This article describes a diagnosis method for optic neuritis confirmed by clinical observations and magnetic resonance imaging (MRI) abnormalities. Clinicians diagnose unilateral optic neuritis by clinical observations only. Unilateral optic nerve impairment is defined as 1) monocular decreased visual acuity not responsible for ocular media or monocular visual field defect with no defect in the other eye, 2) relative afferent pupillary defect positive. Important clinical observations of optic neuritis are disc swelling and ocular pain. If a patient does not have disc swelling, an assessment of relative afferent pupillary defect is necessary. Optic nerve enhancement by fat suppression gadolinium enhanced T1 weighted MRI confirms diagnosis of optic neuritis if other diseases impairing the blood-brain barrier, such as tumor, fungal infection, and radiation, are excluded. Patients with unilateral optic neuritis without autoantibodies meet the inclusion criteria for the Optic Neuritis Treatment Trial and Japanese trial conducted by the Optic Neuritis Treatment Trial Multicenter Cooperative Research Group. Almost all of these patients follow spontaneous recovery of visual function. However, if a patient does not display clinical characteristics revealed by the two treatment trials, clinicians should consider atypical optic neuritis. The most important differential diagnosis is anti-aquaporin-4 antibody-positive optic neuritis in neuromyelitis optica spectrum disorders.

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Anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)–positive optic neuritis is a new type of optic nerve disorder. Cases of optic neuritis associated with MOG-Abs were usually diagnosed by cell-based assays. MOG-Ab seropositive cases had relatively favorable visual acuity outcome but had significant residual visual field deficit. Furthermore, the number of relapses per year was significantly greater in MOG-Ab seropositive cases than in seronegative cases. MOG-Abs may contribute to the heterogeneous clinical picture of optic neuritis, and although visual acuity outcome is favorable, residual visual field tends to deteriorate and relapses may repeatedly occur.

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Recent topics on the diagnosis and treatment of both arteritic (AION) and non-arteritic ischemic optic neuropathy (NAION) were reviewed with reference to our experience with similar cases. I have managed a patient with posterior AION. AION is rare in Japan. Posterior AION is rare even in the United States of America, where AION is encountered more frequently than in Japan. I have also encountered a patient with AION, who presented with a transient visual disturbance. Approximately 30% of all patients with AION are symptomatic, and approximately 60% of these develop permanent visual loss. We need to recognize that AION due to giant cell arteritis in the Japanese might present as posterior AION or with symptoms of transient visual disturbance. Hence, temporal artery biopsy should be performed in such patients with high levels of C-reactive protein and erythrocyte sedimentation rate; moreover, early treatment should be administered to prevent permanent visual loss. Intravenous steroid pulse therapy is the rule in the treatment of AION. Other treatment options, such as immunosuppressive therapy, are used in steroid-resistant patients and in those with recurrence during high-dose steroid therapy and past medical history of severe side effects due to steroids. The efficacies of drugs like tocilizumab and methotrexate have been investigated, and the evidence regarding their effectiveness has not been established. Newly developed fundus cameras have contributed to new insights regarding the diagnosis of NAION. Some investigators have found that the optic nerve blood flow is significantly decreased using laser speckle flowgraphy and that the vessel density in the optic disc is significantly reduced using optical coherence tomography angiography in patients with NAION. We hope that newer machines are developed for the diagnosis of NAION. There is no effective treatment for improving the vision in patients with NAION. We have investigated the effectiveness of L-arginine, which was effective in the rat model of NAION. However, we could not confirm the effectiveness of L-arginine in human patients with NAION. A treatment trial of QPI-1007 involving patients with NAION is ongoing. We hope that this medicine will be effective in patients with NAION.

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In conjunction with a working group for retina-choroidal and optic atrophy funded by the Ministry of Health, Labor, and Welfare (MHLW), the Japanese Neuro-ophthalmology Society has recently defined the Designation Criteria for Leber hereditary optic neuropathy (LHON) and has conducted a nationwide epidemiological survey on LHON to justify its eligibility as a specified intractable disease defined by MHLW. According to the characteristic major symptoms and ancillary prognostic test results, the criteria classify patients with LHON into definite, probable, and possible cases, as well as asymptomatic carriers. Questionnaires were sent to 1\_h,\/397 facilities, which were certified by either the Japanese Ophthalmological Society or the Japanese Neuro-ophthalmology Society, to determine the number and sex of patients with definite and probable LHON who had one of three mitochondrial DNA mutations at nucleotide positions 3460, 11778, or 14484 and had newly developed symptoms during the year 2014. One hundred seventeen patients were estimated to have newly developed LHON (95% confidence interval, 81 to 153). Over 86% and 90% of these patients possessed the 11778 mutation and were male, respectively, and both percentages were higher than those reported previously in foreign countries. The median age at onset was over 30 years, which was older than previously reported. The establishment of the Designation Criteria and identification of the number of patients not only fulfill the eligibility of LHON as a specified intractable disease but also attract general ophthalmologists' interest towards LHON. Moreover, it motivates researchers and pharmaceutical companies to develop new treatment modalities for LHON.

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Hemifacial spasms (HFSs) can be classified into primary and secondary types. A primary HFS is caused by vascular compression of the facial nerve. The arteries that are commonly implicated are the anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA). Twenty-one patients with HFS underwent axial and coronal heavily T2-weighted MRI. We performed three-dimensional evaluation of the vascular compression and the direction of the facial nerve. We also examined the relationship between the vessel compression and clinical signs. In 91% of patients with HFS, the vessels responsible for the neurovascular compression were present in the affected side (i.e., the side showing the spasm) in the cerebellopontine angle cistern. The most frequent vessel was the AICA in the affected side in 9 cases, followed by the PICA in the affected side in 6 cases. Moreover, our results indicate that inferior compression towards the facial nerve is the most probable mechanism of primary HFS. However, MRI abnormalities were found in 40% of unaffected patients, although this was not statistically significant when compared to those in the normal subjects.

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Purpose: To report outcomes of extraocular muscle surgery to improve visual acuity or correct abnormal head posture (AHP) in patients with infantile nystagmus syndrome (INS).
Subject and Methods: Twenty-two patients who underwent extraocular muscle surgery when less than 15 years old (average age at time of surgery, 7.5 years) and were followed up for at least 6 months at Hyogo College of Medicine Hospital from 2006 to 2015 were recruited. The mean follow-up period was 3.7 years. Large recession of all four horizontal rectus muscles was performed to reduce nystagmus. The Anderson or modified Kestenbaum methods were used to correct AHP. We retrospectively analyzed the surgical methods used to treat INS and the effects on visual acuity, AHP, and binocularity.
Results: Twelve patients with a null zone, six without a null zone and jerky nystagmus, three with periodic alternating nystagmus, and one with pendular nystagmus were included. No significant improvement in postoperative binocular visual acuity was observed; however, visual acuity in the more affected eye improved significantly after surgery (p=0.0001). Of the 11 patients with AHP, five (45%) were free from AHP and three (27%) showed improvement after surgery. Two patients exhibited recurrence of AHP. One patient with albinism and macular hypoplasia was unaffected by treatment. Binocularity did not demonstrate improvement for any type of nystagmus (near, p=0.0845; distance, p=0.8516).
Conclusion: Extraocular muscle surgery for INS was effective in improving visual acuity in the more affected eye and AHP. However, there is a possibility that AHP could persist in patients with poor vision accompanied by macular hypoplasia.

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Purpose: To report on the application of optical coherence tomography angiography (OCTA) for the detection of peripapillary abnormalities in 4 eyes with Leber hereditary optic neuritis (LHON).
Case 1: A 23-year-old man was referred to our department with visual impairment in his left eye. A mitochondrial DNA 11778 point mutation was detected. Microscopic examination showed optic disc swelling in both eyes. Fluorescence angiography (FA) demonstrated peripapillary telangiectatic blood vessels and vascular tortuosity without corresponding leakage in both discs. OCTA demonstrated parapapillary telangiectatic blood vessels in both eyes.
Case 2: A 5-year-old boy was referred to our department with visual impairment in his left eye. A mitochondrial DNA 11778 point mutation was detected. Microscopic examination showed optic disc swelling in the right eye and disc pallor in the left eye. FA demonstrated peripapillary telangiectatic blood vessels and vascular tortuosity without corresponding leakage in the right eye only and hyperfluorescence in the left disc. In contrast, OCTA demonstrated parapapillary telangiectatic blood vessels in both eyes
Conclusion: OCTA is a safe and convenient imaging modality that can be used to obtain multiple scans. Further, OCTA may be a useful diagnostic tool for evaluating the eyes of patients with LHON.

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We report on a 68-year-old man who exhibited exotropia as an initial sign of Lambert-Eaton syndrome. He complained of exotropia and diplopia in March and was referred to our hospital for further examinations and treatment in August, where he was diagnosed with conjugate exotropia and cataracts. The patient underwent bilateral cataract surgeries in June after one year and strabismus surgery of the left eye in March after two year. Blepharoptosis and muscle weakness of lower limbs were observed in August after two year and August after three year, respectively. Serum anti-acetylcholine receptor antibody and muscle-specific tyrosine kinase antibody -indicators for the diagnosis of myasthenia gravis-/were not detected. The patient underwent further examination at our Department of Neurology, where his diagnosis of Lambert-Eaton syndrome was confirmed by the presence of serum antibodies against P/Q-type voltage-gated calcium channel and the presence of the waxing sign on evoked electromyography. No malignant lesions were found by positron emission tomography or other serologic examinations. Treatments that have been performed since December after three year include administration of 3,4-diaminopyridine, steroid therapy, immunosuppressant therapy, and immunoglobulin and serum exchange therapy; these treatments have partially reduced the muscle weakness, with some serious complications.

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Idiopathic orbital inflammation is a benign, non-infectious, nongranulomatous orbital lesion that is characterized by sudden onset of orbital pain. While a few cases are diagnosed by tissue biopsy, most are assessed using a diagnostic trial of corticosteroids. However, we often encounter cases that are not completely responsive to corticosteroids, which makes the diagnosis difficult. We report a case of painless idiopathic orbital inflammation diagnosed by tissue biopsy. A 48-year-old man complained of lid swelling of the left eye during the 3 weeks immediately prior to his initial evaluation. The patient presented with a vertical gaze palsy and conjunctival hyperemia without decreased visual acuity or pain in the left eye. Short TI inversion recovery magnetic resonance imaging showed a high intensity area that included the left superior rectus muscle. Since we could not rule out a neoplasm, we performed a biopsy of the lesion. Results revealed the presence of non-specific lymphocytic infiltration without any neoplastic changes. Thus, we diagnosed the patient with idiopathic orbital inflammation. After administration of corticosteroids, his symptoms quickly improved. When diagnosing an idiopathic orbital inflammation, histopathologic diagnosis should be considered in order to rule out a neoplastic lesion.

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Malignant tumors of the paranasal sinuses are most commonly derived from the maxillary sinus. Squamous cell carcinomas from the sphenoidal sinus are quite rare. We report a case of optic neuropathy caused by a squamous cell carcinoma of the sphenoidal sinus. A 72-year-old man presented to the local clinic with a headache and visual disturbance of the left eye. Due to a posterior capsular opacity, a left posterior capsulotomy was performed using a YAG-laser. However, his visual acuity worsened after treatment and he was referred to our hospital. During the initial examination at our hospital, his corrected vision was 1.2 in his right eye and “hand motion” in his left eye. A relative afferent pupillary defect (RAPD) was present on the left side. Ptosis was present on the left side and the left eye movement was limited in all directions. These findings were consistent with left orbital apex syndrome. MRI with gadolinium enhancement revealed a mass lesion that occupied the entire left sphenoidal sinus. In addition, the mass lesion extended to the cavernous sinus and orbital apex region on the left side. A biopsy was performed by an otolaryngologist and the results were consistent with a squamous cell carcinoma. Radiation therapy was performed and his left visual acuity deteriorated to blindness; however, the radiation therapy successfully recuperated his left eye movement with little limitation. A squamous cell carcinoma of the sphenoidal sinus is generally asymptomatic when the tumor remains inside the sinus. After extension into the cavernous sinus and orbital apex, the tumor causes headaches and visual impairment. Thus, ophthalmologists may play a crucial role in identifying an initial symptom of squamous cell carcinoma of the sphenoidal sinus. This case may suggest the importance of basic neuro-ophthalmology skills such as the identification of RAPD.

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We present a patient with a pituitary adenoma suspected of having retrobulbar optic neuritis during pregnancy and delivery. A 24-year-old woman consulted an ophthalmologist for decreased visual acuity in the left eye after delivery and was referred to us on the suspicion of having retrobulbar optic neuritis. Visual acuity was 1.2 in the right eye and 0.4 in the left, the critical flicker frequency was 41 Hz in the right eye and 21 Hz in the left, and relative afferent pupillary defect was present in the left eye. The anterior segment, optic media, and fundus were normal. Goldmann perimetry showed central and paracentral scotoma and enlargement of the Mariotte blind spot in the left eye. Optical coherence tomography (OCT) revealed thinning of the inner retinal layer of the macula at the nasal area of the fovea in the left eye. Magnetic resonance imaging (MRI) revealed a pituitary adenoma. We diagnosed the patient as having a pituitary adenoma with pituitary apoplexy. A week after tumorectomy, preoperative visual loss improved. Six months postoperatively, OCT revealed band atrophy in the left eye. Accordingly, in patients presenting with unilateral visual loss during pregnancy or after delivery, we should keep in mind the possibility of a pituitary adenoma. Detailed examinations for intracranial disease using MRI are useful for the differential diagnosis.

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Purpose: To report a case of non-alcoholic Wernicke encephalopathy presenting with papillitis.
Case: A 46-year-old man with no history of alcohol dependence was referred to us for blurred vision in both eyes. He had developed this visual disturbance along with a severe eye movement disorder. A diagnosis of Wernicke encephalopathy was made following an examination of the patient's magnetic resonance imaging (MRI) scans. Vitamin B1 was administered intravenously and an improvement in the visual disorder was observed by the following day. The patient's ocular movement disorder and papillitis gradually improved, although the endpoint nystagmus that the patient suffered from when looking to the lateral side did not resolve.
Conclusion: A case of Wernicke encephalopathy with papillitis, with no history of alcohol dependence, is presented. Wernicke encephalopathy was diagnosed based on the patient's clinical manifestations and MRI scans, and was resolved by administration of vitamin B1.

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Purpose: To report on the overcorrection of a large-angle esotropia caused by abducens nerve palsy using horizontal rectus muscle surgeries augmented with botulinum toxin A.
Cases: A 76-year-old woman with a distance esotropia of 65 prism diopters (PD) in the primary position received a 6-mm recession of the medial rectus muscle augmented with 5 units of botulinum toxin A and a 6-mm resection of the lateral rectus muscle. Two weeks later, a large-angle overcorrection and transient moderate limitation of adduction were observed. Postoperatively, she had advancement of the recessed muscles to the original insertion and acceptable alignment.
Conclusion: Extraocular muscle surgery augmented with botulinum toxin A may be useful in the treatment of a large-angle paralytic strabismus. However, unpredictable overcorrection may occur and additional surgeries may be necessary.

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Objectives: To describe the neuro-ophthalmic manifestations of intracranial tumors in patients, the type of tumors, and the location of the tumor base on neuro-imaging.
Materials and methos: This retrospective observational study included medical records of patients with intracranial tumors who initialy consulted to ophthalmologists in the neuro-ophthalmology unit of Cicendo Eye Hospital between January 2012 and December 2016. Patients who were diagnosed with intracranial tumors that were confirmed by radiologic examination were included. All data, including patient sex, age, neuro-ophthalmic signs and symptoms, and the type and location of the intracranial tumor, were reviewed.
Results: The study included 132 patients (40.9% male, 59.1% female); the average age was 40.14 years (range, 9-69 years). Neuro-ophthalmologic symptoms included blurred vision (81.06%), visual field complaints (9.09%), double vision (6.82%), and ptosis (3.03%). Neuro-ophthalmologic signs included bilateral optic atrophy (54.54%), unilateral optic atrophy (14.39%), papilledema (12.12%), visual field defect (41.67%) and ocular motor nerve palsy (18.94%). The three most common types of intracranial tumors were meningioma (47.72%), pituitary adenoma (25.75%), and craniopharyngioma (9.10%). All patients were consulted to neurosurgery departmen except 2 patients with nasopharyngeal carcinoma were consulted to otolaryngology department. Only 21 patients (15.91%) returned for a follow up visit.
Conclusion: The most common neuro-ophthalmologic symptom was blurred vision, the most common neuro-ophthalmologic sign was abnormality of the optic discs, particularly optic disc atrophy. Meningioma was the most common tumor that impaired visual function and was most often diagnosed in adult women. The most common tumor location was the chiasm, which manifested as bilateral optic disc atrophy.

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