Homonymous hemianopsia as the presenting sign of migrainous infarctionMigrainous infarction is a rare complication of migraine headaches that accounts for 0.2%−0.5% of ischemic strokes.1,2 This condition frequently occurs in the posterior cerebral circulation and typically affects a single vascular territory.1,2 The most common symptom preceding an acute migrainous infarction is a visual aura (82.3%) that resolves within minutes to hours.1 Visual field defects like partial or complete homonymous hemianopsia (HH) after a migrainous infarction have been reported, but the prevalence is unknown.
Bilateral disc edema in hypertensive emergencyBilateral optic disc edema can be a result of several etiologies and deserves a thorough work-up. Neuroimaging and laboratory studies should be performed to rule out neoplastic, vascular, infectious, ischemic, or inflammatory etiologies, and a lumbar puncture (LP) should be performed to measure intracranial pressure (ICP) if necessary after performing the appropriate imaging studies. Patients with papilledema should be evaluated for alternative causes of increased ICP. We present the case of a patient who presented with bilateral optic disc edema and was found to have increased ICP.
Nine syndrome in a patient with systemic lupus erythematosusSystemic lupus erythematosus (SLE) is an autoimmune disease that can affect many different organ systems and present in a variety of ways. The central nervous system (CNS) is frequently affected, resulting in a wide range of manifestations. Patients may present to an ophthalmologist with ocular symptoms before a diagnosis is established; thus, early recognition is important to avoid delays in diagnosis and treatment. We report the first case of a patient with lupus who developed nine syndrome due to pontine ischemic stroke precipitated by CNS vasculitis.
Orbital metastasis mimicking internuclear ophthalmoplegia: A case report and reviewInternuclear ophthalmoplegia (INO) is caused by a lesion in the medial longitudinal fasciculus (MLF). It is characterized clinically by an ipsilateral adduction deficit (e.g., adduction deficit or lag) and a dissociated, horizontal, abducting nystagmus of the contralateral eye on attempted horizontal gaze. A similar clinical presentation not attributable to a lesion in the MLF, called a pseudo-INO, is usually caused by myasthenia gravis. We present a rare case of an apparent INO resulting from rectal adenocarcinoma metastasis to the orbit.
A rare association: Cushing disease and central serous chorioretinopathyCentral serous chorioretinopathy (CSCR) consists of subretinal accumulation of serous fluid leading to a circumscribed detachment of the neural retina.1,2 The fluid accumulates in the macular region, resulting in decreased visual acuity, blurry vision, color desaturation, metamorphopsia, and impaired dark adaptation.1 Although the pathogenesis of CSCR remains unknown and most cases are idiopathic, excess corticosteroid levels have been reported in association with CSCR.3 This is rare in the setting of Cushing disease.
Perioperative nonarteritic anterior ischemic optic neuropathy in Jehovah’s WitnessesA 62-year-old, female, African American, Jehovah’s Witness experienced bilateral and simultaneous vision loss 2 days after hip arthroplasty. Her 5-hour supine hip replacement surgery resulted in 1 L of blood loss; given her religious status, she refused replacement blood products. Her hemoglobin was 6.3 g/dL preoperatively and 4.7 g/dL postoperatively. The etiology of her preoperative anemia was not known. Her lowest postsurgical blood pressure was 75/45 mm Hg. Her hypotensive medications were discontinued, she was kept in Trendelenburg position, and was treated with intravenous (IV) iron and darbepoetin alfa.
Fulminant idiopathic intracranial hypertension managed with oral acetazolamideIdiopathic intracranial hypertension (IIH), also known as pseudotumour cerebri, is a syndrome of increased intracranial hypertension of unknown cause found predominantly in women of childbearing age and associated with an increased body mass index or a history of recent weight gain.1 The modified Dandy criteria for diagnosing IIH include (i) an imaging study confirming the absence of radiographic hydrocephalus or mass lesion, (ii) elevated cerebrospinal fluid (CSF) opening pressure (OP) upon lumbar puncture (LP) with normal CSF contents, and (iii) an intact neurologic examination with the exception of visual disturbances, sixth cranial nerve palsy, and papilledema.
Bilateral posterior ischemic optic neuropathy associated with the use of Sildenafil for pulmonary hypertensionSildenafil citrate is a selective phosphodiesterase (PDE)-5 inhibitor (PDE5i) and a partial PDE-6 inhibitor that enhances nitric oxide and cyclic guanosine monophosphate (cGMP), leading to smooth muscle relaxation. It has been used as an erectile dysfunction agent in men but also improves pulmonary hemodynamics and exercise capacity in symptomatic pulmonary arterial hypertension.1 PDE5i has been reported to cause transient changes in vision, colour perception (e.g., blue or blue-green tinges), brightness perception, and electroretinography.
Neuro-ophthalmic presentations of clival plasmacytomaNeurologically isolated cranial mononeuropathy (e.g., sixth nerve palsy) leading to diplopia is a common presentation to ophthalmologists. Although rare, clival lesions can cause isolated unilateral or bilateral abducens palsies with or without papilledema. Intracranial plasmacytomas (ICPC) are rare tumours that constitute less than 1% of intracranial neoplasms. They may present as a solitary plasmacytoma or may be part of a systemic malignant plasmacytosis, as in multiple myeloma (MM).
Bilateral optic atrophy in endemic typhusMurine typhus is an infectious disease caused by Rickettsia typhus. The disease is transmitted to humans by rat fleas through direct inoculation.1 Southeast Texas has one of the highest incidence rates of endemic typhus in the United States.2–5 Ophthalmic manifestations of murine typhus include vitritis, retinal lesions, and retinal vascular leakage.6–10 Optic neuropathy in the setting of murine typhus has been rarely described and only in the setting of disc edema or optic neuritis.8–11 We present a rare case of bilateral optic atrophy as the presenting ophthalmic sign of R.
Temporal artery enhancement on cranial magnetic resonance imagingA 68-year-old white female presented to her outside ophthalmologist with acute vision loss in the left eye (OS) described as a “green line across the top half of her vision” and dull pain OS. She then experienced progressive worsening of vision OS, pain with chewing, temple pain, and scalp tenderness. The visual acuity was 20/20 in the right eye (OD) and counting fingers at 1/2-foot distance OS. There was a left relative afferent pupillary defect and optic disc edema OS. The right optic nerve was normal, but the cup-to-disc ratio was 0.4 OD.
Maintenance of pupillary response in a Glaucoma patient with no light perception due to persistence of melanopsin ganglion cellsRecent publications have demonstrated that a subpopulation of retinal ganglion cells (RGCs) that use melanopsin as a visual pigment termed intrinsically photosensitive RGCs or melanopsin RGC (mRGCs) may be more resistant to damage ocular hypertension, glaucoma and Leber hereditary optic neuropathy (LHON) as compared with conventional RGCs.1–5 Although the mRGCs comprise only approximately 0.2% of all ganglion cells, they have been shown to be important in the pupillary response. In this article, we report a patient with no light perception vision caused by secondary glaucoma with a partially retained pupillary response.
Diffusion-weighted imaging in posterior ischemic optic neuropathyDiffusion-weighted imaging (DWI) is a magnetic resonance imaging (MRI) sequence that provides image contrast dependent on the molecular motion of water. Acute ischemia in the central nervous system (CNS) results in disruption of normal cellular metabolism with depletion of ATP causing failure of Na+/K+ ATPase ionic pumps with loss of ionic gradients across cellular membranes. This causes cytotoxic edema with a net shift of water from the extracellular to the intracellular space and changes in the relative volume of these compartments, as well as alterations in their microenvironments.
Paraneoplastic optic neuropathy and retinopathy associated with colon adenocarcinomaA 62-year-old Hispanic female with no prior history of malignancy presented with 3-year history of painless, simultaneous, and progressive bilateral constriction of her visual fields. The patient was otherwise healthy and denied hematochezia, melena, or changes in bowel habit. During this 3-year period, the patient was evaluated by multiple ophthalmologists, but no clear or formal diagnosis was made. Initial examination 3 years before presentation to us showed optic disc edema OU and mild generalized constriction of visual fields OU.
Atonic pupils secondary to presumed systemic vasopressor-related ischemiaA 49-year-old male presented with blurred vision and bilaterally dilated pupils after suffering cardiac arrest following cardiac surgery. Medical history was significant for mitral valve regurgitation and idiopathic hypertrophic cardiomyopathy requiring mitral valve repair and septal myectomy.
Pallid disc edema and choroidal perfusion delay in posthemodialysis nonarteritic ischemic optic neuropathyNonarteritic anterior ischemic optic neuropathy (NAION) is a rare but recognized ophthalmic complication in patients with end-stage renal disease (ESRD) on chronic hemodialysis characterized by unilateral or bilateral acute visual loss.1 Anemia, concomitant vasculopathic risk factors (i.e., diabetes, atherosclerosis), autonomic dysfunction, the hypotensive effect of hemodialysis, and physiologically small cup-to-disc ratios are among the predisposing factors to the development of ischemic optic neuropathy (ION) in this patient population.
Radiographic evidence for denervation atrophy of extraocular muscles due to chronic intracavernous compressive third nerve palsyCase 1: A male in his 70s noted chronic progressive painless diplopia. His right pupil was larger and less reactive to light than his left as a teenager, but he was asymptomatic. Photographs revealed exotropia during his 30s. Ophthalmologic evaluation was suggestive of right third nerve palsy.
Intracranial complications of idiopathic intracranial hypertensionWe report a case of idiopathic intracranial hypertension (IIH) with cerebral venous sinus thrombosis (CVST) and subarachnoid hemorrhage that developed 6 months after initial presentation.
Visual hallucinations (Charles Bonnet syndrome) as the presenting sign of pituitary adenomaVisual hallucinations are a rare presenting sign of pituitary adenomas. We report a patient who presented with the Charles Bonnet syndrome (CBS) whose hallucinations resolved following transsphenoidal resection of a pituitary adenoma. To our knowledge, this is the first such case in the English language literature.