Stacy V. Smith

Evaluation and management of the swollen optic disk in cryptococcal meningitis

Cryptococcal meningitis is the most common and severe form of cryptococcal infection. In addition to infiltrative and inflammatory mechanisms, intracranial hypertension commonly complicates cryptococcal meningitis and may cause significant visual and neurological morbidity and mortality. The mainstays of treatment for cryptococcal meningitis include standard antifungal therapy, management of intracranial hypertension, and treatment of underlying immunosuppressive conditions. Early and aggressive management of intracranial hypertension in accordance with established guidelines reduces the risk of long-term visual and neurological complications and death. Traditional recommendations for treating elevated intracranial pressure in idiopathic intracranial hypertension including acetazolamide, weight loss, and avoiding serial lumbar punctures-are not helpful in cryptococcal meningitis and may be harmful.

Update on Ocular Myasthenia Gravis

Ocular myasthenia gravis (OMG) is a localized form of myasthenia gravis in which autoantibodies directed against acetylcholine receptors block or destroy these receptors at the postsynaptic neuromuscular junction. The hallmark of OMG is a history of painless weakness or fatigability of the extraocular muscles and ptosis with normal pupillary function and visual acuity. Clinical, laboratory, electrophysiologic, and pharmacologic tests are available for diagnosis. Treatment can begin with symptom management; there is no cure. Prognosis is improved by use of immunomodulators. Despite advances in treatment, research is needed, especially in the areas of surgical intervention and medical therapy based on risk stratification.

Metastatic Melanoma of the Optic Nerve Sheath

ABSTRACT A 23-year-old man with a history of metastatic melanoma developed painful vision loss to counting fingers with enhancement of optic nerve on contrast-enhanced magnetic resonance imaging (MRI) and received a diagnosis of optic neuritis from an outside hospital. Despite empiric corticosteroid therapy, the patient worsened and developed secondary central retinal vein occlusion with further deterioration of vision. Repeat MRI demonstrated optic nerve sheath (ONS) involvement suggestive of optic perineuritis (OPN) and an ONS biopsy confirmed a rare case of isolated metastatic melanoma. Our case highlights the clinical and radiographic features that can mimic OPN and delay diagnosis and treatment.

Neuro-Ophthalmology Cases for the Neurologist

Neurologists should be aware of specific urgent and emergent neuro-ophthalmic conditions, including giant cell arteritis, arterial dissection, intracranial aneurysm, pituitary apoplexy, and invasive sino-orbital fungal infection (eg, mucormycosis). Early recognition and treatment can greatly impact patient morbidity and mortality, including the preservation of vision and life. Neurologists should be cognizant of the key and differentiating clinical and radiographic features for these presentations.

Relapsing Painful Ophthalmoplegic Neuropathy: No longer a “Migraine,” but Still a Headache

Purpose of ReviewRecurrent painful ophthalmoplegic neuropathy (RPON), formerly known as ophthalmoplegic migraine, is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. This review discusses the clinical presentation, current understanding of the pathophysiology, key differential diagnoses, and evaluation and treatment of RPON.Recent FindingsThe literature is limited due to the rarity of the disorder. Recent case reports and series continue to suggest the age of first attack is most often during childhood or adolescence as well as a female predominance. Multiple recent case reports and series demonstrate focal enhancement of the affected cranial nerve, as the nerve root exits the brainstem. This finding contributed to the current classification of the disorder as a neuropathy, with the present understanding that it is due to a relapsing-remitting inflammatory or demyelinating process. The link to migraine remains a cause of disagreement in the literature.SummaryRPON is a complex disorder with features of inflammatory neuropathy and an unclear association with migraine. Regardless, the overall prognosis is good for individual episodes, but permanent nerve damage may accumulate with repeated attacks. A better understanding of the pathogenesis is needed to clarify whether it truly represents a single disorder and to guide its treatment. Until that time, a combined approach with acute and preventive therapies can mitigate acute symptoms as well as attempt to limit recurrence of this disabling syndrome.

Idiopathic Orbital Inflammation Associated with Relapsing Polychondritis

The authors describe a patient with recurrent idiopathic orbital inflammatory disease as an unusual presentation of relapsing polychondritis. There are very few reported cases in the literature of relapsing polychondritis associated with idiopathic orbital inflammation. Clinicians should be aware of the orbital and ophthalmic presentations of relapsing polychondritis.

Management of ischemic optic neuropathies

ABSTRACT Introduction: Ischemic optic neuropathy (ION) is the most common acute unilateral optic neuropathy in older patients. Areas covered: A complete review of the literature was performed for ischemic optic neuropathy, including nonarteritic anterior ION, arteritic anterior ION, and posterior ION. The most recent information concerning epidemiology, pathophysiology, and management was included in the review. In particular, the management of each form of ION is discussed, including in special patient populations such as post-surgical patients and hemodialysis patients. Expert commentary: Differentiating arteritic and nonarteritic forms of ION is crucial for appropriate patient management, as those with arteritis should be started on steroid therapy as soon as possible. Nonarteritic ION patients benefit from control of cerebrovascular risk factors, and may benefit systemically from low-dose aspirin therapy.

Cost-effectiveness of systematic population based screening for BRCA1, BRCA2, RAD51C, RAD51D and BRIP1 gene mutations in unselected general population women

Abstract from 16th Biennial Meeting of the International Gynecologic Cancer Society Lisbon, Portugal October 28-31, 2016

Visualization of a Hematoma of the Cloquet Canal

Awhiteman in his mid to late forties with a history of Huntington disease presented with acute painful left eye vision loss. He was diagnosed with acute angle-closure glaucoma secondary to iris neovascularization and underwent diode cyclophotocoagulation after combination topical and systemic medical treatment failed. Workup included ultrasonography examination owing to unsuccessful left fundus visualization. This revealed a hyperechoic lesion within the vitreous, consistent with a Cloquet canal hematoma (Figure, A). Magnetic resonance imaging of the orbit also confirmed the intravitreal hyperintense lesion located anterior to the optic nerve (Figure, B).

Retrobulbar hemorrhage and prasugrel

Retrobulbar hemorrhage (RBH) is the accumulation of blood in the orbit behind the globe. Although some hemorrhages are minor and do not result in significant mass effect, an RBH is an ophthalmologic emergency requiring immediate surgical intervention. An RBH manifests as acute ipsilateral orbital pain and proptosis and can be variably associated with loss of vision, ophthalmoplegia, subconjunctival hemorrhage, and increased intraocular and intraorbital pressure. Most cases of RBH are traumatic or postsurgical, but some occur spontaneously in patients with bleeding diatheses. Another etiology for RBH is retrobulbar anesthesia injection. The use of antiplatelet therapy has not been considered to be a significant risk factor for RBH; yet newer, more potent antiplatelet agents may carry significantly more risk for RBH. We report a case of RBH causing blindness in a patient taking aspirin and prasugrel. To our knowledge, this is the first such case reported in the English-language ophthalmic literature.