Optic Nerve Swelling Research Articles

Impending Central Retinal Vein Occlusion

Central retinal vein occlusion (CRVO) may present with varied clinical manifestations, ranging from mild blurred vision and scattered retinal hemorrhages to severe vision loss, optic nerve swelling, pronounced retinal hemorrhages, collateral retinal vessel formation and neovascularization. Impending CRVO, also known as partial CRVO, is a relatively poorly-defined sub-classification of the CRVO condition. Those affected are either asymptomatic or may complain of mild, often transient episodes of blurring of vision and present with venous dilation and tortuosity but only a few widely scattered flame-shaped retinal hemorrhages. As an impending CRVO may be the prodromal phase of an acute CRVO, this diagnosis requires careful monitoring of the patient for progression. The following case outlines the differential diagnosis, sequelae and inter-professional management of an impending central retinal venous occlusion.

Musculoskeletal/Radiological Manifestations of Mucolipidosis II (I-Cell disease) in late Adolescence/Early Adulthood.

Mucolipidosis type II (I-Cell disease) is a rare autosomal recessive lysosomal disorder, resulting from functional deficiency of lysosomal enzymes due to an impaired targeting of the enzymes to lysosomes, which leads to an abnormal cell architecture and the overflow of lysosomal enzymes into the body fluids. The life expectancy of the patients is poor, with multisystem deterioration leading to death in early childhood. According to the available reports, patients with I-cell disease do not survive beyond the first decade of life. Here, we describe and illustrate various radiological-musculoskeletal manifestations of a rare case of mucolipidosis II who has been a survivor up to now, 20 years old. The course of her disease has been complicated by early severe visual compromise due to optic nerve swelling, hearing loss and mitral valve regurgitation/stenosis, bilateral carpal tunnel, and severe growth impairment. Our case demonstrates several skeletal features of dysostosis multiplex. At the age of 20, she is wheelchair bound and her medical course is complicated by recurrent pneumonia, treated with multiple hospitalizations, antibiotics, and BiPAP. She is on outpatient palliative care, Do Not Resuscitate/Do Not Intubate (DNR/DNI) status.

Optic nerve and retinal damage in a patient with post-hemorrhagic anemia

There is a number of literature data on ischemic optic neuropathy development in acute hemorrhage. However, ocular disorders in prolonged chronic hemorrhage and iron-deficiency anemia are not well studied. We present a clinical case of optic nerve and retinal damage in a patient with prolonged chronic gastrointestinal bleeding. 53-year-old patient S. presented with complaints on dramatic sudden loss of vision of his right eye (visual acuity was 0/02 and was not improving with correction). Visual acuity of the left eye was good. Ophthalmoscopy revealed right optic nerve swelling, flame-shaped disc and peripapillary hemorrhages, and multiple soft exudates along blood vessels of the right eye. Optic nerve head of the left eye was pale pink, with well-defined borders. Multiple soft exudates along blood vessels and few flame-shaped hemorrhages were identified as well. Clinical examination revealed iron-deficiency post-hemorrhagic anemia. The diagnosis of anterior ischemic neuropathy of the right eye, ischemic neuroretinopathy of left eye associated with post-hemorrhagic anemia was established. Conservative treatment increased hemoglobin level up to 82 g/l, the red blood cells count - up to 2,88×1012/L, hematocrit was 0.25%, platelet count reached 344×109/L, but the signs of rectal bleeding remained. The patient underwent surgery for hemorrhoids. After the increase of hemoglobin level, visual acuity of the right eye improved to 0.1, the visual acuity of the left eye was 1.0. According to the results of computed peripheral vision test (Humphrey Full Field 120 Point Screening Test), central scotoma and scotomas in the lower half of the field of vision of the right eye remained. In the field of vision of the left eye, the area of absolute arcuate scotoma in the lower-nasal quadrant decreased significantly. Reduced visual acuity was the main complaint of the patient with a longstanding gastrointestinal bleeding. A careful history and thorough clinical examination allowed to establish the cause of the optic nerve and retinal damage, to assign pathogenetically based treatment, which led to an improvement in visual function.

Lyme disease associated neuroretinitis — Case report

We describe a rare case of Lyme disease complicated by unilateral neuroretinitis in the right eye. We report a case of a 27-year-old woman with blurred vision on her right eye. Because of the suspicion of optic neuritis (multiplex sclerosis) neurological examination was ordered. Surprisingly, computer tomography of the brain revealed incomplete empty sella, which generally results not monocular, but bilateral optic nerve swelling. Opthalmological examination (ophthalmoscopy and optical coherence tomography) indicated not only monocular optic nerve, but retinal oedema next to the temporal part of the right optic disk. Visual evoked potentials (VEP) demonstrated no P100 latency delay and mild differences between the amplitudes of the responses of the left and right eye. Optical coherence tomography (OCT) demonstrated the swelling of the optic nerve head and oedematous retina at the temporal part of the disk. Suspicion of an inflammatory cause of visual disturbance blood tests was ordered. Doxycycline treatment was ordered till the result of the blood test arrived. The Western blot and ELISA test were positive for Borrelia burgdorferi sensu lato. Following one week corticosteroide and ceftriaxone treatments, the patient displayed a clinical improvement. Unilateral neuroretinitis with optic disk swelling due to neuroborreliosis is a rare complication and in many cases it is difficult to distinguish between inflammatory and ischemic lesions. Further difficulty in the diagnosis can occur when intracranial alterations such as empty sella is demonstrated by CT examination.

Bilateral Posterior Scleritis: Analysis of 18 Cases from a Large Cohort of Posterior Scleritis

ABSTRACTPurpose: To describe the clinical and epidemiological characteristics of patients and analyze factors associated with bilateral posterior scleritis.Methods: In this retrospective cohort study, records of patients with diagnosis of bilateral posterior scleritis at two tertiary-care centers in the UK and India were analyzed in comparison with the clinical profile of patients with unilateral scleritis.Results: In total, 18 patients with bilateral posterior scleritis were identified and compared with 96 patients of unilateral posterior scleritis; 14 (77%) were women and the median age was 48 years. Headache (p = 0.04), optic nerve swelling (p = 0.01), and elevated antinuclear antibodies (ANA) titers (p = 0.03) were present more frequently in patients with bilateral than in unilateral posterior scleritis. Seven patients (38.88%) required immunosuppressive therapy to attain resolution of the inflammation and to prevent relapse.Conclusions: Bilateral posterior scleritis is an uncommon but severe inflammation of the posterior sclera. The majority of them are idiopathic, often requiring aggressive treatment to prevent visual loss.

Optical Coherence Tomography for the Radiologist

Optical coherence tomography is an imaging technique using low coherence light sources to produce high-resolution cross-sectional images. This article reviews pertinent anatomy and various pathologies causing optic atrophy (eg, compressive, infiltrating, demyelinating) versus optic nerve swelling (from increased intracranial pressure known as papilledema or other optic nerve intrinsic pathologies). On optical coherence tomography, optic atrophy is often associated with reduced average retinal nerve fiber layer thickness, whereas optic nerve swelling is usually associated with increased average retinal nerve fiber layer thickness.

Applications of optical coherence tomography in pediatric clinical neuroscience.

For nearly two centuries, the ophthalmoscope has permitted examination of the retina and optic nerve-the only axons directly visualized by the physician. The retinal ganglion cells project their axons, which travel along the innermost retina to form the optic nerve, marking the beginning of the anterior visual pathway. Both the structure and function of the visual pathway are essential components of the neurologic examination as it can be involved in numerous acquired, congenital and genetic central nervous system conditions. The development of optical coherence tomography now permits the pediatric neuroscientist to visualize and quantify the optic nerve and retinal layers with unprecedented resolution. As optical coherence tomography becomes more accessible and integrated into research and clinical care, the pediatric neuroscientist may have the opportunity to utilize and/or interpret results from this device. This review describes the basic technical features of optical coherence tomography and highlights its potential clinical and research applications in pediatric clinical neuroscience including optic nerve swelling, optic neuritis, tumors of the visual pathway, vigabatrin toxicity, nystagmus, and neurodegenerative conditions.

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

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.

Birdshot retinochoroidopathy in the absence of typical chorioretinal lesions

Birdshot retinochoroidopathy is an ocular inflammatory disease characterized by depigmented lesions radiating from the optic disc. It may, however, present with isolated optic disc edema, vitritis, or vasculitis. ICG can play a critical role in diagnosising BSRC in cases where characteristic fundus lesions are subtle or absent. We describe a case of Birdshot retinochoroidopathy (BSRC) that presented with isolated optic nerve swelling. Neuroimaging and serum testing for infectious or inflammatory etiologies were unremarkable. Indocyanine angiography (ICG) revealed multiple round dark fundus lesions in both eyes. This raised suspicion for BSRC, which was confirmed with genetic testing. Ocular inflammation resolved following treatment with mycophenolate mofetil (MMF) and a dexamethasone intravitreal implant (Ozurdex®; Allergan Inc, Irvine, CA).

P11.03 * OPTIC NERVE INFILTRATION OF PRIMARY CNS LYMPHOMA

BACKGROUND: Visual impairment in Primary CNS lymphoma (PCNSL) in most of the cases is due to an intraocular lymphomatous involvement, especially uveitis. Optic nerve infiltration (ONI) in PCNSL is a rare condition. We sought to describe its clinical presentation, imaging characteristics, and outcomes. METHODS: Patients with ONI of PCNSL were retrospectively identified from 1998 to 2014 from the databases of the university hospitals Pitie-Salpetriere (Paris) and Rene Huguenin (Saint-Cloud), and the academic teaching hospital Pasteur (Colmar), France. Exclusion criteria were intraocular involvement, orbital lymphoma, or other systemic lymphoma. Clinical presentation, neuroimaging, biological features, treatment and outcomes were assessed from their medical records. RESULTS: Only 6 of 710 PCNSL were identified as presenting ONI. Patients' median age at presentation was 65 years (49-78), 5 were women. 2 patients had ONI at initial diagnosis, 3 at 1st relapse after HD-MTX based chemotherapy, and 1 at 2nd relapse after HD-MTX, salvage chemotherapy and high-dose chemotherapy with autologous stem cell transplant. Clinical presentation: Rapidly progressive visual impairment without red eyes (6/6 patients), painless (4/6), unilateral (4/6), bilateral (2/6), bilateral hemianopia (2/6). MRI: Optic nerve swelling (3/6), contrast-enhancement of the entire ON (5/6) or of the ON sheath (1/6); Localization of ONI: Chiasmic (3/6), cisternal (3/6), intracanalicular (3/6), and intraorbital (4/6). Additional CNS lesions were seen in 4/6. Systemic lymphoma was excluded in all patients; lumbar puncture was done in 4/6 proving leptomeningeal disease in 2. Median delay from symptom onset to treatment: 25 days (10-140). Treatment: Different chemotherapy regimens were administered but lymphoma recurred in the majority. Clinical outcome: Partial recovery (2/6 patients), persisting low vision or blindness (4/6). Radiological outcome: Complete remission (4/6), partial remission (1/6), stable disease (1/6). Median PFS after ONI: 11 months (3-11), OS: 32 months (12-40). CONCLUSIONS: Intraocular involvement is observed in about 15% of PCNSL, clinically marked by slowly progressive visual deterioration over several months, usually caused by posterior uveitis. Lymphomatous infiltration of the optic nerve is characterized by a rapidly progressive severe visual impairment. In patients with ONI as initial presentation of PCNSL, it is difficult to establish the diagnoses as differential diagnosis are broad. In these patients, optic nerve biopsy should be discussed only in absence of cerebral lesions. At relapse, clinical presentation, neuroimaging and the absence of intraocular involvement may help to establish the diagnosis. As optic nerve functional outcome is poor, rapid treatment might improve clinical outcome. ACKNOWLEDGEMENTS: LOC national network (INCa).

Surgery for traumatic optic neuropathy

Traumatic optic neuropathy (TON) is an important cause of severe visual loss following blunt or penetrating head trauma. Following the initial insult optic nerve swelling within the optic nerve canal or compression by bone fragments are thought to result in secondary retinal ganglion cell loss. Optic nerve decompression with steroids or surgical interventions or both have therefore been advocated to improve visual prognosis in TON. To examine the effects and safety of surgical interventions in the management of TON. We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2013, Issue 4), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE, (January 1950 to May 2013), EMBASE (January 1980 to May 2013), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to May 2013), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (http://clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 28 May 2013. We also searched the reference lists of other reviews and book chapters on TON. We also contacted researchers in the field. We planned to include only randomised controlled trials (RCTs) of TON in which any form of surgical intervention either on its own or in combination with steroids was compared to steroids alone or no treatment. Two authors independently assessed the titles and abstracts identified from the search strategy. No studies were found that met our inclusion criteria and therefore none were included for analysis. No studies were found that met our inclusion criteria. The current body of evidence consists mostly of small, retrospective case series. Given the wide range of surgical interventions used in TON it is very difficult to compare these studies, even qualitatively. However, there is a relatively high rate of spontaneous visual recovery and no evidence that surgical decompression of the optic nerve provides any additional benefit. On the other hand, surgery carries a definite risk of complications such as postoperative cerebrospinal fluid leak and meningitis. The decision to proceed with surgery in TON therefore remains controversial and each case needs to be assessed on its own merits. Although there is an urgent need for an adequately powered, RCT of surgical intervention in TON, this will prove a difficult endeavour.

Steroids for traumatic optic neuropathy.

Traumatic optic neuropathy (TON) is an important cause of severe visual loss following blunt or penetrating head trauma. Following the initial injury, optic nerve swelling within the optic nerve canal can result in secondary retinal ganglion cell loss. Optic nerve decompression with steroids or surgical interventions or both has therefore been advocated as a means of improving visual prognosis in TON. The aim of this review was to examine the effectiveness and safety of using steroids in TON. We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2013, Issue 4), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE, (January 1950 to May 2013), EMBASE (January 1980 to May 2013), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to May 2013), Web of Science Conference Proceedings Citation Index- Science (CPCI-S) (January 1990 to May 2013), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (http://clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 21 May 2013. We also searched the reference lists of included studies, other reviews and book chapters on TON to find references to additional trials. The Science Citation Index was used to look for papers that cited the studies included in this review. We did not manually search any journals or conference proceedings. We contacted trial investigators and experts in the field to identify additional published and unpublished studies. We planned to include only randomised controlled trials (RCTs) of TON in which any steroid regime, either on its own or in combination with surgical optic nerve decompression, was compared to surgery alone or no treatment. Two review authors independently assessed the titles and abstracts identified from the electronic searches. We included one study that met our selection criteria; a double-masked, placebo-controlled, randomised trial of high dose intravenous steroids in patients with indirect TON diagnosed within seven days of the initial injury. A total of 31 eligible participants were randomised to receive either high dose intravenous steroids (n = 16) or placebo (n = 15), and they were all followed-up for three months. Mean final best corrected visual acuity (BCVA) was 1.78±1.23 Logarithm of the Minimum Angle of Resolution (LogMAR) in the placebo group, and 1.11±1.14 LogMAR in the steroid group. The mean difference in BCVA between the placebo and steroid groups was 0.67 LogMAR (95% confidence interval -1.54 to 0.20), and this difference was not statistically significant (P = 0.13). At three months follow-up, an improvement in BCVA of 0.40 LogMAR occurred in eight eyes (8/15, 53.3%) in the placebo group, and in 11 eyes (11/16, 68.8%) in the treatment group. This difference was not statistically significant (P = 0.38). There is a relatively high rate of spontaneous visual recovery in TON and there is no convincing data that steroids provide any additional visual benefit over observation alone. Recent evidence also suggests a possible detrimental effect of steroids in TON and further studies are urgently needed to clarify this important issue. Each case therefore needs to be assessed on an individual basis and proper informed consent is paramount.

Optic Disc Edema and Optic Nerve Head Drusen

We are concerned about the conclusions reported by Sarac et al (1) in their article entitled “Differentiation of optic disc edema from optic nerve head drusen with spectral-domain optical coherence tomography” and the application of these conclusions to clinical practice. Sarac et al seek to answer an old and important neuro-ophthalmic question: How can one distinguish between optic disc edema and optic nerve head drusen? Most clinicians have no trouble diagnosing advanced optic disc edema, such as Frisen Stages 3, 4, and 5 (2). Most clinicians have no trouble diagnosing optic nerve head drusen that are visible on ophthalmoscopy (“visible drusen”). Where clinicians do find themselves in a quandary is when they are asked to distinguish mild cases of optic disc edema (Frisen Stages 0, 1, and 2) from optic nerve head drusen that are not visible on ophthalmoscopy (“buried drusen”). Clinicians frequently look to new tools such as optical coherence tomography (OCT) to help sort out these difficult situations. Using time domain OCT, Karam and Hedges (3) concluded that OCT could not be used to differentiate individuals with congenitally crowded optic nerves from individuals with mild papilledema. Conversely, Johnson et al (4) argued that OCT could be used to differentiate optic disc edema from optic nerve head drusen, but these authors included subjects with disc edema that was “mild, moderate, and severe” and drusen that were both visible and buried. These authors also included subjects with papilledema, ischemic optic neuropathy, and optic neuritis in their study population. Lee et al (5) claimed that spectral domain OCT may be used to differentiate optic disc edema from optic nerve head drusen, but they also included subjects in whom the edema ranged from “subtle to severe,” and did not state the etiology of the disc edema in their subjects. In the study by Sarac et al, the optic nerve head drusen group contained eyes with both visible and buried drusen. The optic disc edema group was also heterogeneous, containing subjects with “subtle to severe” optic nerve swelling. In addition, the optic disc edema group contained subjects with papilledema, nonarteritic anterior ischemic optic neuropathy, and optic neuritis. However, most clinicians would have no difficulty distinguishing a patient with optic nerve head drusen from a patient with anterior ischemic optic neuropathy or optic neuritis. We do not dispute the results reported by Sarac et al. Our concern is that clinicians reading this article will inappropriately extrapolate these conclusions to clinical care. When faced with a patient in whom the differential diagnosis includes mild optic disc edema and buried optic nerve head drusen, the guidelines proposed by Sarac et al may not hold. Their study population was not relevant to the clinical question being asked. Currently, it appears that the conclusions reached by Karam and Hedges (3) still hold. Until a study is designed with a clinically relevant population of subjects, the question of the utility of OCT in the differential diagnosis of optic disc edema and optic nerve head drusen remains unanswered.

Radiological evolution and delayed resolution of an optic nerve tuberculoma: Challenges in diagnosis and treatment

Optic nerve tuberculomas are rarely reported and their natural history, prognosis, and duration of required treatment remain unclear. A 40-year-old immunocompetent male presented with complete loss of vision in his right eye, which had evolved over 6 weeks. He had optic atrophy on examination. Initial imaging showed right optic nerve swelling and thickening suggesting an infiltrative inflammatory optic neuropathy (infectious or noninfectious). Serial imaging revealed appearance of ring enhancement with a necrotic centre. Biopsy and culture of the coexistent parietal lobe lesion revealed Mycobacterium tuberculosis. Persistent optic nerve granuloma with evidence of radiological improvement was noted at 18 months follow-up with antituberculous therapy (ATT). Visual recovery could not be achieved. The salient features in this case include the clinical presentation initially mimicking an infiltrative or compressive optic neuropathy, rapidradiological evolution into a tuberculoma, subtle paradoxical radiological worsening after initiation of ATT and persistence of granuloma on follow up scan. The challenges involved in early diagnosis and during the treatment course will be discussed.

Transient optic nerve swelling after acute primary angle closure

Transient optic nerve swelling after acute primary angle closure

Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report

A 33-year-old woman complained of unilateral eyelid edema and blurred vision. Initial ophthalmic examination disclosed anterior chamber reaction with keratic precipitates on the cornea, without posterior abnormalities. Anterior uveitis was treated. Despite that, patient showed rapidly progressive unilateral vision loss with optic nerve swelling. Systemic workup was inconclusive, as well as cranial magnetic resonance imaging and cerebrospinal fluid examination. Based on the hypothesis of optic neuritis, intravenous methylprednisolone pulse was performed with no success. During the following days, the patient presented pericardial effusion and cardiac tamponade, progressing to death. Necropsy was performed and diagnosis of extranodal natural killers/T-cell lymphoma, nasal type with ocular involvement was confirmed by immunohistochemistry.

The translaminar pressure gradient in sustained zero gravity, idiopathic intracranial hypertension, and glaucoma

Papilledema has long been associated with elevated intracranial pressure. Classically, tumors, idiopathic intracranial hypertension, and obstructive hydrocephalus have led to an increase in intracranial pressure causing optic nerve head edema and observable optic nerve swelling. Recent reports describe astronauts returning from prolonged space flight on the International Space Station with papilledema (Mader et al., 2011) [1]. Papilledema has not been observed in shorter duration space flight. Other recent work has shown that the difference in intraocular pressure (IOP) and cerebrospinal fluid pressure (CSFp) may be very important in the pathogenesis of diseases of the optic nerve, especially glaucoma (Berdahl and Allingham, 2009; Berdahl, Allingham, et al., 2008; Berdahl et al., 2008; Ren et al., 2009; Ren et al., 2011) [2–6]. The difference in IOP and CSFp across the lamina cribrosa is known as the translaminar pressure difference (TLPD).We hypothesize that in zero gravity, CSF no longer pools in the caudal spinal column as it does in the upright position on earth. Instead, CSF diffuses throughout the subarachnoid space resulting in a moderate but persistently elevated cranial CSF pressure, including the region just posterior to the lamina cribrosa known as the optic nerve subarachnoid space (ONSAS). This small but chronically elevated CSFp could lead to papilledema when CSFp is greater than the IOP. If the TLPD is the cause of optic nerve head edema in astronauts subjected to prolonged zero gravity, raising IOP and/or orbital pressure may treat this condition and protect astronauts in future space travels from the effect of zero gravity on the optic nerve head. Additionally, the same TLPD concept may offer a deeper understanding of the pathogenesis and treatment options of idiopathic intracranial hypertension (IIH), glaucoma and other diseases of the optic nerve head.

Combing the Globe for Terrorism

A n 83-year-old man with Type 2 diabetes mellitus, systemic hypertension, atrial fibrillation, and successfully treated prostate carcinoma (6 years prior to presentation) complained of progressive painful loss of vision in his left eye for 2 weeks. Examination revealed visual acuity of 20/20 in the right eye and no light perception in the left eye. The left pupil was amaurotic, and there was mild left conjunctival injection and chemosis (Fig. 1), 1 mm of left proptosis, full extraocular movements, and no evidence of anterior or posterior uveitis. The right fundus was normal and left was consistent with combined central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) with optic nerve swelling (Fig. 2). The patient denied temple, jaw, and ear pain, scalp tenderness, migratory arthralgias, and fever. The superficial temporal arteries were palpable and nontender. MRI of the brain and orbits was performed.

Optic nerve swelling and gadolinium contrast enhancement on magnetic resonance imaging in the subacute stage of Leber's hereditary optic neuropathy: A case report

We report the case of a 50-year-old man with subacute onset of bilateral visual field loss and visual acuity loss. His visual acuity was 0.07 OD/0.09 OS and Goldmann perimetry showed central scotomas. The optic fundi were normal bilaterally. Magnetic resonance imaging (MRI) showed hyperintensity in the right optic nerve on T(2) weighted imaging and swelling of the optic chiasm with slight enhancement of the bilateral optic nerves and the optic chiasm on gadolinium-enhanced imaging. Since sensory disturbance in the left hand and leg was noted in addition to the visual problem, multiple sclerosis (MS) was suspected initially. The patient was treated with intravenous methylprednisolone (1,000 mg/day), plasma exchange therapy, and immunosuppressant therapy. However, his visual disturbance did not improve. He had a history of deafness and family history of visual disturbance, because of which we performed an analysis of mitochondrial DNA. G11778A point mutation was found, and a diagnosis of Leber's hereditary optic neuropathy (LHON) was made. Although gadolinium contrast enhancement and swelling of the optic nerve are rare, this case shows that these findings are not in conflict with LHON. The present case also suggests that mitochondrial dysfunction may trigger the onset of MS-like extraocular symptoms in patients with LHON.

Clinical guidelines for diagnosing and managing ocular manifestations in children with mucopolysaccharidosis

The mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans in several tissues and organs. This accumulation results in an array of clinical manifestations and premature death in severe cases. Ocular problems are very common in children with MPS and may involve the cornea, sclera, trabecular meshwork, retina, optic nerve and also the posterior visual pathways. The aims of this study are to give an overview of ocular problems in MPS and to provide clinical guidelines for paediatric ophthalmologists for early diagnosis and management of ocular manifestations in children with MPS. Diagnostic problems may arise in children with severe corneal clouding, hampering visualization of the fundus. Intraocular pressures may be falsely high, even leading to suspicion and unnecessary pressure-lowering treatment. Simple interventions such as the use of prescription glasses or photochromatic glasses can considerably improve quality of life in children with MPS.