between CON with a glaucoma-like disc and glaucoma. There was no laminar remodeling detected by laminar surface position in the patients with CON compared to those with GON.

Unilateral Optic Neuropathy and Acute Angle-Closure Glaucoma following Snake Envenomation

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Osman Okan Olcaysu

2015-01-01

Full Text Available Purpose. We aimed to describe a unique case in which a patient developed unilateral optic neuritis and angle-closure glaucoma as a result of snake envenomation. Case Report. Approximately 18 hours after envenomation, a 67-year-old female patient described visual impairment and severe pain in her left eye (LE. The patient’s best corrected visual acuity was 10/10 in the RE and hand motion in the LE. Cranial magnetic resonance imaging showed signs of neuropathy in the left optic nerve. In the LE, corneal haziness, closure of the iridocorneal angle, and mild mydriasis were observed and pupillary light reflex was absent. Intraocular pressure was 25 mmHg and 57 mmHg in the RE and LE, respectively. The patient was diagnosed with acute angle-closure glaucoma in the LE. Optic neuropathy was treated with intravenous pulse methylprednisolone. Left intraocular pressure was within normal range starting on the fourth day. One month after the incident, there was no sign of optic neuropathy; relative afferent pupillary defect and optic nerve swelling disappeared. Conclusions. Patients with severe headache and visual loss after snake envenomation must be carefully examined for possible optic neuropathy and angle-closure glaucoma. Early diagnosis and treatment of these cases are necessary to prevent permanent damage to optic nerves.

Imaging of macrophage dynamics with optical coherence tomography in anterior ischemic optic neuropathy.

Science.gov (United States)

Kokona, Despina; Häner, Nathanael U; Ebneter, Andreas; Zinkernagel, Martin S

2017-01-01

Anterior ischemic optic neuropathy (AION) is a relatively common cause of visual loss and results from hypoperfusion of the small arteries of the anterior portion of the optic nerve. AION is the leading cause of sudden optic nerve related vision loss with approximately 10 cases per 100'000 in the population over 50 years. To date there is no established treatment for AION and therefore a better understanding of the events occurring at the level of the optic nerve head (ONH) would be important to design future therapeutic strategies. The optical properties of the eye allow imaging of the optic nerve in vivo, which is a part of the CNS, during ischemia. Experimentally laser induced optic neuropathy (eLiON) displays similar anatomical features as anterior ischemic optic neuropathy in humans. After laser induced optic neuropathy we show that hyperreflective dots in optical coherence tomography correspond to mononuclear cells in histology. Using fluorescence-activated flow cytometry (FACS) we found these cells to peak one week after eLiON. These observations were translated to OCT findings in patients with AION, where similar dynamics of hyperreflective dots at the ONH were identified. Our data suggests that activated macrophages can be identified as hyperreflective dots in OCT. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

Treatment strategies for inherited optic neuropathies: past, present and future

OpenAIRE

Yu-Wai-Man, P; Votruba, M; Moore, A T; Chinnery, P F

2014-01-01

Bilateral visual loss secondary to inherited optic neuropathies is an important cause of registrable blindness among children and young adults. The two prototypal disorders seen in clinical practice are Leber hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (DOA). About 90% of LHON cases are due to one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A, and m.14484T>C, which affect critical complex I subunits of the mitochondrial respiratory chain...

Optic neuropathy following an altitude exposure.

Science.gov (United States)

Steigleman, Allan; Butler, Frank; Chhoeu, Austin; O'Malley, Timothy; Bower, Eric; Giebner, Stephen

2003-09-01

This case report describes a 20-yr-old man who presented with retro-orbital pain and blurred vision in his left eye 3 wk after an altitude exposure in a hypobaric chamber. He was found to have significant deficits in color vision and visual fields consistent with an optic neuropathy in his left eye. The patient was diagnosed with decompression sickness and treated with hyperbaric oxygen with a U.S. Navy Treatment Table VI. All signs and symptoms resolved with a single hyperbaric oxygen treatment but recurred. A head MRI revealed a left frontoethmoid sinus opacity. A concomitant sinusitis was diagnosed. The patient had full resolution of symptoms after a total of four hyperbaric oxygen treatments and antibiotic therapy at 6-wk follow-up. Although a para-infectious etiology for this patient's optic neuropathy cannot be excluded, his history of altitude exposure and significant, rapid response to hyperbaric oxygen treatment strongly implies decompression sickness in this case.

New Treatments for Nonarteritic Anterior Ischemic Optic Neuropathy.

Science.gov (United States)

Foroozan, Rod

2017-02-01

Despite increasing knowledge about the risk factors and clinical findings of nonarteritic anterior ischemic optic neuropathy (NAION), the treatment of this optic neuropathy has remained limited and without clear evidence-based benefit. Historical treatments of NAION are reviewed, beginning with the Ischemic Optic Neuropathy Decompression Trial. More recent treatments are placed within the historical context and illustrate the need for evidence-based therapy for ischemic optic neuropathy. Copyright © 2016 Elsevier Inc. All rights reserved.

Radiation-induced bilateral optic neuropathy in cancer of the nasopharynx. Case failure analysis and a review of the literature

International Nuclear Information System (INIS)

Wijers, O.B.; Levendag, P.C.; Klesman-Bradley, J.; Woudstra, E.; Luyten, G.P.M.; Bakker, B.A.; Freling, N.J.M.

1999-01-01

Case Report: A case history of unanticipated radiation-induced bilateral optic neuropathy, 18 months after induction chemotherapy and radiation therapy for a locally advanced nasopharyngeal carcinoma, is presented. Retrospective reanalysis of the radiation therapy technique, with emphasis on the doses received by the optic pathway structures, was performed. These re-calculations revealed unexpectedly high doses in the range 79 and 82 Gy (cumulative external and brachytherapy dose) at the level of the optic nerves, which explained the observed radiation injury. Conclusion: Routine implementation of computed tomography for 3D dose planning purposes is therefore advocated. Review of the current literature confirms the importance of 3D dose planning in avoiding this complication and highlights the role of MRI in establishing the diagnosis of radiation-induced optic neuropathy. (orig.) [de

Bilateral optic neuropathy in acute cryptococcal meningitis

Institute of Scientific and Technical Information of China (English)

Qi Zhe Ngoo; Li Min Evelyn Tai; Wan Hazabbah Wan Hitam; John Tharakan

2016-01-01

We reported a case of cryptococcal meningitis presenting with bilateral optic neuropathy in an immunocompetent patient. A 64-year-old Malay gentleman with no medical comorbidities presented with acute bilateral blurring of vision for a week, which was associated with generalised throbbing headache and low grade fever. He also had som-nolence and altered consciousness. Visual acuity in both eyes was no perception of light with poor pupillary reflexes. Extraocular muscle movements were normal. Anterior segments were unremarkable bilaterally. Fundoscopy revealed bilateral optic disc swelling. CT scan of the brain showed multifocal infarct, but no meningeal enhancement or mass. Cerebrospinal fluid opening pressure was normal, while its culture grew Cryptococcus neoformans. A diagnosis of cryptococcal meningitis with bilateral optic neuropathy was made. Patient was treated with a six-week course of intravenous flu-conazole and started concomitantly on a fortnight's course of intravenous amphotericin B. After that, his general condition improved, but there was still no improvement in his visual acuity. On reviewing at two months post-initiation of treatment, fundi showed bilateral optic atrophy. Bilateral optic neuropathy secondary to cryptococcal meningitis was rare. The prognosis was guarded due to the sequelae of optic atrophy. Anti-fungal medication alone may not be sufficient to manage this condition. However, evidence for other treatment modalities is still lacking and further clinical studies are required.

Hyperbaric oxygenation was effective in a case of radiation-induced optic neuropathy

International Nuclear Information System (INIS)

Saitoh, Ayumi; Dake, Yoshinori; Amemiya, Tsugio

1995-01-01

A 68-year-old female underwent radiation treatment followed by surgical extirpation for olfactory neuroblastoma in the left ethmoidal sinus. Acute optic neuropathy developed 16 months later in her left eye. The visual acuity was reduced to finger counting at 30 cm. Treatment with systemic corticosteroid and hyperbaric oxygenation for 2 months resulted in improvement in fundus findings and improvement of visual acuity to 0.5. The findings show the potential effectiveness of hyperbaric oxygen therapy for radiation-induced optic neuropathy. (author)

Hyperbaric oxygenation was effective in a case of radiation-induced optic neuropathy

Energy Technology Data Exchange (ETDEWEB)

Saitoh, Ayumi; Dake, Yoshinori; Amemiya, Tsugio [Nagasaki Univ. (Japan). School of Medicine

1995-03-01

A 68-year-old female underwent radiation treatment followed by surgical extirpation for olfactory neuroblastoma in the left ethmoidal sinus. Acute optic neuropathy developed 16 months later in her left eye. The visual acuity was reduced to finger counting at 30 cm. Treatment with systemic corticosteroid and hyperbaric oxygenation for 2 months resulted in improvement in fundus findings and improvement of visual acuity to 0.5. The findings show the potential effectiveness of hyperbaric oxygen therapy for radiation-induced optic neuropathy. (author).

Transnasal Endoscopic Optic Nerve Decompression in Post Traumatic Optic Neuropathy.

Science.gov (United States)

Gupta, Devang; Gadodia, Monica

2018-03-01

To quantify the successful outcome in patients following optic nerve decompression in post traumatic unilateral optic neuropathy in form of improvement in visual acuity. A prospective study was carried out over a period of 5 years (January 2011 to June 2016) at civil hospital Ahmedabad. Total 20 patients were selected with optic neuropathy including patients with direct and indirect trauma to unilateral optic nerve, not responding to conservative management, leading to optic neuropathy and subsequent impairment in vision and blindness. Decompression was done via Transnasal-Ethmo-sphenoidal route and outcome was assessed in form of post-operative visual acuity improvement at 1 month, 6 months and 1 year follow up. After surgical decompression complete recovery of visual acuity was achieved in 16 (80%) patients and partial recovery in 4 (20%). Endoscopic transnasal approach is beneficial in traumatic optic neuropathy not responding to steroid therapy and can prevent permanent disability if earlier intervention is done prior to irreversible damage to the nerve. Endoscopic optic nerve surgery can decompress the traumatic and oedematous optic nerve with proper exposure of orbital apex and optic canal without any major intracranial, intraorbital and transnasal complications.

Properties of pattern standard deviation in open-angle glaucoma patients with hemi-optic neuropathy and bi-optic neuropathy.

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Heo, Dong Won; Kim, Kyoung Nam; Lee, Min Woo; Lee, Sung Bok; Kim, Chang-Sik

2017-01-01

To evaluate the properties of pattern standard deviation (PSD) according to localization of the glaucomatous optic neuropathy. We enrolled 242 eyes of 242 patients with primary open-angle glaucoma, with a best-corrected visual acuity ≥ 20/25, and no media opacity. Patients were examined via dilated fundus photography, spectral-domain optical coherence tomography, and Humphrey visual field examination, and divided into those with hemi-optic neuropathy (superior or inferior) and bi-optic neuropathy (both superior and inferior). We assessed the relationship between mean deviation (MD) and PSD. Using broken stick regression analysis, the tipping point was identified, i.e., the point at which MD became significantly associated with a paradoxical reversal of PSD. In 91 patients with hemi-optic neuropathy, PSD showed a strong correlation with MD (r = -0.973, β = -0.965, p < 0.001). The difference between MD and PSD ("-MD-PSD") was constant (mean, -0.32 dB; 95% confidence interval, -2.48~1.84 dB) regardless of visual field defect severity. However, in 151 patients with bi-optic neuropathy, a negative correlation was evident between "-MD-PSD" and MD (r2 = 0.907, p < 0.001). Overall, the MD tipping point was -14.0 dB, which was close to approximately 50% damage of the entire visual field (p < 0.001). Although a false decrease of PSD usually begins at approximately 50% visual field damage, in patients with hemi-optic neuropathy, the PSD shows no paradoxical decrease and shows a linear correlation with MD.

Leber's Hereditary Optic Neuropathy: A Case Report

Directory of Open Access Journals (Sweden)

Chi-Wu Chang

2003-10-01

Full Text Available Leber's hereditary optic neuropathy (LHON is a maternally inherited mitochondrial disease that primarily affects the optic nerve, causing bilateral vision loss in juveniles and young adults. A 12-year-old boy had complained of blurred vision in both eyes for more than 1 year. His best-corrected visual acuity was 0.08 in the right eye and 0.1 in the left. Ophthalmologic examination showed bilateral optic disc hyperemia and margin blurring, peripapillary telangiectasis, and a relative afferent pupil defect in his right eye. Fluorescein angiography showed no stain or leakage around the optic disc in the late phase. Visual field analysis showed central scotoma in the left eye and a near-total defect in the right. Upon examination of the patient's mitochondrial DNA, a point mutation at nucleotide position 11778 was found, and the diagnosis of LHON was confirmed. Coenzyme Q10 was used to treat the patient.

Nonarteritic anterior ischemic optic neuropathy associated with interferon and ribavirin in a patient with hepatitis C.

Science.gov (United States)

Sharif, Walid; Sheikh, Khayam; De Silva, Ian; Elsherbiny, Samer

2017-04-01

To report a case of a temporal artery biopsy negative anterior ischemic optic neuropathy associated with a recently completed course of pegylated interferon 2 α with ribavirin for chronic hepatitis C. Despite the early presentation with symptoms and prompt treatment with systemic intravenous steroids the patient experienced deterioration of their optic neuropathy over the following few days. Although nonarteritic anterior ischemic optic neuropathy is a common disorder with known risk factors, the timing of onset of symptoms in our patient was suggestive of a possible etiology related to treatment with ribavirin and interferon 2 α, as found in the previously reported cases. There have been a few reported cases of the association between the use of interferon/ribavirin for treatment of chronic hepatitis with nonarteritic anterior ischemic optic neuropathy. In these cases stopping the drug caused some improvement of symptoms or halting the progression of optic neuropathy. Having reviewed the literature on previous cases, we postulate that there may be a dose related reaction to explain the delay and deterioration of vision in some cases despite stopping the drugs. We also advise that any person who is started on this treatment for chronic hepatitis are appropriately counselled as to the potential optic nerve side effect of the drug, based on the evidence reported in the literature.

A Case with Symmetrical Intracranial Calcifications and Systemic Lupus Erythematosus Presenting with Optic Neuropathy

Directory of Open Access Journals (Sweden)

Sibel Güler

2012-06-01

Full Text Available 53 years old female patient were evaluated for decrease in right eye vision with sudden onset. Neurological examination revealed no characteristics except 20/200 visual acuity in right eye, significant hyperemia and edema findings in optical disc. On cranial CT scans, symmetrical calcifications were evident in bilateral cerebellar peduncles, cerebral hemispheres, both putamens and thalamus. Laboratory examinations showed positive ANA as well as positive anti-DNA and lymphopenia and the case was diagnosed as lupus erythematosus. SLE case with bilaterally diffuse cerebral calcification showed additionally unilateral optic neuropathy clinical presentation. Being the first case in the literature with these two rare associations because of lupus makes it much more interesting to report

[A rare cause of optic neuropathy: Cassava].

Science.gov (United States)

Zeboulon, P; Vignal-Clermont, C; Baudouin, C; Labbé, A

2016-06-01

Cassava root is a staple food for almost 500 million people worldwide. Excessive consumption of it is a rare cause of optic neuropathy. Ten patients diagnosed with cassava root related optic neuropathy were included in this retrospective study. Diagnostic criteria were a bilateral optic neuropathy preceded by significant cassava root consumption. Differential diagnoses were excluded through a neuro-ophthalmic examination, blood tests and a brain MRI. All patients had visual field examination and OCT retinal nerve fiber layer (RNFL) analysis as well as an evaluation of their cassava consumption. All patients had a bilateral optic nerve head atrophy or pallor predominantly located into the temporal sector. Visual field defects consisted of a central or cecocentral scotoma for all patients. RNFL showed lower values only in the temporal sector. Mean duration of cassava consumption prior to the appearance of visual symptoms was 22.7±11.2 years with a mean of 2.57±0.53 cassava-based meals per week. Cassava related optic neuropathy is possibly due to its high cyanide content and enabled by a specific amino-acid deficiency. Cassava root chronic consumption is a rare, underappreciated cause of optic neuropathy and its exact mechanism is still uncertain. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Bilateral optic neuropathy in acute cr yptococcal meningitis

Directory of Open Access Journals (Sweden)

Qi Zhe Ngoo

2016-11-01

Full Text Available We reported a case of cryptococcal meningitis presenting with bilateral optic neuropathy in an immunocompetent patient. A 64-year-old Malay gentleman with no medical comorbidities presented with acute bilateral blurring of vision for a week, which was associated with generalised throbbing headache and low grade fever. He also had somnolence and altered consciousness. Visual acuity in both eyes was no perception of light with poor pupillary reflexes. Extraocular muscle movements were normal. Anterior segments were unremarkable bilaterally. Fundoscopy revealed bilateral optic disc swelling. CT scan of the brain showed multifocal infarct, but no meningeal enhancement or mass. Cerebrospinal fluid opening pressure was normal, while its culture grew Cryptococcus neoformans. A diagnosis of cryptococcal meningitis with bilateral optic neuropathy was made. Patient was treated with a six-week course of intravenous fluconazole and started concomitantly on a fortnight's course of intravenous amphotericin B. After that, his general condition improved, but there was still no improvement in his visual acuity. On reviewing at two months post-initiation of treatment, fundi showed bilateral optic atrophy. Bilateral optic neuropathy secondary to cryptococcal meningitis was rare. The prognosis was guarded due to the sequelae of optic atrophy. Anti-fungal medication alone may not be sufficient to manage this condition. However, evidence for other treatment modalities is still lacking and further clinical studies are required.

Melanopsin retinal ganglion cells are resistant to neurodegeneration in mitochondrial optic neuropathies

DEFF Research Database (Denmark)

La Morgia, C; Ross-Cisneros, F.N.; Sadun, A.A.

2010-01-01

Mitochondrial optic neuropathies, that is, Leber hereditary optic neuropathy and dominant optic atrophy, selectively affect retinal ganglion cells, causing visual loss with relatively preserved pupillary light reflex. The mammalian eye contains a light detection system based on a subset of retinal...... ganglion cells containing the photopigment melanopsin. These cells give origin to the retinohypothalamic tract and support the non-image-forming visual functions of the eye, which include the photoentrainment of circadian rhythms, light-induced suppression of melatonin secretion and pupillary light reflex...... subjects as in controls, indicating that the retinohypothalamic tract is sufficiently preserved to drive light information detected by melanopsin retinal ganglion cells. We then investigated the histology of post-mortem eyes from two patients with Leber hereditary optic neuropathy and one case...

Epidemic optic neuropathy in Cuba. Eye findings.

Science.gov (United States)

Sadun, A A; Martone, J F; Muci-Mendoza, R; Reyes, L; DuBois, L; Silva, J C; Roman, G; Caballero, B

1994-05-01

To characterize and establish a clinical definition of the optic neuropathy that appeared in epidemic form in Cuba in 1992 and 1993. At the invitation of the Cuban Ministry of Health, Havana, members of ORBIS International and the Pan American Health Organization, assembled teams that traveled to Cuba in May 1993. We were initially briefed by Cuban national experts in the areas of virology, nutrition, toxicology, ophthalmology, neurology, and public health. We then examined 20 patients on our own. Thirteen of these patients underwent a comprehensive neuro-ophthalmologic examination, including neurologic examination, ophthalmologic examination, visual fields, optic nerve function studies, contrast sensitivity studies, and funduscopy. We returned 4 months later to perform an additional 12 comprehensive neuro-ophthalmologic and follow-up examinations. Only seven of the 13 patients who were alleged to have the optic form of the epidemic and who were rigorously and systematically examined on the first visit demonstrated a bilateral optic neuropathy. These seven patients had several features that included decreased visual acuity, poor color vision, central scotomas, decreased contrast sensitivity, saccadic eye movements, and most prominent and distinctive of all, nerve fiber layer wedge defects of the papillomacular bundle. Our clinical definition was then implemented by the Cuban ophthalmologists and epidemiologists. On returning 4 months later, we found that all newly presented patients were correctly diagnosed to have the epidemic disease. With the new case definition and the application of a few simple psychophysical tests, the false-positive rate of diagnosis became much lower. After vitamin therapy, we reexamined the patients seen on our initial visit, and all showed marked improvement. The Cuban epidemic was characterized by an optic neuropathy with features that were similar to those of tobacco/alcohol amblyopia and Leber's optic atrophy. Recent political

Alterations of the outer retina in non-arteritic anterior ischaemic optic neuropathy detected using spectral-domain optical coherence tomography.

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Ackermann, Philipp; Brachert, Maike; Albrecht, Philipp; Ringelstein, Marius; Finis, David; Geerling, Gerd; Aktas, Orhan; Guthoff, Rainer

2017-07-01

A characteristic disease pattern may be reflected by retinal layer thickness changes in non-arteritic anterior ischaemic optic neuropathy measured using spectraldomain optical coherence tomography. Retinal layer segmentation is enabled by advanced software. In this study, retinal layer thicknesses in acute and chronic non-arteritic anterior ischaemic optic neuropathy were compared. A single-centre cross-sectional analysis was used. A total of 27 patients (20 age-matched healthy eyes) were included: 14 with acute (optic neuropathy. Macular volume and 12° peripapillary ring optical coherence tomography scans were used. The peripapillary thicknesses of the following layers were determined by manual segmentation: retinal nerve fibres, ganglion cells + inner plexiform layer, inner nuclear layer + outer plexiform layer, outer nuclear layer + inner segments of the photoreceptors and outer segments of the photoreceptors to Bruch's membrane. Macular retinal layer thicknesses were automatically determined in volume cubes centred on the fovea. Peripapillary retinal swelling in acute nonarteritic anterior ischaemic optic neuropathy was attributable to retinal nerve fibre layer, ganglion cell layer/inner plexiform layer and outer nuclear layer/segments of the photoreceptors thickening. In chronic cases, peripapillary retinal nerve fibre layer, macular ganglion cell layer and inner plexiform layer thinning were observed. In acute non-arteritic anterior ischaemic optic neuropathy, the inner and outer peripapillary retinal layers are affected by thickness changes. In chronic cases, atrophy of the ganglion cells and their axons and dendrites is evident by inner retinal layer thinning. © 2017 Royal Australian and New Zealand College of Ophthalmologists.

Using spectral-domain optical coherence tomography to detect optic neuropathy in patients with craniosynostosis.

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Dagi, Linda R; Tiedemann, Laura M; Heidary, Gena; Robson, Caroline D; Hall, Amber M; Zurakowski, David

2014-12-01

Detecting and monitoring optic neuropathy in patients with craniosynostosis is a clinical challenge due to limited cooperation, and subjective measures of visual function. The purpose of this study was to appraise the correlation of peripapillary retinal nerve fiber layer (RNFL) thickness measured by spectral-domain ocular coherence tomography (SD-OCT) with indication of optic neuropathy based on fundus examination. The medical records of all patients with craniosynostosis presenting for ophthalmic evaluation during 2013 were retrospectively reviewed. The following data were abstracted from the record: diagnosis, historical evidence of elevated intracranial pressure, current ophthalmic evaluation and visual field results, and current peripapillary RNFL thickness. A total of 54 patients were included (mean age, 10.6 years [range, 2.4-33.8 years]). Thirteen (24%) had evidence of optic neuropathy based on current fundus examination. Of these, 10 (77%) demonstrated either peripapillary RNFL elevation and papilledema or depression with optic atrophy. Sensitivity for detecting optic atrophy was 88%; for papilledema, 60%; and for either form of optic neuropathy, 77%. Specificity was 94%, 90%, and 83%, respectively. Kappa agreement was substantial for optic atrophy (κ = 0.73) and moderate for papilledema (κ = 0.39) and for either form of optic neuropathy (κ = 0.54). Logistic regression indicated that peripapillary RNFL thickness was predictive of optic neuropathy (P optic neuropathy than visual field testing (likelihood ratio = 10.02; P = 0.002). Sensitivity and specificity of logMAR visual acuity in detecting optic neuropathy were 15% and 95%, respectively. Peripapillary RNFL thickness measured by SD-OCT provides adjunctive evidence for identifying optic neuropathy in patients with craniosynostosis and appears more sensitive at detecting optic atrophy than papilledema. Copyright © 2014 American Association for Pediatric Ophthalmology and Strabismus. Published by

Nonarteritic anterior ischemic optic neuropathy: cause, effect, and management

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Berry S

2017-09-01

Full Text Available Shauna Berry,1 Weijie V Lin,2 Ama Sadaka,1 Andrew G Lee1–7 1Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, Houston, TX, USA; 2Department of Ophthalmology, Baylor College of Medicine, Houston, TX, USA; 3Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch (UTMB, Galveston, TX, USA; 4Department of Ophthalmology, 5Department of Neurology, 6Department of Neurosurgery, Weill Cornell Medicine, Houston, TX, USA; 7Department of Ophthalmology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Abstract: Nonarteritic anterior ischemic optic neuropathy (NAION is the most common form of ischemic optic neuropathy and the second most common optic neuropathy. Patients are generally over the age of 50 years with vasculopathic risk factors (eg, diabetes mellitus, hypertension, and obstructive sleep apnea. The exact mechanism of NAION is not fully understood. In addition, several treatment options have been proposed. This article summarizes the current literature on the diagnosis, treatment, and management of NAION. Keywords: anterior ischemic optic neuropathy, nonarteritic anterior ischemic optic neuropathy, ischemic optic neuropathy

Longitudinal relationship between traumatic brain injury and the risk of incident optic neuropathy: A 10-year follow-up nationally representative Taiwan survey.

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Chen, Ying-Jen; Liang, Chang-Min; Tai, Ming-Cheng; Chang, Yun-Hsiang; Lin, Tzu-Yu; Chung, Chi-Hsiang; Lin, Fu-Huang; Tsao, Chang-Huei; Chien, Wu-Chien

2017-10-17

Accumulating evidences had shown that traumatic brain injury was associated with visual impairment or vision loss. However, there were a limited number of empirical studies regarding the longitudinal relationship between traumatic brain injury and incident optic neuropathy. We studied a cohort from the Taiwanese National Health Insurance data comprising 553918 participants with traumatic brain injury and optic neuropathy-free in the case group and 1107836 individuals without traumatic brain injury in the control group from 1st January 2000. After the index date until the end of 2010, Cox proportional hazards analysis was used to compare the risk of incident optic neuropathy. During the follow-up period, case group was more likely to develop incident optic neuropathy (0.24%) than the control group (0.11%). Multivariate Cox regression analysis demonstrated that the case group had a 3-fold increased risk of optic neuropathy (HR = 3.017, 95% CI = 2.767-3.289, p optic neuropathy. Our study provided evidence of the increased risk of incident optic neuropathy after traumatic brain injury during a 10-year follow-up period. Patients with traumatic brain injury required periodic and thorough eye examinations for incident optic neuropathy to prevent potentially irreversible vision loss.

Toxocara optic neuropathy: clinical features and ocular findings.

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Choi, Kwang-Dong; Choi, Jae-Hwan; Choi, Seo-Young; Jung, Jae Ho

2018-01-01

We evaluated thirteen eyes of twelve patients diagnosed clinically and serologically with Toxocara optic neuropathy. Eleven patients had unilateral involvement and one patient had bilateral optic neuropathy. Eight patients (66.7%) had a possible infection source to Toxocara. Six patients (50%) had painless acute optic neuropathy. Ten eyes had asymmetric, sectorial optic disc edema with peripapillary infiltration and three eyes had diffuse optic disc edema. Eosinophilia was noted in five patients (41.7%) and optic nerve enhancement was observed in eight of eleven eyes (72.7%) with available orbit magnetic resonance imaging (MRI). Mean visual acuity significantly improved following treatment [mean logarithmic of the minimum angle of resolution (logMAR) 0.94±0.56 at baseline and 0.47±0.59 at the final ( P =0.02)]. Asymmetric optic disc edema with a peripapillary lesion and a history of raw meat ingestion were important clues for diagnosing Toxocara optic neuropathy. Additionally, Toxocara IgG enzyme-linked immunosorbent assay (ELISA) test and evaluating eosinophil may be helpful for diagnosis.

Toxocara optic neuropathy: clinical features and ocular findings

Science.gov (United States)

Choi, Kwang-Dong; Choi, Jae-Hwan; Choi, Seo-Young; Jung, Jae Ho

2018-01-01

We evaluated thirteen eyes of twelve patients diagnosed clinically and serologically with Toxocara optic neuropathy. Eleven patients had unilateral involvement and one patient had bilateral optic neuropathy. Eight patients (66.7%) had a possible infection source to Toxocara. Six patients (50%) had painless acute optic neuropathy. Ten eyes had asymmetric, sectorial optic disc edema with peripapillary infiltration and three eyes had diffuse optic disc edema. Eosinophilia was noted in five patients (41.7%) and optic nerve enhancement was observed in eight of eleven eyes (72.7%) with available orbit magnetic resonance imaging (MRI). Mean visual acuity significantly improved following treatment [mean logarithmic of the minimum angle of resolution (logMAR) 0.94±0.56 at baseline and 0.47±0.59 at the final (P=0.02)]. Asymmetric optic disc edema with a peripapillary lesion and a history of raw meat ingestion were important clues for diagnosing Toxocara optic neuropathy. Additionally, Toxocara IgG enzyme-linked immunosorbent assay (ELISA) test and evaluating eosinophil may be helpful for diagnosis. PMID:29600190

Optic neuropathy in a patient with pyruvate dehydrogenase deficiency

Energy Technology Data Exchange (ETDEWEB)

Small, Juan E. [Massachusetts General Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States); Gonzalez, Guido E. [Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Radiology, Boston, MA (United States); Clinica Alemana de Santiago, Departmento de Imagenes, Santiago (Chile); Nagao, Karina E.; Walton, David S. [Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Ophthalmology, Boston, MA (United States); Caruso, Paul A. [Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Radiology, Boston, MA (United States)

2009-10-15

Pyruvate dehydrogenase (PDH) deficiency is a genetic disorder of mitochondrial metabolism. The clinical manifestations range from severe neonatal lactic acidosis to chronic neurodegeneration. Optic neuropathy is an uncommon clinical sequela and the imaging findings of optic neuropathy in these patients have not previously been described. We present a patient with PDH deficiency with bilateral decreased vision in whom MRI demonstrated bilateral optic neuropathy and chiasmopathy. (orig.)

Optic neuropathy in a patient with pyruvate dehydrogenase deficiency

International Nuclear Information System (INIS)

Small, Juan E.; Gonzalez, Guido E.; Nagao, Karina E.; Walton, David S.; Caruso, Paul A.

2009-01-01

Pyruvate dehydrogenase (PDH) deficiency is a genetic disorder of mitochondrial metabolism. The clinical manifestations range from severe neonatal lactic acidosis to chronic neurodegeneration. Optic neuropathy is an uncommon clinical sequela and the imaging findings of optic neuropathy in these patients have not previously been described. We present a patient with PDH deficiency with bilateral decreased vision in whom MRI demonstrated bilateral optic neuropathy and chiasmopathy. (orig.)

Radiation-induced optic neuropathy 4 years after radiation: report of a case followed up with MRI

International Nuclear Information System (INIS)

Piquemal, R.; Renard, J.P.; Cottier, J.P.; Herbreteau, D.; Arsene, S.; Rospars, C.; Lioret, E.; Jan, M.

1998-01-01

We report a case of radiation-induced optic neuropathy in a 32-year-old man with Cushing's disease and a recurrent tumour of the left cavernous sinus. The patient experienced rapid, painless loss of vision 4 years after treatment without recurrence of tumour or other visual disorder. MRI showed enlargement and contrast enhancement of the optic chiasm. A year later the patient was almost blind and MRI showed atrophy and persistent contrast enhancement of the chiasm. (orig.)

Anterior ischemic optic neuropathy in patients undergoing hemodialysis

NARCIS (Netherlands)

DoorenbosBot, ACC; Geerlings, W; Houtman, IA

Four patients are discussed who underwent hemodialysis and developed anterior ischemic optic neuropathy (AION). Three patients had been treated by hemodialysis for several years. One patient developed bilateral optic neuropathy after the first hemodialysis session, So far, only four hemodialysis

Non-glaucomatous optic neuropathy in Ibadan: Extrapolations to ...

African Journals Online (AJOL)

Background: Optic neuropathy is not a diagnosis in itself, as potential aetiologies are myriad. A pilot study conducted in the Eye Clinic, University College Hospital, Ibadan, between September 2007 and. November 2009, showed that 46.8% of new cases presenting to the neuroophthalmology unit, had non-glaucomatous ...

Radiation-induced optic neuropathy 4 years after radiation: report of a case followed up with MRI

Energy Technology Data Exchange (ETDEWEB)

Piquemal, R.; Renard, J.P. [Service de Medecine Interne A, Hopital Bretonneau, Tours (France); Cottier, J.P.; Herbreteau, D. [Service de Neuroradiologie, Hopital Bretonneau, Tours (France); Arsene, S.; Rospars, C. [Service d`Ophthalmologie, Hopital Bretonneau, Tpurs (France); Lioret, E.; Jan, M. [Service de Neurochirurgie, Hopital Bretonneau, Tours (France)

1998-07-01

We report a case of radiation-induced optic neuropathy in a 32-year-old man with Cushing`s disease and a recurrent tumour of the left cavernous sinus. The patient experienced rapid, painless loss of vision 4 years after treatment without recurrence of tumour or other visual disorder. MRI showed enlargement and contrast enhancement of the optic chiasm. A year later the patient was almost blind and MRI showed atrophy and persistent contrast enhancement of the chiasm. (orig.) With 3 figs., 13 refs.

Acute optic neuropathy associated with a novel MFN2 mutation.

Science.gov (United States)

Leonardi, Luca; Marcotulli, Christian; Storti, Eugenia; Tessa, Alessandra; Serrao, Mariano; Parisi, Vincenzo; Santorelli, F M; Pierelli, Francesco; Casali, Carlo

2015-07-01

Mutations in the mitofusin 2 (MFN2) gene cause CMT2A the most common form of autosomal dominant axonal Charcot-Marie-Tooth (CMT). In addition, mutations in MFN2 have been shown to be responsible for Hereditary Motor Sensory Neuropathy type VI (HSMN VI), a rare early-onset axonal CMT associated with optic neuropathy. Most reports of HMSN VI presented with a sub-acute form of optic neuropathy. Herein, we report a CMT2A patient, who developed very rapidly progressing severe optic neuropathy. A 40-year-old Caucasian man was evaluated for gait disturbance and lower limbs weakness, slowly progressed over the last 2 years. Due to clinical data and family history, a diagnosis of CMT2 was made. The novel heterozygous c.775C > T (p.Arg259Cys) mutation in MFN2 was detected in the patient and his clinical affected mother. Interestingly, the patient developed a severe sudden bilateral visual deterioration few years early, with clinical and instrumental picture suggestive of acute bilateral optic neuropathy. Our report expands the spectrum of MFN2-related manifestation because it indicates that visual symptoms of HMSN VI may enter in the differential with acquired or hereditary acute optic neuropathies, and that severe optic neuropathy is not invariably an early manifestation of the disease but may occur as disease progressed. This report could have an impact on clinicians who evaluate patients with otherwise unexplainable bilateral acute-onset optic neuropathy, especially if associated with a motor and sensory axonal neuropathy.

Nonarteritic anterior ischemic optic neuropathy: cause, effect, and management.

Science.gov (United States)

Berry, Shauna; Lin, Weijie V; Sadaka, Ama; Lee, Andrew G

2017-01-01

Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common form of ischemic optic neuropathy and the second most common optic neuropathy. Patients are generally over the age of 50 years with vasculopathic risk factors (eg, diabetes mellitus, hypertension, and obstructive sleep apnea). The exact mechanism of NAION is not fully understood. In addition, several treatment options have been proposed. This article summarizes the current literature on the diagnosis, treatment, and management of NAION.

MULTIFOCAL RETINAL INFILTRATES WITH PHLEBITIS AND OPTIC NEUROPATHY IN AN HIV-POSITIVE PEDIATRIC PATIENT.

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Kasi, Sundeep K; Vora, Robin A; Martin, Taliva; Cunningham, Emmett T

2015-01-01

To describe an unusual presentation of bilateral HIV-associated multifocal retinal infiltrates with phlebitis and optic neuropathy in a pediatric patient from Zimbabwe, Africa. Retrospective case report of a 15-year-old boy from Zimbabwe, Africa. The patient was found to have bilateral vitritis, multifocal retinitis with phlebitis, and optic neuropathy in the setting of previously unrecognized HIV infection. Vision improved and the clinical findings resolved after treatment with intravenous corticosteroids and highly active retroviral therapy (HAART). The authors describe the occurrence and treatment of bilateral, HIV-associated multifocal retinal infiltrates with phlebitis and HIV-associated optic neuropathy in a pediatric patient from Zimbabwe, Africa.

Linezolid-induced optic neuropathy with a rare pathological change in the inner retina.

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Ishii, Nobuhito; Kinouchi, Reiko; Inoue, Masatomo; Yoshida, Akitoshi

2016-12-01

We report a case of linezolid-induced optic neuropathy with transient microcystic spaces in the inner retina. We observed the retina using Fourier-domain optical coherence tomography (FD-OCT) in a patient with linezolid-induced optic neuropathy. A 49-year-old woman presented to our department with a 1-week history of bilateral photophobia. At the first visit, her best-corrected visual acuity (VA) was 0.6 in the right eye and 0.5 in the left eye. She had moderate optic disk edema and central scotomas bilaterally. FD-OCT showed bilateral microcystic spaces in the retina. Microcystic spaces were seen in the retinal nerve fiber layer (RNFL) and at the border of the RNFL and the retinal ganglion cell layer. Magnetic resonance imaging and laboratory tests showed no positive findings except for an elevated lactic acid level. One week after the first visit, the VA levels decreased to 0.06 and 0.07 in the right and left eyes, respectively. Because the patient had a 7-month history of linezolid treatment for persistent pyogenic arthritis, we suspected linezolid-induced optic neuropathy and immediately terminated treatment with this drug. The optic disk edema and the microcystic spaces in the retina resolved, and the VA improved to 1.2 at 6 weeks after linezolid withdrawal. Microcystic spaces, which resolved with linezolid withdrawal, were observed in linezolid-induced optic neuropathy. The microcystic spaces in the inner retina can be the first retinal sign of some optic neuropathies.

A case of radiation optic neuropathy after resection of a pituitary adenoma

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Yoshii, Masaru; Okisaka, Shigekuni; Yanagawa, Youichi; Takiguchi, Hiroshi [National Defense Medical Coll., Tokorozawa, Saitama (Japan)

1995-06-01

A case of optic neuropathy after postoperative radiation therapy is reported. A 69-year-old woman had a partial resection of a pituitary adenoma in 1990 and was treated with 45 Gy of irradiation to the postoperative pituitary lesion for one month. Seven months later she had sudden right visual field loss. Goldmann perimetry examination revealed remarked visual field defect in her right eye with visual acuity of 1.0. The right relative afferent pupillary defect was positive. The value of critical flicker fusion for her right eye was reduced and the amplitude of steady-state pattern-reversal visually evoked cortical potential was significantly less for the right monocular stimulation than that for the fellow eye stimulation, but Ganzfeld electroretinograms were normal for both eyes. Magnetic resonance imaging using Gadolinium-diethylene triaminepenta-acetic acid revealed enhancement on the right optic nerve, which had not been recognized immediately after the radiation therapy, without any suggestion of right optic nerve compression by the residual pituitary adenoma. (author).

A case of radiation optic neuropathy after resection of a pituitary adenoma

International Nuclear Information System (INIS)

Yoshii, Masaru; Okisaka, Shigekuni; Yanagawa, Youichi; Takiguchi, Hiroshi

1995-01-01

A case of optic neuropathy after postoperative radiation therapy is reported. A 69-year-old woman had a partial resection of a pituitary adenoma in 1990 and was treated with 45 Gy of irradiation to the postoperative pituitary lesion for one month. Seven months later she had sudden right visual field loss. Goldmann perimetry examination revealed remarked visual field defect in her right eye with visual acuity of 1.0. The right relative afferent pupillary defect was positive. The value of critical flicker fusion for her right eye was reduced and the amplitude of steady-state pattern-reversal visually evoked cortical potential was significantly less for the right monocular stimulation than that for the fellow eye stimulation, but Ganzfeld electroretinograms were normal for both eyes. Magnetic resonance imaging using Gadolinium-diethylene triaminepenta-acetic acid revealed enhancement on the right optic nerve, which had not been recognized immediately after the radiation therapy, without any suggestion of right optic nerve compression by the residual pituitary adenoma. (author)

Optic neuropathy following combined proton and photon radiotherapy for base of skull tumors

International Nuclear Information System (INIS)

Kim, June; Munzenrider, John; Maas, Alicea; Finkelstein, Dianne; Liebsch, Norbert; Hug, Eugen; Suit, Herman; Smith, Al; Goitein, Michael

1997-01-01

Purpose/Objective: To evaluate the risk of radiation injury to the optic pathway following high dose radiation therapy (RT) for base of skull tumors with regard to the following variables: diabetes, hypertension, number of surgical procedures, use of patch, patch distance, radiation dose, and volume of optic structures receiving 50, 55, or 60 Cobalt Gray Equivalent (CGE). Materials and Methods: A total of 359 patients with base of skull chordoma or low grade chondrosarcoma received high dose radiation therapy. Patients were treated with external beam radiotherapy utilizing protons alone or combined protons and photons. Protons of 160 MeV were delivered at the Harvard Cyclotron Laboratory using a modulated Bragg peak. The tumor dose ranged from 61 to 76 CGE. CGE was used because modulated protons have an RBE of 1.1 compared to 60 Co. Among 359 patients, 85 patients were excluded from evaluation based on age, tumor location, and pre-RT treatment criteria. All 274 evaluable patients had a minimum follow up of 12 months. Medical records were reviewed to determine the actual cause of vision changes. A total of 12 patients with grade II, III, and IV radiation-induced optic neuropathy were identified. Twenty-four patients without complications who closely matched the aforementioned 12 cases with optic neuropathy were selected from the 274 patients as a control group. Dose volume histograms of 12 cases and 24 controls were reviewed to determine minimum, median, and maximum dose to the optic apparatus as well as dose volume at 50, 55, and 60 CGE. Other information regarding remaining potential risk factors, such as diabetes, hypertension, number of surgical procedures, use of patch, and patch distance, was also obtained. Results: A total of 12 patients (4.4%) developed radiation-induced optic neuropathy: 1 grade II, 9 grade III, and 2 grade IV. Specific sites of involvement were left optic nerve in 9, right optic nerve in 5, and chiasm in 4 cases. The duration to the onset

On the many faces of Leber hereditary optic neuropathy

NARCIS (Netherlands)

Oostra, RJ; Tijmes, NT; Cobben, JM; Bolhuis, PA; vanNesselrooij, BPM; Houtman, WA; deKokNazaruk, MM; BleekerWagemakers, EM

Leber hereditary optic neuropathy (LHON) is a maternally inherited disorder, associated with mutations in the mitochondrial DNA, which is notorious for its aspecific presentations. Two pedigrees are described with cases that are atypical for LHON with respect to sex, age of onset, interval between

On the many faces of Leber hereditary optic neuropathy

NARCIS (Netherlands)

Oostra, R. J.; Tijmes, N. T.; Cobben, J. M.; Bolhuis, P. A.; van Nesselrooij, B. P.; Houtman, W. A.; de Kok-Nazaruk, M. M.; Bleeker-Wagemakers, E. M.

1997-01-01

Leber hereditary optic neuropathy (LHON) is a maternally inherited disorder, associated with mutations in the mitochondrial DNA, which is notorious for its aspecific presentations. Two pedigrees are described with cases that are atypical for LHON with respect to sex, age of onset, interval between

Color Doppler imaging of the retrobulbar circulation in progressive glaucoma optic neuropathy.

Science.gov (United States)

Magureanu, Marineta; Stanila, Adriana; Bunescu, Liviu Valentin; Armeanu, Cristina

2016-01-01

It is known that elevated intraocular pressure (IOP) is the primary risk factor for glaucoma. Recently, more and more evidences have shown that the vascular deficit also plays an important role in the pathogenesis and progressions of glaucomatous optic neuropathy. This issue is backed up by glaucomatous optic neuropathy (GON) cases drug compensated in which the progression of the disease in one or both eyes is ascertained despite a normal and relatively constant IOP. The present study evaluated the hemodynamic parameters in the retrobulbar circulation in patients with progressive glaucomatous optic neuropathy in one eye, who received compensated medication. The hemodynamic parameters (PSV, EDV, IR) were measured by using color Doppler ultrasound and progression was evaluated by a repeated automated perimetry. The obtained values were statistically analyzed and compared with those obtained for the stable eye.

Evaluation of acute radiation optic neuropathy by B-scan ultrasonography

International Nuclear Information System (INIS)

Lovato, A.A.; Char, D.H.; Quivey, J.M.; Castro, J.R.

1990-01-01

We studied the accuracy of B-scan ultrasonography to diagnose radiation-induced optic neuropathy in 15 patients with uveal melanoma. Optic neuropathy was diagnosed by an observer masked as to clinical and photographic data. We analyzed planimetry area measurements of the retrobulbar nerve before and after irradiation. The retrobulbar area of the optic nerve shadow on B-scan was quantitated with a sonic digitizer. Increased optic nerve shadow area was confirmed in 13 of 15 patients who had radiation optic neuropathy (P less than .004). The correct diagnosis was confirmed when the results of ultrasound were compared to fundus photography and fluorescein angiography. In 13 patients there was acute radiation optic neuropathy. Two patients did not show an enlarged retrobulbar optic nerve, and the clinical appearance suggested early progression to optic atrophy. Ultrasonography documents the enlargement of the optic nerve caused by acute radiation changes

Superior ophthalmic vein enlargement and increased muscle index in dysthyroid optic neuropathy.

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Lima, Breno da Rocha; Perry, Julian D

2013-01-01

To compare superior ophthalmic vein diameter and extraocular muscle index in patients with thyroid eye disease with or without optic neuropathy. High-resolution CT scan images of 40 orbits of 20 patients with history of thyroid eye disease (with or without optic neuropathy), who underwent orbital decompression surgery from January 2007 to November 2009, were retrospectively reviewed. Superior ophthalmic vein diameter was measured in coronal and axial planes. Extraocular muscle index was calculated according to the method proposed by Barrett et al. The clinical diagnosis of optic neuropathy was based on characteristic signs that included afferent pupillary defect, decreased visual acuity, visual field defects, and dyschromatopsia. Orbits were divided in 2 groups based on presence or absence of optic neuropathy. Superior ophthalmic vein diameter was significantly higher in orbits with concomitant optic neuropathy (mean 2.4 ± 0.4mm, p optic neuropathy (mean 57.9% ± 5.7%, p = 0.0002). Muscle index greater than 50% was present in all patients with dysthyroid optic neuropathy. This study suggests that patients with thyroid eye disease with enlarged superior ophthalmic vein and increased extraocular muscle index are more likely to have concomitant optic neuropathy.

Subacute peripheral and optic neuropathy syndrome with no evidence of a toxic or nutritional cause.

Science.gov (United States)

Allen, D; Riordan-Eva, P; Paterson, R W; Hadden, R D M

2013-08-01

The syndrome of subacute simultaneous peripheral neuropathy and bilateral optic neuropathy is known to occur in tropical countries, probably due to malnutrition or toxicity, but not often seen in developed countries. We report seven patients in London who were not malnourished or alcoholic, and in whom no clear cause was found. We retrospectively reviewed the case notes and arranged some further investigations. All patients developed peripheral and bilateral optic neuropathy within 6 months. Patients were aged 30-52, and all of Jamaican birth and race but lived in the UK. Most had subacute, painful ataxic sensory axonal neuropathy or neuronopathy, some with myelopathy. Nerve conduction studies revealed minor demyelinating features in two cases. The optic neuropathy was symmetrical, subacute and monophasic, usually with marked reduction in visual acuity. CSF protein concentration was usually elevated but other laboratory investigations were normal. Patients showed only modest improvement at follow-up. These patients share a common clinical and electrophysiological phenotype, age, ethnicity and elevated CSF protein, but otherwise normal laboratory investigations. The syndrome is a cause of significant morbidity in young people. The cause remains uncertain despite thorough investigation. Copyright © 2013 Elsevier B.V. All rights reserved.

Anterior ischemic optic neuropathy after conventional coronary artery bypass graft surgery.

Science.gov (United States)

Dorecka, Mariola; Miniewicz-Kurkowska, Joanna; Romaniuk, Dorota; Gajdzik-Gajdecka, Urszula; Wójcik-Niklewska, Bogumiła

2011-06-01

Perioperative optic neuropathy is a disease which can lead to serious, irreversible damage of vision. This complication could be the result of non-ocular surgery, for example, cardiac or spinal procedures. We present a case of anterior ischemic neuropathy (AION) which occurred following a conventional coronary artery bypass graft procedure. A 57-year-old man, 4 days after Conventional Coronary Artery Bypass Graft surgery as result of multi-vessel stabile coronary artery disease and history of anterolateral wall myocardial infarction, was admitted to the Eye Clinic due to significant loss of vision in his right eye. The patient had hypertension and was a heavy smoker. On admission, the slit lamp examination revealed a relative afferent pupillary defect in the right eye. The fundus examination showed optic disc edema with the presence of flame hemorrhages. Best corrected visual acuity (BCVA) was 0.02. The results of eye examination and fluorescein angiography confirmed the diagnosis of AION. Anti-aggregation and antithrombotic treatment was continued with steroids and vasodilators. After 7 days of this treatment we noticed the improvement of BCVA to 0.2. At 6-month follow-up, the vision was stable, and fundus examination revealed optic disc atrophy. After cardiac surgical operations, such as coronary artery bypass graft procedures, anterior ischemic optic neuropathy may occur. In those cases, close cooperation between the various specialists is necessary.

Quantitative comparison of disc rim color in optic nerve atrophy of compressive optic neuropathy and glaucomatous optic neuropathy.

Science.gov (United States)

Nakano, Eri; Hata, Masayuki; Oishi, Akio; Miyamoto, Kazuaki; Uji, Akihito; Fujimoto, Masahiro; Miyata, Manabu; Yoshimura, Nagahisa

2016-08-01

The purpose was to investigate an objective and quantitative method to estimate the redness of the optic disc neuroretinal rim, and to determine the usefulness of this method to differentiate compressive optic neuropathy (CON) from glaucomatous optic neuropathy (GON). In our study there were 126 eyes: 40 with CON, 40 with normal tension glaucoma (NTG), and 46 normal eyes (NOR). Digital color fundus photographs were assessed for the redness of disc rim color using ImageJ software. We separately measured the intensity of red, green, and blue pixels from RGB images. Three disc color indices (DCIs), which indicate the redness intensity, were calculated through existing formulas. All three DCIs of CON were significantly smaller than those of NOR (P  -6 dB), in which the extent of retinal nerve fiber layer thinning is comparable, the DCIs of mild CON were significantly smaller than those of mild NTG (P optic disc color was useful in differentiating early-stage CON from GON and NOR.

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review.

Science.gov (United States)

Siemerink, Martin J; Freling, Nicole J M; Saeed, Peerooz

2017-10-01

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.

[Clinical feature of chronic compressive optic neuropathy without optic atrophy].

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Jiang, Libin; Shi, Jitong; Liu, Wendong; Kang, Jun; Wang, Ningli

2014-12-01

To investigate the clinical feature of the chronic compressive optic neuropathy without optic atrophy. Retrospective cases series study. The clinical data of 25 patients (37 eyes) with chronic compressive optic neuropathy without optic atrophy, treated in Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, from October, 2005 to March, 2014, were collected. Those patients had been showing visual symptoms for 6 months or longer, but missed diagnosed or misdiagnosed as other eye diseases due to their normal or slightly changed fundi. The collected data including visual acuities, visual fields, neuroimaging and/or pathologic diagnosis were analyzed. Among the 25 patients, there were 5 males and 20 females, and their ages range from 9 to 74 years [average (47.5 ± 13.4) years]. All patients suffered progressive impaired vision in single eye or both eyes, without exophthalmos or abnormal eye movements. Except one patient had a headache, other patients did not show systemic symptoms. The corrected visual acuities were between HM to 1.0, and their appearances of optic discs and colors of fundi were normal. After neuroimaging and/or pathological examination, it was proven that 14 patients suffered tuberculum sellae meningiomas, 5 patients with hypophysoma, 3 patient with optic nerve sheath meningioma in orbital apex, 1 patient with cavernous hemangioma, 1 patient with vascular malformation in orbital apex and 1 patient with optic nerve glioma. Among the 19 patients whose suffered occupied lesions of saddle area, 14 patients underwent visual field examinations, and only 4 patients showed classic visual field defects caused by optic chiasmal lesions. Occult progressive visual loss was the most important clinical feature of the disease.

Bilateral Ischemic Optic Neuropathy Developed under Interferon Therapy

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Fatih Selcukbiricik

2012-01-01

Full Text Available Introduction. Interferon is a glycoprotein produced by assigned cells of immune system. It has been used in many different diseases. Although flu-like syndrome, myalgia, rash, hypotension, thrombocytopenia and peripheral neuropathy due to interferon use are encountered frequently, ocular side effects are rare, generally mild and transient. Case Report. 47-year-old female patient, presented with a mass lesion in right renal pelvis. Right radical nephrectomy was applied and the histopathological examination was consistent with papillary renal cell carcinoma. Interferon alpha treatment was started subcutaneously at the dose of 5 MIU/3 times in a week. Four weeks after the interferon therapy, suddenly bilateral visual loss developed. We discussed the diagnosis, followup, and treatment of the patient who developed irreversible ischemic optic neuropathy and had no previous known primary systemic disease to cause this condition. Conclusion. We suggest that patients should be screened for risk factors causing optic ischemic neuropathy, before interferon therapy. Although there was no adequate information in the literature for the followup, patients should be monitorized before, 1 month after, and 2 months after the treatment. And if there is no complication, we suggest that they should be followed up at 3-month intervals.

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review

NARCIS (Netherlands)

Siemerink, Martin J.; Freling, Nicole J. M.; Saeed, Peerooz

2017-01-01

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and

Posterior Ischemic Optic Neuropathy following Herpes Zoster Ophthalmicus

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Mohammad Pakravan

2009-01-01

Full Text Available

PURPOSE: To report a case of posterior ischemic optic neuropathy (PION following herpes zoster ophthalmicus (HZO. CASE REPORT: A 58-year-old woman with history of recent HZO in her right eye presented with acute painless loss of vision in the same eye to no light perception. Examination revealed a positive relative afferent pupillary defect and a normal appearing optic disc. Inflammatory and infiltrative lesions of the optic nerve were ruled out by laboratory and imaging studies. The patient received systemic acyclovir and prednisolone. Three months later, visual acuity improved to counting fingers, but the optic disc became pale and atrophic leading to a presumptive diagnosis of PION. Considering the positive PCR test for varicella zoster virus and the short time interval between the two presentations, HZO was considered as the most probable cause of the optic neuropathy. CONCLUSION: Herpes zoster ophthalmicus can be associated with PION.

Increased Mortality and Comorbidity Associated With Leber's Hereditary Optic Neuropathy

DEFF Research Database (Denmark)

Vestergaard, Nanna; Rosenberg, Thomas; Torp-Pedersen, Christian

2017-01-01

Purpose: Leber's hereditary optic neuropathy (LHON) is a mitochondrial genetic disease in which optic neuropathy is considered a key feature. Several other manifestations of LHON have been reported; however, only little is known of their incidence and the life expectancy in LHON patients. Methods...... patients (RR: 4.26, 95% CI: 1.91-9.48; P neuropathy, and alcohol-related disorders. Conclusions: The manifestation of LHON was associated...

Nonarteritic anterior ischemic optic neuropathy following pars plana vitrectomy for macular hole treatment: case report.

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Cunha, Leonardo Provetti; Cunha, Luciana Virgínia Ferreira Costa; Costa, Carolina Ferreira; Monteiro, Mário Luiz Ribeiro

2016-01-01

Herein, we report a case of nonarteritic anterior ischemic optic neuropathy (NAION) following uneventful pars plana vitrectomy for macular hole treatment. A 56-year-old previously healthy woman presented with a full-thickness macular hole in right eye (OD) and small cup-to-disc ratios in both eyes. Five days after surgery, she noticed sudden painless loss of vision in OD and was found to have an afferent pupillary defect and intraocular pressure of 29 mmHg. Fundus examination showed right optic disc edema and the resolution of a macular hole with an inferior altitudinal visual field defect. Erythrocyte sedimentation rate, C-reactive protein levels, and general physical examination findings were normal. She was treated with hypotensive eyedrops and oral prednisone, resulting in mild visual improvement and a pale optic disc. A combination of face-down position and increased intraocular pressure due to a small optic disc cup were considered as potential mechanisms underlying NAION in the present case. Vitreoretinal surgeons should be aware of NAION as a potentially serious complication and be able to recognize associated risk factors and clinical findings.

Objective evaluation of improvement in optic neuropathy following radiation therapy for thyroid eye disease

International Nuclear Information System (INIS)

Rush, Stephen; Winterkorn, Jacqueline; Zak, Rochelle

2000-01-01

Purpose: While the literature supports the use of radiation therapy for thyroid eye disease, it does not sufficiently describe in detail the results of radiation therapy for optic neuropathy associated with thyroid eye disease. The objective of this study is to quantify the changes in parameters of optic neuropathy after orbital irradiation for thyroid eye disease. Methods and Materials: Twelve consecutive patients with optic neuropathy from thyroid eye disease were followed by a single neuro-ophthalmology practice and treated by one radiation oncologist with radiation therapy from 1991 through 1995. All cases were prospectively followed for visual acuity, color vision, mean deviation, and/or foveal sensitivity and afferent pupillary defect. All patients received 2000 cGy in 10 fractions with megavoltage irradiation to the orbits. Results: Ten of 12 patients were evaluated for follow-up (one moved out of this country and one had a stroke, which confounded interpretation of examination results). An analysis was performed retrospectively while treatment and evaluation remained uniform. Five men and five women formed the basis of this study with a median age of 60 years (35-76 years). Nineteen eyes were evaluated for thyroid optic neuropathy. Improvement in optic nerve function occurred in eight of ten patients. Improvement was seen either during radiotherapy or within 2 weeks of completion. No long-term adverse effects were noted. Conclusion: This study objectively demonstrates improvement in optic neuropathy from radiation therapy for thyroid eye disease

A case of radiation optic neuropathy after irradiation for pituitary adenoma

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Ohta, Masahiro; Sasaki, Ushio; Shinohara, Nobuya; Takeda, Tetsuji; Chaki, Takanori; Nishigakiuchi, Keiji; Kusunoki, Katsusuke (Ehime Prefectural Central Hospital, Matsuyama (Japan))

1992-05-01

A 60-year-old woman with radiation optic neuropathy 21 months after irradiation is reported. The patient received a total dose of 50 Gy in 25 fractions for 39 days for pituitary adenoma. She presented with bitemporal hemianopsia and loss of recent memory. Gadolinium-enhanced T1-weighted imaging was very useful for detecting lesions in the optic nerves and chiasm to the hypothalamus including mamillary bodies. Two-month steroid therapy was effective in preventing the disease progression, although visual loss and loss of recent memory were not improved. (N.K.).

A case of radiation optic neuropathy after irradiation for pituitary adenoma

International Nuclear Information System (INIS)

Ohta, Masahiro; Sasaki, Ushio; Shinohara, Nobuya; Takeda, Tetsuji; Chaki, Takanori; Nishigakiuchi, Keiji; Kusunoki, Katsusuke

1992-01-01

A 60-year-old woman with radiation optic neuropathy 21 months after irradiation is reported. The patient received a total dose of 50 Gy in 25 fractions for 39 days for pituitary adenoma. She presented with bitemporal hemianopsia and loss of recent memory. Gadolinium-enhanced T1-weighted imaging was very useful for detecting lesions in the optic nerves and chiasm to the hypothalamus including mamillary bodies. Two-month steroid therapy was effective in preventing the disease progression, although visual loss and loss of recent memory were not improved. (N.K.)

Reduced risk of compressive optic neuropathy using orbital radiotherapy in patients with active thyroid eye disease.

Science.gov (United States)

Shams, Pari N; Ma, Roy; Pickles, Tom; Rootman, Jack; Dolman, Peter J

2014-06-01

To compare the risk of developing compressive optic neuropathy in patients with active thyroid eye disease (TED) treated with corticosteroids with or without orbital radiotherapy. Retrospective single-center case-control study. The clinical charts of 351 patients with active TED who received corticosteroids with or without orbital radiotherapy between 1999 and 2010 were reviewed. Patients with compressive optic neuropathy at the time of presentation were excluded. Group 1 received corticosteroids only and Group 2 received corticosteroids as well as orbital radiotherapy. The primary outcome measure was the development of compressive optic neuropathy. Secondary outcome measures were changes in other parameters indicating the activity of TED, including soft tissue inflammation, diplopia, ocular motility restriction, and appearance. There were 144 cases in Group 1 and 105 in Group 2. Both groups were matched for age, sex, and stability of thyroid function. The 2 groups differed only in the modality of treatment for active TED. The main indication for treatment in both groups was soft tissue inflammation. Corticosteroids were initiated an average of 2.6 months following symptom onset in Group 1 and 2.5 months in Group 2. Group 2 received orbital radiotherapy on average 4.2 months following the initiation of corticosteroid therapy and 8% (9/105) were intolerant to corticosteroids. At an average of 3.2 years follow-up, compressive optic neuropathy had developed in 17% (25/144) of Group 1 and 0% of Group 2 (P optic neuropathy was significantly lower and improvement in ocular motility greater in patients receiving orbital radiotherapy in addition to corticosteroids. Patients with active TED appear to have an effective and sustained response to orbital radiotherapy combined with corticosteroids that is protective against disease progression and the development of compressive optic neuropathy. Copyright © 2014 Elsevier Inc. All rights reserved.

Mitochondrial optic neuropathies – Disease mechanisms and therapeutic strategies

Science.gov (United States)

Yu-Wai-Man, Patrick; Griffiths, Philip G.; Chinnery, Patrick F.

2011-01-01

Leber hereditary optic neuropathy (LHON) and autosomal-dominant optic atrophy (DOA) are the two most common inherited optic neuropathies in the general population. Both disorders share striking pathological similarities, marked by the selective loss of retinal ganglion cells (RGCs) and the early involvement of the papillomacular bundle. Three mitochondrial DNA (mtDNA) point mutations; m.3460G>A, m.11778G>A, and m.14484T>C account for over 90% of LHON cases, and in DOA, the majority of affected families harbour mutations in the OPA1 gene, which codes for a mitochondrial inner membrane protein. Optic nerve degeneration in LHON and DOA is therefore due to disturbed mitochondrial function and a predominantly complex I respiratory chain defect has been identified using both in vitro and in vivo biochemical assays. However, the trigger for RGC loss is much more complex than a simple bioenergetic crisis and other important disease mechanisms have emerged relating to mitochondrial network dynamics, mtDNA maintenance, axonal transport, and the involvement of the cytoskeleton in maintaining a differential mitochondrial gradient at sites such as the lamina cribosa. The downstream consequences of these mitochondrial disturbances are likely to be influenced by the local cellular milieu. The vulnerability of RGCs in LHON and DOA could derive not only from tissue-specific, genetically-determined biological factors, but also from an increased susceptibility to exogenous influences such as light exposure, smoking, and pharmacological agents with putative mitochondrial toxic effects. Our concept of inherited mitochondrial optic neuropathies has evolved over the past decade, with the observation that patients with LHON and DOA can manifest a much broader phenotypic spectrum than pure optic nerve involvement. Interestingly, these phenotypes are sometimes clinically indistinguishable from other neurodegenerative disorders such as Charcot-Marie-Tooth disease, hereditary spastic

The significance of computed tomography in optic neuropathy

International Nuclear Information System (INIS)

Awai, Tsugumi; Yasutake, Hirohide; Ono, Yoshiko; Kumagai, Kazuhisa; Kairada, Kensuke

1981-01-01

Computed tomography (CT scan) has become one of the important and useful modes of examination for ophthalmological and neuro-ophthalmological disorders. CT scan (EMI scan) was performed on 21 patients with optic neuropathy in order to detect the cause. Of these 21 patients, the CT scan was abnormal in six. These six patients were verified, histopathologically, as having chromophobe pituitary adenoma, craniopharyngioma, plasmocytoma from sphenoidal sinus, optic nerve glioma and giant aneurysma of anterior communicating artery. The practical diagnostic value of CT scan for optic neuropathy is discussed. (author)

Mitochondrial DNA analysis as a diagnostic tool in singleton cases of Leber's hereditary optic neuropathy

NARCIS (Netherlands)

Oostra, R. J.; Bolhuis, P. A.; Bleeker-Wagemakers, E. M.

1993-01-01

Leber's hereditary optic neuropathy (LHON) is characterized by subacute loss of central vision due to bilateral optic nerve atrophy accompanied by several nonspecific clinical findings. The only pathognomonic feature is its strictly maternal inheritance. It was therefore impossible to establish the

Radiation optic neuropathy after external beam radiation therapy for acromegaly: report of two cases

International Nuclear Information System (INIS)

Bergh, Alfons C.M. van den; Hoving, Marjanke A.; Links, Thera P.; Dullaart, Robin P.F.; Ranchor, Adelita V.; Weeme, Cees A. ter; Canrinus, Alof A.; Szabo, Ben G.; Pott, Jan-Willem R.

2003-01-01

For diagnosing radiation optic neuropathy (RON) ophthalmological and imaging data were evaluated from 63 acromegalic patients, irradiated between 1967 and 1998. Two patients developed RON: one patient in one optic nerve 10 years and another patient in both optic nerves 5 months after radiation therapy. RON is a rare complication after external beam radiation therapy for acromegaly, which can occur after a considerable latency period

Optic neuropathy causing vertical unilateral hemianopsia after pars plana vitrectomy for a macular hole: A case report.

Science.gov (United States)

Kawashima, Hirohiko; Nagai, Norihiro; Shinoda, Hajime; Tsubota, Kazuo; Ozawa, Yoko

2018-04-01

Recent progress in medical technology has resulted in improved surgical outcomes of pars plana vitrectomy (PPV); with microincision systems, the incidence of procedure-related complications during surgery has been reduced. However, unpredictable visual field defects after PPV remain an unresolved issue. A few reports have shown that damage to the retinal neurofibers owing to dry-up during air/fluid exchange or retinal neurotoxicity of the dye used to visualize the internal limiting membrane (ILM), as well as unintentional removal of retinal neurofibers during ILM peeling, are responsible for such visual field disorders. In this report, we present a case of extensive visual field defect due to optic neuropathy exhibiting vertical hemianopsia after PPV. A 50-year-old woman underwent PPV and cataract surgery for a macular hole and mild cataract under retrobulbar anesthesia with 3.5 mL of xylocaine. At the time of opening an infusion cannula for PPV, the intraocular lens was herniating, with an acute increase in pressure from the posterior eyeball; thus, intraocular pressure configuration level had to be decreased from the default level, whereas the other procedures including 20% SF6 injection were performed without any modification. The macular hole was closed postoperatively. However, the patient experienced nasal hemianopsia, which turned out to be optic neuropathy, as assessed via electric physiological examinations. The pattern of the visual field defect was not typical for glaucoma or anterior ischemic optic neuropathy. Her optic nerve head was pale at the temporal side soon after the surgery, and her blood pressure was low, suggesting that there may have been a congestion of the optic nerve feeder vessels because of the relatively high pressure in the orbit. The space occupancy with xylocaine and extensively stretched and plumped out eye ball with infusion during PPV may have pressed the surrounding tissue of the optic nerve and the feeder vessels. PPV is safe

Radiation-induced bilateral optic neuropathy in cancer of the nasopharynx. Case failure analysis and a review of the literature

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Wijers, O.B.; Levendag, P.C.; Klesman-Bradley, J.; Woudstra, E. [University Hospital Rotterdam (Netherlands). Dept. of Radiation Oncology; Luyten, G.P.M. [University Hospital Rotterdam (Netherlands). Dept. of Ophthalmology; Bakker, B.A. [Hospital De Baronie, Breda (Netherlands). Dept. of Ophthalmology; Freling, N.J.M. [University Hospital Rotterdam (Netherlands). Dept. of Radiology

1999-01-01

Case Report: A case history of unanticipated radiation-induced bilateral optic neuropathy, 18 months after induction chemotherapy and radiation therapy for a locally advanced nasopharyngeal carcinoma, is presented. Retrospective reanalysis of the radiation therapy technique, with emphasis on the doses received by the optic pathway structures, was performed. These re-calculations revealed unexpectedly high doses in the range 79 and 82 Gy (cumulative external and brachytherapy dose) at the level of the optic nerves, which explained the observed radiation injury. Conclusion: Routine implementation of computed tomography for 3D dose planning purposes is therefore advocated. Review of the current literature confirms the importance of 3D dose planning in avoiding this complication and highlights the role of MRI in establishing the diagnosis of radiation-induced optic neuropathy. (orig.) [Deutsch] Kasuistik: Ein Fallbericht mit unerwarteter strahleninduzierter beidseitiger Optikusneuropathie 18 Monate nach eingeleiteter Chemo- und Strahlentherapie wegen eines lokal fortgeschrittenen Nasopharynxkarzinoms wird praesentiert. Eine retrospektive Analyse der Radiotherapietechnik mit Berechnung der Dosisbelastung der Sehbahnstrukturen wurde durchgefuehrt. Aus dieser Berechnung wurde deutlich, dass die Komplikation durch eine unerwartet hohe Dosis in den Sehnerven (79 bis 82 Gy) verursacht wurde. Schlussfolgerung: Eine routinemaessige Durchfuehrung einer Computertomographie fuer die dreidimensionale (Dosis-)Planung wird befuerwortet. Ein Rueckblick auf die aktuelle Literatur bestaetigt die Notwendigkeit einer dreidimensionalen (Dosis-)Planung, um diese Komplikationen zu vermeiden. Die Rolle des MRI beim Nachweis der Diagnose der strahleninduzierten Optikusneuropathie ist hervorzuheben. (orig.)

Anomalous coagulation factors in non-arteritic anterior ischemic optic neuropathy with central retinal vein occlusion: A case report.

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Kim, Ji Hong; Kang, Min Ho; Seong, Mincheol; Cho, Heeyoon; Shin, Yong Un

2018-04-01

Non-arteritic anterior ischemic optic neuropathy (NAION) is characterized by sudden, painless visual loss and optic disc edema. NAION occurs mainly in the presence of cardiovascular disease and hypercoagulability, mainly in patients over 50 years of age. We experienced a case of NAION associated with central retinal vein occlusion (CRVO) in a young man with no underlying disease. A 46-year-old man was referred to our clinic following a sudden loss of vision in his right eye. The patient exhibited no underlying disease and reported no ongoing medication. Significant visual loss and visual disturbance of the right eye were observed. The pupil of the right eye was enlarged and an afferent pupillary defect was observed. On fundus examination, retinal hemorrhage was observed in the peripheral retina; macular edema was observed in optical coherence tomography analysis. However, optic disc edema was not evident. No abnormal findings were found in routine blood tests for hypercoagulability. After 3 days of steroid intravenous injection, macular edema disappeared and visual acuity was improved, but optic disc edema began to appear. One week later, optic disc edema was evident and visual acuity was significantly reduced; thus, the patient was diagnosed with NAION. In fluorescein angiography, peripheral retinal ischemia was observed, suggesting that CRVO was complicated. Blood tests, including analysis of coagulation factors, were performed again, showing that coagulation factors IX and XI were increased. Anomalous coagulation factors in non-arteritic anterior ischemic optic neuropathy with central retinal vein occlusion. Systemic steroids were administered. One month later, optic disc edema and retinal hemorrhage gradually diminished and eventually disappeared; however, visual acuity did not recover. In young patients without underlying disease, cases of NAION require careful screening for coagulation disorders. Even if there is no abnormality in the test for routine

Could Buerger's disease cause nonarteritic anterior ischemic optic neuropathy?: a rare case report.

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Korkmaz, Anil; Karti, Omer; Top Karti, Dilek; Yüksel, Bora; Zengin, Mehmet Ozgur; Kusbeci, Tuncay

2018-04-05

We present an interesting case with nonarteritic anterior ischemic optic neuropathy (NAION) accompanied by Buerger's disease. A 43-year-old man was referred to our neuro-ophthalmology clinic with a complaint of visual deterioration in the left eye that started 5 days ago. He suffered from Buerger's disease, and he had acute pain in the right lower limb below the knee. His best corrected visual acuity was 10/10 in the right eye and 2/10 in the left eye by Snellen chart. There was a relative afferent pupil defect in the left eye. The right optic disc was normal on fundus examination, and blurring, hemorrhagic swelling was found at the left optic disc. Inferior altitudinal visual field defect was observed in the left eye. Neurological examination was normal. Computed tomography angiography scan revealed occlusion in the right posterior tibial artery. Brain imaging and laboratory tests such as blood analyses, genetic screening, coagulation, and lipid panels were unremarkable. NAION may occur in patients with Buerger's disease, but it is extremely rare. Therefore, clinicians should be aware of this rare association.

Pupillometric evaluation of the melanopsin containing retinal ganglion cells in mitochondrial and non-mitochondrial optic neuropathies

DEFF Research Database (Denmark)

Ba-Ali, Shakoor; Lund-Andersen, Henrik

2017-01-01

of pupillary light reflex is primarily driven by the ipRGCs. Optic neuropathies i.e. Leber hereditary optic neuropathy (LHON), autosomal dominant optic atrophy (ADOA), nonarteritic anterior ischemic optic neuropathy (NAION), glaucoma, optic neuritis and idiopathic intracranial hypertension (IIH) are among...... the diseases, which have been subject to pupillometric studies. The ipRGCs are differentially affected in these various optic neuropathies. In mitochondrial optic neuropathies, the ipRGCs are protected against degeneration, whereas in glaucoma, NAION, optic neuritis and IIH the ipRGCs are damaged. Here, we...... will review the results of pupillometric, histopathological and animal studies evaluating the ipRGCs in mitochondrial and non-mitochondrial optic neuropathies....

Screening for Electrophysiological Abnormalities in Chronic Hepatitis C Infection: Peripheral Neuropathy and Optic Neuropathy.

Science.gov (United States)

Köşkderelioğlu, Aslı; Ortan, Pınar; Ari, Alpay; Gedizlioğlu, Muhteşem

2016-03-01

To investigate the existence of peripheral and optic neuropathies in asymptomatic individuals with hepatitis C infection. Thirty consecutive patients who were followed in a hepatitis C outpatient clinic were recruited for electrophysiological evaluation together with 30 age- and gender-compatible healthy controls. All patients had a detailed neurological examination. The information regarding the disease duration and management with interferons were collected. Nerve conduction studies and visual evoked potentials (VEP) were recorded in all subjects. The results of the patient and control groups were statistically compared. Of the patients with hepatitis C infection, 16 were females and 14 males. The mean age was 57.5 years, and the average disease duration was 6.43 years. The P100 latencies in the patient group were within normal limits, while the amplitudes were meaningfully small by comparison with the controls. There were some abnormalities in the nerve conduction studies of 15 patients. Sensorial neuropathy was detected in two patients, sensorimotor polyneuropathy in four, carpal tunnel syndrome in seven, and carpal tunnel syndrome and sensorimotor polyneuropathy as comorbid states in another two patients. The nerve conduction studies and VEP parameters were entirely normal in the control group. Hepatitis C-related neurological abnormalities may occur both in the central and peripheral nervous system. Mononeuritis multiplex, sensorial axonal neuropathy, and multiple mononeuropathies are some of the presentations of the peripheral nervous system involvement. The mode of infection is considered to be via vasculitic mechanisms. In addition, optic neuropathy is a known complication of interferon treatment. Autoantibodies, cytokines, chemokines, and cryoglobulins are accused to play roles in the pathogenesis. In this study, we investigated the involvement of the peripheral nervous system and optic nerves in a group of patients with hepatitis C. The results were in

Bilateral vision loss due to Leber's hereditary optic neuropathy after long-term alcohol, nicotine and drug abuse.

Science.gov (United States)

Maass, Johanna; Matthé, Egbert

2018-04-01

Leber's hereditary optic neuropathy is relatively rare, and no clinical pathognomonic signs exist. We present a rare case of bilateral vision loss of a patient with multiple drug abuse in the history. A 31-year-old man presented with a history of progressive, decreased vision in both eyes for 6 month. On examination, his visual acuity was hand motion in both eyes. Funduscopy demonstrated a temporal pallor of the optic disc. Goldmann visual field perimetry showed a crescent visual field in the right eye and a circular decrease to less than 50 ° in the left eye. Electroretinogram showed a scotopic b-wave amplitude reduction. Optical coherence tomographies, Heidelberg Retina tomography, visual evoked potentials, and magnetic resonance imaging with contrast as well as blood tests were normal. The patient reported to consume various kinds of drugs as well as recreational drug use and alcohol consumption since he was 16 years old. We started a hemodilution therapy, believing the patient suffered from a bilateral, toxic optic neuropathy due to his lifestyle. Laboratory results later on showed Leber's hereditary optic neuropathy. Leber's hereditary optic neuropathy is a rare disease without a typical, pathognomonic presentation. Even though the patient gave good reasons for a toxic optic neuropathy, one should never stop to test for other diseases.

Pathological Confirmation of Optic Neuropathy in Familial Dysautonomia.

Science.gov (United States)

Mendoza-Santiesteban, Carlos E; Palma, Jose-Alberto; Hedges, Thomas R; Laver, Nora V; Farhat, Nada; Norcliffe-Kaufmann, Lucy; Kaufmann, Horacio

2017-03-01

Clinical data suggest that optic neuropathy and retinal ganglion cell loss are the main cause of visual decline in patients with familial dysautonomia, but this has not previously been confirmed by pathological analyses. We studied retinas and optic nerves in 6 eyes from 3 affected patients obtained at autopsy. Analyses included routine neurohistology and immunohistochemistry for neurofilaments, cytochrome c oxidase (COX), and melanopsin-containing ganglion cells. We observed profound axon loss in the temporal portions of optic nerves with relative preservation in the nasal portions; this correlated with clinical and optical coherence tomography findings in 1 patient. Retinal ganglion cell layers were markedly reduced in the central retina, whereas melanopsin-containing ganglion cells were relatively spared. COX staining was reduced in the temporal portions of the optic nerve indicating reduced mitochondrial density. Axonal swelling with degenerating lysosomes and mitochondria were observed by electron microscopy. These findings support the concept that there is a specific optic neuropathy and retinopathy in patients with familial dysautonomia similar to that seen in other optic neuropathies with mitochondrial dysfunction. This raises the possibility that defective expression of the IkB kinase complex-associated protein (IKAP) resulting from mutations in IKBKAP affects mitochondrial function in the metabolism-dependent retinal parvocellular ganglion cells in this condition. © 2017 American Association of Neuropathologists, Inc. All rights reserved.

Anterior ischemic optic neuropathy in association with optic nervehead drusen

Directory of Open Access Journals (Sweden)

Bharathi Megur

2014-01-01

Full Text Available Optic nerve head drusen (ONHD are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration′s (TVO, the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.

A retrospective review of 26 cases of dysthyroid optic neuropathy

International Nuclear Information System (INIS)

Panzo, G.J.; Tomsak, R.L.

1983-01-01

Sixteen patients (14 women and two men) with dysthyroid optic neuropathy (26 involved eyes) were treated with either oral corticosteroids, orbital irradiation, surgical orbital decompression, combined corticosteroids and irradiation, or combined corticosteroids and surgical decompression. Thirteen of 16 eyes responded favorably to corticosteroid therapy but eight of the 13 relapsed upon discontinuation of treatment. Two of four eyes responded to irradiation initially but later relapsed. The response to orbital decompression was almost uniformly beneficial (eight of nine eyes responded) and lasting in all. Combined modes of therapy offered no additional advantage

Recovery of Visual Function in a Patient with an Onodi Cell Mucocele Compressive Optic Neuropathy Who Had a 5-Week Interval between Onset and Surgical Intervention: A Case Report

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Wencan Wu

2010-01-01

Full Text Available Purpose. To report on a patient with compressive optic neuropathy secondary to an Onodi cell mucocele, who fully recovered visual function following surgery. Method. Case report. Results. A 28-year-old male was admitted with a right visual acuity of 20/100 following treatment for an initial diagnosis of optic neuritis. Subsequent examination suggested compressive optic neuropathy, and neuroimaging confirmed the presence of an Onodi mucocele compressing the optic nerve. The patient underwent a right endonasal sphenoethmoidectomy with decompression 5 weeks after the initial onset of symptoms. Three weeks following surgery, the visual acuity was 20/20, and there was complete resolution of the visual field defect, which has remained stable at 1 year. Conclusion. Onodi cell mucocele should be included in the differential diagnosis of a young patient with compressive optic neuropathy. Surgical decompression should be considered even when symptoms have been present for over a month.

Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

International Nuclear Information System (INIS)

Park, Ju Young; Lee, In Ho; Song, Chang June; Hwang, Hee Youn

2012-01-01

A 57-year-old woman experienced bilateral acute ischemic optic neuropathy after spine surgery. Routine MR imaging sequence, T2-weighted image, showed subtle high signal intensity on bilateral optic nerves. A contrast-enhanced T1 weighted image showed enhancement along the bilateral optic nerve sheath. Moreover, diffusion-weighted image (DWI) and an apparent diffusion coefficient map showed markedly restricted diffusion on bilateral optic nerves. Although MR findings of T2-weighted and contrast enhanced T1-weighted images may be nonspecific, the DWI finding of cytotoxic edema of bilateral optic nerves will be helpful for the diagnosis of acute ischemic optic neuropathy after spine surgery.

Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

Energy Technology Data Exchange (ETDEWEB)

Park, Ju Young; Lee, In Ho; Song, Chang June [Chungnam National University Hospital, Daejeon (Korea, Republic of); Hwang, Hee Youn [Eulji University Hospital, Daejeon(Korea, Republic of)

2012-03-15

A 57-year-old woman experienced bilateral acute ischemic optic neuropathy after spine surgery. Routine MR imaging sequence, T2-weighted image, showed subtle high signal intensity on bilateral optic nerves. A contrast-enhanced T1 weighted image showed enhancement along the bilateral optic nerve sheath. Moreover, diffusion-weighted image (DWI) and an apparent diffusion coefficient map showed markedly restricted diffusion on bilateral optic nerves. Although MR findings of T2-weighted and contrast enhanced T1-weighted images may be nonspecific, the DWI finding of cytotoxic edema of bilateral optic nerves will be helpful for the diagnosis of acute ischemic optic neuropathy after spine surgery.

Is there treatment for nonarteritic anterior ischemic optic neuropathy.

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Katz, David M; Trobe, Jonathan D

2015-11-01

Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common cause of an acute optic neuropathy over the age 50 with an annual incidence of 2-10/100 000. Most patients are left with a permanent decrease in visual acuity and visual field loss. No approved treatment has conclusively reversed the process or prevented a second event that typically involves the previously unaffected eye. Many medical and surgical treatments have been proposed with conflicting results. The goal of this review is to present current data in order to permit clinicians and patients to make an educated decision about treatment. Recently, there has been a flurry of case reports, small clinical trials and testing in animal models of NAION for various treatments for NAION and this review attempts to present the data concisely with the authors' opinions about the reliability of the data. To date, there is no class I evidence of benefit for the treatment of NAION; however, the aphorism attributed to Carl Sagan, PhD aptly applies: 'Absence of evidence is not evidence of absence'.

Relative Frequencies of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy in an Arab Population.

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Gruener, Anna M; Chang, Jessica R; Bosley, Thomas M; Al-Sadah, Zakeya M; Kum, Clarissa; McCulley, Timothy J

2017-12-01

To evaluate the relative frequencies of arteritic and nonarteritic anterior ischemic optic neuropathy (AION) in an Arab population and to compare and contrast these findings with known epidemiological data from Caucasian populations. A retrospective review of the medical records of all patients diagnosed with AION at the King Khaled Eye Specialist Hospital (KKESH) in Riyadh, Saudi Arabia, between 1997 and 2012. Of 171 patients with AION, 4 had biopsy-proven giant-cell arteritis (GCA). The relative frequencies of arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION) in this Arab cohort were 2.3% and 97.7%, respectively. The relative frequencies of arteritic anterior ischemic optic neuropathy and nonarteritic anterior ischemic optic neuropathy differ between Arab and North American clinic-based populations, with giant-cell arteritis-related ischemia being much less frequent in Saudi Arabia.

Evolution of optic nerve and retina alterations in a child with indirect traumatic neuropathy as assessed by optical coherence tomography

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Julia Dutra Rossetto

Full Text Available ABSTRACT Herein, we describe the case of a 4-year-old child with indirect traumatic optic neuropathy and serial changes of the optic nerve head and retinal nerve fiber layer (RNFL documented using optical coherence tomography (OCT. Visual acuity improved despite progressive RNFL thinning and optic disc pallor. We concluded that OCT may be useful for monitoring axonal loss but may not predict the final visual outcome.

Nonarteritic ischemic optic neuropathy secondary to severe ocular hypertension masked by interface fluid in a post-LASIK eye.

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Pham, Mai T; Peck, Rachel E; Dobbins, Kendall R B

2013-06-01

We report a case of ischemic optic neuropathy arising from elevated intraocular pressure (IOP) masked by interface fluid in a post-laser in situ keratomileusis (LASIK) eye. A 51-year-old man, who had had LASIK 6 years prior to presentation, sustained blunt trauma to the left eye that resulted in a hyphema and ocular hypertension. Elevated IOP resulted in accumulation of fluid in the stromal bed-LASIK flap interface, leading to underestimation of IOP when measured centrally over the flap. After days of unrecognized ocular hypertension, ischemic optic neuropathy developed. To our knowledge, this is the first reported case of ischemic optic neuropathy resulting from underestimated IOP measurements in a post-LASIK patient. It highlights the inaccuracy of IOP measurements in post-LASIK eyes and a vision-threatening potential complication. No author has a financial or proprietary interest in any material or method mentioned. Copyright © 2013 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.

The metabolomic signature of Leber's hereditary optic neuropathy reveals endoplasmic reticulum stress.

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Chao de la Barca, Juan Manuel; Simard, Gilles; Amati-Bonneau, Patrizia; Safiedeen, Zainab; Prunier-Mirebeau, Delphine; Chupin, Stéphanie; Gadras, Cédric; Tessier, Lydie; Gueguen, Naïg; Chevrollier, Arnaud; Desquiret-Dumas, Valérie; Ferré, Marc; Bris, Céline; Kouassi Nzoughet, Judith; Bocca, Cinzia; Leruez, Stéphanie; Verny, Christophe; Miléa, Dan; Bonneau, Dominique; Lenaers, Guy; Martinez, M Carmen; Procaccio, Vincent; Reynier, Pascal

2016-11-01

Leber's hereditary optic neuropathy (MIM#535000), the commonest mitochondrial DNA-related disease, is caused by mutations affecting mitochondrial complex I. The clinical expression of the disorder, usually occurring in young adults, is typically characterized by subacute, usually sequential, bilateral visual loss, resulting from the degeneration of retinal ganglion cells. As the precise action of mitochondrial DNA mutations on the overall cell metabolism in Leber's hereditary optic neuropathy is unknown, we investigated the metabolomic profile of the disease. High performance liquid chromatography coupled with tandem mass spectrometry was used to quantify 188 metabolites in fibroblasts from 16 patients with Leber's hereditary optic neuropathy and eight healthy control subjects. Latent variable-based statistical methods were used to identify discriminating metabolites. One hundred and twenty-four of the metabolites were considered to be accurately quantified. A supervised orthogonal partial least squares discriminant analysis model separating patients with Leber's hereditary optic neuropathy from control subjects showed good predictive capability (Q 2cumulated = 0.57). Thirty-eight metabolites appeared to be the most significant variables, defining a Leber's hereditary optic neuropathy metabolic signature that revealed decreased concentrations of all proteinogenic amino acids, spermidine, putrescine, isovaleryl-carnitine, propionyl-carnitine and five sphingomyelin species, together with increased concentrations of 10 phosphatidylcholine species. This signature was not reproduced by the inhibition of complex I with rotenone or piericidin A in control fibroblasts. The importance of sphingomyelins and phosphatidylcholines in the Leber's hereditary optic neuropathy signature, together with the decreased amino acid pool, suggested an involvement of the endoplasmic reticulum. This was confirmed by the significantly increased phosphorylation of PERK and eIF2α, as well as

Bilateral optic neuropathy in a patient with familial amyloidotic polyneuropathy

DEFF Research Database (Denmark)

Hamann, Steffen; Jensen, Peter Koch; Fledelius, Hans Callø

2013-01-01

Amyloidogenic transthyretin (ATTR)-related familial amyloidotic polyneuropathy (FAP) is an autosomal-dominant hereditary disease characterised by slowly progressive peripheral sensorimotor and autonomic neuropathy and tissue involvement of the heart, kidneys and central nervous system. Secondary...... ATTR Val30Met mutation. After 11 years of ophthalmic follow-up best-corrected visual acuity was 20/100 in his seeing eye, which further had visual field findings suggestive of optic neuropathy. This was also the diagnosis underlying the preceding insidious full loss of vision in the fellow eye......, with colour Doppler imaging to support an ischaemic aetiology. To our knowledge, this is the first report of ischaemic optic neuropathy in this familial amyloid disorder....

Unilateral anterior ischemic optic neuropathy

DEFF Research Database (Denmark)

Herbst, Kristina; Sander, Birgit; Lund-Andersen, Henrik

2013-01-01

of this study was to investigate the ipRGC mediated pupil response in patients with a unilateral non-arteritic anterior ischemic optic neuropathy (NAION). Consensual pupil responses during and after exposure to continuous 20 s blue (470 nm) or red (660 nm) light of high intensity (300 cd/m(2)) were recorded...

Hyperbaric oxygen in the treatment of radiation-induced optic neuropathy

International Nuclear Information System (INIS)

Guy, J.; Schatz, N.J.

1986-01-01

Four patients with radiation-induced optic neuropathies were treated with hyperbaric oxygen. They had received radiation therapy for treatment of pituitary tumors, reticulum cell sarcoma, and meningioma. Two presented with amaurosis fugax before the onset of unilateral visual loss and began hyperbaria within 72 hours after development of unilateral optic neuropathy. Both had return of visual function to baseline levels. The others initiated treatment two to six weeks after visual loss occurred in the second eye and had no significant improvement of vision. Treatment consisted of daily administration of 100% oxygen under 2.8 atmospheres of pressure for 14-28 days. There were no medical complications of hyperbaria. While hyperbaric oxygen is effective in the treatment of radiation-induced optic neuropathy, it must be instituted within several days of deterioration in vision for restoration of baseline function

Diagnostic ability of Barrett's index to detect dysthyroid optic neuropathy using multidetector computed tomography

International Nuclear Information System (INIS)

Monteiro, Mario L.R.; Goncalves, Allan C.P.; Silva, Carla T.M.; Moura, Janete P.; Ribeiro, Carolina S.; Gebrim, Eloisa M.M.S.; Universidade de Sao Paulo; Universidade de Sao Paulo

2008-01-01

Objectives: The objective of this study was to evaluate the ability of a muscular index (Barrett's Index), calculated with multidetector computed tomography, to detect dysthyroid optic neuropathy in patients with Graves' orbitopathy. Methods: Thirty-six patients with Graves' orbitopathy were prospectively studied and submitted to neuro-ophthalmic evaluation and multidetector computed tomography scans of the orbits. Orbits were divided into two groups: those with and without dysthyroid optic neuropathy. Barrett's index was calculated as the percentage of the orbit occupied by muscles. Sensitivity and specificity were determined for several index values. Results: Sixty-four orbits (19 with and 45 without dysthyroid optic neuropathy) met the inclusion criteria for the study. The mean Barrett's index values (±SD) were 64.47% ± 6.06% and 49.44% ± 10.94% in the groups with and without dysthyroid optic neuropathy, respectively (p 60% should be carefully examined and followed for the development of dysthyroid optic neuropathy. (author)

Diagnostic ability of barrett's index to detect dysthyroid optic neuropathy using multidetector computed tomography

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Mário L. R. Monteiro

2008-01-01

Full Text Available OBJECTIVES: The objective of this study was to evaluate the ability of a muscular index (Barrett's Index, calculated with multidetector computed tomography, to detect dysthyroid optic neuropathy in patients with Graves' orbitopathy. METHODS: Thirty-six patients with Graves' orbitopathy were prospectively studied and submitted to neuro-ophthalmic evaluation and multidetector computed tomography scans of the orbits. Orbits were divided into two groups: those with and without dysthyroid optic neuropathy. Barrett's index was calculated as the percentage of the orbit occupied by muscles. Sensitivity and specificity were determined for several index values. RESULTS: Sixty-four orbits (19 with and 45 without dysthyroid optic neuropathy met the inclusion criteria for the study. The mean Barrett's index values (± SD were 64.47% ± 6.06% and 49.44% ± 10.94%in the groups with and without dysthyroid optic neuropathy, respectively (p60% should be carefully examined and followed for the development of dysthyroid optic neuropathy.

Association between nasopharyngeal carcinoma and risk of optic neuropathy: A population-based cohort study.

Science.gov (United States)

Fan, Chao-Yueh; Jen, Yee-Min; Su, Yuan-Chih; Chao, Hsing-Lung; Lin, Chun-Shu; Huang, Wen-Yen; Lin, Miao-Jung; Kao, Chia-Hung

2018-04-16

The purpose of this study was to assess the predictive factors of optic neuropathy among patients with nasopharyngeal carcinoma (NPC). The analysis included 16 297 patients with NPC and 65 187 controls. Each patient with NPC was randomly frequency-matched with 4 individuals without NPC by age, sex, and index year. Cox proportional hazard models were applied to measure the hazard ratios (HRs) and 95% confidence intervals (CIs) of optic neuropathy development associated with NPC. The risk of optic neuropathy was significantly higher in the NPC cohort (adjusted HR [aHR] 3.42; 95% CI 2.85-4.09; P optic neuropathy among patients with NPC included stroke (aHR 1.7; 95% CI 1.07-2.7; P = .03) and receipt of chemotherapy (aHR 1.55; 95% CI 1.17-2.06; P = .002). The risk of optic neuropathy was significantly higher in patients with NPC than in the general population. © 2018 Wiley Periodicals, Inc.

Macular retinoschisis in eyes with glaucomatous optic neuropathy: Vitrectomy and natural course.

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Yoshikawa, Tadanobu; Yamanaka, Chihiro; Kinoshita, Takamasa; Morikawa, Shohei; Ogata, Nahoko

2018-02-01

Our purpose was to determine the effectiveness of vitrectomy in resolving the macular retinoschisis in an eye with glaucomatous optic neuropathy and also to determine the natural course of macular retinoschisis. This was a retrospective case series of patients who were diagnosed with macular retinoschisis and glaucomatous optic neuropathy. Fourteen eyes of 13 patients were studied. Patients with high myopia, vitreomacular traction syndrome, and the pit macular syndrome were excluded. There were three men and ten women, and 12 had unilateral and one had bilateral macular retinoschisis. Vitrectomy was performed for a serous retinal detachment, macular hole, or severe visual loss in five eyes. The mean follow-up time was 68.8 months in these five eyes, and the macular retinoschisis was resolved and the best-corrected visual acuity (BCVA) at the final visit was significantly improved in all eyes (P = 0.007). However, two of these fiv e eyes developed a macular hole and required a second vitrectomy. Of the nine eyes without treatment with a mean follow-up time of 29.0 months, the BCVA at the final visit remained unchanged from the baseline BCVA in all eyes. The macular retinoschisis was resolved or reduced in three eyes without treatment. Vitrectomy was effective for the resolution of macular retinoschisis in eyes with glaucomatous optic neuropathy and serous retinal detachment or macular hole or severe reduction of the BCVA. Macular retinoschisis can be resolved without a reduction of the BCVA in some cases without treatment.

MRI in Leber's hereditary optic neuropathy

DEFF Research Database (Denmark)

Matthews, Lucy; Enzinger, Christian; Fazekas, Franz

2015-01-01

BACKGROUND: Leber's hereditary optic neuropathy (LHON) and a multiple sclerosis (MS)-like illness appear to coexist 50 times more frequently than would be expected by chance. This association of LHON and MS (LMS) raises an important question about whether there could be a common pathophysiological...

Neurophthalmological conditions mimicking glaucomatous optic neuropathy: analysis of the most common causes of misdiagnosis.

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Dias, Diego Torres; Ushida, Michele; Battistella, Roberto; Dorairaj, Syril; Prata, Tiago Santos

2017-01-10

To analyze the most common neurophthalmological conditions that may mimic glaucomatous optic neuropathy and to determine which most often lead to misdiagnosis when evaluated by a glaucoma specialist. We reviewed the charts of consecutive patients with optic neuropathies caused by neurophthalmological conditions screened in a single Eye Clinic within a period of 24 months. Within these enrolled patients, we selected the eyes whose fundoscopic appearance could resemble glaucoma based in pre-defined criteria (vertical cup-to-disc ratio ≥0.6, asymmetry of the cup-to-disc ratio ≥0.2 between eyes, presence of localized retinal nerve fiber layer and/or neuroretinal rim defects, and disc haemorrhages). Then, color fundus photographs and Humphrey Visual Field tests (HVF) of these eyes were mixed with tests from 21 consecutive glaucomatous patients (42 eyes with normal tension glaucoma). These images were mixed randomly and a masked glaucoma specialist was asked to distinguish if each set of exams was from a patient with glaucoma or with a neurophthalmologic condition. Among the 101 eyes (68 patients) enrolled with neurophthalmological diseases, 16 (15.8%) were classified as conditions that could mimic glaucoma. The most common diagnoses were ischemic optic neuropathy (25%), compressive optic neuropathy (18.7%) and hereditary optic neuropathy (18.7%). Based on the analysis of fundus photographs and HVF tests, 25% of these were misdiagnosed as glaucoma (two ischemic optic neuropathies and two congenital optic disc anomalies). Conversely, 11.9% of the glaucomatous neuropathies were misdiagnosed as neurophthalmological disorders. Overall, the glaucoma specialist correctly diagnosed 84.5% of the eyes. Some neurophthalmological disorders can mimic glaucoma. In our study, isquemic and compressive optic neuropathies were the ones that most often did so. Almost one quarter of the eyes were misdiagnosed when evaluated by a glaucoma specialist, which can lead to inadequate

Magnetic resonance imaging of radiation optic neuropathy

International Nuclear Information System (INIS)

Zimmerman, C.F.; Schatz, N.J.; Glaser, J.S.

1990-01-01

Three patients with delayed radiation optic neuropathy after radiation therapy for parasellar neoplasms underwent magnetic resonance imaging. The affected optic nerves and chiasms showed enlargement and focal gadopentetate dimeglumine enhancement. The magnetic resonance imaging technique effectively detected and defined anterior visual pathway changes of radionecrosis and excluded the clinical possibility of visual loss because of tumor recurrence

Evaluation of retinal nerve fiber layer thickness measurements using optical coherence tomography in patients with tobacco-alcohol-induced toxic optic neuropathy

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Moura Frederico

2010-01-01

Full Text Available Three patients with progressive visual loss, chronic alcoholism and tabagism were submitted to a complete neuro-ophthalmic examination and to retinal nerve fiber layer (RNFL measurements using optical coherence tomography (OCT scanning. Two patients showed marked RNFL loss in the temporal sector of the optic disc. However, a third patient presented RNFL measurements within or above normal limits, based on the Stratus-OCT normative database. Such findings may be due to possible RNFL edema similar to the one that may occur in the acute phase of toxic optic neuropathies. Stratus-OCT was able to detect RNFL loss in the papillomacular bundle of patients with tobacco-alcohol-induced toxic optic neuropathy. However, interpretation must be careful when OCT does not show abnormality in order to prevent diagnostic confusion, since overestimation of RNFL thickness measurements is possible in such cases.

Optical coherence tomography angiography in acute arteritic and non-arteritic anterior ischemic optic neuropathy.

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Balducci, Nicole; Morara, Mariachiara; Veronese, Chiara; Barboni, Piero; Casadei, Nicoletta Lelli; Savini, Giacomo; Parisi, Vincenzo; Sadun, Alfredo A; Ciardella, Antonio

2017-11-01

The purpose of our study was to describe the feature of acute non-arteritic or arteritic anterior ischemic optic neuropathy (NA-AION and A-AION) using optical coherence tomography angiography (OCT-A) and to compare it with fluorescein angiography (FA) and indocyanine green angiography (ICGA). In this retrospective, observational case-control study four NA-AION patients and one A-AION patient were examined by FA, ICGA and OCT-A within 2 weeks from disease presentation. The characteristics of the images were analyzed. Optic nerve head (ONH) and radial peripapillary capillaries (RPC) vessel densities (VDs) were compared between NA-AION and controls. In two of four NA-AION cases and in the A-AION patient, OCT-A clearly identified the boundary of the ischemic area at the level of the optic nerve head, which was comparable to optic disc filling defects detected by FA. In the other two NA-AION cases, a generalized leakage from the disc was visible with FA, yet OCT-A still demonstrated sectorial peripapillary capillary network reduction. Both ONH and RPC VDs were reduced in NA-AION patients, when compared to controls. OCT-A was able to identify microvascular defects and VD reduction in cases of acute optic disc edema due to NA-AION and A-AION. OCT-A provides additional information in ischemic conditions of the optic nerve head.

A Novel Rodent Model of Posterior Ischemic Optic Neuropathy

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Wang, Yan; Brown, Dale P.; Duan, Yuanli; Kong, Wei; Watson, Brant D.; Goldberg, Jeffrey L.

2014-01-01

Objectives To develop a reliable, reproducible rat model of posterior ischemic optic neuropathy (PION) and study the cellular responses in the optic nerve and retina. Methods Posterior ischemic optic neuropathy was induced in adult rats by photochemically induced ischemia. Retinal and optic nerve vasculature was examined by fluorescein isothiocyanate–dextran extravasation. Tissue sectioning and immunohistochemistry were used to investigate the pathologic changes. Retinal ganglion cell survival at different times after PION induction, with or without neurotrophic application, was quantified by fluorogold retrograde labeling. Results Optic nerve injury was confirmed after PION induction, including local vascular leakage, optic nerve edema, and cavernous degeneration. Immunostaining data revealed microglial activation and focal loss of astrocytes, with adjacent astrocytic hypertrophy. Up to 23%, 50%, and 70% retinal ganglion cell loss was observed at 1 week, 2 weeks, and 3 weeks, respectively, after injury compared with a sham control group. Experimental treatment by brain-derived neurotrophic factor and ciliary neurotrophic factor remarkably prevented retinal ganglion cell loss in PION rats. At 3 weeks after injury, more than 40% of retinal ganglion cells were saved by the application of neurotrophic factors. Conclusions Rat PION created by photochemically induced ischemia is a reproducible and reliable animal model for mimicking the key features of human PION. Clinical Relevance The correspondence between the features of this rat PION model to those of human PION makes it an ideal model to study the pathophysiologic course of the disease, most of which remains to be elucidated. Furthermore, it provides an optimal model for testing therapeutic approaches for optic neuropathies. PMID:23544206

Nonarteritic anterior ischaemic optic neuropathy in Addison's disease.

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Ross, A H; Haider, S; Bailey, C C

2010-10-01

To report three cases of Nonarteritic anterior ischaemic optic neuropathy (NAAION) in patients with Addison's disease. We present a retrospective review of patients presenting with NAAION with underlying Addison's disease. Three eyes of two young patients presented with NAAION. Both patients had underlying Addison's disease with episodes of prolonged hypotension. To our knowledge, this is the first published report of NAAION associated with Addison's disease. As hypotension may be one of the few situations, in which NAAION may be treatable and the visual loss reversible, it is important to recognize and treat sustained episodes of hypotension in these individuals.

Corticosteroid therapy in patients with non-arteritic anterior ischemic optic neuropathy.

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Vidović, Tomislav; Cerovski, Branimir; Perić, Sanja; Kordić, Rajko; Mrazovac, Danijela

2015-03-01

Non-arteritic anterior ischemic optic neuropathy is one of the most common conditions affecting the optic nerve in the elderly. It may lead to severe visual loss. Typical symptoms are painless impairment of visual function accompanied by relative afferent pupillary defect, edema of the optic disc and visual field defects. Aim is to present 38 patients with nonarteritic anterior ischemic optic neuropathy who were treated with corticosteroid therapy. This prospective study involved 38 patients, 20 men and 18 women aged 60-75 years who were treated with corticosteroid therapy. The study included patients with visual acuity in the affected eye from 0.1 to 0.8 according to Snellen. Every patient underwent clinical examination, the Octopus 900 perimetry in G program, laboratory testing, while the compressive optic neuropathy was rule out with MSCT of the brain and orbits. The most common forms of visual field defect are altitudinal defect and diffuse depression. Corticosteroid therapy led to recovery in 65% of patient, in 30% of patients did not change, while the deterioration occurred in 5% of patients.

Morphological functional criteria of neuroprotective therapy efficacy in glaucomatous optic neuropathy

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Tszin Dan

2015-01-01

Full Text Available Electrophysiological tests may be used to detect early glaucomatous changes and glaucoma progression risk and to monitor treatment efficacy. Most important pathogenic aspects of glaucomatous process, pathogenesis and multifactorial nature of glaucomatous optic neuropathy are described. Major triggers of glaucomatous optic neuropathy are mechanical and vascular. Principles of neuroprotective therapy, neuroprotective drugs, and mechanisms of action of direct and indirect neuroprotective agents are presented. IOPcc is a basis for neuroprotective therapy selection and its efficacy monitoring. Amongst neuroprotective drugs, NMDA agonists, antioxidants, peptides, and calcium channel blockers are of special importance. Structural damage and functional deficiency (e.g., visual field loss in glaucoma and the most informative and accurate methods of their detection are characterized. Confocal laser microscopy, optical coherence tomography, and scanning laser polarimetry are compared. These techniques are used to study optic nerve head and retinal nerve fiber layer. They are proposed as diagnostic and monitoring tools for glaucoma, glaucoma suspicion, and ocular hypertension. The most sensitive and specific electrophysiological tests for glaucomatous optic neuropathy are pattern electroretinography, multfocal electroretinography, and multifocal visually evoked potentials. 

Radiation optic neuropathy after megavoltage external-beam irradiation: Analysis of time-dose factors

International Nuclear Information System (INIS)

Parsons, J.T.; Bova, F.J.; Million, R.R.

1994-01-01

To investigate the risk of radiation-induced optic neuropathy according to total radiotherapy dose and fraction size, based on both retrospective and prospectively collected data. Between October 1964 and May 1989, 215 optic nerves in 131 patients received fractionated external-beam irradiation during the treatment of primary extracranial head and neck tumors. All patients had a minimum of 3 years of ophthalmologic follow-up (range, 3 to 21 years). The clinical end point was visual acuity of 20/100 or worse as a result of optic nerve injury. Anterior ischemic optic neuropathy developed in five nerves (at mean and median times of 32 and 30 months, respectively, and a range of 2-4 years). Retrobulbar optic neuropathy developed in 12 nerves (at mean and median times of 47 and 28 months, respectively, and a range of 1-14 years). No injuries were observed in 106 optic nerves that received a total dose of <59 Gy. Among nerves that received doses of ≥ 60 Gy, the dose per fraction was more important than the total dose in producing optic neuropathy. The 15-year actuarial risk of optic compared with 47% when given in fraction sizes ≥1.9 Gy. The data also suggest an increased risk of optic nerve injury with increasing age. As there is no effective treatment of radiation-induced optic neuropathy, efforts should be directed at its prevention by minimizing the total dose, paying attention to the dose per fraction to the nerve, and using reduced field techniques where appropriate to limit the volume of tissues that receive high-dose irradiation. 32 refs., 5 figs., 5 tabs

Reversible optic neuropathy with OPA1 exon 5b mutation

DEFF Research Database (Denmark)

Cornille, K.; Milea, D.; Amati-Bonneau, P.

2008-01-01

A new c.740G>A (R247H) mutation in OPA1 alternate spliced exon 5b was found in a patient presenting with bilateral optic neuropathy followed by partial, spontaneous visual recovery. R247H fibroblasts from the patient and his unaffected father presented unusual highly tubular mitochondrial network......, significant increased susceptibility to apoptosis, oxidative phosphorylation uncoupling, and altered OPA1 protein profile, supporting the pathogenicity of this mutation. These results suggest that the clinical spectrum of the OPA1-associated optic neuropathies may be larger than previously described...

EFFICACY OF INTRAVENOUS METHYLPREDNISOLONE THERAPY IN TRAUMATIC OPTIC NEUROPATHY WITH ORBITAL WALL FRACTURES: A PROSPECTIVE COHORT STUDY

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Srinivasan

2016-05-01

Full Text Available BACKGROUND Craniofacial injury due to road traffic accidents, blunt trauma and other accidents leading to traumatic optic neuropathy were managed with high dose of steroids rather than wait and observation and surgical decompression of optic nerve or nerve sheath (in case of sheath hematoma. Motor vehicles and bikes are most frequent causes for traumatic optic neuropathy, accounting for 17%-63% of cases. Our study was conducted to assess the visual loss due to traumatic optic neuropathy in association with orbital bone and wall fracture due to various types of ocular injuries and the response to medical line of management by intravenous methylprednisolone was observed. MATERIALS AND METHODS The prospective cohort study conducted at Department of Ophthalmology, Government Vellore Medical College Hospital, Vellore. Total number of ocular injury cases included in this study were 200. The study period was from November 2014 to December 2015. The ocular injury patients reported as outpatients in eye department as well as referred patients from Trauma Ward. RESULTS In our study, the ocular injuries of age group between 21-40 years is (121/200 60.5%. All cases of traumatic optic neuropathy manifestation individuals fall in that age group with severe form of ocular injuries. But the visual recovery reported with intravenous methylprednisolone and oral prednisolone alone because of neuropraxia and surrounding oedema of tissues as well as incomplete fracture of orbital wall without extending into optic canal level and without impingement of bone chips to the optic nerve. With improvement of colour vision apart from visual acuity improvement, visual field changes disappeared with the treatment. In our study, instead of wait and observation management where there was danger for total loss of vision or surgical decompression which carried the risk of orbital apex structure and other intracranial structure damage, iatrogenic direct and indirect optic nerve

The Prevalence of diabetic optic neuropathy in type 2 diabetes mellitus

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Ali A. Taqi Al-Saffar

2017-12-01

Full Text Available Background and objective: As diabetes mellitus a common health problem, it is well known that it can lead to optic neuropathy that affects the optic nerve functions. It is important to monitor the effect of this metabolic disease on the optic nerve that can lead ultimately to decrease visual acuity that can be irreversible. This study aimed to find out the prevalence of diabetic optic nerve diseases and to evaluate the patient characteristics and fundus findings. Methods: Screening examination was done for 2213 patients with type 2 diabetic patients presented to the diabetic center from October 2007 to September 2009. The examination includes visual acuity test using conventional E chart, slit lamp exam, followed by installing short acting Mydriatics (tropicamide 1% eye drops for fundoscopy examination using +76.D or +90 D. Results: Eighty eight patients (approximately 4% had optic nerve problems; 50 females and 38 males. The mean age was 59 years. A total of 58 (116 eyes patients were bilaterally affected, 42 patients with optic papillopathy, 8 patients with anterior ischemic optic neuropathy and profound loss of vision, 8 with glaucomatous cupping and pallor and 30 patients with end stage optic atrophy. A total of 63 (71.5% patients had poor metabolic control. Conclusions: Patients with type 2 diabetes mellitus have 4% prevalence of diabetic optic neuropathy.

Antiretroviral therapy-induced Leber's hereditary optic neuropathy

African Journals Online (AJOL)

2014-06-01

Jun 1, 2014 ... Optic neuropathy in HIV-infected patients results from the HIV ... individuals was triggered by NRTI drugs lamivudine and tenofovir when ... in disseminated tuberculosis where its prolonged use over ... Fungal: cryptococcal meningitis .... Genetic testing of LHON by polymerase chain reaction and restriction.

Linezolid-Associated Optic Neuropathy in Drug-Resistant Tuberculosis Patients in Mumbai, India.

Science.gov (United States)

Mehta, Salil; Das, Mrinalini; Laxmeshwar, Chinmay; Jonckheere, Sylvie; Thi, Sein Sein; Isaakidis, Petros

2016-01-01

Patients on linezolid-containing drug-resistant TB (DR-TB) regimen often develop adverse-events, particularly peripheral and optic neuropathy. Programmatic data and experiences of linezolid-associated optic neuropathy from high DR-TB burden settings are lacking. The study aimed to determine the frequency of and risk-factors associated with linezolid-associated optic neuropathy and document the experiences related to treatment/care of DR-TB patients on linezolid-containing regimens. This was a retrospective cohort study using routine clinical and laboratory data in Médecins Sans Frontières (MSF) HIV/DR-TB clinic in collaboration with Lilavati Hospital & Research Center, Mumbai, India. All DR-TB patients on linezolid-containing treatment regimens were included in the study and underwent routine evaluations for systemic and/or ocular complaints. Ophthalmological evaluation by a consultant ophthalmologist included visual-acuity screening, slit-lamp examination and dilated fundus examination. During January 2013-April 2016, 86 of 136 patients (with/without HIV co-infection) initiated linezolid-containing DR-TB treatment. The median age of these 86 patients was 25 (20-35) years and 47% were males. 20 percent of them had HIV co-infection. Of 86, 24 (27.9%) had at least one episode of ocular complaints (the majority blurred-vision) and among them, five (5.8%) had optic neuropathy. Patients received appropriate treatment and improvements were observed. None of the demographic/clinical factors were associated with optic neuropathy in Poissons or multivariate binary logistic-regression models. This is the first report focusing on optic neuropathy in a cohort of complex DR-TB patients, including patients co-infected with HIV, receiving linezolid-containing regimens. In our study, one out of four patients on linezolid had at least one episode of ocular complaints; therefore, systematic monitoring of patients by primary physicians/nurses, and access to specialized diagnostic

Linezolid-Associated Optic Neuropathy in Drug-Resistant Tuberculosis Patients in Mumbai, India

Science.gov (United States)

Mehta, Salil; Das, Mrinalini; Laxmeshwar, Chinmay; Jonckheere, Sylvie; Thi, Sein Sein; Isaakidis, Petros

2016-01-01

Background Patients on linezolid-containing drug-resistant TB (DR-TB) regimen often develop adverse-events, particularly peripheral and optic neuropathy. Programmatic data and experiences of linezolid-associated optic neuropathy from high DR-TB burden settings are lacking. The study aimed to determine the frequency of and risk-factors associated with linezolid-associated optic neuropathy and document the experiences related to treatment/care of DR-TB patients on linezolid-containing regimens. Methods This was a retrospective cohort study using routine clinical and laboratory data in Médecins Sans Frontières (MSF) HIV/DR-TB clinic in collaboration with Lilavati Hospital & Research Center, Mumbai, India. All DR-TB patients on linezolid-containing treatment regimens were included in the study and underwent routine evaluations for systemic and/or ocular complaints. Ophthalmological evaluation by a consultant ophthalmologist included visual-acuity screening, slit-lamp examination and dilated fundus examination. Results During January 2013-April 2016, 86 of 136 patients (with/without HIV co-infection) initiated linezolid-containing DR-TB treatment. The median age of these 86 patients was 25 (20–35) years and 47% were males. 20 percent of them had HIV co-infection. Of 86, 24 (27.9%) had at least one episode of ocular complaints (the majority blurred-vision) and among them, five (5.8%) had optic neuropathy. Patients received appropriate treatment and improvements were observed. None of the demographic/clinical factors were associated with optic neuropathy in Poissons or multivariate binary logistic-regression models. Discussion This is the first report focusing on optic neuropathy in a cohort of complex DR-TB patients, including patients co-infected with HIV, receiving linezolid-containing regimens. In our study, one out of four patients on linezolid had at least one episode of ocular complaints; therefore, systematic monitoring of patients by primary physicians

The risk of ischemic optic neuropathy post phacoemulsification cataract surgery.

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Al-Madani, Mousa Victor; Al-Raqqad, Nancy Khalaf; Al-Fgarra, Naser Abdallah; Al-Thawaby, Amal Mousa; Jaafar, Ahmed Abdelra'of

2017-01-01

The aim was to study the risk of non arteritic ischemic optic neuropathy after phacoemulsification cataract surgery. This study was conducted at King Hussein Medical Center during the period between January 2015 and July 2016. Patients attending ophthalmology clinic complaining of decreased vision due to lens opacity were evaluated. Patients were divided into two groups. First group included patients with no medical illness and second group included patients with diabetes mellitus, hypertension or hyperlipidemia. The two groups were further divided into two subgroups. First subgroup included patients who had phacoemulsification surgery and second subgroup did not have surgery. All patients were followed up for 6 months. They were assessed by neuro-ophthalmologist looking for ischemic optic neuropathy. A total number of 568 patients were enrolled. Group 1A included patients with no medical illness who underwent surgery and group 1B did not undergo surgery. The number of patients in these two subgroups was 119 and 103 respectively. Number of patients in group 2A (medical illness and surgery) was 188 and number of patients in group 2B (medical illness and no surgery) was 130. The incidence of ischemic optic neuropathy was 4.3 % in group 2A, 4.2 % in group 1A, 0.8% in group 2B, and 0% in group 1B. Phacoemulsification is a risk factor for non arteritic ischemic optic neuropathy independent of the presence of medical risk factors. Suggested mechanisms would be local anaesthesia, intraocular pressure fluctuation and local intraocular inflammation.

Unilateral Acute Anterior Ischemic Optic Neuropathy in a Patient with an Already Established Diagnosis of Bilateral Optic Disc Drusen

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Ziya Ayhan

2015-01-01

Full Text Available Optic disc drusen (ODD are calcific deposits that form in the optic nerve head secondary to abnormalities in axonal metabolism and degeneration. Anterior ischemic optic neuropathy, central retinal artery, and vein occlusion are among the rare vascular complications of disc drusen. We reported the clinical course of a 51-year-old patient with a unilateral acute nonarteritic anterior ischemic optic neuropathy (NAION who received the diagnosis of bilateral optic disc drusen five years earlier and thereby reiterated the association of ODD and acute NAION.

Long-term retinal nerve fiber layer changes following nonarteritic anterior ischemic optic neuropathy

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Dotan G

2013-04-01

Full Text Available Gad Dotan,1 Michaella Goldstein,1 Anat Kesler,1 Barry Skarf21Department of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; 2Eye Care Services, Henry Ford Hospital, Detroit, MI, USABackground: In cases of nonarteritic anterior ischemic optic neuropathy (NAION, retinal nerve fiber layer (RNFL thickness changes have been described during the first 12 months following the acute event. The purpose of this study was to report on the long-term RNFL changes in these eyes beyond the first year following onset of NAION.Methods: Fourteen eyes of 13 patients with NAION were analyzed in this retrospective observational case series study. Uninvolved eyes served as controls. All patients underwent a complete neuro-ophthalmological examination and repeat measurements of peripapillary RNFL thickness using Stratus optical coherence tomography.Results: On optical coherence tomography scan performed on average 6 months following onset of NAION, the mean global RNFL thickness (59.8 ± 11.8 μm was significantly thinner (P < 0.001 compared with uninvolved eyes (95.1 ± 13.9 μm. In a second optical coherence tomography scan performed on average 13 (range 12–23 months later, the mean global RNFL thickness (58.9 ± 6.5 μm was not significantly different (P = 0.702 from the first scan.Conclusion: There appears to be no further RNFL loss beyond the first 6 months following an acute event of NAION.Keywords: optical coherence tomography, retinal nerve fiber layer, nonartertic anterior ischemic optic neuropathy

Visual loss related to macular subretinal fluid and cystoid macular edema in HIV-related optic neuropathy

DEFF Research Database (Denmark)

Gautier, David; Rabier, Valérie; Jallet, Ghislaine

2012-01-01

Optic nerve involvement may occur in various infectious diseases, but is rarely reported after infection by the human immunodeficiency virus (HIV). We report the atypical case of a 38-year-old patient in whom the presenting features of HIV infection were due to a bilateral optic neuropathy associ...... associated with macular subretinal fluid and cystoid macular edema, which responded well to antiretroviral therapy....

Choroidal thickness in traumatic optic neuropathy.

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Lee, Ju-Yeun; Eo, Doo-Ri; Park, Kyung-Ah; Oh, Sei Yeul

2017-12-01

To examine the choroidal thickness in patients with indirect traumatic optic neuropathy (TON) Methods: Patients with unilateral traumatic optic neuropathy over a period of 4 years were included in this study. Horizontal and vertical enhanced-depth imaging (EDI) from spectral-domain optical coherence tomography (SD-OCT) scans of the fovea were obtained in patients with unilateral TON within 2 weeks of injury. The main outcome measure was the choroidal thickness at nine locations. The choroidal thickness was compared between affected and unaffected eyes in the TON group, and the mean difference in the choroidal thickness in both eyes was compared between TON and control groups. A total of 16 patients and 20 control subjects were included. The choroidal thickness at horizontal, vertical and average subfoveal, inner temporal, and outer inferior locations was significantly thicker (13-23%) in affected eyes than in unaffected fellow eyes (p = 0.042, 0.046, 0.024, 0.013, 0.018, and 0.027, respectively). The mean difference value between choroidal thickness measurements in both eyes was significantly larger in the TON group than in the control group at the horizontal, vertical and average subfoveal, inner temporal, inner nasal, inner superior, inner inferior, and outer superior locations (p = 0.001, 0.011,  0.05). Eyes affected by TON showed a regionally thicker choroid than unaffected fellow eye. This thick choroid might be due to impaired blood circulation and vascular remodeling of the optic nerve head and choroid. These results help to better understand the pathophysiology of TON.

Diagnostic ability of Barrett's index to detect dysthyroid optic neuropathy using multidetector computed tomography

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Monteiro, Mario L.R.; Goncalves, Allan C.P.; Silva, Carla T.M.; Moura, Janete P.; Ribeiro, Carolina S.; Gebrim, Eloisa M.M.S. [Universidade de Sao Paulo (USP), SP (Brazil). Hospital das Clinicas. Division of Ophthalmology; Universidade de Sao Paulo (USP), SP (Brazil). Hospital das Clinicas. Dept. of Endocrinology; Universidade de Sao Paulo (USP), SP (Brazil). Hospital das Clinicas. Division of Radiology]. E-mail: mlrmonteiro@terra.com.br

2008-07-01

Objectives: The objective of this study was to evaluate the ability of a muscular index (Barrett's Index), calculated with multidetector computed tomography, to detect dysthyroid optic neuropathy in patients with Graves' orbitopathy. Methods: Thirty-six patients with Graves' orbitopathy were prospectively studied and submitted to neuro-ophthalmic evaluation and multidetector computed tomography scans of the orbits. Orbits were divided into two groups: those with and without dysthyroid optic neuropathy. Barrett's index was calculated as the percentage of the orbit occupied by muscles. Sensitivity and specificity were determined for several index values. Results: Sixty-four orbits (19 with and 45 without dysthyroid optic neuropathy) met the inclusion criteria for the study. The mean Barrett's index values ({+-}SD) were 64.47% {+-} 6.06% and 49.44% {+-} 10.94% in the groups with and without dysthyroid optic neuropathy, respectively (p<0.001). Barrett's index sensitivity ranged from 32% to 100%, and Barrett's index specificity ranged from 24% to 100%. The best combination of sensitivity and specificity was 79%/72% for BI=60% (odds ratio: 9.2). Conclusions: Barrett's Index is a useful indicator of dysthyroid optic neuropathy and may contribute to early diagnosis and treatment. Patients with a Barrett's index >60% should be carefully examined and followed for the development of dysthyroid optic neuropathy. (author)

Ischemic Optic Neuropathy in Cardiac Surgery: Incidence and Risk Factors in the United States from the National Inpatient Sample 1998 to 2013.

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Rubin, Daniel S; Matsumoto, Monica M; Moss, Heather E; Joslin, Charlotte E; Tung, Avery; Roth, Steven

2017-05-01

Ischemic optic neuropathy is the most common form of perioperative visual loss, with highest incidence in cardiac and spinal fusion surgery. To date, potential risk factors have been identified in cardiac surgery by only small, single-institution studies. To determine the preoperative risk factors for ischemic optic neuropathy, the authors used the National Inpatient Sample, a database of inpatient discharges for nonfederal hospitals in the United States. Adults aged 18 yr or older admitted for coronary artery bypass grafting, heart valve repair or replacement surgery, or left ventricular assist device insertion in National Inpatient Sample from 1998 to 2013 were included. Risk of ischemic optic neuropathy was evaluated by multivariable logistic regression. A total of 5,559,395 discharges met inclusion criteria with 794 (0.014%) cases of ischemic optic neuropathy. The average yearly incidence was 1.43 of 10,000 cardiac procedures, with no change during the study period (P = 0.57). Conditions increasing risk were carotid artery stenosis (odds ratio, 2.70), stroke (odds ratio, 3.43), diabetic retinopathy (odds ratio, 3.83), hypertensive retinopathy (odds ratio, 30.09), macular degeneration (odds ratio, 4.50), glaucoma (odds ratio, 2.68), and cataract (odds ratio, 5.62). Female sex (odds ratio, 0.59) and uncomplicated diabetes mellitus type 2 (odds ratio, 0.51) decreased risk. The incidence of ischemic optic neuropathy in cardiac surgery did not change during the study period. Development of ischemic optic neuropathy after cardiac surgery is associated with carotid artery stenosis, stroke, and degenerative eye conditions.

Treatment strategies for inherited optic neuropathies: past, present and future

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Yu-Wai-Man, P; Votruba, M; Moore, A T; Chinnery, P F

2014-01-01

Bilateral visual loss secondary to inherited optic neuropathies is an important cause of registrable blindness among children and young adults. The two prototypal disorders seen in clinical practice are Leber hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (DOA). About 90% of LHON cases are due to one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A, and m.14484T>C, which affect critical complex I subunits of the mitochondrial respiratory chain. The majority of patients with DOA harbour pathogenic mutations within OPA1, a nuclear gene that codes for a multifunctional inner mitochondrial membrane protein. Despite their contrasting genetic basis, LHON and DOA share overlapping pathological and clinical features that serve to highlight the striking tissue-specific vulnerability of the retinal ganglion cell (RGC) layer to disturbed mitochondrial function. In addition to severe visual loss secondary to progressive optic nerve degeneration, a subgroup of patients will also develop a more aggressive syndromic phenotype marked by significant neurological deficits. The management of LHON and DOA remains largely supportive, but major advances in our understanding of the mechanisms underpinning RGC loss in these two disorders are paving the way for novel forms of treatment aimed at halting or reversing visual deterioration at different stages of the disease process. In addition to neuroprotective strategies for rescuing RGCs from irreversible cell death, innovative in vitro fertilisation techniques are providing the tantalising prospect of preventing the germline transmission of pathogenic mtDNA mutations, eradicating in so doing the risk of disease in future generations. PMID:24603424

Treatment strategies for inherited optic neuropathies: past, present and future.

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Yu-Wai-Man, P; Votruba, M; Moore, A T; Chinnery, P F

2014-05-01

Bilateral visual loss secondary to inherited optic neuropathies is an important cause of registrable blindness among children and young adults. The two prototypal disorders seen in clinical practice are Leber hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (DOA). About 90% of LHON cases are due to one of three mitochondrial DNA (mtDNA) point mutations: m.3460G>A, m.11778G>A, and m.14484T>C, which affect critical complex I subunits of the mitochondrial respiratory chain. The majority of patients with DOA harbour pathogenic mutations within OPA1, a nuclear gene that codes for a multifunctional inner mitochondrial membrane protein. Despite their contrasting genetic basis, LHON and DOA share overlapping pathological and clinical features that serve to highlight the striking tissue-specific vulnerability of the retinal ganglion cell (RGC) layer to disturbed mitochondrial function. In addition to severe visual loss secondary to progressive optic nerve degeneration, a subgroup of patients will also develop a more aggressive syndromic phenotype marked by significant neurological deficits. The management of LHON and DOA remains largely supportive, but major advances in our understanding of the mechanisms underpinning RGC loss in these two disorders are paving the way for novel forms of treatment aimed at halting or reversing visual deterioration at different stages of the disease process. In addition to neuroprotective strategies for rescuing RGCs from irreversible cell death, innovative in vitro fertilisation techniques are providing the tantalising prospect of preventing the germline transmission of pathogenic mtDNA mutations, eradicating in so doing the risk of disease in future generations.

Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2

NARCIS (Netherlands)

Züchner, Stephan; de Jonghe, Peter; Jordanova, Albena; Claeys, Kristl G.; Guergueltcheva, Velina; Cherninkova, Sylvia; Hamilton, Steven R.; van Stavern, Greg; Krajewski, Karen M.; Stajich, Jeffery; Tournev, Ivajlo; Verhoeven, Kristien; Langerhorst, Christine T.; de Visser, Marianne; Baas, Frank; Bird, Thomas; Timmerman, Vincent; Shy, Michael; Vance, Jeffery M.

2006-01-01

OBJECTIVE: Charcot-Marie-Tooth (CMT) neuropathy with visual impairment due to optic atrophy has been designated as hereditary motor and sensory neuropathy type VI (HMSN VI). Reports of affected families have indicated autosomal dominant and recessive forms, but the genetic cause of this disease has

Orbital Radiotherapy Combined With Corticosteroid Treatment for Thyroid Eye Disease-Compressive Optic Neuropathy.

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Gold, Katherine G; Scofield, Stacy; Isaacson, Steven R; Stewart, Michael W; Kazim, Michael

To evaluate the effectiveness of orbital radiotherapy (ORT) in the treatment of thyroid eye disease (TED)-compressive optic neuropathy. A retrospective review of patients with corticosteroid-responsive compressive optic neuropathy due to TED treated with ORT. Study was conducted in compliance with Health Insurance Portability and Accountability Act. One hundred four patients (163 orbits) with a mean age of 61.7 years met inclusion criteria. Seventy-four percent (77/104) were female, and 32.7% (34/104) were current or previous smokers. A total absorbed dose of 2000 cGy fractionated in 10 treatment doses over the course of 2 weeks was administered to the retroocular tissues according to a standard protocol. The primary end point was failure of ORT, defined as persistent optic neuropathy following completion of radiotherapy that mandated urgent orbital decompression surgery. Ninety-eight of 104 (94%) patients or 152 of 163 (93.3%) orbits did not require orbital decompression surgery during the acute phase. Patients who responded successfully to ORT had similar improvements in visual acuity, color vision, Humphrey threshold visual field testing, and afferent pupillary defects compared with patients who failed ORT and underwent urgent decompression surgery. Only 36.7% of successfully treated patients ultimately underwent elective surgery, including orbital decompression, strabismus, or eyelid surgery, during the inactive phase of TED. The data from this study, the largest retrospective review reported to date, supports the use of ORT in eyes with corticosteroid-responsive TED-compressive optic neuropathy. ORT may favorably alter the natural history of active-phase TED by preventing recurrent compressive optic neuropathy after withdrawal of corticosteroids.

Characterizing Intraorbital Optic Nerve Changes on Diffusion Tensor Imaging in Thyroid Eye Disease Before Dysthyroid Optic Neuropathy.

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Lee, Hwa; Lee, Young Hen; Suh, Sang-Il; Jeong, Eun-Kee; Baek, Sehyun; Seo, Hyung Suk

The aim of this study was to determine whether the optic nerve is affected by thyroid eye disease (TED) before the development of dysthyroid optic neuropathy with diffusion-tensor imaging (DTI). Twenty TED patients and 20 controls were included. The mean, axial, and radial diffusivities and fractional anisotropy (FA) value were measured at the optic nerves in DTI. Extraocular muscle diameters were measured on computed tomography. The diffusivities and FA of the optic nerves were compared between TED and controls and between active and inactive stages of TED. The correlations between these DTI parameters and the clinical features were determined. The mean, axial, and radial diffusivities were lower in TED compared with the controls (P optic nerve before dysthyroid optic neuropathy in TED. The FA, in particular, reflected TED activity and severity.

Evaluation of dysthyroid optic neuropathy using T2-relaxation time of extraocular muscle as parameter

International Nuclear Information System (INIS)

Yu, Fumihiko; Maeda, Toshine; Inoue, Toyoko; Inoue, Yoichi

2001-01-01

The T2 value of magnetic resonance imaging (MRI) is useful in evaluating the activity of dysthyroid ophthlamopathy. We applied this method in evaluating dysthyroid optic neuropathy in 15 affected eyes of 15 patients. Another group of 40 eyes of 20 patients of dysthyroid opthalmopathy without hypertrophy of extraocular muscles served as control. The T2 value in dysthyroid optic neuropathy significantly decreased following treatment with corticosteroid but the value was still higher than that in control eyes. The findings show that the T2 value of MRI is useful in evaluating the therapeutic effect of dysthyroid optic neuropathy. (author)

Evaluation of dysthyroid optic neuropathy using T2-relaxation time of extraocular muscle as parameter

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Yu, Fumihiko; Maeda, Toshine; Inoue, Toyoko; Inoue, Yoichi [Olympia Eye Hospital, Tokyo (Japan)

2001-11-01

The T2 value of magnetic resonance imaging (MRI) is useful in evaluating the activity of dysthyroid ophthlamopathy. We applied this method in evaluating dysthyroid optic neuropathy in 15 affected eyes of 15 patients. Another group of 40 eyes of 20 patients of dysthyroid opthalmopathy without hypertrophy of extraocular muscles served as control. The T2 value in dysthyroid optic neuropathy significantly decreased following treatment with corticosteroid but the value was still higher than that in control eyes. The findings show that the T2 value of MRI is useful in evaluating the therapeutic effect of dysthyroid optic neuropathy. (author)

Dissection of internal carotid artery presenting as isolated ischaemic optic neuropathy

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Serdar Oruc

2016-08-01

Full Text Available Carotid artery dissections are one of the important reasons of cerebrovascular events that are observed before the age of 45. Besides the local findings such as head, neck and face pains, Horner syndrome findings, pulsatile tinnitus and cranial nerve involvements, some other symptoms such as ischemic stroke, transient ischemic attacks and amaurosis fugax can also be observed in the approximately three quarters of patients. Ischemic optic neuropathy may be seen as %4 in the carotid artery dissections and it mostly accompanies other ischemic local symptoms. It is rare to observe the ischemic optic neuropathy as the first and unique finding in the carotid artery dissections. In this study, a 55 year old male patient with carotid artery dissection was represented. He did not have any other complaint, except the sudden unilateral visual loss and he was sent to our clinics from the opthalmology clinics in order to search for the etiology of ischemic optic neuropathy. It should be kept in mind that there can be a possibility to have carotid artery dissections in patients with unilateral visual loss.

Utility of coronal contrast-enhanced fat-suppressed FLAIR in the evaluation of optic neuropathy and atrophy.

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Boegel, Kevin H; Tyan, Andrew E; Iyer, Veena R; Rykken, Jeffrey B; McKinney, Alexander M

2017-01-01

Evaluating chronic sequelae of optic neuritis, such as optic neuropathy with or without optic nerve atrophy, can be challenging on whole brain MRI. This study evaluated the utility of dedicated coronal contrast-enhanced fat-suppressed FLAIR (CE-FS-FLAIR) MR imaging to detect optic neuropathy and optic nerve atrophy. Over 4.5 years, a 3 mm coronal CE-FS-FLAIR sequence at 1.5T was added to the routine brain MRIs of 124 consecutive patients, 102 of whom had suspected or known demyelinating disease. Retrospective record reviews confirmed that 28 of these 102 had documented onset of optic neuritis >4 weeks prior to the brain MRI. These 28 were compared to the other 22 ("controls") of the 124 patients who lacked a history of demyelinating disease or visual symptoms. Using coronal CE-FS-FLAIR, two neuroradiologists separately graded each optic nerve (n = 50 patients, 100 total nerves) as either negative, equivocal, or positive for optic neuropathy or atrophy. The scoring was later repeated. The mean time from acute optic neuritis onset to MRI was 4.1 ± 4.6 years (range 34 days-17.4 years). Per individual nerve grading, the range of sensitivity, specificity, and accuracy of coronal CE-FS-FLAIR in detecting optic neuropathy was 71.4-77.1%, 93.8-95.4%, and 85.5-89.0%, respectively, with strong interobserver (k = 0.667 - 0.678, p optic atrophy, interobserver agreement was moderate (k = 0.437 - 0.484, p optic neuropathy years after the onset of acute optic neuritis, but is less useful in detecting optic nerve atrophy.

Leber hereditary optic neuropathy: current perspectives

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Meyerson, Cherise; Van Stavern, Greg; McClelland, Collin

2015-01-01

Leber hereditary optic neuropathy (LHON) is one of the most common inherited optic neuropathies causing bilateral central vision loss. The disorder results from point mutations in mitochondrial DNA and subsequent mitochondrial dysfunction. The primary cell type that is lost in LHON is the retinal ganglion cell, which is highly susceptible to disrupted ATP production and oxidative stress. Inheritance of LHON follows that of mitochondrial genetics, and it has a highly variable clinical phenotype, as other genetic and environmental factors also play a role. Although LHON usually presents with isolated vision loss, some patients suffer other neurological sequelae. For ill-defined reasons, male LHON mutation carriers are more affected than females. Most LHON patients remain legally blind, but a small proportion can experience spontaneous partial recovery, often within the first year of symptom onset. Unfortunately, at this time there are no established curative interventions and treatment is largely supportive. Patients should be offered low vision services and counseled on mitigating risk factors for additional vision loss, such as smoking and consuming alcohol. Encouraging treatments currently undergoing investigation includes ubiquinone analogs, such as idebenone, as well as gene therapy and stem cells to restore ATP synthesis and provide neuroprotection to surviving retinal ganglion cells. PMID:26170609

Automatic computer-aided diagnosis of retinal nerve fiber layer defects using fundus photographs in optic neuropathy.

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Oh, Ji Eun; Yang, Hee Kyung; Kim, Kwang Gi; Hwang, Jeong-Min

2015-05-01

To evaluate the validity of an automatic computer-aided diagnosis (CAD) system for detection of retinal nerve fiber layer (RNFL) defects on fundus photographs of glaucomatous and nonglaucomatous optic neuropathy. We have proposed an automatic detection method for RNFL defects on fundus photographs in various cases of glaucomatous and nonglaucomatous optic neuropathy. In order to detect the vertical dark bands as candidate RNFL defects, the nonuniform illumination of the fundus image was corrected, the blood vessels were removed, and the images were converted to polar coordinates with the center of the optic disc. False positives (FPs) were reduced by using knowledge-based rules. The sensitivity and FP rates for all images were calculated. We tested 98 fundus photographs with 140 RNFL defects and 100 fundus photographs of healthy normal subjects. The proposed method achieved a sensitivity of 90% and a 0.67 FP rate per image and worked well with RNFL defects with variable depths and widths, with uniformly high detection rates regardless of the angular widths of the RNFL defects. The average detection accuracy was approximately 0.94. The overall diagnostic accuracy of the proposed algorithm for detecting RNFL defects among 98 patients and 100 healthy individuals was 86% sensitivity and 75% specificity. The proposed CAD system successfully detected RNFL defects in optic neuropathies. Thus, the proposed algorithm is useful for the detection of RNFL defects.

Contrast sensitivity and the diagnosis dysthyroid optic neuropathy

NARCIS (Netherlands)

Mourits, M. P.; Suttorp-Schulten, M. S.; Tijssen, R. O.; Apkarian, P.

1990-01-01

Contrast sensitivity function (CSF) was investigated in 19 patients (34 eyes) with clinical signs and symptoms of dysthyroid optic neuropathy (DON). CSF was disturbed in 33 eyes and was shown to improve after orbital decompression. These results indicate that the CSF may be a useful supplementary

Baseline demographics, clinical features, and treatment protocols of 240 patients with optic neuropathy: experiences from a neuro-ophthalmological clinic in the Aegean region of Turkey.

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Karti, Omer; Karti, Dilek Top; Kilic, İlay Hilal; Gokcay, Figen; Celebisoy, Nese

2017-12-19

To analyze the demographic patterns, clinical characteristics, and treatment protocols of optic neuropathies. The hospital data of patients with optic neuropathy admitted to the Department of Neuro-ophthalmology in a tertiary referral center in Turkey between January 2010 to January 2017 were retrospectively analyzed. Demographic patterns, clinical features, treatment protocols, and the natural disease courses were assessed. The total number of patients with optic neuropathy seen over this period was 240, which consist of 43 with idiopathic optic neuritis (17.9%), 40 with multiple sclerosis-related optic neuritis (16.7%), 12 with chronic relapsing inflammatory optic neuritis (5.0%), 12 with atypical optic neuritis (5.0%), 11 with neuromyelitis optica spectrum disorders-related optic neuritis (4.6%), 90 with non-arteritic ischemic optic neuropathy (37.5%), 4 with arteritic ischemic optic neuropathy (1.7%), 10 with traumatic optic neuropathy (4.1%), 6 with compressive optic neuropathy (2.5%), and 12 with mitochondrial optic neuropathy [9 with toxic optic neuropathy (3.7%) and 3 with Leber's hereditary optic neuropathy (1.2%)]. There were 101 males (42%) and 139 females (58%). The mean age was 43.34 ± 15.86 years. This study reported the demographics, clinical characteristics, and treatment protocols of optic neuropathies in a neuro-ophthalmology specialty clinic at a tertiary referral center in Turkey during the past decade. The data may be useful in assessing the global status of optic neuropathies.

Clinical variability in hereditary optic neuropathies: Two novel mutations in two patients with dominant optic atrophy and Wolfram syndrome.

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Galvez-Ruiz, Alberto

2015-01-01

Dominant optic atrophy (DOA) and Wolfram syndrome share a great deal of clinical variability, including an association with hearing loss and the presence of optic atrophy at similar ages. The objective of this paper was to discuss the phenotypic variability of these syndromes with respect to the presentation of two clinical cases. We present two patients, each with either DOA or Wolfram syndrome, and contribute to the research literature through our findings of two novel mutations. The overlapping of several clinical characteristics in hereditary optic neuropathies can complicate the differential diagnosis. Future studies are needed to better determine the genotype-phenotype correlation for these diseases.

Radiation-induced optic neuropathy: A magnetic resonance imaging study

International Nuclear Information System (INIS)

Guy, J.; Mancuso, A.; Beck, R.; Moster, M.L.; Sedwick, L.A.; Quisling, R.G.; Rhoton, A.L. Jr.; Protzko, E.E.; Schiffman, J.

1991-01-01

Optic neuropathy induced by radiation is an infrequent cause of delayed visual loss that may at times be difficult to differentiate from compression of the visual pathways by recurrent neoplasm. The authors describe six patients with this disorder who experienced loss of vision 6 to 36 months after neurological surgery and radiation therapy. Of the six patients in the series, two had a pituitary adenoma and one each had a metastatic melanoma, multiple myeloma, craniopharyngioma, and lymphoepithelioma. Visual acuity in the affected eyes ranged from 20/25 to no light perception. Magnetic resonance (MR) imaging showed sellar and parasellar recurrence of both pituitary adenomas, but the intrinsic lesions of the optic nerves and optic chiasm induced by radiation were enhanced after gadolinium-diethylenetriaminepenta-acetic acid (DTPA) administration and were clearly distinguishable from the suprasellar compression of tumor. Repeated MR imaging showed spontaneous resolution of gadolinium-DTPA enhancement of the optic nerve in a patient who was initially suspected of harboring recurrence of a metastatic malignant melanoma as the cause of visual loss. The authors found the presumptive diagnosis of radiation-induced optic neuropathy facilitated by MR imaging with gadolinium-DTPA. This neuro-imaging procedure may help avert exploratory surgery in some patients with recurrent neoplasm in whom the etiology of visual loss is uncertain

Distinguishing ischaemic optic neuropathy from optic neuritis by ganglion cell analysis.

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Erlich-Malona, Natalie; Mendoza-Santiesteban, Carlos E; Hedges, Thomas R; Patel, Nimesh; Monaco, Caitlin; Cole, Emily

2016-12-01

To determine whether a pattern of altitudinal ganglion cell loss, as detected and measured by optical coherence tomography (OCT), can be used to distinguish non-arteritic ischaemic optic neuropathy (NAION) from optic neuritis (ON) during the acute phase, and whether the rate or severity of ganglion cell loss differs between the two diseases. We performed a retrospective, case-control study of 44 patients (50 eyes) with ON or NAION and 44 age-matched controls. Non-arteritic ischaemic optic neuropathy and ON patients had OCT at presentation and four consecutive follow-up visits. Controls had OCT at one point in time. The ganglion cell complex (GCC) was evaluated in the macula, and the retinal nerve fibre layer (RNFL) was evaluated in the peripapillary region. Ganglion cell complex thickness, RNFL thickness and GCC mean superior and inferior hemispheric difference were compared between NAION and ON patients at each time-point using unpaired t-tests and between disease and control subjects at first measurement using paired t-tests. Mean time from onset of symptoms to initial presentation was 10.7 ± 6.6 days in NAION and 11.7 ± 8.6 days in ON (p = 0.67). There was a significantly greater vertical hemispheric difference in GCC thickness in NAION patients than ON patients at all time-points (5.5-10.7 μm versus 3.1-3.6 μm, p = 0.01-0.049). Mean GCC thickness was significantly decreased at less than 2 weeks after onset in NAION compared to age-matched controls (72.1 μm versus 82.1 μm, p < 0.001), as well as in ON compared to age-matched controls (74.3 μm versus 84.5 μm, p < 0.001). Progression and severity of GCC and RNFL loss did not differ significantly between NAION and ON. A quantitative comparison of mean superior and inferior hemispheric GCC thickness with OCT may be used to distinguish NAION from ON. © 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

Lamina cribrosa position and Bruch's membrane opening differences between anterior ischemic optic neuropathy and open-angle glaucoma.

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Rebolleda, Gema; Pérez-Sarriegui, Ane; Díez-Álvarez, Laura; De Juan, Victoria; Muñoz-Negrete, Francisco J

2018-06-01

To compare the optic nerve head morphology among primary open-angle glaucoma, non-arteritic anterior ischemic optic neuropathy eyes, their fellow healthy eyes and control eyes, using spectral-domain optical coherence tomography with enhanced depth imaging. Observational cross-sectional study including 88 eyes of 68 patients. In this study, 23 non-arteritic anterior ischemic optic neuropathy eyes, 17 fellow unaffected eyes, 25 primary open-angle glaucoma eyes, and 23 age-matched control eyes were included. Peripapillary retinal nerve fiber layer thickness and optic disk area were evaluated. Bruch's membrane opening diameter, optic cup depth, anterior lamina cribrosa depth, and prelaminar tissue thickness were assessed. Non-arteritic anterior ischemic optic neuropathy and primary open-angle glaucoma eyes had similar visual field mean deviation and peripapillary retinal nerve fiber layer thickness (P = 0.6 and P = 0.56, respectively). Bruch's membrane opening diameter was significantly larger in primary open-angle glaucoma eyes than in control eyes (P = 0.02). Lamina cribrosa and disk cup were deeper in eyes with primary open-angle glaucoma than both control and non-arteritic anterior ischemic optic neuropathy eyes (P open-angle glaucoma eyes than in non-arteritic anterior ischemic optic neuropathy eyes (P opening diameter was found in primary open-angle glaucoma eyes compared with control eyes. This issue has clinical implications because Bruch's membrane opening has been considered a stable reference for disk-related measures.

Visual recovery from optic atrophy following acute optic neuropathy in the fellow eye.

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Ornek, Kemal; Ornek, Nurgül

2012-06-01

The left eye of a 65-year-old male was blind due to optic atrophy and only seeing eye had also dry type age-related macular degeneration. An anterior ischemic optic neuropathy developed in the better seeing eye. Vision recovered in the blind eye in a short time after losing the better eye. Gaining some vision in a blind eye may be an adaptation of visual pathway in such patients.

Radioinduced optic nerve neuropathy after treatment for nasopharynx cancer. About two cases and literature review

International Nuclear Information System (INIS)

Mnejja, W.; Siala, W.; Daoud, J.; Fki, J.; Ghorbel, M.; Frikha, M.

2007-01-01

The radioinduced neuropathy of the optic nerve is a rare and delayed complication. Its incidence is difficult to evaluate in the literature. It depends on the whole irradiation dose, fractionation and irradiated volume. Currently, it does not exist any efficient treatment, only the prevention play an important part on avoiding the high doses, and the broad irradiation volumes. The innovating techniques using conformal radiotherapy with or without modulated intensity could contribute to reduce this toxicity incidence. (N.C.)

Choroidal thickness in Chinese patients with non-arteritic anterior ischemic optic neuropathy.

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Jiang, Libin; Chen, Lanlan; Qiu, Xiujuan; Jiang, Ran; Wang, Yaxing; Xu, Liang; Lai, Timothy Y Y

2016-08-31

Non-arteritic anterior ischemic optic neuropathy (NA-AION) is one of the most common types of ischemic optic neuropathy. Several recent studies suggested that abnormalities of choroidal thickness might be associated with NA-AION. The main objective of this case-control study was to evaluate whether choroidal thickness is an ocular risk factor for the development of NA-AION by evaluating the peripapillary and subfoveal choroidal thicknesses in affected Chinese patients. Forty-four Chinese patients with unilateral NA-AION were recruited and compared with 60 eyes of 60 normal age and refractive-error matched control subjects. Peripapillary and subfoveal choroidal thicknesses were measured by enhanced depth imaging optical coherence tomography. Choroidal thicknesses of eyes with NA-AION and unaffected fellow eyes were compared with normal controls. Choroidal thicknesses of NA-AION eyes with or without optic disc edema were also compared. The correlation between choroidal thickness and retinal nerve fiber layer (RNFL) thickness, logMAR best-corrected visual acuity (BCVA), and the mean deviation (MD) of Humphrey static perimetry in NA-AION eyes were analyzed. The peripapillary choroidal thicknesses at the nasal, nasal inferior and temporal inferior segments in NA-AION eyes with optic disc edema were significantly thicker compared with that of normal subjects (P optic disc edema and normal eyes (all P > 0.05). No significant correlation between choroidal thickness and RNFL thickness, logMAR BCVA and perimetry MD was found in eyes affected by NA-AION (all P > 0.05). Increase in peripapillary choroid thickness in some segments was found in NA-ION eyes with optic disc edema. However, our findings do not support the hypothesis that choroidal thickness is abnormal in Chinese patients with NA-AION compared with normal subjects with similar age and refractive error status.

Tocilizumab for giant cell arteritis with corticosteroid-resistant progressive anterior ischemic optic neuropathy.

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Vionnet, Julien; Buss, Guillaume; Mayer, Cédric; Sokolov, Arseny A; Borruat, François-Xavier; Spertini, François

2017-10-01

Giant cell arteritis is an inflammatory disorder of the medium- and large-size arteries. Permanent visual loss related to arteritic anterior ischemic optic neuropathy is among the most serious complications of this disease and initial treatment usually consists of high dose corticosteroids. There is no consensus in the literature concerning the optimal therapeutic approach in giant cell arteritis patients with corticosteroid-resistant arteritic anterior ischemic optic neuropathy. A 73-year-old Caucasian female with biopsy-proven giant cell arteritis developed an acute visual loss of the right eye due to arteritic anterior ischemic optic neuropathy. Despite 5 daily methylprednisolone pulses, systemic symptoms persisted and rapid involvement of the controlateral eye was documented. Therefore, tocilizumab (humanised monoclonal antibody binding the human interleukin-6 receptor) was introduced as a potential salvage therapy with a swift consecutive resolution of the systemic symptoms and stabilization of the ophthalmic lesions. Although a late effect of steroids pulses cannot be formally ruled out in this dramatic situation, tocilizumab likely offered a decisive effect in preventing bilateral blindness and may have contributed to steroid tapering. Tocilizumab may represent a new early effective second-line treatment option in corticosteroid-resistant anterior ischemic optic neuropathy. More data are needed to confirm this observation and to evaluate the safety profile of this treatment. Copyright © 2017 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Toxic optic neuropathy: An unusual cause

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Hema L Ramkumar

2014-01-01

Full Text Available A 60-year-old woman with a history of chronic alcoholism and tobacco use presented with the complaint of a painless decrease in vision in both eyes. She lost vision first in the left eye then in the right eye. She admitted consuming at least one 16 ounce bottle of over the counter mouthwash daily and denied consumption of any other alcohols, methanol, or antifreeze. She stated that her vision had been continuing to deteriorate in both eyes. Her best-corrected visual acuity was 4/200 in each eye. Color vision was nil in each eye. Her pupils were sluggish bilaterally, and her optic discs were flat and hyperemic with peripapillary hemorrhages. Her visual fields revealed central scotomas bilaterally. The magnetic resonance imaging of the brain and lumbar puncture were within normal limits. Antinuclear antibody, human leukocyte antigen-B27 genotyping, and B12 were normal; serum thiamine was low. While continuing to ingest mouthwash, her vision decreased to count fingers at 2 feet, and maculopapillary bundle pallor developed. She was started on folate and thiamine supplementation. Once she discontinued mouthwash, her vision improved to 20/400 bilaterally, and her central scotomas improved. This case demonstrates an alcohol-induced toxic optic neuropathy from mouthwash ingestion with some visual recovery after discontinuation of the offending agent.

Utility of coronal contrast-enhanced fat-suppressed FLAIR in the evaluation of optic neuropathy and atrophy

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Kevin H. Boegel

Full Text Available Background and purpose: Evaluating chronic sequelae of optic neuritis, such as optic neuropathy with or without optic nerve atrophy, can be challenging on whole brain MRI. This study evaluated the utility of dedicated coronal contrast-enhanced fat-suppressed FLAIR (CE-FS-FLAIR MR imaging to detect optic neuropathy and optic nerve atrophy. Materials and methods: Over 4.5 years, a 3 mm coronal CE-FS-FLAIR sequence at 1.5T was added to the routine brain MRIs of 124 consecutive patients, 102 of whom had suspected or known demyelinating disease. Retrospective record reviews confirmed that 28 of these 102 had documented onset of optic neuritis >4 weeks prior to the brain MRI. These 28 were compared to the other 22 (“controls” of the 124 patients who lacked a history of demyelinating disease or visual symptoms. Using coronal CE-FS-FLAIR, two neuroradiologists separately graded each optic nerve (n = 50 patients, 100 total nerves as either negative, equivocal, or positive for optic neuropathy or atrophy. The scoring was later repeated. Results: The mean time from acute optic neuritis onset to MRI was 4.1 ± 4.6 years (range 34 days-17.4 years. Per individual nerve grading, the range of sensitivity, specificity, and accuracy of coronal CE-FS-FLAIR in detecting optic neuropathy was 71.4–77.1%, 93.8–95.4%, and 85.5–89.0%, respectively, with strong interobserver (k = 0.667 − 0.678, p < 0.0001, and intraobserver (k = 0.706 − 0.763, p < 0.0001 agreement. For optic atrophy, interobserver agreement was moderate (k = 0.437 − 0.484, p < 0.0001, while intraobserver agreement was moderate-strong (k = 0.491 − 0.596, p < 0.0001. Conclusion: Coronal CE-FS-FLAIR is quite specific in detecting optic neuropathy years after the onset of acute optic neuritis, but is less useful in detecting optic nerve atrophy. Keywords: Optic

Anterior ischemic optic neuropathy precipitated by acute primary angle closure

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Choudhari Nikhil

2010-01-01

Full Text Available A 59-year-old man with a history of longstanding systemic hypotension developed asymmetric non-arteritic anterior ischemic optic neuropathy (NAION apparently precipitated by bilateral sequential acute primary angle closure. NAION is very rarely reported in association with raised intraocular pressure. In contrast to optical coherence tomography, the failure of scanning laser polarimetry to detect axonal swelling was another interesting finding. Possible reasoning for these observations is discussed.

The Central Bright Spot Sign: A Potential New MR Imaging Sign for the Early Diagnosis of Anterior Ischemic Optic Neuropathy due to Giant Cell Arteritis.

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Remond, P; Attyé, A; Lecler, A; Lamalle, L; Boudiaf, N; Aptel, F; Krainik, A; Chiquet, C

2017-07-01

A rapid identification of the etiology of anterior ischemic optic neuropathy is crucial because it determines therapeutic management. Our aim was to assess MR imaging to study the optic nerve head in patients referred with anterior ischemic optic neuropathy, due to either giant cell arteritis or the nonarteritic form of the disease, compared with healthy subjects. Fifteen patients with giant cell arteritis-related anterior ischemic optic neuropathy and 15 patients with nonarteritic anterior ischemic optic neuropathy from 2 medical centers were prospectively included in our study between August 2015 and May 2016. Fifteen healthy subjects and patients had undergone contrast-enhanced, flow-compensated, 3D T1-weighted MR imaging. The bright spot sign was defined as optic nerve head enhancement with a 3-grade ranking system. Two radiologists and 1 ophthalmologist independently performed blinded evaluations of MR imaging sequences with this scale. Statistical analysis included interobserver agreement. MR imaging scores were significantly higher in patients with giant cell arteritis-related anterior ischemic optic neuropathy than in patients with nonarteritic anterior ischemic optic neuropathy ( P ≤ .05). All patients with giant cell arteritis-related anterior ischemic optic neuropathy (15/15) and 7/15 patients with nonarteritic anterior ischemic optic neuropathy presented with the bright spot sign. No healthy subjects exhibited enhancement of the anterior part of the optic nerve. There was a significant relationship between the side of the bright spot and the side of the anterior ischemic optic neuropathy ( P ≤ .001). Interreader agreement was good for observers (κ = 0.815). Here, we provide evidence of a new MR imaging sign that identifies the acute stage of giant cell arteritis-related anterior ischemic optic neuropathy; patients without this central bright spot sign always had a nonarteritic pathophysiology and therefore did not require emergency corticosteroid

Optic neuropathy in thyroid eye disease: results of the balanced decompression technique.

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Baril, Catherine; Pouliot, Denis; Molgat, Yvonne

2014-04-01

To determine the efficacy of combined endoscopic medial and external lateral orbital decompression for the treatment of compressive optic neuropathy (CON) in thyroid eye disease (TED). A retrospective review of all patients undergoing combined surgical orbital decompression for CON between 2000 and 2010 was conducted. Fifty-nine eyes of 34 patients undergoing combined surgical orbital decompression for CON. Clinical outcome measures included visual acuity, Hardy-Rand-Rittler (HRR) colour plate testing, relative afferent pupillary defect, intraocular pressure measurement, and Hertel exophthalmometry. A CON score was calculated preoperatively and postoperatively based on the visual acuity and the missed HRR plates. A higher CON score correlates with more severe visual dysfunction. All patients had improvement of their optic neuropathy after surgical decompression. CON score was calculated for 54 eyes and decreased significantly from a mean of 13.2 ± 10.35 preoperatively to a mean of 8.51 ± 10.24 postoperatively (p < 0.0001). Optic neuropathy was completely resolved in 93.22% (55/59 eyes). Eighteen of 34 patients (52.94%) experienced development of new-onset postoperative strabismus that required subsequent surgical intervention. Endoscopic medial combined with external lateral orbital decompression is an effective technique for the treatment of TED-associated CON. © 2013 Canadian Ophthalmological Society Published by Canadian Ophthalmological Society All rights reserved.

Contrast sensitivity function in Graves' ophthalmopathy and dysthyroid optic neuropathy

NARCIS (Netherlands)

Suttorp-Schulten, M. S.; Tijssen, R.; Mourits, M. P.; Apkarian, P.

1993-01-01

Contrast sensitivity function was measured by a computer automated method on 38 eyes with dysthyroid optic neuropathy and 34 eyes with Graves' ophthalmopathy only. The results were compared with 74 healthy control eyes. Disturbances of contrast sensitivity functions were found in both groups when

A case of nonarteritic anterior ischemic optic neuropathy of a male with family history of the disease after receiving sildenafil

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Felekis T

2011-10-01

Full Text Available T Felekis1, I Asproudis1, K Katsanos2, EV Tsianos21University Eye Clinic of Ioannina, Ioannina, Greece; 2First Department of Internal Medicine, University Hospital of Ioannina, Ioannina, GreeceAbstract: A 51-year-old male was referred to the University Eye Clinic of Ioannina with nonarteritic anterior ischemic optic neuropathy (NAION 12 hours after receiving sildenafil citrate (Viagra®. Examination for possible risk factors revealed mild hypercholesterolemia. Family history showed that his father had suffered from bilateral NAION. Although a cause-and-effect relationship is difficult to prove, there are reports indicating an association between the use of erectile dysfunction agents and the development of NAION. Physicians might need to investigate the presence of family history of NAION among systemic or vascular predisposing risk factors before prescribing erectile dysfunction drugs.Keywords: sildenafil, nonarteritic anterior ischemic optic neuropathy, erectile dysfunction drugs, family history

Mitochondrial DNA Mutation Associated with Leber's Hereditary Optic Neuropathy

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Wallace, Douglas C.; Singh, Gurparkash; Lott, Marie T.; Hodge, Judy A.; Schurr, Theodore G.; Lezza, Angela M. S.; Elsas, Louis J.; Nikoskelainen, Eeva K.

1988-12-01

Leber's hereditary optic neuropathy is a maternally inherited disease resulting in optic nerve degeneration and cardiac dysrhythmia. A mitochondrial DNA replacement mutation was identified that correlated with this disease in multiple families. This mutation converted a highly conserved arginine to a histidine at codon 340 in the NADH dehydrogenase subunit 4 gene and eliminated an Sfa NI site, thus providing a simple diagnostic test. This finding demonstrated that a nucleotide change in a mitochondrial DNA energy production gene can result in a neurological disease.

[Bilateral non-arteritic ischemic optic neuropathy during treatment of viral hepatitis C with pegylated interferon and Ribavirin].

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Iferkhass, S; Elasri, F; Chatioui, S; Khoyaali, A; Bargach, T; Reda, K; Oubaaz, A

2015-01-01

Hepatitis C is a serious viral infection, for which the current treatment is based on the combination of pegylated interferon (IFN) and Ribavirin(®). Ophthalmic complications observed with PEG-IFN are infrequent and of variable prognosis. They often include an ischemic retinopathy with typical cotton-wool spots, hemorrhage and retinal edema, and rarely acute non-arteritic anterior ischemic optic neuropathy as illustrated by our report. We report the case of a 51-year-old man followed for chronic active hepatitis C, who presented in the fourth month of treatment with pegylated interferon and vidarabine with a sharp decline in visual acuity secondary to acute bilateral non-arteritic anterior ischemic optic neuropathy. The hepatitis C treatment was discontinued. His course was notable by the third week for a significant regression of papilledema with improvement in visual acuity in the right eye and no change in the left eye, remaining at counting fingers. After regressing for four years, the disease progressed to bilateral temporal optic atrophy without change in visual acuity. Pegylated interferon and Ribavirin(®) are commonly used in the treatment of chronic hepatitis C. They are the source of various ophthalmologic complications of varied severity. The pathophysiology of this ocular toxicity currently remains hypothetical. Non-arteritic ischemic optic neuropathy is still a relatively rare complication with a poor functional prognosis, often requiring discontinuation of treatment. Thus, careful ophthalmologic monitoring before and during antiviral treatment of patients with hepatitis C appears necessary. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Visual Rehabilitation of Persons with Leber's Hereditary Optic Neuropathy.

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Rudanko, S.-L.

1995-01-01

This article presents results of a noncontrolled clinical study of 20 persons with Leber's hereditary optic neuropathy who were treated from 1976 to 1990 at the Low Vision Centre of the Finnish Federation of the Visually Handicapped. The importance of early functional visual rehabilitation is emphasized, as is the use of low vision aids to help…

Progression of asymptomatic optic disc swelling to non-arteritic anterior ischaemic optic neuropathy.

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Subramanian, Prem S; Gordon, Lynn K; Bonelli, Laura; Arnold, Anthony C

2017-05-01

The time of onset of optic disc swelling in non-arteritic anterior ischaemic optic neuropathy (NAION) is not known, and it is commonly assumed to arise simultaneously with vision loss. Our goal is to report the presence and persistence of optic disc swelling without initial vision loss and its subsequent evolution to typical, symptomatic NAION. Clinical case series of patients with optic disc swelling and normal visual acuity and visual fields at initial presentation who progressed to have vision loss typical of NAION. All subjects underwent automated perimetry, disc photography and optic coherence tomography and/or fluorescein angiography to evaluate optic nerve function and perfusion. Four patients were found to have sectoral or diffuse optic disc swelling without visual acuity or visual field loss; the fellow eye of all four had either current or prior NAION or a 'disc at risk' configuration. Over several weeks of clinical surveillance, each patient experienced sudden onset of visual field and/or visual acuity loss typical for NAION. Current treatment options for NAION once vision loss occurs are limited and may not alter the natural history of the disorder. Subjects with NAION may have disc swelling for 2-10 weeks prior to the occurrence of visual loss, and with the development of new therapeutic agents, treatment at the time of observed disc swelling could prevent vision loss from NAION. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy

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Huai-En Lu

2018-04-01

Full Text Available Leber's hereditary optic neuropathy (LHON is a maternally inherited mitochondrial disease caused by homoplasmic point mutations in complex I subunit genes of mitochondrial DNA. In this report, we generated an induced pluripotent stem cell (iPSCs line, TVGH-iPSC-010-09, from the peripheral blood mononuclear cells of a female patient with Leber's hereditary optic neuropathy (LHON by using the Sendai-virus delivery system. The resulting iPSCs retained the disease-causing mitochondrial DNA mutation, expressed pluripotent markers and could differentiate into the three germ layers. We believe LHON patient-specific iPSCs provide a powerful in vitro model for evaluating the pathological phenotypes of the disease.

Neuropathies optiques héréditaires

DEFF Research Database (Denmark)

Milea, D; Verny, C

2012-01-01

Hereditary optic neuropathies are a group of heterogeneous conditions affecting both optic nerves, with an autosomal dominant, autosomal recessive, X-related or mitochondrial transmission. The two most common non-syndromic hereditary optic neuropathies (Leber's hereditary optic neuropathy...... and autosomal dominant optic atrophy) are very different in their clinical presentation and their genetic transmission, leading however to a common, non-specific optic nerve atrophy. Beyond the optic atrophy-related visual loss, which is the clinical hallmark of this group of diseases, other associated...

Post-Cataract Surgery Optic Neuropathy: Prevalence, Incidence, Temporal Relationship, and Fellow Eye Involvement.

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Moradi, Ahmadreza; Kanagalingam, Sivashakthi; Diener-West, Marie; Miller, Neil R

2017-03-01

To reassess the prevalence and incidence of post-cataract surgery optic neuropathy (PCSON) in the modern era. Retrospective cohort study. Setting: Single-center tertiary care practice. All patients with a diagnosis of nonarteritic anterior ischemic optic neuropathy (NAION) seen in the Wilmer Eye Network system between January 1, 2010 and December 31, 2014 were included. Inclusion was based on the following: (1) a history of an acute unilateral decrease in vision, (2) a visual field defect consistent with NAION, (3) a relative afferent pupillary defect, (4) observed optic disc swelling, and (5) no other etiology being found. The prevalence and incidence of PCSON and the temporal association between surgery and onset of PCSON. The secondary outcome was the risk of PCSON in the fellow eye of patients with prior unilateral spontaneous NAION. One hundred eighty-eight patients had developed NAION during the study period. Of these, 18 (9.6%) had undergone cataract surgery (CS) during the year prior to developing NAION. There was no significant temporal pattern associated with the distribution of NAION cases (P = .28). The incidence of PCSON in patients who had noncomplex CS was 10.9 cases per 100 000 (95% CI, 1.3, 39.4). Our data indicate that both the prevalence and incidence of NAION after modern CS are comparable to those of the general population and that there is no significant temporal relationship between modern CS and the subsequent development of NAION in the operated eye. Thus, although this study has inherent biases owing to its retrospective nature, concern regarding an increased risk of PCSON in the fellow eye in patients who have experienced it or spontaneous NAION in 1 eye may be unwarranted. Copyright © 2016 Elsevier Inc. All rights reserved.

Orbital apex cyst: a rare cause of compressive optic neuropathy post-functional endoscopic sinus surgery

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Koh YN

2017-07-01

Full Text Available Yi Ni Koh,1,2 Shu Fen Ho,2 Letchumanan Pathma,3 Harvinder Singh,3 Embong Zunaina1 1Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia; 2Department of Ophthalmology, 3Department of Otorhinolaryngology, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia Abstract: There are various causes that can lead to compressive optic neuropathy. We present here orbital apex cyst as an unusual cause of compressive optic neuropathy in a 49-year-old male. He presented with 2 weeks painless loss of vision in the left eye with left-sided headache. He had had left functional endoscopic sinus surgery for left nasal polyps 4 years earlier. Magnetic resonance imaging of brain and orbit revealed a left discrete orbital nodule, possibly orbital cyst or mucocele, which was compressing on the left optic nerve. Left eye vision improved markedly from hand movement to 6/36 pinhole 6/18 after initiation of intravenous dexamethasone. A subsequent endoscopic endonasal left optic nerve decompression found the orbital nodule lesion to be an orbital cyst. Marsupialization was performed instead of excision, as the cyst ruptured intraoperatively. Postoperative vision improved to 6/7.5 with normal optic nerve function postoperatively. Possible cause of orbital apex cyst is discussed. Keywords: orbital cyst, compressive optic neuropathy, functional endoscopic sinus surgery

Potential risk factors for diabetic neuropathy: a case control study

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Nooraei Mahdi

2005-12-01

Full Text Available Abstract Background Diabetes mellitus type II afflicts at least 2 million people in Iran. Neuropathy is one of the most common complications of diabetes and lowers the patient's quality of life. Since neuropathy often leads to ulceration and amputation, we have tried to elucidate the factors that can affect its progression. Methods In this case-control study, 110 diabetic patients were selected from the Shariati Hospital diabetes clinic. Michigan Neuropathic Diabetic Scoring (MNDS was used to differentiate cases from controls. The diagnosis of neuropathy was confirmed by nerve conduction studies (nerve conduction velocity and electromyography. The multiple factors compared between the two groups included consumption of angiotensin converting enzyme inhibitors (ACEI, blood pressure, serum lipid level, sex, smoking, method of diabetes control and its quality. Results Statistically significant relationships were found between neuropathy and age, gender, quality of diabetes control and duration of disease (P values in the order: 0.04, 0.04, Conclusion In this study, hyperglycemia was the only modifiable risk factor for diabetic neuropathy. Glycemic control reduces the incidence of neuropathy, slows its progression and improves the diabetic patient's quality of life. More attention must be paid to elderly male diabetic patients with poor diabetes control with regard to regular foot examinations and more practical education.

Endoscopic optic nerve decompression for nontraumatic compressive optic neuropathy

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Cheng-long REN

2015-11-01

Full Text Available Objective　To describe the preliminary experience with endoscopic optic nerve decompression (EOND for nontraumatic compressive optic neuropathies (NCONs. Methods　The clinical data of 10 patients, male 5 and female 5, with a mean age of 44.3±5.1 years, who underwent EOND for visual loss (n=5 or visual deterioration (n=5 due to tumor compression in General Hospital of Armed Police Forces of China in the period from April 2013 to April 2014 were analyzed retrospectively. Preoperative and 6-month-postoperative clinical and imaging data of these patients were reviewed and analyzed. Results　Among 5 patients who lost light perception (including 2 patients with bilateral optic nerve compression before operation, 4 of them showed visual improvement to different degrees on the 7th day after operation (with improvement of bilateral visual acuity. The other 5 patients with visual impairment before operation recovered their visual acuity to different extent after the operation. All of the patients had no obvious post-operative complications. Conclusion　EOND is a safe, effective, and minimally invasive surgical technique affording recovery of visual function to NCON patients. DOI: 10.11855/j.issn.0577-7402.2015.11.12

Shape Analysis of the Peripapillary RPE Layer in Papilledema and Ischemic Optic Neuropathy

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Kupersmith, Mark J.; Rohlf, F. James

2011-01-01

Purpose. Geometric morphometrics (GM) was used to analyze the shape of the peripapillary retinal pigment epithelium–Bruch's membrane (RPE/BM) layer imaged on the SD-OCT 5-line raster in normal subjects and in patients with papilledema and ischemic optic neuropathy. Methods. Three groups of subjects were compared: 30 normals, 20 with anterior ischemic optic neuropathy (AION), and 25 with papilledema and intracranial hypertension. Twenty equidistant semilandmarks were digitized on OCT images of the RPE/BM layer spanning 2500 μm on each side of the neural canal opening (NCO). The data were analyzed using standard GM techniques, including a generalized least-squares Procrustes superimposition, principal component analysis, thin-plate spline (to visualize deformations), and permutation statistical analysis to evaluate differences in shape variables. Results. The RPE/BM layer in normals and AION have a characteristic V shape pointing away from the vitreous; the RPE/BM layer in papilledema has an inverted U shape, skewed nasally inward toward the vitreous. The differences were statistically significant. There was no significant difference in shapes between normals and AION. Pre- and posttreatment OCTs, in select cases of papilledema, showed that the inverted U-shaped RPE/BM moved posteriorly into a normal V shape as the papilledema resolved with weight loss or shunting. Conclusions. The shape difference in papilledema, absent in AION, cannot be explained by disc edema alone. The difference is a consequence of both the translaminar pressure gradient and the material properties of the peripapillary sclera. GM offers a novel way of statistically assessing shape differences of the peripapillary optic nerve head. PMID:21896851

The morphological difference between glaucoma and other optic neuropathies

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Burgoyne, Claude

2016-01-01

The clinical phenomenon of cupping has two principal pathophysiologic components in all optic neuropathies: prelaminar thinning and laminar deformation. We define prelaminar thinning to be the portion of cup enlargement that results from thinning of the prelaminar tissues due to physical compression and/or loss of Retinal Ganglion Cell axons. We define laminar deformation or laminar cupping to be the portion of cup enlargement that results from permanent, intraocular pressure-(IOP) induced deformation of the lamina cribrosa and peripapillary scleral connective tissues following damage and/or remodeling. We propose that the defining phenomenon of glaucomatous cupping is deformation and/or remodeling of the neural and connective tissues of the optic nerve head (ONH), which is governed by the distribution of IOP-related connective tissue stress and strain, regardless of the mechanism of insult or the level of IOP at which that deformation and/or remodeling occurs. Said in another way, “glaucomatous cupping” is the term clinicians use to describe the clinical appearance and behavior the ONH assumes as its neural and connective tissues deform, remodel or mechanically fail: 1) in a pattern and 2) by the several pathophysiologic processes governed by IOP-related connective tissue stress and strain. ONH Biomechanics explains why a given optic nerve head will demonstrate a certain form of “cupping” and at what level of IOP that might happen. Animal models are allowing us to tease apart the important components of cupping in IOP-related and non-IOP-related forms of optic neuropathy. A paradigm change in spectral domain optical coherence tomography ONH, retinal nerve fiber layer and Macular imaging should improve our ability to phenotype all forms of damage to the visual system including glaucoma. PMID:26274837

Linezolid-associated optic neuropathy in a pediatric patient with Mycobacterium nonchromogenicum: A case report.

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González Saldaña, Napoleón; Galvis Trujillo, Diego Mauricio; Borbolla Pertierra, Ana Maria; Mondragón Pineda, Ana Ivette; Juárez Olguín, Hugo

2017-12-01

Toxic optic neuropathies are alterations of the optic nerve and can be caused by environmental, pharmacological, or nutritional agents. It is about a 7-year-old male patient, a native of the State of Mexico, Mexico who was diagnosed with cervical mycobacterial lymphadenitis that required management with linezolid. After 7 months of treatment, visual acuity of the left eye decreased and was accompanied by headache. Neuroinfection and other central nervous system affections were discarded. An adverse effect related to treatment with linezolid was suspected, and linezolid was suspended. The symptoms subsided after discontinuation; however, the patient continued to show decreased visual acuity of the left eye, assessed by his ability to count 2 fingers. The right eye remained unaffected. Neurotoxicity can be decreased by reducing the total dose of linezolid or by administrating it in an intermittent form. To avoid progression and loss of vision, we suggest frequent periodic ophthalmological evaluation in patients treated with linezolid. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

Optic neuropathies--importance of spatial distribution of mitochondria as well as function.

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Yu Wai Man, C Y; Chinnery, P F; Griffiths, P G

2005-01-01

Optic neuropathies such as Leber's hereditary optic neuropathy, dominant optic atrophy and toxic amblyopia are an important cause of irreversible visual failure. Although they are associated with a defect of mitochondrial energy production, their pathogenesis is poorly understood. A common feature to all these disorders is relatively selective degeneration of the papillomacular bundle of retinal ganglion cells resulting central or caecocentral visual field defects. The striking similarity in the pattern of clinical involvement seen with these disparate disorders suggests a common pathway in their aetiology. The existing hypothesis that the optic nerve head has higher energy demands than other tissues making it uniquely dependent on oxidative phosporylation is not satisfactory. First, other ocular tissues such as photoreceptors, which are more dependent on oxidative phosporylation are not affected. Second, other mitochondrial disorders, which have a greater impact on mitochondrial energy function, do not affect the optic nerve. The optic nerve head has certain unique ultra structural features. Ganglion cell axons exit the eye through a perforated collagen plate, the lamina cribrosa. There is a sharp discontinuity in the density of mitochondria at the optic nerve head, with a very high concentration in the prelaminar nerve fibre layer and low concentration behind the lamina. This has previously been attributed to a mechanical hold up of axoplasmic flow, which has itself been proposed as a factor in the pathogenesis of a number of optic neuropathies. More recent evidence shows that mitochondrial distribution reflects the different energy requirements of the unmyelinated prelaminar axons in comparison to the myelinated retrolaminar axons. The heterogeous distribution of mitochondria is actively maintained to support conduction through the optic nerve head. We propose that factors that disrupt the heterogeneous distribution of mitochondria can result in ganglion cell

Ischemic optic neuropathy as a model of neurodegenerative disorder: A review of pathogenic mechanism of axonal degeneration and the role of neuroprotection.

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Khalilpour, Saba; Latifi, Shahrzad; Behnammanesh, Ghazaleh; Majid, Amin Malik Shah Abdul; Majid, Aman Shah Abdul; Tamayol, Ali

2017-04-15

Optic neuropathy is a neurodegenerative disease which involves optic nerve injury. It is caused by acute or intermittent insults leading to visual dysfunction. There are number of factors, responsible for optic neuropathy, and the optic nerve axon is affected in all type which causes the loss of retinal ganglion cells. In this review we will highlight various mechanisms involved in the cell loss cascades during axonal degeneration as well as ischemic optic neuropathy. These mechanisms include oxidative stress, excitotoxicity, angiogenesis, neuroinflammation and apoptosis following retinal ischemia. We will also discuss the effect of neuroprotective agents in attenuation of the negative effect of factors involve in the disease occurrence and progression. Copyright © 2016. Published by Elsevier B.V.

The Correlation between Subjective and Objective Visual Function Test in Optic Neuropathy Patients

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Ungsoo Kim

2012-10-01

Full Text Available Purpose: To investigate the correlation between visual acuity and quantitative measurements of visual evoked potentials (VEP, optical coherence tomography (OCT, and visual field test (VF in optic neuropathy patients. Methods: We evaluated 28 patients with optic neuropathy. Patients who had pale disc, visual acuity of less than 0.5 and abnormal visual field defect were included. At the first visit, we performed visual acuity and VF as subjective methods and OCT and VEP as objective methods. In the spectral domain OCT, rim volume, average and temporal quadrant retinal nerve fiber layer (RNFL thickness were measured. And pattern VEP (N75, P100, N135 latency, and P100 amplitude and Humphrey 24-2 visual field test (mean deviation and pattern standard deviation were obtained. Using Spearman's correlation coefficient, the correlation between visual acuity and various techniques were assessed. Results: Visual acuity was most correlated with the mean deviation of Humphrey perimetry.

Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy.

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Miller, N R; Arnold, A C

2015-01-01

Nonarteritic anterior ischaemic optic neuropathy (NAION) is the most common acute optic neuropathy in patients over the age of 50 and is the second most common cause of permanent optic nerve-related visual loss in adults after glaucoma. Patients typically present with acute, painless, unilateral loss of vision associated with a variable visual field defect, a relative afferent pupillary defect, a swollen, hyperaemic optic disc, and one or more flame-shaped peripapillary retinal haemorrhages. The pathogenesis of this condition is unknown, but it occurs primarily in patients with structurally small optic discs that have little or no cup and a variety of underlying vascular disorders that may or may not be known at the time of visual loss. There is no consistently beneficial medical or surgical treatment for the condition, but there are now animal models that allow testing of various potential therapies. About 40% of patients experience spontaneous improvement in visual acuity. Patients in whom NAION occurs in one eye have a 15-19% risk of developing a similar event in the opposite eye over the subsequent 5 years.

Does altered fractionation influence the risk of radiation-induced optic neuropathy?

International Nuclear Information System (INIS)

Bhandare, Niranjan; Monroe, Alan T.; Morris, Christopher G.; Bhatti, M. Tariq; Mendenhall, William M.

2005-01-01

Purpose: To analyze the parameters that influence the risk of radiation-induced optic neuropathy (RION) after radiotherapy for head-and-neck tumors. Methods and Materials: Between 1964 and 2000, 273 patients with tumors of the nasopharynx, paranasal sinuses, nasal cavity, and hard palate adenoid cystic carcinomas were treated with curative intent and had radiation fields that included the optic nerves and/or chiasm. Patients were followed for at least 1 year after radiotherapy. Results: Radiation-induced optic neuropathy developed in 32 eyes of 24 patients (9%). The 5-year rates of freedom from RION according to the total dose and once- vs. twice-daily fractionation were as follows: ≤63 Gy once daily, 95%; ≤63 Gy twice daily, 98%; >63 Gy once daily, 78%; and >63 Gy twice daily, 91%. Multivariate analysis revealed that the total dose affected the risk of RION (p = 0.0047), with patient age (p = 0.0909), once-daily vs. twice-daily fractionation (p = 0.0684), and overall treatment time (p = 0.0972) were marginally significant. The use of adjuvant chemotherapy did not significantly influence the likelihood of developing RION. Conclusion: The likelihood of developing RION is primarily influenced by the total dose. Hyperfractionation may reduce the risk of experiencing this complication

Bilateral Glaucomatous Optic Neuropathy Caused by Eye Rubbing.

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Savastano, Alfonso; Savastano, Maria Cristina; Carlomusto, Laura; Savastano, Silvio

2015-01-01

In this report, we describe a particular condition of a 52-year-old man who showed advanced bilateral glaucomatous-like optic disc damage, even though the intraocular pressure resulted normal during all examinations performed. Visual field test, steady-state pattern electroretinogram, retinal nerve fiber layer and retinal tomographic evaluations were performed to evaluate the optic disc damage. Over a 4-year observational period, his visual acuity decreased to 12/20 in the right eye and counting fingers in the left eye. Visual fields were severely compromised, and intraocular pressure values were not superior to 14 mm Hg during routine examinations. An accurate anamnesis and the suspicion of this disease represent a crucial aspect to establish the correct diagnosis. In fact, our patient strongly rubbed his eyes for more than 10 h per day. Recurrent and continuous eye rubbing can induce progressive optic neuropathy, causing severe visual field damage similar to the pathology of advanced glaucoma.

Genetic and Clinical Analyses of DOA and LHON in 304 Chinese Patients with Suspected Childhood-Onset Hereditary Optic Neuropathy.

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Yadi Li

Full Text Available Leber hereditary optic neuropathy (LHON and dominant optic atrophy (DOA, the most common forms of hereditary optic neuropathy, are easily confused, and it is difficult to distinguish one from the other in the clinic, especially in young children. The present study was designed to survey the mutation spectrum of common pathogenic genes (OPA1, OPA3 and mtDNA genes and to analyze the genotype-phenotype characteristics of Chinese patients with suspected childhood-onset hereditary optic neuropathy. Genomic DNA and clinical data were collected from 304 unrelated Chinese probands with suspected hereditary optic neuropathy with an age of onset below 14 years. Sanger sequencing was used to screen variants in the coding and adjacent regions of OPA1, OPA3 and the three primary LHON-related mutation sites in mitochondrial DNA (mtDNA (m.3460G>A, m.11778G>A and m.14484T>C. All patients underwent a complete ophthalmic examination and were compared with age-matched controls. We identified 89/304 (29.3% primary mtDNA mutations related to LHON in 304 probands, including 76 mutations at m.11778 (76/89, 85.4% of all mtDNA mutations, four at m.3460 (4/89, 4.5% and nine at m.14484 (9/89, 10.1%. This result was similar to the mutation frequency among Chinese patients with LHON of any age. Screening of OPA1 revealed 23 pathogenic variants, including 11 novel and 12 known pathogenic mutations. This study expanded the OPA1 mutation spectrum, and our results showed that OPA1 mutation is another common cause of childhood-onset hereditary optic neuropathy in Chinese pediatric patients, especially those with disease onset during preschool age.

Efficacy of spinal cord stimulators in treating peripheral neuropathy: a case series.

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Abd-Elsayed, Alaa; Schiavoni, Nick; Sachdeva, Harsh

2016-02-01

Peripheral neuropathy is a common cause of pain, and it is increasing in prevalence. Peripheral neuropathic pain is very hard to treat and can be resistant to multiple pain management modalities. Our series aimed at testing the efficacy of spinal cord stimulators (SCSs) in treating resistant painful peripheral neuropathy. Case 1: A 79-year-old man presented to our clinic with long-standing history of painful peripheral diabetic neuropathy resistant to conservative management. After failure of all possible modalities, we offered the patient an SCS trial that was very successful, and we proceeded with the permanent implant that continued to help with his pain and allowed the patient to wean down his medications. Case 2: A 60-year-old man presented with chronic peripheral neuropathy secondary to HIV, patient failed all conservative and procedural management. Patient then had an SCS trial that relieved his pain significantly. Unfortunately, we did not proceed with the implant due to deterioration of the patient general health. Case 3: A 39-year-old woman presented with painful peripheral neuropathy secondary to chemotherapy for breast cancer. After failure of medication management and procedures, patient had a SCS trial that improved her pain and we then proceeded with performing the permanent implant that controlled her pain. We presented 3 cases with chronic painful peripheral neuropathy secondary to HIV, diabetes mellitus, and chemotherapy that was resistant to conservative pain management and procedures that was successfully treated with neurostimulation. Copyright © 2016 Elsevier Inc. All rights reserved.

Drug-induced peripheral neuropathy

DEFF Research Database (Denmark)

Vilholm, Ole Jakob; Christensen, Alex Alban; Zedan, Ahmed

2014-01-01

Peripheral neuropathy can be caused by medication, and various descriptions have been applied for this condition. In this MiniReview, the term 'drug-induced peripheral neuropathy' (DIPN) is used with the suggested definition: Damage to nerves of the peripheral nervous system caused by a chemical...... substance used in the treatment, cure, prevention or diagnosis of a disease. Optic neuropathy is included in this definition. A distinction between DIPN and other aetiologies of peripheral neuropathy is often quite difficult and thus, the aim of this MiniReview is to discuss the major agents associated...

The Decrease in Mitochondrial DNA Mutation Load Parallels Visual Recovery in a Leber Hereditary Optic Neuropathy Patient

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Sonia Emperador

2018-02-01

Full Text Available The onset of Leber hereditary optic neuropathy is relatively rare in childhood and, interestingly, the rate of spontaneous visual recovery is very high in this group of patients. Here, we report a child harboring a rare pathological mitochondrial DNA mutation, present in heteroplasmy, associated with the disease. A patient follow-up showed a rapid recovery of the vision accompanied by a decrease of the percentage of mutated mtDNA. A retrospective study on the age of recovery of all childhood-onset Leber hereditary optic neuropathy patients reported in the literature suggested that this process was probably related with pubertal changes.

Peripheral neuropathy in a diabetic child treated with linezolid for multidrug-resistant tuberculosis: a case report and review of the literature.

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Swaminathan, Aravind; du Cros, Philipp; Seddon, James A; Mirgayosieva, Shamsiya; Asladdin, Rajabov; Dusmatova, Zulfiya

2017-06-12

Extensively drug-resistant (XDR) tuberculosis (TB) and multidrug resistant (MDR)-TB with additional resistance to injectable agents or fluoroquinolones are challenging to treat due to lack of available, effective drugs. Linezolid is one of the few drugs that has shown promise in treating these conditions. Long-term linezolid use is associated with toxicities such as peripheral and optic neuropathies. Diabetes mellitus (DM), especially when uncontrolled, can also result in peripheral neuropathy. The global burden of DM is increasing, and DM has been associated with a three-fold increased risk of developing TB disease. TB and DM can be a challenging combination to treat. DM can inhibit the host immune response to tuberculosis infection; and TB and some anti-TB drugs can worsen glycaemic control. A child experiencing neuropathy that is a possible complication of both DM and linezolid used to treat TB has not been reported previously. We report peripheral neuropathy in a 15-year-old boy with type 1 DM, diagnosed with MDR-TB and additional resistance to injectable TB medications. The boy was treated with a linezolid-based regimen, but after 8 months developed peripheral neuropathy. It was unclear whether the neuropathy was caused by the DM or the linezolid therapy. He had clinical improvement following cessation of linezolid and was declared cured following 21 months of treatment. Following completion of treatment, nerve conduction studies demonstrated significant improvement in neuropathy. To the best of our knowledge, this is the first case of peripheral neuropathy reported in a diabetic child on long-term linezolid therapy for tuberculosis. This case study underlines the importance of stringent follow-up for side effects of linezolid, especially when associated with co-morbidity such as DM that increases the chances of adverse effects. The presence of both DM and TB should alert a physician to strive for optimal glycaemic control to minimize the risk of

Risk factors and long-term changes of non-arteritis anterior ischaemic optic neuropathy

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Ying-Xin Cui

2016-01-01

Full Text Available AIM:To investigate the risk factors and long-term changes of non-arteritis anterior ischaemic optic neuropathy(NAION. METHODS:Three hundred and sixty cases of patients with NAION in our hospital from January 2010 to Juny 2015 were used as patients group and another 400 people undergoing health examination were used as control group. The clinical data was collected. Optical coherence tomography(OCTwas performed. RESULTS:There were significant difference on gender, history of diabetes or hypertension, arteriosclerosis history, disc area, cup area, rim area, cup/disc area ratio, horizontal cup-disc ratio, vertical cup-disc ratio, FBG and TG of two groups(PPP>0.05. CONCLUSION:Male, with diabetes, history of hypertension, arteriosclerosis history, disc area, cup area, rim area, cup/disc area ratio, horizontal cup-disc ratio, vertical cup-disc ratio, FBG and TG are independent risk factors of NAION. Long-term damage of RNFL may not aggravate.

Factors predicting optic nerve axonal degeneration after methanol-induced acute optic neuropathy: a 2-year prospective study in 54 patients

Czech Academy of Sciences Publication Activity Database

Zakharov, S.; Nurieva, O.; Kotíková, K.; Urban, P.; Navrátil, Tomáš; Pelclová, D.

2016-01-01

Roč. 147, č. 1 (2016), s. 251-261 ISSN 0026-9247 Institutional support: RVO:61388955 Keywords : methanol optic neuropathy * visual evoked potentials * axonal degeneration Subject RIV: CF - Physical ; Theoretical Chemistry Impact factor: 1.282, year: 2016

Structure-Function Analysis of Nonarteritic Anterior Ischemic Optic Neuropathy and Age-Related Differences in Outcome.

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Sun, Ming-Hui; Liao, Yaping Joyce

2017-09-01

The optic nerve head is vulnerable to ischemia leading to anterior ischemic optic neuropathy (AION), the most common acute optic neuropathy in those older than 50 years of age. We performed a cross-sectional study of 55 nonarteritic anterior ischemic optic neuropathy (NAION) eyes in 34 patients to assess clinical outcome and perform structure-function correlations. The peak age of NAION onset was between 50 and 55 years. Sixty-seven percent of patients presented with their first event between the ages of 40 and 60 years, and 32% presented at ≤50 years. Those with NAION onset at age ≤50 years did not have significantly better visual outcome per logMAR visual acuity, automated perimetric mean deviation (PMD) or optical coherence tomography (OCT) measurements. Kaplan-Meier survival curve and multivariate Cox proportional regression analysis showed that age >50 years at NAION onset was associated with greater risk of second eye involvement, with hazard ratio of 20. Older age at onset was significantly correlated with greater thinning of the ganglion cell complex (GCC) (P = 0.022) but not with logMAR visual acuity, PMD, or thinning of retinal nerve fiber layer (RNFL). Using area under receiver operating characteristic curve analyses, we found that thinning of RNFL and GCC was best able to predict visual outcome, and that mean RNFL thickness >65 μm or macular GCC thickness >55 μm significantly correlated with good visual field outcome. We showed that NAION onset at age >50 years had a greater risk of second eye involvement. Patients with OCT mean RNFL thickness >65 μm and mean macular ganglion cell complex thickness >55 μm had better visual outcomes.

Optic neuropathies: the tip of the neurodegeneration iceberg

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Carelli, Valerio; La Morgia, Chiara; Ross-Cisneros, Fred N.; Sadun, Alfredo A.

2017-01-01

Abstract The optic nerve and the cells that give origin to its 1.2 million axons, the retinal ganglion cells (RGCs), are particularly vulnerable to neurodegeneration related to mitochondrial dysfunction. Optic neuropathies may range from non-syndromic genetic entities, to rare syndromic multisystem diseases with optic atrophy such as mitochondrial encephalomyopathies, to age-related neurodegenerative diseases such as Alzheimer’s and Parkinson’s disease where optic nerve involvement has, until recently, been a relatively overlooked feature. New tools are available to thoroughly investigate optic nerve function, allowing unparalleled access to this part of the central nervous system. Understanding the molecular pathophysiology of RGC neurodegeneration and optic atrophy, is key to broadly understanding the pathogenesis of neurodegenerative disorders, for monitoring their progression in describing the natural history, and ultimately as outcome measures to evaluate therapies. In this review, the different layers, from molecular to anatomical, that may contribute to RGC neurodegeneration and optic atrophy are tackled in an integrated way, considering all relevant players. These include RGC dendrites, cell bodies and axons, the unmyelinated retinal nerve fiber layer and the myelinated post-laminar axons, as well as olygodendrocytes and astrocytes, looked for unconventional functions. Dysfunctional mitochondrial dynamics, transport, homeostatic control of mitobiogenesis and mitophagic removal, as well as specific propensity to apoptosis may target differently cell types and anatomical settings. Ultimately, we can envisage new investigative approaches and therapeutic options that will speed the early diagnosis of neurodegenerative diseases and their cure. PMID:28977448

Vitamin B6 in Health Supplements and Neuropathy: Case Series Assessment of Spontaneously Reported Cases.

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van Hunsel, Florence; van de Koppel, Sonja; van Puijenbroek, Eugène; Kant, Agnes

2018-05-08

In the literature, vitamin B 6 has been linked to the development of polyneuropathy. Most often, these complaints were seen when taking high doses of vitamin B 6 for a long time. Evidence as to whether a lower dosage range of vitamin B 6 (< 50 mg/day) can also induce neuropathy is scarce. We aim to comprehensively describe the cases of neuropathy associated with vitamin B 6 received by the Netherlands Pharmacovigilance Centre Lareb and to assess the case series concerning the use of vitamin B 6 and neuropathic complaints. We describe the number and nature of the reported cases, including suspect product, dosage, duration of use, and vitamin B 6 serum levels. In addition, we describe the causality for the individual cases (Naranjo Probability Scale) and for the entire case series (Bradford Hill criteria). In total, 90 reports on products containing vitamin B 6 included at least one adverse drug reaction in the standardized Medical Dictionary for Regulatory Activities (MedDRA ® ) query (SMQ; broad) 'peripheral neuropathy'. The amount of vitamin B 6 in the products varied between 1.4 and 100 mg per tablet. The serum vitamin B 6 level was known in 36 cases (88-4338 nmol/l), and the mean serum vitamin B 6 level was 907 nmol/l. However, no statistical correlation between dosage and vitamin B 6 blood levels was found. Causality assessment of the case series of 90 reports to Lareb shows it is plausible for the vitamin B 6 supplements to have caused complaints such as neuropathies. This is especially the case with higher dosages and prolonged use, but dosages < 50 mg/day also cannot be excluded.

Clinical features of dysthyroid optic neuropathy: a European Group on Graves' Orbitopathy (EUGOGO) survey

NARCIS (Netherlands)

McKeag, David; Lane, Carol; Lazarus, John H.; Baldeschi, Lelio; Boboridis, Kostas; Dickinson, A. Jane; Hullo, A. Iain; Kahaly, George; Krassas, Gerry; Marcocci, Claudio; Marinò, Michele; Mourits, Maarten P.; Nardi, Marco; Neoh, Christopher; Orgiazzi, Jacques; Perros, Petros; Pinchera, Aldo; Pitz, Susanne; Prummel, Mark F.; Sartini, Maria S.; Wiersinga, Wilmar M.

2007-01-01

BACKGROUND: This study was performed to determine clinical features of dysthyroid optic neuropathy (DON) across Europe. METHODS: Forty seven patients with DON presented to seven European centres during one year. Local protocols for thyroid status, ophthalmic examination and further investigation

Paraneoplastic neuropathies.

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Antoine, Jean-Christophe; Camdessanché, Jean-Philippe

2017-10-01

To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease. Neuropathies improving with tumor treatment are occasional, occur with a variety of cancer and include motor neuron disease, chronic inflammatory demyelinating neuropathy and nerve vasculitis. If antibodies toward intracellular antigens are well characterized, it is not the case for antibodies toward cell membrane proteins. Contactin-associated protein-2 antibodies occur with neuromyotonia and thymoma with the Morvan's syndrome in addition to Netrin 1 receptor antibodies but may not be responsible for peripheral nerve hyperexcitability. The treatment of AL amyloidosis, POEMS syndrome, anti-MAG neuropathy and cryoglobulinemia is now relatively well established. It is not the case with onconeural antibodies for which the rarity of the disorders and a short therapeutic window are limiting factors for the development of clinical trials. A strict definition of paraneoplastic neuropathies helps their identification and is necessary to allow an early diagnosis of the underlying tumor.

Topiramate-induced Neuropathy Mimicking Carpal Tunnel Syndrome: A Case Report

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Jigar S. Gandhi

2018-01-01

Full Text Available Carpel tunnel syndrome (CTS is a condition in which median nerve compression results in paresthesias and pain in thewrist and hand. We are going to report a rare case of topiramate-induced neuropathy which clinically resembles CTS.Discontinuation of topiramate resulted in spontaneous resolution of numbness, paresthesia and pain in a few days. Highclinical suspicion is advised in patients who are on topiramate and present with signs of compressive neuropathy.

Neuropatia óptica isquêmica anterior e poliarterite nodosa: relato de caso Anterior ischemic optic neuropathy and polyarteritis nodosa: case report

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Christie Michelle Graf

2006-02-01

Full Text Available Descreve-se uma paciente com diagnóstico de poliarterite nodosa, em tratamento com citostáticos e corticosteróides que desenvolveu neuropatia óptica isquêmica anterior, uma manifestação considerada bastante rara para esta doença.We describe a patient with polyarteritis nodosa being treated with cytostatics and corticosteroids who developed an anterior ischemic optic neuropathy, a rare manifestation of this pathology.

Bilateral Simultaneous Nonarteritic Anterior Ischemic Optic Neuropathy after Ingestion of Sildenafil for Erectile Dysfunction

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Anna Tarantini

2012-01-01

Full Text Available Purpose. To describe a patient who developed bilateral, simultaneous nonarteritic anterior ischemic optic neuropathy (NAION after ingestion of Sildenafil citrate (Viagra for erectile dysfunction. Methods. Observational case report. Results. A 60-year-old diabetic man noted sudden decrease of vision in both eyes 16 hours after his third consecutive 50 mg daily Sildenafil ingestion. A diagnosis of bilateral NAION was made and he was treated for three days with methylprednisolone 1 g/d intravenously, followed by oral prednisone 75 mg/d. Final visual acuity was 20/50 right eye (OD and 20/20 left eye (OS. He had preexisting diabetes. Conclusion. This is the first reported case of simultaneous bilateral NAION occurred in a diabetic patient early after Sildenafil intake. Patients with predisposing conditions such as diabetes have to be warned against the use of PDE inhibitors.

Neutrophil-to-Lymphocyte Ratio as a Marker in Patients with Non-arteritic Anterior Ischemic Optic Neuropathy

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Onur Polat

2015-12-01

Full Text Available Background: Non-arteritic anterior ischemic optic neuropathy (NAION is the most common acute optic neuropathy in patients over the age of 50 and is the second most common cause of permanent optic nerve-related visual loss in adults after glaucoma. Although the precise cause of NAION remains elusive, the etiology of NAION is believed to be multifactorial. Aims: To evaluate the utility of neutrophil-to-lymphocyte ratio (NLR as a simple and readily available prognostic factor for clinical disease activity in patients with NAION. Study Design: Case-control study. Methods: Forty-five patients with the diagnosis of NAION and 50 age- and sex-matched controls with/without any systemic or ocular diseases except cataract were retrospectively enrolled in the study. Demographic characteristics and laboratory findings including complete blood count of all patients and control subjects were obtained from the electronic medical record. The neutrophil and lymphocyte counts were recorded and the NLR was calculated. Results: White blood cell, neutrophil, NLR and platelet values of the NAION patients were significantly higher than those of the controls (p<0.001, p<0.001, p=0.004, p=0.037, respectively. Initial NLR values were negatively correlated with initial and the third month best corrected visual acuity levels in the study group. The optimum NLR cut-off point for NAION was 1.94. Conclusion: NLR could be considered as a new inflammatory marker for assessment of the severity of inflammation in NAION patients with its quick, cheap, easily measurable property with routine complete blood count analysis.

Inspection methods progression of diabetic optic neuropathy

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Yan Sun

2015-06-01

Full Text Available Increasing incidence of diabetes, diet restructuring with excessive intake of high-calorie foods closely related with this. Currently diabetes prevalence rate increased from 7% in 2003 to 14% in 2010. Diabetes can cause a variety of eye diseases, such as corneal ulcers, glaucoma, vitreous hemorrhage and so on. Diabetic retinopathy and cataract are the most common and greater impact on patients. At present, study for diabetic retinopathy(DRis wider than diabetes optic neuropathy(DON. Clinical manifestations of DON are not specific, but DON occurred extensively, also contributed to an important cause of blindness.In this paper, we collected a variety of inspection and early diagnosis methods, try to achieve early detection, interventional therapy and good treatment for this disease. Here to make a presentation on the various types of inspection methods.

Topiramate induced peripheral neuropathy: A case report and review of literature.

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Hamed, Sherifa Ahmed

2017-12-16

Drug-induced peripheral neuropathy had been rarely reported as an adverse effect of some antiepileptic drugs (AEDs) at high cumulative doses or even within the therapeutic drug doses or levels. We describe clinical and diagnostic features of a patient with peripheral neuropathy as an adverse effect of chronic topiramate (TPM) therapy. A 37-year-old woman was presented for the control of active epilepsy (2010). She was resistant to some AEDs as mono- or combined therapies (carbamazepine, sodium valproate, levetiracetam, oxcarbazepine and lamotrigine). She has the diagnosis of frontal lobe epilepsy with secondary generalization and has a brother, sister and son with active epilepsies. She became seizure free on TPM (2013-2017) but is complaining of persistent distal lower extremities paresthesia in a stocking distribution. Neurological examination revealed presence of diminished Achilles tendon reflexes, stocking hypesthesia and delayed distal latencies, reduced conduction velocities and amplitudes of action potentials of posterior tibial and sural nerves, indicating demyelinating and axonal peripheral neuropathy of the lower extremities. After exclusion of the possible causes of peripheral neuropathy, chronic TPM therapy is suggested as the most probable cause of patient's neuropathy. This is the first case report of topiramate induced peripheral neuropathy in the literature.

Blunt Facial Trauma Causing Isolated Optic Nerve Hematoma

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R. Parab

2013-01-01

Full Text Available Traumatic optic neuropathy is an uncommon, yet serious, result of facial trauma. The authors present a novel case of a 59-year-old gentleman who presented with an isolated blunt traumatic left optic nerve hematoma causing vision loss. There were no other injuries or fractures to report. This case highlights the importance of early recognition of this rare injury and reviews the current literature and management of traumatic optic neuropathy.

Optic atrophy, cataracts, lipodystrophy/lipoatrophy, and peripheral neuropathy caused by a de novo OPA3 mutation

OpenAIRE

Bourne, Stephanie C.; Townsend, Katelin N.; Shyr, Casper; Matthews, Allison; Lear, Scott A.; Attariwala, Raj; Lehman, Anna; Wasserman, Wyeth W.; van Karnebeek, Clara; Sinclair, Graham; Vallance, Hilary; Gibson, William T.

2017-01-01

We describe a woman who presented with cataracts, optic atrophy, lipodystrophy/lipoatrophy, and peripheral neuropathy. Exome sequencing identified a c.235C > G p.(Leu79Val) variant in the optic atrophy 3 (OPA3) gene that was confirmed to be de novo. This report expands the severity of the phenotypic spectrum of autosomal dominant OPA3 mutations.

Femoral neuropathy due to patellar dislocation in a theatrical and jazz dancer: a case report.

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Shin, Chris S; Davis, Brian A

2005-06-01

This case report describes a teenage female, high-level modern dancer who suffered multiple left patellar dislocations. Her history is atypical in that after her fifth dislocation, her recovery was hindered secondary to persistent weakness and atrophy of her quadriceps out of proportion to disuse alone. Electrodiagnostic studies and magnetic resonance imaging showed evidence of a subacute femoral neuropathy correlating chronologically with her most recent patellar dislocation. This case suggests that further diagnostic study may be warranted in patients with persistent quadriceps weakness or atrophy after a patellar dislocation, because this may suggest the presence of a femoral neuropathy. This is important because the strength training goals and precautions differ in disuse atrophy and a neuropathy. We believe this is the first reported case of a femoral neuropathy associated with the mechanism of a patellar dislocation.

Celiac Disease Presenting with Peripheral Neuropathy in Children: A Case Report.

Science.gov (United States)

Pacitto, Alessandra; Paglino, Alessandra; Di Genova, Lorenza; Leonardi, Alberto; Farinelli, Edoardo; Principi, Nicola; di Cara, Giuseppe; Esposito, Susanna

2017-07-14

Background: Clinically relevant neurological manifestations in children with celiac disease (CD) are unusual, especially when they are considered as signs of the onset of the disease. In this paper, a case of Guillain-Barrè syndrome (GBS) as the first manifestation of CD in a 23-month-old child is reported. Case presentation: We describe a case of CD onset with peripheral neuropathy in a 23-month-old Bulgarian boy presenting with a sudden refusal to walk and absence of deep tendon reflexes in both lower limbs. Neurological symptoms were preceded by two months of gastrointestinal symptoms such as vomiting, abdominal distention, and clear signs of malnutrition and weight loss. When we evaluated the child six months after the onset of the symptoms, clinical and laboratory findings showed clear signs of peripheral neuropathy associated with malnutrition. Serum deamidated gliadin and tissue transglutaminase antibodies were therefore measured. The anti-gliadin levels were more than sixteen times higher than normal and the IgA anti-transglutaminase levels were four times higher than normal. Anti-endomysium antibodies were positive, and human leukocyte antigens (HLA) II typing confirmed a genetic predisposition to CD (DQ2 positive and DQ8 negative). Given the association between the clinical evidence of the disease and the results of the celiac screening tests, a diagnosis of CD was made without biopsy confirmation of the enteropathy. The child began a restricted gluten-free diet that led to complete recovery of the peripheral neuropathy, walking, reflexes, and overall improvement after three months on the diet. Conclusion: Our case underlines the rare but possible associations between CD and peripheral neuropathy in children as an onset symptom, even in the absence of gastrointestinal manifestations, thus suggesting that CD should always be considered in the differential diagnosis of peripheral neuropathy in children. A good knowledge of the extra

Antivascular Endothelial Growth Factor Bevacizumab for Radiation Optic Neuropathy: Secondary to Plaque Radiotherapy

International Nuclear Information System (INIS)

Finger, Paul T.; Chin, Kimberly J.

2012-01-01

Purpose: To evaluate the intravitreal antivascular endothelial growth factor, bevacizumab, for treatment of radiation optic neuropathy (RON). Methods and Materials: A prospective interventional clinical case series was performed of 14 patients with RON related to plaque radiotherapy for choroidal melanoma. The RON was characterized by optic disc edema, hemorrhages, microangiopathy, and neovascularization. The entry criteria included a subjective or objective loss of vision, coupled with findings of RON. The study subjects received a minimum of two initial injections of intravitreal bevacizumab (1.25 mg in 0.05 mL) every 6–8 weeks. The primary objectives included safety and tolerability. The secondary objectives included the efficacy as measured using the Early Treatment Diabetic Retinopathy Study chart for visual acuity, fundus photography, angiography, and optical coherence tomography/scanning laser ophthalmoscopy. Results: Reductions in optic disc hemorrhage and edema were noted in all patients. The visual acuity was stable or improved in 9 (64%) of the 14 patients. Of the 5 patients who had lost vision, 2 had relatively large posterior tumors, 1 had had the vision decrease because of intraocular hemorrhage, and 1 had developed optic atrophy. The fifth patient who lost vision was noncompliant. No treatment-related ocular or systemic side effects were observed. Conclusions: Intravitreal antivascular endothelial growth factor bevacizumab was tolerated and generally associated with improved vision, reduced papillary hemorrhage, and resolution of optic disc edema. Persistent optic disc neovascularization and fluorescein angiographic leakage were invariably noted. The results of the present study support additional evaluation of antivascular endothelial growth factor medications as treatment of RON.

Antivascular Endothelial Growth Factor Bevacizumab for Radiation Optic Neuropathy: Secondary to Plaque Radiotherapy

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Finger, Paul T., E-mail: pfinger@eyecancer.com [New York Eye Cancer Center, New York, NY (United States); Chin, Kimberly J. [New York Eye Cancer Center, New York, NY (United States)

2012-02-01

Purpose: To evaluate the intravitreal antivascular endothelial growth factor, bevacizumab, for treatment of radiation optic neuropathy (RON). Methods and Materials: A prospective interventional clinical case series was performed of 14 patients with RON related to plaque radiotherapy for choroidal melanoma. The RON was characterized by optic disc edema, hemorrhages, microangiopathy, and neovascularization. The entry criteria included a subjective or objective loss of vision, coupled with findings of RON. The study subjects received a minimum of two initial injections of intravitreal bevacizumab (1.25 mg in 0.05 mL) every 6-8 weeks. The primary objectives included safety and tolerability. The secondary objectives included the efficacy as measured using the Early Treatment Diabetic Retinopathy Study chart for visual acuity, fundus photography, angiography, and optical coherence tomography/scanning laser ophthalmoscopy. Results: Reductions in optic disc hemorrhage and edema were noted in all patients. The visual acuity was stable or improved in 9 (64%) of the 14 patients. Of the 5 patients who had lost vision, 2 had relatively large posterior tumors, 1 had had the vision decrease because of intraocular hemorrhage, and 1 had developed optic atrophy. The fifth patient who lost vision was noncompliant. No treatment-related ocular or systemic side effects were observed. Conclusions: Intravitreal antivascular endothelial growth factor bevacizumab was tolerated and generally associated with improved vision, reduced papillary hemorrhage, and resolution of optic disc edema. Persistent optic disc neovascularization and fluorescein angiographic leakage were invariably noted. The results of the present study support additional evaluation of antivascular endothelial growth factor medications as treatment of RON.

Bilateral non-arteritic ischemic optic neuropathy in a transsexual woman using excessive estrogen dosage.

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Wierckx, Katrien; De Zaeytijd, Julie; Elaut, Els; Heylens, Gunter; T'Sjoen, Guy

2014-02-01

We present a case report on a 53-year-old transsexual woman who developed acute painless vision loss in both eyes during cross-sex hormone treatment. After 10 months of cross-sex hormone treatment, she experienced total vision loss of the right eye and, 6 months later, vision loss to 20/63 in the left eye. After a full ophthalmic exam, bilateral sequential non-arteritic ischemic optic neuropathy (NA-ION) was diagnosed. Extensive etiological work-up revealed no cardiac abnormalities or inherited blood-clotting disorders. A manifest self-administered overdose of transdermal estrogen treatment with serum estradiol levels of 5,765 pg/ml was possibly related to the sequential bilateral NA-ION resulting in nearly total vision loss in this transsexual woman.

Ophthalmople gic cranial neuropathy: clinical case

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N. S. Dozorova

2018-01-01

Full Text Available Ophthalmoplegic cranial neuropathy (OCN is a disease with unknown etiology, which manifests itself by episodes of intense headache, accompanied by completely or partially reversible dysfunction of the oculomotor nerve: ptosis, mydriasis and ophthalmoplegia. It is assumed that the pathology is demyelinating in nature, therefore in the International classification of headaches OCN excluded from rubric migraine and related to the painful cranial neuropathies. The question of the prevention and treatment of this disease is still controversial, the issue of the appointment of corticosteroids, calcium channel blockers and β-blockers, methods of surgical correction of strabismus and botulin therapy.The article describes OCN in an 11-year-old boy. In the clinical picture headache attacks were observed. These attacks were with signs of selective lesions of the oculomotor nerve on one side. These functional changes are recurrent, and fully regress between attacks. Laboratory and instrumental examinations revealed no pathology that could cause this symptom, including myasthenia. The described case demonstrates the classical picture of OCN with a favorable course and the partial damage of the oculomotor nerve on one side.

Non arteritic anterior ischemic optic neuropathy; does anticoagulation help?

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Aftab, A.M.; Iqbal, M.; Ali, A.; Rauf, A.

2017-01-01

Non Arteritic Anterior Ischemic Optic Neuropathy (NAION) is the most common acute optic neuropathy in patients over 50 years of age. This study was conducted to determine the beneficial effects of anticoagulation with Heparin and Warfarin in patients with NAION presenting within 4 weeks of onset of symptoms Methods: A prospective, interventional, pilot study was conducted in Eye- A unit of Khyber Teaching Hospital from July 2010 onwards on patients with NAION presenting within 4 weeks of onset of symptoms. Patients underwent complete ophthalmological examination including Snellen's visual acuity (latter converted to Log MAR), pupil examination, fundus examination and automated Humphrey visual field analysis. Hematologic tests, Thrombophilia screening, Echocardiography and carotid Doppler ultrasound were carried on patients. All patients were anticoagulated with Heparin and Warfarin after obtaining informed written consent. Patients were examined at 1 Month, 3 months and 6 months' time period. Primary parameter measured was improvement in visual acuity. Results: Total number of patients in our study was 24. Regarding visual outcome total number of patients having significant improvement of visual acuity in our study was 16 (66.6 percent), while 4 (16.7 percent) patients had marginal improvement of visual acuity. Three (12.5 percent) patients maintained stable visual acuity of 6/6 throughout the study period in presence of thrombophilic disorders. One patient (4.1 percent) suffered a decline in visual acuity compared to VA at baseline presentation. Conclusions: Anticoagulation using heparin and warfarin does benefit patients with NAION presenting within 4 weeks of onset of symptoms. In our study a higher proportion of patients experienced significant improvement of visual acuity following anticoagulation as compared to the highest reported spontaneous improvement in such patients. (author)

Nonarteritic Anterior Ischemic Optic Neuropathy and Double Thrombophilic Defect: A New Observation

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Eleni Papageorgiou

2012-02-01

Full Text Available We report the first case of nonarteritic anterior ischemic neuropathy (NAION associated with double thrombophilia: protein S deficiency and prothrombin G20210A mutation. A 58-year-old man is presented including the clinical and laboratory findings, cardiovascular profile and thrombophilia screening. The patient presented with 3/10 vision and an inferior altitudinal defect in the right eye. Funduscopic examination of the right eye revealed a hyperemic optic disk with blurred superior optic disk border and sectoral nerve fiber layer edema. Complete blood count, erythrocyte sedimentation rate and C-reactive protein were normal, suggesting a NAION. A workup of cardiovascular risk factors revealed hyperlipidemia, arterial hypertension and high-risk asymptomatic coronary artery disease. Due to the family history of deep vein thrombosis in the patient’s daughter, a thrombophilia screening was additionally performed. The results revealed a double thrombophilic defect, namely congenital protein S deficiency and heterozygosity for prothrombin G20210A mutation, which were also identified in the patient’s daughter. Anticoagulant warfarin therapy was initiated and the patient underwent a triple bypass surgery. At three-month follow-up, the right optic disk edema had resolved, leaving a pale superior optic nerve head. Visual acuity in the right eye had slightly improved to 4/10; however, the dense inferior altitudinal field defect had remained unchanged. The patient is currently treated with warfarin, atorvastatin, irbesartan and metoprolol. This case suggests that the first line of investigation in all patients with NAION involves assessment of cardiovascular risk factors. However, careful history taking will identify NAION patients who are eligible for additional thrombophilia screening: young patients without vasculopathic risk factors, bilateral or recurrent NAION, idiopathic or recurrent venous thromboembolism (VTE, positive family history of VTE

Four novel cases of periaxin-related neuropathy and review of the literature.

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Marchesi, C; Milani, M; Morbin, M; Cesani, M; Lauria, G; Scaioli, V; Piccolo, G; Fabrizi, G M; Cavallaro, T; Taroni, F; Pareyson, D

2010-11-16

To report 4 cases of autosomal recessive hereditary neuropathy associated with novel mutations in the periaxin gene (PRX) with a review of the literature. Periaxin protein is required for the maintenance of peripheral nerve myelin. Patients with PRX mutations have early-onset autosomal recessive demyelinating Charcot-Marie-Tooth disease (CMT4F) or Déjèrine-Sottas neuropathy (DSN). Only 12 different mutations have been described thus far. Case reports and literature review. Four patients from 3 unrelated families (2 siblings and 2 unrelated patients) were affected by an early-onset, slowly progressive demyelinating neuropathy with relevant sensory involvement. All carried novel frameshift or nonsense mutations in the PRX gene. The 2 siblings were compound heterozygotes for 2 PRX null mutations (p.Q547X and p.K808SfsX2), the third patient harbored a homozygous nonsense mutation (p.E682X), and the last patient had a homozygous 2-nt insertion predicting a premature protein truncation (p.S259PfsX55). Electrophysiologic analysis showed a severe slowing of motor nerve conduction velocities (MNCVs, between 3 and 15.3 m/s) with undetectable sensory nerve action potentials (SNAPs). Sural nerve biopsy, performed in 2 patients, demonstrated a severe demyelinating neuropathy and onion bulb formations. Interestingly, we observed some variability of disease severity within the same family. These cases and review of the literature indicate that PRX-related neuropathies have early onset but overall slow progression. Typical features are prominent sensory involvement, often with sensory ataxia; a moderate-to-dramatic reduction of MNCVs and almost invariable absence of SNAPs; and pathologic demyelination with classic onion bulbs, and less commonly myelin folding and basal lamina onion bulbs.

Leber hereditary optic neuropathy due to a new ND1 mutation

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Soldath, Patrick; Wegener, Marianne; Sander, Birgit

2017-01-01

We report a proband with Leber hereditary optic neuropathy (LHON), in whom we have identified a novel homoplasmic m.3,395A>G mutation in the ND1 gene. The mutation alters a highly conserved amino acid in codon 30 which previously has been associated with LHON and leads to a severe selective complex...... and is present in the early stage of the disease. Furthermore, evaluation of two unaffected mutation carriers disclosed asymptomatic borderline ganglion cell loss and thin pRNFL in one....

Prevalence, dynamics, and biochemical predictors of optic nerve remyelination after methanol-induced acute optic neuropathy: a 2-year prospective study in 54 patients

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Nurieva, O.; Kotíková, K.; Urban, P.; Pelclová, D.; Petřík, V.; Navrátil, Tomáš; Zakharov, S.

2016-01-01

Roč. 147, č. 1 (2016), s. 239-249 ISSN 0026-9247 Institutional support: RVO:61388955 Keywords : methanol optic neuropathy * visual evoked potentials * remyelination Subject RIV: CF - Physical ; Theoretical Chemistry Impact factor: 1.282, year: 2016

Delayed autonomic neuropathy in a patient with diethylene glycol poisoning: a case report.

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Kamada, Hiroki; Suzuki, Hideaki; Yamamoto, Saori; Nomura, Ryosuke; Kushimoto, Shigeki

2017-07-01

A 72-year-old man presented to our hospital after ingesting insecticide containing approximately 2 mL/kg diethylene glycol, which exceeded the lethal dose of 1 mL/kg. The patient recovered from critical symptoms on acute phase until day 3, but received artificial ventilation for muscle weakness secondary to sensorimotor neuropathy on days 11-54. Even after marked improvement from sensorimotor neuropathy, the patient continued to complain of orthostatic hypotension. Autonomic neuropathy was identified by positive result of a head-up tilt test, and reduction in coefficient of variation of R-R intervals and cardiac iodine-123-metaiodobenzylguanidine uptake for the assessment of cardiac sympathetic activity. The patient's symptoms fully recovered 2 years after the exposure to diethylene glycol. This case shows the first report of delayed autonomic neuropathy after recovery from severe sensorimotor neuropathy, and suggests the importance of continuous monitoring for late-onset neurological complications.

“Peripheral Neuropathy Crippling Bronchial Asthma”: Two Rare Case Reports of Churg-Strauss Syndrome

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Kamal Kishore Pandita

2014-01-01

Full Text Available Churg-Strauss syndrome (CSS is a rare cause of vasculitic neuropathy. Although rare and potentially fatal, Churg-Strauss syndrome (CSS is easily diagnosable and treatable. The presence of bronchial asthma with peripheral neuropathy in a patient alerts a physician to this diagnosis. This is vividly illustrated by the presented two cases who had neuropathy associated with bronchial asthma, eosinophilia, sinusitis, and positive perinuclear antineutrophil cytoplasmic antibodies (p-ANCA test, which improved with administration of steroids.

Anterior ischemic optic neuropathy in a patient with Churg-Strauss syndrome.

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Lee, Ji Eun; Lee, Seung Uk; Kim, Soo Young; Jang, Tae Won; Lee, Sang Joon

2012-12-01

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.

Cyclosporine A does not prevent second-eye involvement in Leber's hereditary optic neuropathy.

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Leruez, Stéphanie; Verny, Christophe; Bonneau, Dominique; Procaccio, Vincent; Lenaers, Guy; Amati-Bonneau, Patrizia; Reynier, Pascal; Scherer, Clarisse; Prundean, Adriana; Orssaud, Christophe; Zanlonghi, Xavier; Rougier, Marie-Bénédicte; Tilikete, Caroline; Miléa, Dan

2018-02-17

Evaluation of the efficacy of oral cyclosporine A as a prophylactic agent in preventing second-eye involvement in Leber's hereditary optic neuropathy (LHON) in a prospective, open-label, non-randomized, multicenter pilot study. Only LHON patients aged 18 years or more, with confirmed primary mitochondrial DNA mutations and strictly unilateral optic neuropathy occurring within 6 months prior to enrolment, were included in the study. All these patients, receiving treatment with oral cyclosporine (Neoral®, Novartis) at 2.5 mg/kg/day, were examined at three-month intervals for a year. The primary endpoint was the best corrected visual acuity in the unaffected eye; the secondary endpoints were the best corrected visual acuity in the first eye affected, the mean visual field defect on automated perimetry, the thickness of the perifoveal retinal ganglion cell inner plexiform layer, and the thickness of the peripapillary retinal nerve fiber layer in both eyes. Among the 24 patients referred to our institution with genetically confirmed LHON, between July 2011 and April 2014, only five patients, four males and one female, fulfilled the inclusion criteria. Age at enrolment ranged from 19 to 42 years (mean: 27.2 years; median: 26 years), four patients harbored the m.11778G > A pathogenic variant, and one the m.14484 T > C pathogenic variant. The time-interval between the onset of symptoms and inclusion in the study ranged from 7 to 17 weeks (mean: 11.8 weeks; median: 9 weeks). Despite treatment with oral cyclosporine A, all patients eventually experienced bilateral eye involvement, occurring within 11-65 weeks after the initiation of treatment. Over the study time period, the average best corrected visual acuity worsened in the first eye affected; by the end of the study, both eyes were equally affected. Oral cyclosporine, at 2.5 mg/kg/day, did not prevent second-eye involvement in patients with strictly unilateral Leber's hereditary optic neuropathy

Bilateral retrobulbar optic neuropathy as the only sign of zoledronic acid toxicity.

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Lavado, Félix Manco; Prieto, Marta Para; Osorio, María Rosalba Ramoa; Gálvez, María Isabel López; Leal, Lucía Manzanas

2017-10-01

Bisphosphonates may rarely cause ocular adverse effects and retrobulbar optic neuropathy (RON) secondary to zoledronic acid is very rare. A 67-year-old man was referred because of progressive and painless decrease vision in the left eye. He had been treated with 7 cycles of zoledronic acid infusions because of metastatic prostate cancer. On examination, VA was 20/20 in the right eye (OD) and 20/50 in the left eye (OS). The optic nerve was unremarkable OU. Pattern visual evoked potentials (pVEP) and electroretinography were performed with the result of VEP responses abolished in OS, and the VEP waveform within the normal range amplitude and delayed peak latencies in OD. Due to the high suspicion of bilateral RON secondary to zoledronic acid, we decided to discontinue the treatment. 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. The diagnosis of bilateral RON secondary to zoledronic acid infusions was confirmed, and it was only partially reversible. Zoledronic acid is a potent new generation bisphosphonate increasingly used in oncologic patients and it is usually well tolerated. Optic nerve toxicity is not a side effect recognised by either the Food and Drug Administration or the drug manufacturers, and to our knowledge, this is the first case of zoledronic acid-related bilateral RON with late onset. In conclusion, patients treated with bisphosphonates should be informed about the possibility of ocular side-effects, and ophthalmologists should be consider discontinuing the drug. Copyright © 2017 Elsevier Ltd. All rights reserved.

Hydroquinone neuropathy following use of skin bleaching creams: case report.

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Karamagi, C; Owino, E; Katabira, E T

2001-04-01

A 30-year old black woman presented with gradual onset of weakness of the legs associated with burning sensation in the feet for two months. She had been using two hydroquinone based skin bleaching creams (MGC by M. G. C. International, MEKAKO by Anglo Fabrics BOLTON Ltd) for about four years. Her BP was 80/40 mm Hg supine with un-recordable diastolic pressure on standing. She had decreased power (Grade 3/5), loss of deep tendon reflexes and impairment of deep sensation in the lower limbs. A complete blood count, urinalysis, serum electrolytes, serum creatinine and uric acid were all normal. Oral GTT, VDRL and brucella tests were negative. Chest and abdominal radiographs did not show any abnormalities. A diagnosis of peripheral neuropathy with autonomic neuropathy possibly due to hydroquinone toxicity was made and she was advised to stop using hydroquinone based skin bleaching creams. Four months later she was asymptomatic, her BP was 120/80 mmHg supine and standing, and neurological examination was normal. The case raises the question of whether hydroquinone based skin bleaching creams could be a cause of peripheral neuropathy and underscores the need for research on hydroquinone based skin bleaching creams and neuropathy particularly in black women involved in the sale and/or use of skin bleaching creams.

Stem Cell Ophthalmology Treatment Study (SCOTS): bone marrow-derived stem cells in the treatment of Leber's hereditary optic neuropathy

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Weiss, Jeffrey N.; Levy, Steven; Benes, Susan C.

2016-01-01

The Stem Cell Ophthalmology Treatment Study (SCOTS) is currently the largest-scale stem cell ophthalmology trial registered at ClinicalTrials.gov (identifier: NCT01920867). SCOTS utilizes autologous bone marrow-derived stem cells (BMSCs) to treat optic nerve and retinal diseases. Treatment approaches include a combination of retrobulbar, subtenon, intravitreal, intra-optic nerve, subretinal, and intravenous injection of autologous BMSCs according to the nature of the disease, the degree of visual loss, and any risk factors related to the treatments. Patients with Leber's hereditary optic neuropathy had visual acuity gains on the Early Treatment Diabetic Retinopathy Study (ETDRS) of up to 35 letters and Snellen acuity improvements from hand motion to 20/200 and from counting fingers to 20/100. Visual field improvements were noted. Macular and optic nerve head nerve fiber layer typically thickened. No serious complications were seen. The increases in visual acuity obtained in our study were encouraging and suggest that the use of autologous BMSCs as provided in SCOTS for ophthalmologic mitochondrial diseases including Leber's hereditary optic neuropathy may be a viable treatment option. PMID:27904503

Optical Coherence Tomography Study of Experimental Anterior Ischemic Optic Neuropathy and Histologic Confirmation

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Ho, Joyce K.; Stanford, Madison P.; Shariati, Mohammad A.; Dalal, Roopa; Liao, Yaping Joyce

2013-01-01

Purpose. The optic nerve is part of the central nervous system, and interruption of this pathway due to ischemia typically results in optic atrophy and loss of retinal ganglion cells. In this study, we assessed in vivo retinal changes following murine anterior ischemic optic neuropathy (AION) by using spectral-domain optical coherence tomography (SD-OCT) and compared these anatomic measurements to that of histology. Methods. We induced ischemia at the optic disc via laser-activated photochemical thrombosis, performed serial SD-OCT and manual segmentation of the retinal layers to measure the ganglion cell complex (GCC) and total retinal thickness, and correlated these measurements with that of histology. Results. There was impaired perfusion and leakage at the optic disc on fluorescein angiography immediately after AION and severe swelling and distortion of the peripapillary retina on day-1. We used SD-OCT to quantify the changes in retinal thickness following experimental AION, which revealed significant thickening of the GCC on day-1 after ischemia followed by gradual thinning that plateaued by week-3. Thickness of the peripapillary sensory retina was also increased on day-1 and thinned chronically. This pattern of acute retinal swelling and chronic thinning on SD-OCT correlated well with changes seen in histology and corresponded to loss of retinal ganglion layer cells after ischemia. Conclusions. This was a serial SD-OCT quantification of acute and chronic changes following experimental AION, which revealed changes in the GCC similar to that of human AION, but over a time frame of weeks rather than months. PMID:23887804

Structural changes of macula and optic disk of the fellow eye in patients with nonarteritic anterior ischemic optic neuropathy.

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Duman, R; Yavas, G F; Veliyev, I; Dogan, M; Duman, R

2018-05-10

The aim was to assess the ganglion cell complex (GCC) thickness, retinal nerve fiber layer (RNFL) thickness and optic disk features in the affected eyes (AE) and unaffected fellow eyes (FE) of subjects with unilateral nonarteritic anterior ischemic optic neuropathy (NAION) and to compare with healthy control eyes (CE) using spectral domain-optical coherence tomography (SD-OCT). This study included 28 patients and age, sex and refraction-matched 28 control subjects. Mean GCC thickness and peripapillary RNFL thickness in four quadrants measured by cirrus SD-OCT were evaluated in both AE and FE of patients and CE. In addition, optic disk measurements obtained with OCT were evaluated. Mean GCC thickness was significantly lower in AE compared with both FE and CE (P optic disk cupping compared with both FE and CE (P optic disk features between the CE and FE. And significantly greater optic disk cupping in the AE compared with both FE and CE supports the acquired enlargement of cupping after the onset of NAION.

Olfactory Neuroblastoma: A Rare Cause of External Ophthalmoplegia, Proptosis and Compressive Optic Neuropathy.

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Kartı, Ömer; Zengin, Mehmet Özgür; Çelik, Ozan; Tokat, Taşkın; Küsbeci, Tuncay

2018-04-01

Olfactory neuroblastoma (ONB), which is a neuroectodermal tumor of the nasal cavity, is a rare and locally aggressive malignancy that may invade the orbit via local destruction. In this study, we report a patient with proptosis, external ophthalmoplegia, and compressive optic neuropathy caused by ONB. A detailed clinical examination including ocular imaging and histopathological studies were performed. The 62-year-old female patient presented to our clinic with complaints of proptosis and visual deterioration in the left eye. Her complaints started 2 months prior to admission. Visual acuity in the left eye was counting fingers from 2 meters. There was relative afferent pupillary defect. She had 6 mm of proptosis and limitation of motility. Fundus examination was normal in the right eye, but there was a hyperemic disc, and increased vascular tortuosity and dilation of the retinal veins in the left eye. Computerized tomography and magnetic resonance imaging of the brain and orbits demonstrated a large heterogeneous mass in the left superior nasal cavity with extensions into the ethmoidal sinuses as well as into the left orbit, compressing the medial rectus muscle and optic nerve. Endoscopic biopsy of the lesion was consistent with an ONB (Hyams' grade III). Orbital invasion may occur in patients with ONB. Therefore, it is important to be aware of this malignancy because some patients present with ophthalmic signs such as external ophthalmoplegia, proptosis, or compressive optic neuropathy.

Subretinal fluid is common in experimental non-arteritic anterior ischemic optic neuropathy

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Yu, C; Ho, J K; Liao, Y J

2014-01-01

Purpose Anterior ischemic optic neuropathy (AION) is an important cause of acute vision loss for which several animal models exist. It has been associated with subretinal fluid in a previous study on patients but not yet so in animal models. Patients and Methods A patient presented with acute non-arteritic AION (NAION) and underwent ophthalmic evaluation and testing including fluorescein angiography and spectral-domain optical coherence tomography (SD-OCT). On the basis of the patient's findings, we used SD-OCT circular and volume scans to analyze retinal changes in a murine model of NAION. Results One week after left eye vision loss, the patient had clinical and imaging findings consistent with NAION. On SD-OCT, there was prominent peripapillary retinal thickening consistent with intra-retinal edema and sub-foveolar fluid. Inspired by the findings in human AION, we looked for similar changes in murine NAION using SD-OCT. The circular scan did not adequately detect the presence of subretinal fluid. Using the 25-line scan, which covered a larger part of the posterior pole, we found that 100% of murine AION resulted in subretinal fluid at day 1. The subretinal fluid resolved by week 1. Conclusion This study detailed a case of clinical NAION associated with intra-retinal and subretinal fluid. We also found that subretinal fluid was common in murine photochemical thrombosis model of AION and could be found far away from the optic disc. PMID:25257770

Laser speckle flowgraphy for differentiating between nonarteritic ischemic optic neuropathy and anterior optic neuritis.

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Maekubo, Tomoyuki; Chuman, Hideki; Nao-I, Nobuhisa

2013-07-01

The aim of this study was to investigate the usefulness of laser speckle flowgraphy (LSFG) for the differentiation of acute nonarteritic ischemic optic neuropathy (NAION) from anterior optic neuritis (ON). To investigate blood flow in the optic disc under normal conditions, NAION, and anterior ON, we compared the tissue blood flow of the right eye with that of the left eye in the control group, and that of the affected eye with that of the unaffected eye in the NAION and anterior ON groups. In the normal control group, the tissue blood flow did not significantly differ between the right and left eyes. In the NAION group, all 6 patients had decreased optic disc blood flow in the NAION eye when compared with the unaffected eye. By contrast, in the anterior ON group, all 6 patients had increased optic disc blood flow in the anterior ON eye when compared with the unaffected eye. In the NAION group, the mean blur rate (MBR) of the affected eyes was 29.5 % lower than that of the unaffected eyes. In the anterior ON group, the MBR of the affected eyes was 15.9 % higher than that of the unaffected eyes. LSFG could be useful in differentiating between NAION and anterior ON. In addition, this imaging technique saves time and is noninvasive.

Improving the conditions of regional hemodinamics of the eyes as a method of treatment in optic neuropathy at short-sightedness of high degree.

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N. G. Zavgorodnyaya

2014-04-01

Full Text Available The aim of research. To increase treatment efficacy in optic neuropathy with high myopia by performing revascularization operations in order to improve the performance of regional hemodynamics of the eyeball and improve of visual functions. Materials and methods. The study involved 56 patients (78 eyes with high myopia in age from 18 to 36 years, 32 were men (57.1% , 24 - women (42.9% . All patients underwent standard ophthalmologic examination, had nonprogressive form of myopia with signs of optic neuropathy . Exclusion criteria were : myopia more than 6.0 diopters , progressive form of the disease , opaque optical surroundings, clinically significant comorbidity (glaucoma, diabetes, etc.. Study group comprised 28 patients (32 eyes. Complex of medications included vasodilators, nootropics and neuroprotective agents , vitamins. Compression bandaging of the superficial temporal artery was used with revascularization purpose. The control group consisted of 28 patients (46 eyes who received a similar course of drug therapy. Examination of patients in both groups were performed before treatment and after 1, 6 and 12 months. Results. In the main group positive changes in visual acuity and visual fields was more pronounced and more cases of negative dynamics during the observation period were recorded. Patients in the control group in most cases achieved stabilization of the process, there was a negative trend of visual function during long-term follow-up. Analyzing the performance of retinal tomography HRT II for the reporting period in the study group noted the absence of further thinning of the nerve fibers. In the control group had a negative trend 8 eyes ( 17.39 % having an average thickness loss of retinal nerve fibers was 0,007 ±0,00046 mm( 7.12 % of the initial level . Prior to treatment the linear velocity of blood flow was reduced and averaged speed was 28,77 ±4,12 cm/ sec , and the resistance index increased (averaged 0,9 ± 0

Neuropatia óptica auto-imune: relato de caso Autoimmune optic neuropathy: case report

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Laura Martins C. Duprat Cardoso

2006-08-01

Full Text Available Descrevemos uma paciente de 9 anos, sexo feminino, com perda visual bilateral grave tratada com corticóide por via oral apresentando melhora em apenas um olho. Nove anos depois apresentou recidiva que inicialmente respondeu à pulsoterapia corticóide mas foi seguida de perda progressiva e completa da visão após a redução do tratamento. Novas tentativas terapêuticas não melhoraram a visão. Avaliação clínico-laboratorial não revelou doença sistêmica, mas apresentava anticorpos antinucleares (1/640, anti-Ro e anti-La positivos. Neuropatia óptica auto-imune é uma afecção rara, que simula neurite óptica idiopática e se caracteriza por perda visual aguda, sem doença sistêmica, mas com evidências laboratoriais de doença auto-imune, em especial o FAN positivo. Biópsia de pele freqüentemente mostra evidências de vasculite. Esta condição deve ser tratada agressivamente, com corticóide e imunossupressores, uma vez que o acometimento visual geralmente é mais grave do que o da neurite óptica idiopática/desmielinizante e pode ser irreversível.We report on a 9-year-old female patient who had bilateral severe visual loss and was treated with oral corticosteroids. Visual improvement occurred in one eye. Nine years later she presented relapse of visual loss in her only seeing eye. Pulse corticosteroid therapy resulted in dramatic visual improvement followed, however, by progressive and complete visual loss as soon as the corticosteroid was tapered. Repeat treatment did not result in visual improvement. Clinical and laboratory investigation failed to find a systemic disease but the patient had positive antinuclear (1/640, anti-Ro and anti-La antibodies. Autoimmune optic neuropathy is a rare condition that may mimic an idiopathic optic neuritis and is characterized by acute visual loss, without systemic disease but with laboratory evidence of an autoimmune disorder, usually a positive ANA. A skin biopsy usually shows evidence of

Nonarteritic anterior ischemic optic neuropathy (NAION) and its experimental models

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Bernstein, Steven L.; Johnson, Mary A.; Miller, Neil R.

2011-01-01

Anterior ischemic optic neuropathy (AION) can be divided into nonarteritic (NAION) and arteritic (AAION) forms. NAION makes up ~85% of all cases of AION, and until recently was poorly understood. There is no treatment for NAION, and its initiating causes are poorly understood, in part because NAION is not lethal, making it difficult to obtain fresh, newly affected tissue for study. In-vivo electrophysiology and post-mortem studies reveal specific responses that are associated with NAION. New models of NAION have been developed which enable insights into the pathophysiological events surrounding this disease. These models include both rodent and primate species, and the power of a `vertically integrated' multi-species approach can help in understanding the common cellular mechanisms and physiological responses to clinical NAION, and to identify potential approaches to treatment. The models utilize laser light to activate intravascular photoactive dye to induce capillary vascular thrombosis, while sparing the larger vessels. The observable optic nerve changes associated with rodent models of AION (rAION) and primate NAION (pNAION) are indistinguishable from that seen in clinical disease, including sectoral axonal involvement, and in-vivo electrophysiological data from these models are consistent with clinical data. Early post-infarct events reveal an unexpected inflammatory response, and changes in intraretinal gene expression for both stress response, while sparing outer retinal function, which occurs in AAION models. Histologically, the NAION models reveal an isolated loss of retinal ganglion cells by apoptosis. There are changes detectable by immunohistochemistry suggesting that other retinal cells mount a brisk response to retinal ganglion cell distress without themselves dying. The optic nerve ultimately shows axonal loss and scarring. Inflammation is a prominent early histological feature. This suggests that clinically, specific modulation of inflammation may

Ophthalmople gic cranial neuropathy: clinical case

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N. S. Dozorova; A. S. Kotov; E. V. Mukhina

2018-01-01

Ophthalmoplegic cranial neuropathy (OCN) is a disease with unknown etiology, which manifests itself by episodes of intense headache, accompanied by completely or partially reversible dysfunction of the oculomotor nerve: ptosis, mydriasis and ophthalmoplegia. It is assumed that the pathology is demyelinating in nature, therefore in the International classification of headaches OCN excluded from rubric migraine and related to the painful cranial neuropathies. The question of the prevention and ...

Leukemic optic neuropathy.

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Brown, G C; Shields, J A; Augsburger, J J; Serota, F T; Koch, P

1981-03-01

The clinical course and ophthalmic manifestations of an eight year old child with acute undifferentiated leukemia and unilateral blindness secondary to leukemic optic nerve head infiltration are described. At autopsy the involved nerve head and peripapillary retina demonstrated massive leukemic cell infiltration and hemorrhagic necrosis. This manifestation of leukemia is quite uncommon and prognosis for life in such cases is poor with existing methods of therapy.

Optic nerve histopathology in a case of Wolfram Syndrome

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Ross-Cisneros, Fred N; Pan, Billy X; Silva, Ruwan A

2013-01-01

Mitochondrial dysfunction in Wolfram Syndrome (WS) is controversial and optic neuropathy, a cardinal clinical manifestation, is poorly characterized. We here describe the histopathological features in postmortem retinas and optic nerves (ONs) from one patient with WS, testing the hypothesis...

Peripapillary Pachychoroid in Nonarteritic Anterior Ischemic Optic Neuropathy

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Nagia, Lina; Huisingh, Carrie; Johnstone, John; Kline, Lanning B.; Clark, Mark; Girard, Michael J. A.; Mari, Jean Martial; Girkin, Christopher A.

2016-01-01

Purpose This study examined the peripapillary choroidal thickness (PCT) in nonarteritic ischemic optic neuropathy (NAION) in comparison to contralateral eyes and normal eyes. Methods We used enhanced depth imaging spectral-domain optical coherence tomography to image the optic nerve head of 20 NAION, 10 contralateral eyes, and 102 normal eyes. Following compensation, the scans were manually delineated to identify relevant surfaces including Bruch's membrane opening (BMO), Bruch's membrane, and anterior sclera. The PCT was defined as the measurement between Bruch's membrane and the anterior sclera and was measured at increasing distance from BMO. Models adjusted for age, BMO area, and axial length were used to compare the mean PCT between NAION and normal eyes, and contralateral eyes and normal eyes. Paired t-tests were used to compare the PCT between NAION and contralateral eyes. Results The mean PCT was thicker in NAION and contralateral eyes when compared with normal eyes at all distances from BMO (P < 0.001). The PCT was not significantly thicker in contralateral eyes when compared with affected NAION eyes. Choroidal thickness was thinnest in the inferior quadrant in all eyes regardless of the group. Conclusions Increased peripapillary choroidal thickness was noted in both NAION and contralateral eyes. The thicker choroid may be an associated feature or a result of the disorder. Although further longitudinal study is required to determine causation, these findings may suggest that a thickened peripapillary choroid may be a component of the disk-at-risk clinical phenotype. PMID:27583829

The enigma of nonarteritic anterior ischemic optic neuropathy: an update for the comprehensive ophthalmologist.

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Gaier, Eric D; Torun, Nurhan

2016-11-01

Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common cause of acute optic nerve injury, and frequently presents to comprehensive ophthalmologists. We review the typical and atypical clinical features and current literature on various treatment modalities for NAION. The epidemiology and clinical presentation of this disease can be variable, making a definitive diagnosis difficult in many cases. In addition, the differential diagnoses for this disorder, although comprising much less prevalent entities, are quite broad and can have substantial systemic implications if these alternatives go unrecognized. NAION has many systemic associations and comorbidities that deserve inquiry when the diagnosis is made. There are currently no widely accepted, evidence-based treatments for NAION. All recommendations made to patients to reduce their risk of sequential eye involvement, including avoidance of potential nocturnal hypotension, erectile dysfunction medication, and treatment of obstructive sleep apnea, have theoretical bases. NAION is a common cause of acute vision loss in adult and older patients, and thus, comprehensive ophthalmologists need to be able to diagnose and appropriately manage this disorder. We anticipate fruitful results from current and future trials aimed at neuroprotection in the affected eye and prevention of sequential eye involvement.

Neuroprotective and Anti-Inflammatory Effects of Rhus coriaria Extract in a Mouse Model of Ischemic Optic Neuropathy

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Saba Khalilpour

2018-04-01

Full Text Available Modulating oxidative stresses and inflammation can potentially prevent or alleviate the pathological conditions of diseases associated with the nervous system, including ischemic optic neuropathy. In this study we evaluated the anti-neuroinflammatory and neuroprotective activities of Rhus coriaria (R. coriaria extract in vivo. The half maximal inhibitory concentration (IC50 for DPPH, ABTS and β–carotene were 6.79 ± 0.009 µg/mL, 10.94 ± 0.09 µg/mL, and 6.25 ± 0.06 µg/mL, respectively. Retinal ischemia was induced by optic nerve crush injury in albino Balb/c mice. The anti-inflammatory activity of ethanolic extract of R. coriaria (ERC and linoleic acid (LA on ocular ischemia was monitored using Fluorescence Molecular Tomography (FMT. Following optic nerve crush injury, the mice treated with 400 mg/kg of ERC and LA exhibited an 84.87% and 86.71% reduction of fluorescent signal (cathepsin activity respectively. The results of this study provide strong scientific evidence for the neuroprotective activity of the ERC, identifying LA as one of the main components responsible for the effect. ERC may be useful and worthy of further development for its adjunctive utilization in the treatment of optic neuropathy.

Diagnostic capability of retinal thickness measures in diabetic peripheral neuropathy

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Sangeetha Srinivasan

2017-10-01

Conclusions: The GCC FLV can differentiate individuals with diabetic neuropathy from healthy controls, while the inferior RNFL thickness is able to differentiate those with greater degrees of neuropathy from those with mild or no neuropathy, both with an acceptable level of accuracy. Optical coherence tomography represents a non-invasive technology that aids in detection of retinal structural changes in patients with established diabetic neuropathy. Further refinement of the technique and the analytical approaches may be required to identify patients with minimal neuropathy.

Stem Cell Ophthalmology Treatment Study (SCOTS) for retinal and optic nerve diseases: a case report of improvement in relapsing auto-immune optic neuropathy.

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Weiss, Jeffrey N; Levy, Steven; Benes, Susan C

2015-09-01

We present the results from a patient with relapsing optic neuropathy treated within the Stem Cell Ophthalmology Treatment Study (SCOTS). SCOTS is an Institutional Review Board approved clinical trial and has become the largest ophthalmology stem cell study registered at the National Institutes of Health to date (www.clinicaltrials.gov Identifier NCT 01920867). SCOTS utilizes autologous bone marrow-derived stem cells (BMSCs) for treatment of retinal and optic nerve diseases. Pre-treatment and post-treatment comprehensive eye exams of a 54 year old female patient were performed both at the Florida Study Center, USA and at The Eye Center of Columbus, USA. As a consequence of a relapsing optic neuritis, the patient's previously normal visual acuity decreased to between 20/350 and 20/400 in the right eye and to 20/70 in the left eye. Significant visual field loss developed bilaterally. The patient underwent a right eye vitrectomy with injection of BMSCs into the optic nerve of the right eyeand retrobulbar, subtenon and intravitreal injection of BMSCs in the left eye. At 15 months after SCOTS treatment, the patient's visual acuity had improved to 20/150 in the right eye and 20/20 in the left eye. Bilateral visual fields improved markedly. Both macular thickness and fast retinal nerve fiber layer thickness were maximally improved at 3 and 6 months after SCOTS treatment. The patient also reduced her mycophenylate dose from 1,500 mg per day to 500 mg per day and required no steroid pulse therapy during the 15-month follow up.

A Female Patient with Down Syndrome and Low-Penetrance Leber's Hereditary Optic Neuropathy

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Starleen E. Frousiakis

2014-11-01

Full Text Available We present the case of a 19-year-old female with a history of Down syndrome (DS who was referred to our neuro-ophthalmology clinic for evaluation of Leber's hereditary optic neuropathy (LHON. The patient's family history was significant for a known G11778A mutation in a maternal relative, consistent with LHON. The patient was also positive for the G11778A mutation; however, the genotype demonstrated low penetrance in the pedigree, with only 1 out of 10 adult male offspring showing signs or symptoms of the disease. Mitochondrial mutations implicated in LHON have been shown to impair complex I of the electron transport chain and thereby reducing the effective generation of adenosine triphosphate and increasing the production of toxic reactive oxygen species. Although the partial or complete triplicate of chromosome 21 constitutes the etiology of DS, some of the pleiotropic phenotypes of the syndrome have been attributed to oxidative stress and mitochondrial dysfunction. Given the low penetrance of the mutation and the patient's sex, this case illustrates the possibility that the mitochondrial mutation demonstrated increased penetrance due to pre-existing mitochondrial dysfunction related to DS.

Multifocal Choroiditis with Retinal Vasculitis, Optic Neuropathy, and Keratoconus in a Young Saudi Male.

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Dhafiri, Yousef; Al Rubaie, Khalid; Kirat, Omar; May, William N; Nguyen, Quan D; Kozak, Igor

2017-01-01

The purpose of this study is to describe an association of unilateral multifocal choroiditis (MFC), retinal vasculitis, optic neuropathy, and bilateral keratoconus in a young Saudi male. A 27-year-old male patient with stable bilateral keratoconus presented with a painless vision loss in his left eye. Ophthalmic examinations revealed multiple foci of idiopathic chorioretinitis, retinal vasculitis, and mild optic disc leakage on fluorescein angiography, all of which resolved on systemic therapy with mycophenolate mofetil and prednisone after 3 months. Systemic medication was stopped after 8 months. One year after presentation, patient's visual acuity has improved and remained stable. Systemic immunomodulatory therapy can be effective in managing and leading to resolution of MFC, retinal vasculitis, and optic disc leak in young patients.

Brain white matter 1 H MRS in Leber optic neuropathy mutation carriers

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Ostojic, Jelena; Jancic, Jasna; Kozic, Dusko

2009-01-01

OBJECTIVE: This study was conducted in order to test the hypothesis that proton MR spectroscopic (1H MRS) profile of Leber's hereditary optic neuropathy (LHON) mutation carriers group (including both symptomatic and asymptomatic) differs from group of healthy individuals and to determine metabolite...... or ratio that contributes most to differentiation. PATIENTS AND METHODS: We performed single voxel 1H MRS in normal appearing white matter of eighteen LHON mtDNA mutation carriers bearing one of three LHON mtDNA point mutations and in fifty control subjects. RESULTS: ANOVA showed significant difference...

Delayed autonomic neuropathy in a patient with diethylene glycol poisoning: a case report

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Kamada, Hiroki; Suzuki, Hideaki; Yamamoto, Saori; Nomura, Ryosuke; Kushimoto, Shigeki

2017-01-01

Case A 72‐year‐old man presented to our hospital after ingesting insecticide containing approximately 2 mL/kg diethylene glycol, which exceeded the lethal dose of 1 mL/kg. The patient recovered from critical symptoms on acute phase until day 3, but received artificial ventilation for muscle weakness secondary to sensorimotor neuropathy on days 11–54. Outcome Even after marked improvement from sensorimotor neuropathy, the patient continued to complain of orthostatic hypotension. Autonomic neur...

Posterior tibial neuropathy by a Baker's cyst: case report.

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Lee, J H; Jun, J B; Lee, H S; Yun, H R; Choi, C H; Park, S B; Hong, E K; Yoo, D H; Kim, S Y

2000-01-01

Baker's cysts are rare cause of peripheral nerve entrapment and only a few cases of tibial nerve entrapment resulting from the popliteal cyst in the calf muscle have been reported in the literature. We present a case of rheumatoid arthritis complicated by a Baker's cyst with a tibial nerve entrapment. It is important to diagnose a Baker's cyst early and to differentiate it from thrombophlebitis, a popliteal aneurysm, tumor or muscle tear to effect optimal therapy and to obviate a potential neuropathy. Prompt recognition of these cases may save the patients unnecessary procedures and delay in treatment.

Penicillamin-induced neuropathy in rheumatoid arthritis

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Pedersen, P B; Hogenhaven, H

1990-01-01

A case of penicillamin-induced severe polyradiculopathy in rheumatoid arthritis is presented. The neuropathy was of demyelinating type, purely motor, proximal and clinically fully reversible when the drug ceased. In case of a progressive neuropathy, during penicillamin treatment, this adverse...

Error diagnóstico en la neuropatía óptica epidémica Diagnostic error in epidemic optic neuropathy

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Rosaralis Santiesteban Freixas

2005-12-01

Full Text Available La forma óptica de la epidemia de neuropatía, aparecida en el decenio pasado en Cuba, ha sido motivo de discusión por el posible hiperdiagnóstico, de los más de 22 000 casos notificados. El propósito principal de este trabajo es profundizar en la caracterización de esta enfermedad y dar a conocer la congruencia que debe de existir entre la agudeza visual y la visión de colores en las neuropatías de este tipo; además, estimar sobre la base de este elemento la posible cifra de errores diagnósticos de dos grupos de pacientes con neuropatía optica epidérmica y en un tercer grupo de pacientes que no mejoraron con tratamiento, donde se reunieron 78 casos remitidos porque no mejoraban con tratamiento vitamínico. Los resultados demostraron congruencia entre el estado de afectación de la agudeza visual y la visión de colores en más del 90 % de los 960 casos de los grupos 1 y 2, con diagnóstico de neuropatía óptica epidémica comprobados por expertos, a diferencia de los casos del tercer grupo, en los que se detectó que el 96,1 % tuvieron de inicio falsos diagnósticos de esta enfermedad p The optic form of the neuropathy epidemic that appeared in the last decade in Cuba has been discussed due to the possible hyperdiagnosis of the more than 22 000 notified cases. The main objective of this paper was to go deep into the characterization of this disease, to make known the congruency that should exist between the visual acuity and the vision of colors in the neuropathies of this type, and to estimate, on the basis of this element, the possible figure of diagnostic errors in 2 groups of patients with EON and of a third group of patients that did not improve with the treatment. In this group, there were 78 cases that were referred because they did not respond to vitamin therapy. The results showed congruency between the state of affection of visual acuity and the vision of colors in more than 90 % of the 960 cases from groups 1 and 2 with

Peripheral neuropathy associated with mitochondrial disease in children.

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Menezes, Manoj P; Ouvrier, Robert A

2012-05-01

Mitochondrial diseases in children are often associated with a peripheral neuropathy but the presence of the neuropathy is under-recognized because of the overwhelming involvement of the central nervous system (CNS). These mitochondrial neuropathies are heterogeneous in their clinical, neurophysiological, and histopathological characteristics. In this article, we provide a comprehensive review of childhood mitochondrial neuropathy. Early recognition of neuropathy may help with the identification of the mitochondrial syndrome. While it is not definite that the characteristics of the neuropathy would help in directing genetic testing without the requirement for invasive skin, muscle or liver biopsies, there appears to be some evidence for this hypothesis in Leigh syndrome, in which nuclear SURF1 mutations cause a demyelinating neuropathy and mitochondrial DNA MTATP6 mutations cause an axonal neuropathy. POLG1 mutations, especially when associated with late-onset phenotypes, appear to cause a predominantly sensory neuropathy with prominent ataxia. The identification of the peripheral neuropathy also helps to target genetic testing in the mitochondrial optic neuropathies. Although often subclinical, the peripheral neuropathy may occasionally be symptomatic and cause significant disability. Where it is symptomatic, recognition of the neuropathy will help the early institution of rehabilitative therapy. We therefore suggest that nerve conduction studies should be a part of the early evaluation of children with suspected mitochondrial disease. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

Radioinduced optic nerve neuropathy after treatment for nasopharynx cancer. About two cases and literature review; Neuropathie radio-induite du nerf optique apres traitement pour cancer du nasopharynx. A propos de deux cas et revue de la litterature

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Mnejja, W.; Siala, W.; Daoud, J. [Centre Hospitalier Universitaire Habib-Bourguiba, Service de Radiotherapie Carcinologique, Sfax (Tunisia); Fki, J. [Centre Hospitalier Universitaire Habib-Bourguiba, Service d' Ophtalmologie, Sfax (Tunisia); Ghorbel, M. [Centre Hospitalier Universitaire Habib-Bourguiba, Service d' ORL, Sfax (Tunisia); Frikha, M. [Centre Hospitalier Universitaire Habib-Bourguiba, Service de Carcinologie Medicale, Sfax (Tunisia)

2007-11-15

The radioinduced neuropathy of the optic nerve is a rare and delayed complication. Its incidence is difficult to evaluate in the literature. It depends on the whole irradiation dose, fractionation and irradiated volume. Currently, it does not exist any efficient treatment, only the prevention play an important part on avoiding the high doses, and the broad irradiation volumes. The innovating techniques using conformal radiotherapy with or without modulated intensity could contribute to reduce this toxicity incidence. (N.C.)

An unusual case of suprascapular nerve neuropathy: a case report

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Kyriakides Theodoros

2011-08-01

Full Text Available Abstract Introduction Suprascapular nerve neuropathy constitutes an unusual cause of shoulder weakness, with the most common etiology being nerve compression from a ganglion cyst at the suprascapular or spinoglenoid notch. We present a puzzling case of a man with suprascapular nerve neuropathy that may have been associated with an appendectomy. The case was attributed to nerve injury as the most likely cause that may have occurred during improper post-operative patient mobilization. Case presentation A 23-year-old Caucasian man presented to an orthopedic surgeon with a history of left shoulder weakness of several weeks' duration. The patient complained of pain and inability to lift minimal weight, such as a glass of water, following an appendectomy. His orthopedic clinical examination revealed obvious atrophy of the supraspinatus and infraspinatus muscles and 2 of 5 muscle strength scores on flexion resistance and external rotation resistance. Magnetic resonance imaging showed diffuse high signal intensity within the supraspinatus and infraspinatus muscles and early signs of minimal fatty infiltration consistent with denervation changes. No compression of the suprascapular nerve in the suprascapular or spinoglenoid notch was noted. Electromyographic studies showed active denervation effects in the supraspinatus muscle and more prominent in the left infraspinatus muscle. The findings were compatible with damage to the suprascapular nerve, especially the part supplying the infraspinatus muscle. On the basis of the patient's history, clinical examination, and imaging studies, the diagnosis was suspected to be associated with a possible traction injury of the suprascapular nerve that could have occurred during the patient's transfer from the operating table following an appendectomy. Conclusion Our case report may provide important insight into patient transfer techniques used by hospital personnel, may elucidate the clinical significance of careful

OCT angiography of acute non-arteritic anterior ischemic optic neuropathy.

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Rougier, M-B; Delyfer, M-N; Korobelnik, J-F

2017-02-01

To describe changes of the retinal peripapillary microvasculature on optical coherence tomography angiography (OCT-A) in non-arteritic anterior ischemic optic (NAION) neuropathy. Observational study of 10 patients at the acute phase of NAION. OCT-A was performed using a 3mm×3mm square centered on the optic disc (Cirrus HD-OCT with Angioplex, Carl Zeiss Meditec, Dublin, CA). A qualitative comparison was made with the healthy fellow eye of each patient. All patients had a fluorescein angiography (HRA2, Heidelberg, Germany) and a visual field examination (Octopus 101 ® , Haag-Streit, USA). In the affected eyes, OCT-A showed clear modifications in the radial peripapillary network. In all these eyes, a focal disappearance of the superficial capillary radial pattern was present, twisted and irregular. In 8 eyes, there was also a lack of vascularization in some focal areas, appearing as dark areas. No correlation was found between the topography of the vascular alteration shown on OCT-A and visual field pattern defects. OCT-A is a new imaging technology able to demonstrate easily and safely the changes in the peripapillary capillary network during the acute phase of NAION. These changes are likely related to a decrease of the prelaminar optic nerve blood flow during the acute phase of NAION. Visual field defects are not correlated with OCT-A images, suggesting that they may be due mainly to disturbances in posterior ciliary artery blood flow. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Neuroprotective effects of recombinant human granulocyte colony-stimulating factor (G-CSF) in a rat model of anterior ischemic optic neuropathy (rAION).

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Chang, Chung-Hsing; Huang, Tzu-Lun; Huang, Shun-Ping; Tsai, Rong-Kung

2014-01-01

The purpose of this study was to investigate the neuroprotective effects of recombinant human granulocyte colony stimulating factor (G-CSF), as administered in a rat model of anterior ischemic optic neuropathy (rAION). Using laser-induced photoactivation of intravenously administered Rose Bengal in the optic nerve head of 60 adult male Wistar rats, an anterior ischemic optic neuropathy (rAION) was inducted. Rats either immediately received G-CSF (subcutaneous injections) or phosphate buffered saline (PBS) for 5 consecutive days. Rats were euthanized at 4 weeks post infarct. Density of retinal ganglion cells (RGCs) was counted using retrograde labeling of Fluoro-gold. Visual function was assessed by flash visual-evoked potentials (FVEP) at 4 weeks. TUNEL assay in the retinal sections and immunohistochemical staining of ED1 (marker of macrophage/microglia) were investigated in the optic nerve (ON) specimens. The RGC densities in the central and mid-peripheral retinas in the G-CSF treated rats were significantly higher than those of the PBS-treated rats (survival rate was 71.4% vs. 33.2% in the central retina; 61.8% vs. 22.7% in the mid-peripheral retina, respectively; both p optic nerve sections of G-CSF-treated rats (16 ± 6/HPF vs. 35 ± 10/HPF; p = 0.016). In conclusion, administration of G-CSF is neuroprotective in the rat model of anterior ischemic optic neuropathy, as demonstrated both structurally by RGC density and functionally by FVEP. G-CSF may work via the dual actions of anti-apoptosis for RGC surviving as well as anti-inflammation in the optic nerves as evidenced by less infiltration of ED1-povitive cells. Copyright © 2013 Elsevier Ltd. All rights reserved.

The 14th Hoyt Lecture: Ischemic Optic Neuropathy: The Evolving Profile, 1966-2015.

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Arnold, Anthony C

2016-06-01

Since the first English language description by Miller and Smith in 1966 of ischemic optic neuropathy as a distinct ophthalmic syndrome, a long series of studies has refined the clinical profile to what we consider to be accurate today. From the specifics of pathogenesis to the clinical appearance to the effect of therapy, the basic tenets of diagnosis and management have evolved over 5 decades. What we thought we knew about the following topics has changed: location of vasculopathy; incidence; age at onset; optic disc appearance; risk factors for development; natural history; rate of fellow eye involvement; ischemia as an all-or-none phenomenon; and treatment. A look back at these discoveries shows both how far we have come and how far we have to go in managing this disorder.

Optical coherence tomography findings in methanol toxicity.

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Klein, Kendra A; Warren, Alexis K; Baumal, Caroline R; Hedges, Thomas R

2017-01-01

Methanol toxicity poses a significant public health problem in developing countries, and in Southeast Asia, where the most common source of poisoning is via adulterated liquor in local drinks. Methanol toxicity can have devastating visual consequences and retinal specialists should be aware of the features of this toxic optic neuropathy. The authors report a case of severe systemic methanol toxicity and relatively mild optic neuropathy demonstrating unique retinal changes on optical coherence tomography (OCT). A previously healthy student developed ataxia, difficulty breathing and loss of consciousness hours after drinking homemade alcohol while traveling in Indonesia. She was found to have a serum pH of 6.79 and elevated methanol levels. She was treated with intravenous ethanol, methylprednisolone and sodium bicarbonate. When she awoke she had bilateral central scotomas. At presentation, she had central depression on visual field testing. OCT of the retinal nerve fiber layer (RNFL) was normal but ganglion cell layer analysis (GCL) showed highly selective loss of the nasal fibers in both eyes. Further, OCT of the macula demonstrated inner nuclear layer (INL) microcysts in the corresponding area of selective GCL loss in both eyes. The selective involvement of the papillomacular bundle fibers is common in toxic optic neuropathies and represents damage to the small caliber axons rich in mitochondria. Despite severe systemic toxicity, the relative sparing of the optic nerve in this case enabled characterization of the evolution of methanol toxicity with segmental GCL involvement and preservation of the RNFL, corresponding to the papillomacular bundle. This is the first reported case of INL microcysts in methanol optic neuropathy and supports that they are a non-specific finding, and may represent preferential damage to the papillomacular bundle.

Immune mediated neuropathy following checkpoint immunotherapy.

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Gu, Yufan; Menzies, Alexander M; Long, Georgina V; Fernando, S L; Herkes, G

2017-11-01

Checkpoint immunotherapy has revolutionised cancer therapy and is now standard treatment for many malignancies including metastatic melanoma. Acute inflammatory neuropathies, often labelled as Guillain-Barre syndrome, are an uncommon but potentially severe complication of checkpoint immunotherapy with individual cases described but never characterised as a group. We describe a case of acute sensorimotor and autonomic neuropathy following a single dose of combination ipilimumab and nivolumab for metastatic melanoma. A literature search was performed, identifying 14 other cases of acute neuropathy following checkpoint immunotherapy, with the clinical, electrophysiological and laboratory features summarised. Most cases described an acute sensorimotor neuropathy (92%) with hyporeflexia (92%) that could occur from induction up till many weeks after the final dose of therapy. In contrast to Guillain-Barre syndrome, the cerebrospinal fluid (CSF) analysis often shows a lymphocytic picture (50%) and the electrophysiology showed an axonal pattern (55%). Treatment was variable and often in combination. 11 cases received steroid therapy with only 1 death within this group, whereas of the 4 patients who did not receive steroid therapy there were 3 deaths. In conclusion checkpoint immunotherapy - induced acute neuropathies are distinct from and progress differently to Guillain-Barre syndrome. As with other immunotherapy related adverse events corticosteroid therapy should be initiated in addition to usual therapy. Copyright © 2017 Elsevier Ltd. All rights reserved.

Penicillamin-induced neuropathy in rheumatoid arthritis

DEFF Research Database (Denmark)

Pedersen, P B; Hogenhaven, H

1990-01-01

A case of penicillamin-induced severe polyradiculopathy in rheumatoid arthritis is presented. The neuropathy was of demyelinating type, purely motor, proximal and clinically fully reversible when the drug ceased. In case of a progressive neuropathy, during penicillamin treatment, this adverse eff...... effect should be born in mind, and discontinuation of the drug considered....

Crystallins are regulated biomarkers for monitoring topical therapy of glaucomatous optic neuropathy.

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Verena Prokosch

Full Text Available Optic nerve atrophy caused by abnormal intraocular pressure (IOP remains the most common cause of irreversible loss of vision worldwide. The aim of this study was to determine whether topically applied IOP-lowering eye drugs affect retinal ganglion cells (RGCs and retinal metabolism in a rat model of optic neuropathy. IOP was elevated through cauterization of episcleral veins, and then lowered either by the daily topical application of timolol, timolol/travoprost, timolol/dorzolamide, or timolol/brimonidine, or surgically with sectorial iridectomy. RGCs were retrogradely labeled 4 days prior to enucleation, and counted. Two-dimensional polyacrylamide gel electrophoresis (2D-PAGE, matrix-assisted laser desorption ionization mass spectrometry, Western blotting, and immunohistochemistry allowed the identification of IOP-dependent proteomic changes. Genomic changes were scrutinized using microarrays and qRT-PCR. The significant increase in IOP induced by episcleral vein cauterization that persisted until 8 weeks of follow-up in control animals (p<0.05 was effectively lowered by the eye drops (p<0.05. As anticipated, the number of RGCs decreased significantly following 8 weeks of elevated IOP (p<0.05, while treatment with combination compounds markedly improved RGC survival (p<0.05. 2D-PAGE and Western blot analyses revealed an IOP-dependent expression of crystallin cry-βb2. Microarray and qRT-PCR analyses verified the results at the mRNA level. IHC demonstrated that crystallins were expressed mainly in the ganglion cell layer. The data suggest that IOP and either topically applied antiglaucomatous drugs influence crystallin expression within the retina. Neuronal crystallins are thus suitable biomarkers for monitoring the progression of neuropathy and evaluating any neuroprotective effects.

Establishing an experimental model of photodynamic induced anterior ischemic optic neuropathy

Institute of Scientific and Technical Information of China (English)

Runsheng Wang; Xiaodi Wang; Peilin Lü; Jianwei Bai; Jianzhou Wang; Xiaoqin Lei; Xiaoliang Zhou; Hongfen Sun; Aizhu Pan

2006-01-01

BACKGROUND: Scholars have supposed to establish animal models of optic neuropathy by pressing and partially amputating optic nerve, increasing intraocular pressure and injecting vasoconstrictor, etc., but the models are greatly different from anterior ischemia optic neuropathy. Therefore, a more ideal method is needed to establish animal model of anterior ischemic optic neuropathy (AION).OBJECTIVE: To establish AION models in rats, observe the functional changes of fundus, fundus fluorescein angiography (FFA), optical coherence tomography (OCT), flash visual evoked potential (F-VEP), and histopathologically confirm its reliability.DESIGN: A randomized control trial.SETTINGS: Department of Ophthalmology, Xi'an Fourth Hospital; Xi'an Institute of Ocular Fundus Diseases.MATERIALS: The experiments were carried out in the research room of Xi'an Institute of Ocular Fundus Diseases from February 2005 to May 2006. Thirty healthy male SD rats of 4-5 weeks old, weighing 140-160 g,were provided by the animal experimental center of the Fourth Military Medical University of Chinese PLA [SCXK (Military)2002-005], and those without eye disease examined by slit lamp and direct ophthalmoscope after mydriasis were enrolled. The conditions for feeding mice without special pathogen were strictly followed.The rats were randomly divided into blank control group (n =5), laser group (n =5), hematoporphyrin derivative (HPD) group and AION group (n =15), each group was numbered randomly. For each rat, the right eye was taken as the experimental eye, and the left one as the control one.METHODS: In the AION group, the rats were injected with HPD (10 mg/kg) via caudal vein, and then the optic discs were exposed to krypton red (647 nm, 80 mV) for 120 s, and the rats were in avoidance of light for 2 weeks postoperatively. Rats in the laser group were only exposed to krypton red (647 nm, 80 mV) for 120 s, and in avoidance of light for 2 weeks postoperatively; Those in the HPD group were only

Imaging of neuropathies about the hip

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Martinoli, Carlo, E-mail: carlo.martinoli@unige.it [Radiologia – DISC, Università di Genova, Largo Rosanna Benzi 8, I-16132 Genoa (Italy); Miguel-Perez, Maribel [Unit of Human Anatomy and Embryology, Department of Pathology and Experimental Therapy, Faculty of Medicine (C Bellvitge), University of Barcelona, Barcelona (Spain); Padua, Luca [Fondazione Don Gnocchi Onlus and Department of Neurology, Policlinico “A. Gemelli”, Università Cattolica del Sacro Cuore, Rome (Italy); Gandolfo, Nicola [IM2S – Institut Monégasque de Médecine and Chirurgie Sportive, Montecarlo (Monaco); Zicca, Anna [Radiologia – DISC, Università di Genova, Largo Rosanna Benzi 8, I-16132 Genoa (Italy); Tagliafico, Alberto [Radiologia – National Institute for Cancer Research, Genoa (Italy)

2013-01-15

Neuropathies about the hip may be cause of chronic pain and disability. In most cases, these conditions derive from mechanical or dynamic compression of a segment of a nerve within a narrow osteofibrous tunnel, an opening in a fibrous structure, or a passageway close to a ligament or a muscle. Although the evaluation of nerve disorders primarily relies on neurological examination and electrophysiology, diagnostic imaging is currently used as a complement to help define the site and aetiology of nerve compression and exclude other disease possibly underlying the patient’ symptoms. Diagnosis of entrapment neuropathies about the hip with US and MR imaging requires an in-depth knowledge of the normal imaging anatomy and awareness of the anatomic and pathologic factors that may predispose or cause a nerve injury. Accordingly, the aim of this article is to provide a comprehensive review of hip neuropathies with an emphasis on the relevant anatomy, aetiology, clinical presentation, and their imaging appearance. The lateral femoral cutaneous neuropathy (meiralgia paresthetica), femoral neuropathy, sciatic neuropathy, obturator neuropathy, superior and inferior gluteal neuropathies and pudendal neuropathy will be discussed.

Ultrasound in the diagnosis of a median neuropathy in the forearm: case report

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Yoon Joon-Shik

2007-12-01

Full Text Available Abstract Background Electrodiagnostic studies are traditionally used in the diagnosis of focal neuropathies, however they lack anatomical information regarding the nerve and its surrounding structures. The purpose of this case is to show that high-resolution ultrasound used as an adjunct to electrodiagnostic studies may complement this lack of information and give insight to the cause. Case presentation A 60-year-old male patient sustained a forearm traction injury resulting in progressive weakness and functional loss in the first three digits of the right hand. High-resolution ultrasound showed the presence of an enlarged nerve and a homogenous soft-tissue structure appearing to engulf the nerve. The contralateral side was normal. Surgery revealed fibrotic bands emanating from the flexor digitorum profundus muscle compressing the median nerve thus confirming the ultrasound findings. Conclusion A diagnostically challenging case of median neuropathy in the forearm is presented in which high-resolution ultrasound was valuable in establishing an anatomic etiology and directing appropriate management.

Incipient nonarteritic anterior ischemic optic neuropathy.

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Hayreh, Sohan Singh; Zimmerman, M Bridget

2007-09-01

To describe the clinical entity of incipient nonarteritic anterior ischemic optic neuropathy (NAION). Cohort study. Fifty-four patients (60 eyes) seen in our clinic from 1973 through 2000. At their first visit to our clinic, all patients gave a detailed ophthalmic and medical history and underwent a comprehensive ophthalmic evaluation, color fundus photography, and fluorescein fundus angiography. At each follow-up visit (of 49 patients [55 eyes]), the same ophthalmic evaluation was performed, except for fluorescein fundus angiography. Clinical features of incipient NAION. Mean age (+/- standard deviation) of the patients was 58.7+/-15.9 years. Median follow-up time was 6.3 years (interquartile range [IQR], 2.1-8.5). At initial visit, all had optic disc edema (ODE) without any visual loss attributable to NAION. In 55%, the fellow eye had classic NAION; in 25%, incipient progressed to classic NAION (after a median time of 5.8 weeks [IQR, 3.2-10.1]); and in 20%, classic NAION developed after resolution of the first episode of incipient NAION. Patients with incipient, compared with classic, NAION had a greater prevalence of diabetes mellitus (Pheart disease (P = 0.046). Patients who progressed to classic NAION versus those who did not were significantly younger (P = 0.025), and their visual acuity worsened in 31% and 0%, respectively, and remained stable in 62% and 98%, respectively; in the eyes with progression, central (in 31%) and peripheral (in 77%) visual fields worsened compared with only 1 eye and 2 eyes, respectively, that did not (P = 0.01 and Pversus 9.6 weeks (IQR, 6.0-17.7) in those who did not progress. The results show that incipient NAION is a distinct clinical entity, with asymptomatic ODE and no visual loss attributable to NAION. When a patient seeks treatment with asymptomatic ODE, incipient NAION must be borne in mind as a strong possibility in those who have had classic NAION in the fellow eye, in diabetics of all ages, and in those with high risk

Neuropatía óptica hereditaria de Leber Leber´s hereditary optic neuropathy

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Yannara Elina Columbié Garbey

2012-06-01

Full Text Available La neuropatía óptica hereditaria de Leber es una enfermedad de herencia materna que se caracteriza por la pérdida subaguda, indolora y bilateral, aunque por lo general no siempre al unísono de la visión central. Predomina en hombres jóvenes y es causada por mutaciones puntuales del ADN mitocondrial. Esta es una de las neuropatías ópticas hereditarias más frecuentes y altamente invalidante, cuyo diagnóstico de certeza lo constituyen los estudios moleculares. El propósito de esta revisión es alertar en cuanto a su diagnóstico y posible incremento en condiciones ambientales favorecedoras. Se realizó una búsqueda automatizada de artículos científicos relacionados con el tema, en PUBMED e Hinari, que resultó en 37 publicaciones realizadas durante los años 1988-2010. Se estudiaron y discutieron aspectos de la enfermedad tales como antecedentes históricos, factores de riesgo, epidemiología, genética, características clínicas, diagnóstico y tratamiento; además de profundizar en su estado actual en nuestro contexto. En Cuba actualmente se conoce de la existencia de varias familias que padecen la neuropatía óptica hereditaria de Leber. El alza de la incidencia probablemente se debió a las condiciones medioambientales que favorecen o son factores de riesgo de esta entidad, como ocurrió durante la pasada epidemia de neuropatía óptica en Cuba. Cada día se producen más avances en el campo de la genética, que permiten identificar un número mayor de mutaciones asociadas a esta entidad. Esto unido al conocimiento de las características clínicas de la enfermedad ha permitido identificar las familias afectadas y actuar sobre los factores de riesgo.Leber´s hereditary optic neuropathy is a maternally inherited disease characterized by subacute, painless and bilateral loss of the central vision, although not always at the same time. It predominates in young men and is caused by mitochondrial DNA spot mutations. This is one of

Burn-related peripheral neuropathy: A systematic review.

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Tu, Yiji; Lineaweaver, William C; Zheng, Xianyou; Chen, Zenggan; Mullins, Fred; Zhang, Feng

2017-06-01

Peripheral neuropathy is the most frequent disabling neuromuscular complication of burns. However, the insidious and progressive onset of burn neuropathy makes it often undiagnosed or overlooked. In our study, we reviewed the current studies on the burn-related peripheral neuropathy to summarize the morbidity, mechanism, detecting method and management of peripheral neuropathy in burn patients. Of the 1533 burn patients included in our study, 98 cases (6.39%) were presented with peripheral neuropathy. Thermal and electrical burns were the most common etiologies. Surgical procedures, especially nerve decompression, showed good effect on functional recovery of both acute and delayed peripheral neuropathy in burn patients. It is noteworthy that, for early detection and prevention of peripheral neuropathy, electrodiagnostic examinations should be performed on burn patients independent of symptoms. Still, the underlying mechanisms of burn-related peripheral neuropathy remain to be clarified. Copyright © 2016 Elsevier Ltd and ISBI. All rights reserved.

Posterior Ischemic Optic Neuropathy Following Percutaneous Nephrolithotomy

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Mohammad Pakravan

2008-12-01

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PURPOSE: To report a case of posterior ischemic optic neuropathy (PION following percutaneous nephrolithotomy (PCNL. CASE REPORT: A 57-year-old man with history of diabetes mellitus, hyperlipidemia and mild anemia underwent PCNL for treatment of nephrolithiasis. He noticed painless visual loss in both eyes immediately after the procedure. Visual acuity was light perception, however ophthalmologic examinations were unremarkable and the optic discs were pink with no swelling. Visual fields were severely affected, but neuro-imaging was normal. Within three months, visual acuity and visual fields improved dramatically but the optic discs became slightly pale. CONCLUSION: This is the first report of PION following PCNL. PION is a rare cause of severe visual loss following surgery. Severe blood loss, hypotension, anemia and body position during surgery are the most important risk factors. Ophthalmologists, urologists and anesthesiologists should be aware of this condition and this rare possibility should be considered prior to surgery.