Vigabatrin（VGB, Sabril^®）is a new antiepileptic drug for infantile spasms, which was approved in Japan in 2016. VGB is the first-line therapy for infantile spasms, as is ACTH. Although apparent efficacy has been reported, a significant side effect（visual field loss）has received much attention. In Japan, all patients who use VGB should receive regular ophthalmologic examination with electroretinogram. In this section, the efficacy of VGB therapy and the management of side effects are explained.

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　Chloroquine retinopathy is a type of retinopathy in which the macula is impaired bilaterally from long-term administration of chloroquine（CQ）. It is one of the three major drug toxicities in Japan. Consequently, CQ use has been limited for over 50 years. In 2015, hydroxychloroquine（HCQ）was approved for systemic and cutaneous lupus erythematosus. Similar retinopathy may occur with oral administration of HCQ, although the frequency is relatively low compared to CQ retinopathy. The treatment is drug cessation, but progression or worsening may occur even after the cessation; therefore, early detection is important. Factors such as the cumulative dose, liver and renal dysfunction, and aging influence its onset, and collaboration between the doctor prescribing HCQ and the ophthalmologist is important.

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　Digoxin is a type of cardiac glycoside used to treat congestive heart failure and arrhythmia. Because the difference between the therapeutic concentration range and toxic concentrations is very small, digoxin toxicity can easily occur. Digoxin toxicity results predominantly in ocular symptoms（such as xanthopsia, photophobia, hazy vision, visual loss, and central scotoma）, digestive symptoms（such as diarrhea and vomiting）, and cardiac symptoms（such as bradycardia and arrhythmia）. A decrease in 30 Hz-flicker ERG signal amplitude is the accepted visual electrophysiological diagnostic of digoxin toxicity. Digoxin toxicity is treated by stopping the medication. Recovery from digoxin toxicity is assessed by monitoring of symptoms.

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　Currently, cancer affects one in every two people. Therefore, development in anticancer drugs has recently been remarkable, and reports of ocular side effects have increased. These side effects are associated with divergence, and, neuro-ophthalmologically, with reduction in visual acuity, visual field loss, diplopia, and photophobia. Damage sites are the optic nerve, retina, extraocular muscles, and cranial nerves. The representative causative agents include cytotoxic drugs（paclitaxel, docetaxel, cisplatin, 5-fluorouracil, etc.）, hormone drugs（tamoxifen）, molecular target drugs（crizotinib, imatinib, etc.）, and immune checkpoint inhibitors（nivolumab, pembrolizumab, ipilimumab, etc.）. Uveitis is a common side effect of immune checkpoint inhibitors, while optic neuropathy and myasthenia gravis are occasional side effects. Ocular myasthenia gravis tends to suddenly shift to the systemic type; therefore, the clinical course should be observed. Balance between side effects（risk）and therapeutic effects（benefit）is important in cancer therapy, and early detection of side effects and medical cooperation are required.

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　Benzodiazepines（BZD）often clinically include thienodiazepines because of pharmacological similarity. Non-benzodiazepines also bind to gamma-butyric acid（GABA）A receptors. These are used as anticonvulsants, hypnotics, sedatives, and muscle relaxants. Adverse ophthalmological effects of these agents have drawn less attention. In this paper, we reviewed the followings: 1）visual side effects, 2）drug-induced blepharospasm, and 3）withdrawal syndrome of BZD. Acute anti-cholinergic effects of narrow angle glaucoma are well-known. However, ophthalmologists should monitor more frequent and key adverse effects of these drugs, because these agents are used in large doses especially in Japan.

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Purpose: To perform a bibliometric analysis of the biomedical publications on pupil research over the past 50 years and to identify the 100 most frequently cited articles that have been influential in pupil research.
Materials and Methods: We accessed Science Citation Index Expanded provided online by the Web of Science（Clarivate Analytics, November 1918）and searched the database for articles titled with “pupil” or its derivatives that were published between 1968 and 2017, which enabled us to retrieve 3,013 articles for a bibliometric analysis. Furthermore, we counted the number of citations each article had received since its publication and determined the most-cited articles, i.e., the “pupil research citation classics.”
Results: The annual count of publications on pupil research remained about 50 until the early 2000s, followed by a steady increase to over 100 articles in the recent decade. The country of origin was mostly the United States of America（34.1％）, followed by the United Kingdom（10.8％）, Germany（8.4％）, Japan（7.1％）, and People's Republic of China（3.5％）. Pupil studies have been conducted in a wide range of biomedical specialties, and their decreasing order by number of publications is ophthalmology, neurology, neurophysiology, neuroscience, neuropharmacology, and neuropsychology. Remarkable study topics included tonic pupil, fixed dilated pupil, pupillary escape, melanopsin-related pupillary reflexes, cholinergic deficiency in Alzheimer’s disease, task-evoked psychic pupillary reflexes, higher order aberration associated with cataract and refractive surgery, and application of pupillometry on the objective perimetry. The citation counts of articles ranged from 0 to 640, and the most-cited articles dealt with task-evoked pupillary responses（Beatty, 1982）, Stiles–Crawford effect（Stiles and Crawford, 1933）, pupil diameter and load on memory（Kahneman, 1966）, pupil as a measure of emotional arousal（Bradley, 2008）, and melanopsin capable of driving pupillary constriction（Lucas, 2003）.
Conclusions: The bibliometric analysis indicated that diverse biomedical specialties have participated in a wide range of basic and clinical pupil studies. It is remarkable that neurophysiology and neuropsychology have become increasingly popular in the recent decade.

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　Two definite and two probable cases of myasthenia gravis（MG）with antibodies against the low-density lipoprotein receptor-related protein 4（Lrp4）were reported. Clinical, serological, and radiological examinations were conducted. The clinical course and treatment were also investigated. The patients included 3 women and 1 man, with the mean onset age of 55 years. They showed ocular or mild-to-moderate generalized MG and had double-seronegative MG with anti-Lrp4 antibodies. Three of the 4 patients had anti-thyroid peroxidase antibodies. Computed tomography did not reveal thymoma in any patient. Corticosteroids improved the symptoms in 3 patients. An immunosuppressive agent was effective in one patient and an acetylcholinesterase inhibitor in another.

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　Vici syndrome is an autosomal recessive, congenital multisystem disorder first reported by Vici et al. in 1988. It presents with agenesis of the corpus callosum, congenital cataracts, oculocutaneous albinism, and mixed immunodeficiency syndrome. Many children with Vici syndrome die before 3 years of age because of heart failure or infection. The disease is attributed to a mutation in the ectopic P-granules autophagy protein 5（EPG5）gene, which is involved in regulating the intracellular protein metabolism. Many patients with Vici syndrome exhibit neuroophthalmological abnormalities, such as congenital cataracts, retinal hypopigmentation, optic atrophy, and nystagmus. We treated a patient with Vici syndrome who has survived to 17 years of age, making it possible to perform objective tests such as electroretinography（ERG）, visually evoked cortical potential（VECP）tests, and infrared pupillometry. Superficial punctate keratitis due to bilateral eyelid closure insufficiency and bilateral optic nerve atrophy were observed; however, congenital cataracts and retinal hypopigmentation, which are typical of this disease, were absent. ERG recorded negative waveforms, and VECP showed a clear response in both eyes; however, the pupillary light reflex was absent. To our knowledge, this patient is the oldest person to survive with Vici syndrome, reported to date.

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Purpose: We present a case of unilateral retinal degeneration that was incorrectly diagnosed and treated as optic neuritis.
Case: A 21-year-old woman was referred to Kurume University Hospital for further examination of left optic neuritis. She received steroid pulse therapy after a diagnosis of left optic neuritis 7 years ago; however, her visual acuity did not improve. On presentation, her best-corrected visual acuity was 1.5 in the right eye and 0.03 in the left eye, with a relative afferent pupillary defect in the left eye. Fundus examination of the left eye showed pallor of the optic nerve head, mottling of the retinal pigment epithelium, and attenuated retinal vessels in the mid periphery. Fundus examination of the right eye revealed normal findings. A diagnosis of unilateral retinal degeneration in the left eye was made based on the results of optical coherence tomography, fundus autofluorescence, electroretinogram, and fundus fluorescein angiography.
Conclusion: The present case emphasizes on the importance of a complete ocular examination, including the peripheral retina, to rule out retinal abnormalities before optic neuritis is presumed to be the cause of unilateral visual loss.

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　A 10-year-old girl who complained of left upper eyelid pain and diplopia was diagnosed with left oculomotor nerve palsy by a previous doctor. However, computed tomography, magnetic resonance imaging, blood examination, and spinal fluid analysis revealed no abnormalities. The patient underwent steroid pulse therapy, which was not successful. She was referred to our department for a second opinion. The clinical features were ptosis, ocular motility disorder, disorders of accommodation, and reduced pupillary light reflex in the left eye. At 2 months after onset, ptosis and ocular motility disorder completely resolved. At 25 months after onset, the disorders of accommodation slightly improved but the pupillary light reflex did not. Moreover, light-near dissociation, segmental palsy of the iris sphincter, and denervation supersensitivity by an ocular injection of pilocarpine hydrochloride（0.125％）in the pupil of the affected eye were observed. Therefore, the lesion responsible for tonic pupil in the patient was considered to be of postganglionic origin.

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　This report describes a case involving a Japanese patient with distance esotropia, who was followed-up for 4 years using magnetic resonance imaging（MRI）. A 68-year-old woman with high myopia exhibited esotropia of 10 prism diopters（∆）and right hypotropia of 5∆； her axial lengths were 31.0 mm oculus dextrus（OD）and 30.7 mm oculus sinister（OS）. Bilateral enlarged lateral rectus（LR）-superior rectus（SR）bands and posterior staphylomata were observed on orbital MRI. At the 4-year follow-up, her ocular deviation had deteriorated to esotropia of 18∆ and right hypotropia of 8∆； however, her axial lengths had scarcely changed（31.0 mm OD; 30.8 mm OS）. The enlargement and rupturing of the bilateral LR-SR bands had worsened. Progressive enlargement and rupturing of the LR-SR bands, which are presumably related to aging and possibly linked to progressive connective tissue degeneration, may increase ocular deviation.

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