Andrew G. Lee

Visual hallucinations (Charles Bonnet syndrome) associated with neurosarcoidosis

The Charles Bonnet syndrome (CBS) refers to lucid and complex visual hallucinations in cognitively normal patients with acquired vision loss. It can be associated with any type of vision loss including that related to macular degeneration, corneal disease, diabetic retinopathy, and occipital infarct. Neurosarcoidosis, a multi-systemic inflammatory granulomatous disease affecting both the central and peripheral nervous systems, is rarely associated with CBS. We report a patient with biopsy-confirmed neurosarcoidosis who experienced visual hallucinations following the development of a right seventh-nerve palsy, right facial paresthesia, and bilateral progressive visual loss. This case highlights the importance of recognizing that the CBS can occur in visual loss of any etiology.

Homonymous Hemianopsia Due to Posterior Cortical Atrophy

ABSTRACT A 63-year-old woman presented to her ophthalmologist complaining of reading difficulties for two years. Ophthalmological examination revealed a homonymous hemianopsia. Brain magnetic resonance imaging (MRI) scan was interpreted as normal, but positron emission tomography (PET) showed areas of posterior brain hypometabolism. This case highlights the high diagnostic suspicion that ophthalmologists should have regarding posterior cortical atrophy (including the visual variant of Alzheimer disease) in patients complaining of reading difficulties in the setting of a normal ophthalmic examination.

Metastatic Papillary Thyroid Carcinoma Presenting as Abducens Palsy Complicated by Ocular Neuromyotonia

ABSTRACT Papillary thyroid carcinoma (PTC) is a type of well-differentiated thyroid cancer that accounts for the majority of thyroid malignancies. The prognosis of PTC is very good and distant metastases are rare, especially to the skull base. The authors report the case of a 47-year-old woman with biopsy-proven PTC treated with surgery and radiation therapy who presented with headache and diplopia after 5 years and was found to have clivus and cavernous sinus metastasis. Following radiation therapy for her skull base and cavernous sinus lesion, she subsequently developed sixth nerve ocular neuromyotonia. Possible causes and treatments are reviewed.

Neuro-ophthalmology as a career

This essay was written to discuss the reasoning behind the personal decisions made by 2 current neuro-ophthalmology fellows to pursue neuro-ophthalmology as a career. It is meant to enlighten the reader about what role neuro-ophthalmologists play in clinical practice, what makes neuro-ophthalmology unique to all other sub-specialties, and how this contributes to making neuro-ophthalmology not only one of the most medically interesting, yet rewarding sub-specialties in ophthalmology.

Systemic corticosteroids in nonarteritic ischemic optic neuropathy

Nonarteritic ischemic optic neuropathy (NAION) is one of the most prevalent optic nerve disorders seen in ophthalmic practice. The role of corticosteroid therapy in NAION remains a highly controversial area of debate in ophthalmology. This brief review will provide an overview of the current clinical evidence on this topic as well as some comment on the medical debate.

Rhino-orbital Mucormycosis Treated Successfully with Posaconazole without Exenteration

Abstract Mucormycosis is a rare and often fatal opportunistic angioinvasive infection seen mostly in immunocompromised patients, such as those with diabetes mellitus, cancer, or renal failure. Ophthalmic manifestations of orbital mucormycosis include ocular pain, periocular oedema, visual loss, ophthalmoplegia, proptosis, and ptosis. Although therapy for orbital mucormycosis consists of maximally tolerated doses of antifungal agents (e.g., amphotericin B) and extensive surgical debridement, treatment remains ineffective in up to 20% of cases. We describe two patients with rhino-orbitalmucormycosis who were successfully treated with posaconazole in conjunction with intravenous (IV) amphotericin B and sinus surgical debridement. These cases highlight several unusual early manifestations of orbital mucormycosis, including disc oedema and amaurosis fugax, as well as the applicability of a new extended-spectrum antifungal agent in management of orbital zygomycosis.

Geriatric Cross-Cutting Issues in Ophthalmology

A gray tsunami of aging baby boomers has entered into the health care system (Bierman et al., Improving the Health Care of Older Americans. A Report of the AHRQ Task Force on Aging, 2001).

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.

Bilateral Adie’s Tonic Pupil and Hyperopic Shift in Idiopathic Sclerochoroidal Calcification

Abstract Sclerochoroidal calcification is a rare but recognised ophthalmic manifestation seen mostly in elderly Caucasian individuals. The lesions, often bilateral, appear as yellow-white irregular subretinal lesions usually found along the mid-peripheral fundus. Though typically asymptomatic, sclerochoroidal calcification has rarely been associated with parafoveal involvement, choroidal neovascularisation, and serous detachment of the calcifications. Visual involvement is typically minimal, and neovascularisation is often visually insignificant. We present a rare case of sclerochoroidal calcification in a 64-year-old Caucasian female who presented with painless progressive bilateral vision loss and a hyperoptic shift with subsequent development of bilateral sequential Adie’s tonic pupil. To the best of our knowledge, this is the first such report in the English language literature.

Autoimmune-related retinopathy and optic neuropathy accompanied by anti-GAD antibodies

Abstract Autoimmune-related retinopathy and optic neuropathy is characterized by visual loss in the presence of antibodies against retina or optic nerve antigens in the absence of neoplasia. We report a case with progressive central visual loss accompanied by latent autoimmune diabetes in an adult. Visual fields, multimodal imaging and electrophysiological testing showed characteristic changes without evidence of neoplasia on positron emission tomography. Immunologic testing revealed abnormal reactivity against a 20 kDa optic nerve antigen and 40 kDa and 62 kDa retina antigens along with antibodies against anti-glutamic acid decarboxylase, a constellation which we believe to be unique.