Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG.

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Aims: The aim was to assess the etiology of sixth nerve palsy and on the basis of our data, to formulate a diagnostic algorithm for the management in sixth nerve palsy. Design: Retrospective chart review. Results: Of the 104 neurologically isolated cases, 9 cases were attributable to trauma, and 95 (86.36%) cases were classified as nontraumatic, neurologically isolated cases. Of the 95 nontraumatic, isolated cases of sixth nerve palsy, 52 cases were associated with vasculopathic risk factors, namely diabetes and hypertension and were classified as vasculopathic sixth nerve palsy (54.7%), and those with a history of sixth nerve palsy from birth (6 cases) were classified as congenital sixth nerve palsy (6.3%). Of the rest, neuroimaging alone yielded a cause in 18 of the 37 cases (48.64%). Of the other 19 cases where neuroimaging did not yield a cause, 6 cases were attributed to preceding history of infection (3 upper respiratory tract infection and 3 viral illnesses), 2 cases of sixth nerve palsy were found to be a false localizing sign in idiopathic intracranial hypertension and in 11 cases, the cause was undetermined. In these idiopathic cases of isolated sixth nerve palsy, neuroimaging yielded no positive findings. Conclusions: In the absence of risk factors, a suggestive history, or positive laboratory and clinical findings, neuroimaging can serve as a useful diagnostic tool in identifying the exact cause of sixth nerve palsy. Furthermore, we recommend an algorithm to assess the need for neuroimaging in sixth nerve palsy.

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Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children.

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Background: Idiopathic intracranial hypertension (IIH) has been increasing in prevalence in the past decade, following the obesity epidemic. When medical treatment fails, surgical treatment options must be considered. However, controversy remains as to which surgical procedure is the preferred surgical option - optic nerve sheath fenestration (ONSF) or cerebrospinal fluid (CSF) shunting - for the long-term treatment of this syndrome. Purpose: To provide a clinical update of the pros and cons of ONSF versus shunt placement for the treatment of IIH. Design: This was a retrospective review of the current literature in the English language indexed in PubMed. Methods: The authors conducted a PubMed search using the following terms: Idiopathic IIH, pseudotumor cerebri, ONSF, CSF shunts, vetriculo-peritoneal shunting, and lumbo-peritoneal shunting. The authors included pertinent and significant original articles, review articles, and case reports, which revealed the new aspects and updates in these topics. Results: The treatment of IIH remains controversial and lacks randomized controlled clinical trial data. Treatment of IIH rests with the determination of the severity of IIH-related visual loss and headache. Conclusion: The decision for ONSF versus shunting is somewhat institution and surgeon dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache. There are positive and negative aspects of both procedures, and a prospective, randomized, controlled trial is needed (currently underway). This article will hopefully be helpful in allowing the reader to make a more informed decision until that time.

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Background: There is an increase in the incidence of traumatic optic neuropathy (TON) due to increasing urbanization and rapid spurt in the number of motor vehicles on the road. Despite early presentation and ease of diagnosis the visual outcomes in TON are still limited. There is also significant confusion about the timing, dose and efficacy of steroid treatment in its management. Purpose: To provide a clinical update of the pros and cons of steroid therapy for TON. Design: The paper is a retrospective review of the currently available literature in the English language indexed in PubMed. Methods: A PubMed search was conducted by the authors using the following terms: Traumatic optic neuropathy, megadose, steroids, methylprednisolone. Relevant original articles, review articles, and case reports related to the topic of discussion were evaluated and discussed in the paper. Results: There is no prospective randomized control trial evaluating the effect of steroids in TON. There are varying reports on the effect of steroid therapy from significant improvement to no difference compared to observation. Conclusion: The decision to give steroids to patients with TON has to be on an individual case to case basis and must involve informed consent from the patient. There are documented advantages and disadvantages of steroid therapy and a prospective, randomized, controlled trial is necessary comparing steroids, surgery and observation before definitive management can be evolved.

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Aims: The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. Setting and Design: Review article. Materials and Methods: Existing literature and the authors' experience were reviewed. Results: Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri. Conclusion: While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis.

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Background: Optic nerve atrophy is an important ophthalmological sign that may be associated with serious systemic conditions having a significant bearing on the overall morbidity of the child. Studies specific to etiology of childhood optic atrophy are scarce, this being the first such study from India to the best of our knowledge. Aim: The aim was to analyze the clinical features and etiology of diagnosed cases of optic nerve atrophy in children <16 years of age. Materials and Methods: Retrospective review of records of children diagnosed with optic nerve atrophy between the ages of 0 and 16 years from 2006 to 2011. Results: A total of 324 children (583 eyes) were identified. Among these 160 (49%) presented with defective vision, 71 (22%) with strabismus, 18 (6%) with only nystagmus. Rest had a combination of two or three of the above symptoms. Sixty-five patients (20%) had a unilateral affection. Hypoxic ischemic encephalopathy seen in 133 patients (41%) was the most frequent cause of childhood optic atrophy, followed by idiopathic in 98 (30%), hydrocephalus in 24 (7%), compressive etiology in 18 (5%), infective in 19 (6%), congenital in 6 (2%), inflammatory in 5 (2%) patients, respectively. Conclusion: Hypoxic ischemic encephalopathy appears to be the most common cause of optic atrophy in children in this series. The most common presenting complaint was defective vision.

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Aims: The aim was to highlight recent advances in the treatment of thyroid eye disease. Settings and Design: Review article. Materials and Methods: Existing literature and the authors' experience was reviewed. Results: Thyroid ophthalmopathy is a disfiguring and vision-threatening complication of autoimmune thyroid disease that may develop or persist even in the setting of well-controlled systemic thyroid status. Treatment response can be difficult to predict, and optimized algorithms for disease management do not exist. Thyroid ophthalmopathy should be graded for both severity and disease activity before choosing a treatment modality for each patient. The severity of the disease may not correlate directly with the activity; medical treatment is most effective in active disease, and surgery is usually reserved for quiescent disease with persistent proptosis and/or eyelid changes. Conclusions: Intravenous pulsed corticosteroids, orbital radiotherapy, and orbital surgical techniques form the mainstay of current management of thyroid ophthalmopathy. Immunosuppressive and biologic agents may have a role in treating active disease although additional safety and efficacy studies are needed.

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Context: Optic neuritis (ON) is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or "typical" ON, usually associated with multiple sclerosis. This is in contrast to "atypical" causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision. Aims: The aim was to describe the clinical presentations of atypical ON and their treatments. Settings and Design: Review article. Materials and Methods: Literature review. Results: Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression. Conclusions: Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

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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.

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This essay was written to discuss the reasoning behind the personal decisions made by 2 current neuro-ophthalmology fellows to pursue neuro-ophthalmology as a career. It is meant to enlighten the reader about what role neuro-ophthalmologists play in clinical practice, what makes neuro-ophthalmology unique to all other sub-specialties, and how this contributes to making neuro-ophthalmology not only one of the most medically interesting, yet rewarding sub-specialties in ophthalmology.

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