A 28-year-old caucasian woman presented to the emergency room with a history of a pressure-type frontal headache during the previous week; there was no exacerbation when lying down nor with Valsalva maneuvers and no accompanying features, namely, visual symptoms. Medical history was unremarkable except for venous thrombosis on her left leg 5 years before. She was not taking any drugs other than oral contraceptives. She had a son who was diagnosed with chickenpox two weeks before. She was febrile (temperature of 38°C), and there was a rash suggestive of chickenpox. Uncorrected visual acuity was 20/20 in both eyes, and the optic discs were swollen and hyperemic, with ophthalmological and neurological examinations otherwise normal. Two days later, she complained of ocular pain on eye movement and bilateral blurred vision. By that time, examination revealed reduced visual acuity of 20/32 in her right eye (OD) and 20/60 in her left eye (OS) and relative afferent pupillary defect on her left eye. She reported a difference in brightness of the red color, with less saturation in the left eye.\nRoutine blood testing and immunological workup (ANA, ANCA, anticardiolipin antibodies, and complement levels) were normal. HIV serology was negative as well as VDRL, Toxoplasma, Bartonella, and Borrelia antibodies.\nCerebral computed tomography (CT) scan and magnetic resonance imaging (MRI) were normal.\nCerebrospinal fluid (CSF) examination was unremarkable: clear fluid with a normal opening pressure of 130–140 mm H2O with no block when pressing the jugular vein, normal protein and glucose levels, less than 5 leukocytes, and no oligoclonal bands. Antibodies for the following microorganisms were negative in the CSF: Cytomegalovirus, Epstein Barr virus, Parvovirus, Herpes simplex virus, Varicella zoster virus, Mycoplasma pneumoniae, Borrelia burgdorferi, and Leptospira species. VDRL was also negative in the CSF.\nGoldmann perimetry showed an enlarged blind spot and constriction of the nasal field, especially in her left eye (). Farnsworth Munsell 100 color vision test was abnormal in both eyes (in the red axis), with more pronounced defect in her left eye.\nShe was started on acyclovir 4 g/day per os, as is indicated to treat adult chickenpox.\nTwo days after the initial visual symptoms, visual acuity began to increase, and one week later, the optic disc edema had almost resolved (). Visual acuity continued to improve, and two months later, she had a visual acuity of 20/20 bilaterally. Retinal fluorescein angiography one month after presentation was normal. She has been free of further attacks of optic neuritis for three years after the admission.
A 41-year-old man complaining of left visual disturbance visited our department. He had a history of IVH associated with moyamoya disease three months ago. He was comatose at the initial presentation. Computed tomography (CT) at the first visiting hospital demonstrated IVH with hydrocephalus (). Cerebral angiography showed severe bilateral stenosis at the distal internal carotid artery and basal moyamoya vessels. It was compatible with moyamoya disease (). Emergent extraventricular drainage was performed. His mentality gradually improved to alert. Ophthalmologic examination at our hospital detected a vitreous hemorrhage in his left eye (), with right eye remaining normal (). In spite of three months conservative care, his visual acuity was not improved. Vitrectomy with epiretinal membrane removal was performed (). After operation his left visual acuity was recovered.