Neuro-Ophthalmology Japan
Online ISSN : 2188-2002
Print ISSN : 0289-7024
ISSN-L : 0289-7024
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Guest Articles
  • [in Japanese]
    2020 Volume 37 Issue 2 Pages 119
    Published: June 25, 2020
    Released: July 08, 2020
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  • Toshiaki Osato, Hirohiko Nakamura
    2020 Volume 37 Issue 2 Pages 120-128
    Published: June 25, 2020
    Released: July 08, 2020
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     Cerebrovascular diseases have high morbidity and mortality rates and include cerebral infarction, cerebral hemorrhage, and subarachnoid hemorrhage. Cerebral hemorrhage accounted for 80%of cerebrovascular diseases in the 1960s. However, in elderly patients, cerebral infarction caused by atherosclerosis and embolisms caused by atrial fibrillation currently account for 70%of these diseases. This paper describes the medical and surgical treatments for cerebrovascular diseases, the social system of stroke treatment in Japan, and particularly, thrombectomy in patients with acute ischemic stroke who are undergoing endovascular treatment, because the clinical results of this treatment have been improving, especially in recent years.

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  • Ken Johkura
    2020 Volume 37 Issue 2 Pages 129-139
    Published: June 25, 2020
    Released: July 08, 2020
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     Although horizontal eye movement disorders caused by pontine lesions are not yet fully understood, their evaluation is useful for the localization and diagnosis of pontine lesions. For example, medial longitudinal fasciculus (MLF) lesions often cause exotropia along with internuclear ophthalmoplegia (INO) characterized by impaired adduction of the ipsilateral eye.

    INO associated exotropia is usually seen in the contralateral eye (the contralateral exotropia is called pontine exotropia) . This exotropia has recently been found to be caused by the coexisting paramedian pontine reticular formation (PPRF) impairment. When PPRF is not damaged at all,INO associated exotropia is seen in the ipsilateral eye (the ipsilateral exotropia is called wall-eyed monocular internuclear ophthalmoplegia) . Conversely, PPRF lesions without INO, usually cause ipsilateral horizontal gaze palsy, sometimes cause abduction limitation and esotropia of the ipsilateral eye, because the effects of PPRF impairment are not equal between abduction and adduction. Rostral pontine lesions may cause impairment of ipsilateral smooth-pursuit eye movements and contralateral hemisensory syndrome. In contrast, caudal pontine lesions may cause impairment of contralateral smooth-pursuit eye movements and ipsilateral horizontal gaze palsy.

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  • Kaori Hanai
    2020 Volume 37 Issue 2 Pages 140-146
    Published: June 25, 2020
    Released: July 08, 2020
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     Terson syndrome is characterized by intraocular hemorrhage associated with subarachnoid hemorrhage (SAH) or non-traumatic intracerebral hemorrhage. This syndrome has been recognized worldwide since Terson described a patient with vitreous hemorrhage following as SAH in 1900. Previously, there have been several reports on the clinical epidemiology of Terson syndrome. However, statistics from most of these retrospective studies are variable because it is difficult to perform an ophthalmologic examination for all patients with SAH referred to an emergency hospital prospectively. We attempted to conduct a prospective clinical study of all patients with SAH in our hospital with the cooperation of our neurosurgery staff.

     Here, we describe the results of our research on the vitreosurgical outcome, characteristics of vitreous hemorrhage during microsurgical observation, and the mechanism of Terson syndrome.

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Original Articles
  • Keiichi Tanzawa, Mayumi Oka, Ayumi Amejima, Kaho Shimada, Mayu Nishika ...
    2020 Volume 37 Issue 2 Pages 147-153
    Published: June 25, 2020
    Released: July 08, 2020
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    Purpose: To investigate photometric harmony with pupil perimetry in healthy adults.

    Methods: We included 21 right eyes of 21 healthy adults (mean age, 20.6±0.5 years) . Pupillary light reflexes were measured based on the pupil perimeter using a device produced experimentally by the Kawasaki University of Medical Welfare. Light stimuli had five levels of intensity (area×luminance) , which were paired with the same intensity but at different areas (2° to 16°) and luminance (1 to 16 cd/m2) . Stimuli sites were positioned at five points (45°, 135°, 225°, and 315°) with eccentricities of 0° and 15°. The pupillary contraction ratio (PCR) was measured to evaluate the pupillary light reflex.

    Results: At each stimulus site, PCR showed a logarithm of intensity and linear regression (R2 > 0.9) . When using stimuli of the same intensity, PCRs with an eccentricity of 0° were greater than PCRs with an eccentricity of 15°. PCR did not differ between pairs of same intensities at any stimulus site (p>0.05) .

    Conclusions: In the central visual field with an eccentricity of 15° or less, photometric harmony with pupil perimetry was observed using light stimuli of the same intensity.

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  • Kazuko Totsuka, Toshiaki Goseki, Hitoshi Ishikawa, Tomoya Handa, Chiek ...
    2020 Volume 37 Issue 2 Pages 154-159
    Published: June 25, 2020
    Released: July 08, 2020
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    Purpose: To measure cyclofusion in healthy adults and patients with superior oblique palsy using the ORTe and synoptophore and compare the results obtained with the two devices.

    Subjects and Methods: We examined 17 subjects (28.9±6.3 years old) in the control group and 17 patients (46.1±23.9 years old) in the superior oblique palsy group. Cyclofusion was evaluated in the primary, 15° upward, and 15° downward positions using the ORTe and synoptophore.

    Results: In the control group, cyclofusion ranged from 9.6° to 10.2° with ORTe and from 13.6° to 14.4° with the synoptophore. In the superior oblique palsy group, cyclofusion ranged from 12.0° to 13.5° with ORTe and from 16.5° to 18.4° with the synoptophore. In the control group, ORTe showed significantly smaller cyclofusion in all directions, and in the superior oblique palsy group, ORTe showed significantly smaller cyclofusion in the primary and 15° upward positions. There was no significant difference between the two groups in the results obtained with either device.

    Conclusion: ORTe helped evaluate cyclofusion with the same efficacy as the synoptophore. The reason for the small amount of cyclofusion was presumed to be the difference in the binocular separation method between the ORTe and synoptophore.

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Case Reports
  • Yuko Harada, Toshiaki Goseki, Kazutoyo Kaneda, Hitoshi Ishikawa, Shuji ...
    2020 Volume 37 Issue 2 Pages 160-164
    Published: June 25, 2020
    Released: July 08, 2020
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     Here, we report the case of a patient with strabismus fixus without high myopia whose eyeball and orbital volumes differed, as confirmed with the eyeball-to-orbital area ratio. The patient was treated using the SR-LR union suture.

     Case: A 58-year-old woman had gradually worsening esotropia for 20 years. Her visual acuity was 1.2 ×-3.75 D OD, and 0.6 ×-3.75 D (C-1.00 D; 170°) OS. Her eye alignment was 90 prism diopters (PD) esotropia and 20 PD left hypotropia at distance and 100 PD esotropia and 25 PD left hypotropia at near. Aversion was full in the right eye, restricting all movements without adduction or depression in adduction in the left eye. The lateral rectus muscle showed an inferior shift and the superior rectus muscle showed a nasal shift. The lateral rectus–superior rectus muscle band was thin and ruptured. Orbital magnetic resonance imaging (MRI) revealed a herniated eyeball from the muscle cone in the left eye. The axis lengths were normal, but the orbital lengths were shorter than normal. The eyeball-to-orbital area ratio was below the average in both eyes. We performed the left SR-LR union suture with left medial rectus muscle recession. The eye alignment improved to 10 PD esotropia at distance and 6 PD esophoria at near.

     We assumed that strabismus fixus occurred from the discordance in volume between the eyeball and orbit without high myopia. The eyeball-to-orbital area ratio was useful for the diagnosis of strabismus fixus without high myopia, and the SR-LR union suture was useful for treatment.

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  • Kazuko Haruishi, Atsushi Miki, Syunsuke Araki, Katsutoshi Goto, Hiroto ...
    2020 Volume 37 Issue 2 Pages 165-170
    Published: June 25, 2020
    Released: July 08, 2020
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     We report a case of adrenoleukodystrophy (ALD) in a 10-year-old boy with visual disturbance. He presented with developmental disorders and a progressive intellectual disability. The patient was referred to our hospital by a neighboring ophthalmologist because of vision loss and gait disturbance for 3 weeks. At the first visit, his visual acuity was 0.08 in the right eye and 0.04 in the left eye. Both eyes showed abduction. Symmetric areas of high signal intensity in the white matter on head magnetic resonance imaging (MRI) with the T2-weighted or fluid-attenuated inversion recovery sequence, elevated blood very-long-chain fatty acid, and mutation in ABCD1 were found. He was diagnosed with childhood cerebral ALD. Although umbilical cord blood transplantation was successfully performed 4 months after the diagnosis, the patient died at 13 years of age. In this case, the diagnosis and treatment were delayed because the initial clinical symptoms were overlooked owing to a history of developmental disorders.

     ALD should be considered in a boy patients with vision loss, gait disturbance, and progressive intellectual disability. MRI is useful for establishing the early diagnosis and therapy of ALD.

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  • Minori Tanabe, Shinji Ohkubo, Sachiko Udagawa, Daisuke Takemoto, Kazuh ...
    2020 Volume 37 Issue 2 Pages 171-175
    Published: June 25, 2020
    Released: July 08, 2020
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     A 36-year-old woman complained of deformation of her right pupil to a“teardrop shape.”She was diagnosed with anisocoria (R>L) at an eye clinic and referred to our hospital. She presented with right mydriasis and anisocoria in the light room. The right pupil revealed hypersensitivity to 0.125% pilocarpine, and she was diagnosed with Adie syndrome in the right eye. During the follow-up, both eyes showed hypersensitivity to 0.125% pilocarpine, and she was diagnosed with bilateral Adie syndrome. The tadpole-shaped pupil was not observed at the first visit. The diagnosis of tadpole pupil was confirmed with self-acquired photographs from a smartphone, which were useful in diagnosing episodic deformation of the pupil. The tadpole-shaped pupil might have occurred after the early onset of Adie syndrome and before the pupillary sphincter completely palsied.

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Trend & Development
  • Hiroshi Ohguro, Fumihito Hikage
    2020 Volume 37 Issue 2 Pages 176-180
    Published: June 25, 2020
    Released: July 08, 2020
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     Cancer-associated retinopathy (CAR) is recognized as retinitis pigmentosa-like retinal degeneration caused by anti-retinal autoimmunity, in some patients with cancer. Our group has been studying the molecular mechanisms of CAR and has found that the retina-specific calcium-binding protein recoverin plays a central role in the pathogenesis.

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  • Motohisa Yamamoto, Hirotoshi Tanaka
    2020 Volume 37 Issue 2 Pages 181-187
    Published: June 25, 2020
    Released: July 08, 2020
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     Immunoglobulin (Ig) G4-related disease is a chronic inflammatory disorder that can cause irreversible dysfunction in many organs. The eyes, lacrimal glands, extraocular muscles, and optic nerves can be affected. Diagnosis is based on any of the comprehensive diagnostic criteria, criteria for Mikulicz’s disease, and criteria for IgG4-related ophthalmic disease. It is especially important to exclude malignant lymphoma in cases of head and neck lesions. We must evaluate the gene rearrangement of Ig heavy chains. At the time of diagnosis, we must also perform systemic screening of other organ involvements and malignancies. Regarding the pathogenesis, type 2 helper T (Th2) cell inflammation acts on follicular helper T cells to form hyperplastic germinal centers and differentiate into IgG4-bearing plasma cells. Meanwhile, fibroblast proliferation is induced by periostin via interleukin-13, which is produced by Th2 cells. Transforming growth factor-β produced by regulatory T cells and CD4+cytotoxic T lymphocytes also induces fibrosis. In the future, omics analyses are expected to cause a shift from the current glucocorticoid treatment to molecular-targeted treatments for disease control and prevention of relapse.

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Introductory Series 116
Contribution
  • Jonathan C. Horton, Daniel L. Adams, John R. Economides
    2020 Volume 37 Issue 2 Pages 196-202
    Published: June 25, 2020
    Released: July 08, 2020
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     Patient with exotropia frequently alternate fixation, looking at something with one eye and then switching their attention to acquire a new target with the other eye. Which eye informs the brain about the location of the new target? To address this issue, we presented targets dichoptically to 16 exotropes that were visible to the fixating eye, the deviated eye, or to both eyes. We then compared the subjectsʼ choice of eye for target acquisition with the organization of their suppression scotomas. There was a correspondence between suppression scotoma maps and the eye used to acquire peripheral targets. In other words, a target perceived via an eye was also fixated by it. These studies reveal how patients with alternating strabismus, despite eye misalignment, manage to localize and fixate efficiently visual targets in their environment.

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  • [in Japanese]
    2020 Volume 37 Issue 2 Pages 203-206
    Published: June 25, 2020
    Released: July 08, 2020
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Neuro-Ophthalmology Series with Sourcebooks
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Asian Section
  • Kazuko Uchida, Motohiro Kiyosawa, Masato Wakakura
    2020 Volume 37 Issue 2 Pages 237-243
    Published: June 25, 2020
    Released: July 08, 2020
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    Purpose: To provide a novel non-invasive treatment for blepharospasm, we developed an ultra-thin adhesive plaster that can improve narrowing of lid fissure that causes blepharoptosis by acting as a type of geste antagoniste/sensory trick known as a forcible trick. The tape was also developed to cover skin irregularities, but here we only report its outcome for quality of life in blepharospasm patients.

    Patients and methods: Subjects consisted of 131 patients being treated for blepharospasm. Tape was attached to the forehead, upper parts of the brows, and other facial areas. Patient satisfaction with appearance was surveyed before, immediately after, and 3 weeks after the treatment using a visual analog scale as well as before and 3 weeks after the treatment using the WHO QOL-26.

    Results: Survey results 3 weeks post-treatment were obtained from 69 out of 131 subjects. The mean visual analog scale score decreased from 78.6 at baseline to 22.3 immediately after treatment and to 48.8 3 weeks post-treatment. There was a significant increase in the mean WHO QOL-26 scores of the overall and physical domains. In addition, photophobia and patient satisfaction with their physical appearance improved.

    Conclusion: The ultra-thin adhesive tape prolongs the forcible trick effect to serve as a non-invasive treatment option for blepharospasm. The tape can be applied directly by the patient. It can also be combined with invasive treatments such as botulinum toxin type A injections and oral medications. We proposed the tape as a new symptomatic treatment for blepharospasm.

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