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PURPOSE: To investigate the association between optical coherence tomography (OCT) parameters and long-term visual recovery following optic chiasm decompression surgery. DESIGN: Prospective cohort study. METHODS: Consecutive patients who underwent pituitary or parasellar tumor resection between January 2009 to December 2018 were recruited in a single-center, 2-year prospective, longitudinal cohort study. Best-corrected visual acuity, visual fields, and OCT retinal nerve fiber layer (RNFL) thickness, macular thickness and volume were assessed preoperatively, and at 6 weeks, 6 months, and 2 years postoperatively. Long-term visual field recovery and maintenance were defined as a mean deviation of >-3 at 24 months, and visual acuity recovery and maintenance were defined as a logarithm of minimal angle of resolution (logMAR) of 0 (Snellen 20/20) or better at 24 months. RESULTS: A total of 239 patients (129 men, 110 women; mean ± SD age: 52 ± 16 years) were included. Multiple logistic regression analysis demonstrated that increased inferior RNFL thickness (per 10 μm) was associated with higher odds of long-term visual field recovery and maintenance (odds ratio [OR]: 1.26; 95% confidence interval [CI]: 1.12-1.41; Q < 0.001), and greater superior RNFL thickness (per 10 μm) was associated with higher odds of visual acuity recovery and maintenance (OR: 1.13; 95% CI: 1.03-1.27; Q = 0.031). A multivariable risk prediction model developed for long-term visual field recovery and maintenance that incorporated age, preoperative visual function, and RNFL thickness demonstrated C-statistics of 0.83 (95% CI: 0.72-0.94). CONCLUSION: Preoperative RNFL thickness was associated with long-term visual recovery and maintenance following chiasmal decompression. The multivariable risk prediction model developed in the present study may assist with preoperative patient counseling and prognosis.

Purpose To assess the clinical utility of the American College of Rheumatology criteria for the diagnosis of giant cell arteritis (GCA) in patients with positive and negative temporal artery biopsies. Design Retrospective case series of all patients undergoing temporal artery biopsy. Methods Retrospective chart review of all patients seen in the Neuro-ophthalmology Service of the Wills Eye Institute undergoing biopsy. One hundred twelve patients were identified between October 2001 and May 2006. Charts were reviewed for American College of Rheumatology criteria, biopsy results, and progression of visual loss after diagnosis. Results Nine of 35 patients (25.7%) with positive biopsies would not have been diagnosed with GCA using American College of Rheumatology criteria alone. An additional 16 patients (45.7%) met only 2 criteria and required the positive biopsy to establish the American College of Rheumatology diagnosis of GCA. Eleven of 39 patients (28.2%) with negative biopsies met the criteria and would have been diagnosed with GCA. Diagnostic agreement between the American College of Rheumatology criteria without biopsy results and biopsy results alone was 51.4%; with the addition of biopsy results to the criteria, this increased to 73.0%. Conclusions The current American College of Rheumatology criteria should not be used to diagnose GCA and all patients suspected of having GCA should undergo a temporal artery biopsy.

Purpose To report three cases of nonarteritic anterior ischemic optic neuropathy (NAION) that occurred in patients with sleep apnea syndrome (SAS) while being treated with continuous positive airway pressure (CPAP). Design A prospective, noncomparative case series. Methods Prospective review of 108 patients with NAION diagnosed between 2002 and 2003 to identify any patients who developed NAION while being treated for SAS with CPAP. Results One patient had bilateral sequential NAION and two patients had unilateral NAION despite treatment with CPAP for SAS. All the patients were being treated with CPAP for a period ranging from 4 months to 6 years before the onset of NAION. Conclusions In this series, CPAP did not prevent the development of NAION in SAS patients. Larger observational studies are needed to study the benefit of CPAP in preventing NAION in patients with SAS.

PURPOSE: To describe a patient with congenital nystagmus that decreased in one eye after the development of a cavernous sinus meningioma. DESIGN: Interventional case report. METHODS: A 45-year-old man with congenital nystagmus, albinism, and high myopia presented with right upper eyelid ptosis that was corrected surgically on three occasions during a 3-year period. An episode of syncope was followed by diagnostic evaluation that disclosed a cavernous sinus meningioma. He commented that his right eye nystagmus progressively decreased. He was found to have ophthalmoplegia on the right eye due to nerve compression by the meningioma. RESULTS: A magnetic resonance imaging scan disclosed a cavernous sinus meningioma extending into the parachiasmal and clival areas. A biopsy specimen of the lesion demonstrated a meningioma. CONCLUSIONS: Any patient with acquired ptosis should have a complete ophthalmic examination, including evaluation of extraocular motility.

Purpose To report a case of isolated optic nerve lymphoma diagnosed by optic nerve biopsy. Design Case report. Methods A 66-year-old woman was referred to the Neuro-Ophthalmology Service because of a decrease in visual acuity and right optic disk edema. Results A magnetic resonance image of the brain showed only enhancement of the optic nerve. An examination that included ANA, c-ANCA, p-ANCA, Lyme titers, FTA-ABS, ACE level, chest x-ray, and lumbar puncture was negative. Because of rapid progression on clinical examination and serial imaging, an optic nerve biopsy was performed, which showed B-cell lymphoma. Conclusion Optic nerve lymphoma can be confused with a variety of inflammatory and neoplastic infiltrations of the optic nerve on clinical and radiographic examinations. Optic nerve biopsy can be valuable in diagnosing isolated optic nerve lymphoma if other diagnostic tests are unrevealing, but the procedure carries considerable risk of loss of visual acuity and should be recommended judiciously.

Purpose Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder. Methods We examined three patients who initially complained of a nonspecific, insidious visual disturbance. Results The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease. Conclusion At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.

PURPOSE: To report a case of metastatic synovial sarcoma to the parasellar area and orbit. DESIGN: Interventional case report. METHODS: A 43-year-old woman with a history of a right forearm synovial sarcoma treated with excision, radiation, and chemotherapy 16 months previously presented with blurred vision of the right eye, pain with eye movement, epistaxis, and a right superior division cranial nerve III palsy. Magnetic resonance imaging showed a right parasellar and right orbital apex mass. RESULTS: Pathologic examination of the parasellar biopsy revealed metastatic synovial sarcoma. At 6-month follow-up, the neuro-ophthalmic examination was unchanged, and repeat magnetic resonance imaging showed no recurrence of the tumor. CONCLUSIONS: Metastatic synovial sarcoma should be added to the differential diagnosis of parasellar and orbital tumors.

Purpose To describe a patient whose initial sign of giant cell arteritis was a branch retinal artery occlusion. Methods We examined a 77-year-old woman who developed arteritic anterior ischemie optic neuropathy three weeks after an isolated nonembolic branch retinal artery occlusion occurred in the same eye. Results The diagnosis of giant cell arteritis was confirmed by temporal artery biopsy after the patient was treated with intravenous corticosteroids. Conclusions Although a rare cause of branch retinal vascular occlusion, giant cell arteritis should be considered in the differential diagnosis of a nonembolic branch retinal artery occlusion in elderly patients.

PURPOSE To assess visual function more than 10 years after an episode of optic neuritis in patients enrolled in the Optic Neuritis Treatment Trial. DESIGN Longitudinal follow-up of a randomized clinical trial. METHODS Vision testing included measures of visual acuity, contrast sensitivity, and visual field. Quality of life was assessed with the National Eye Institute Visual Function Questionnaire. RESULTS Examinations were completed on 319 patients. In most patients, visual function test results in the eyes that experienced optic neuritis at study entry ("affected eyes") were normal or only slightly abnormal after 9.9 to 13.7 years. Visual acuity in the affected eyes was >or=20/20 in 74%, 20/25 to 20/40 in 18%

PURPOSE: To determine if tobacco or alcohol consumption is associated with vision loss among sibships harboring pathogenic mitochondrial mutations associated with Leber hereditary optic neuropathy. METHODS: Retrospective case-control study with questionnaires obtained from both affected and unaffected siblings from 80 sibships with Leber hereditary optic neuropathy. Sibships harbored molecularly confirmed mitochondrial DNA mutations at nucleotide positions 11778 (63), 14484 (10), and 3460 (7). Exposure in affected individuals was calculated based on reported consumption before vision loss. RESULTS: For male probands (67 sibships), the recurrence risk within a sibship was 10.3% (eight of 78) for males and 3.1% (three of 98) for females. For female probands (13 sibships), the recurrence risk within a sibship was 17.6% (three of 17) for males and 0% (zero of 22) for females. Greater risk of vision loss was associated with male sex (odds ratio [OR] = 6.63; 95% confidence interval [CI] = 2.96 to 14.84; P = .00001) and harboring a 3460 or 14484 in comparison with the 11778 mutation (OR = 2.071; 95% CI=1.19 to 3.58; P = .0095). No significant association of maximal intensity of smoking or cumulative smoking, whether light or heavy, with vision loss was observed. Light (OR = 0.31; 95% CI=0.17 to 0.56; P = .0001) and heavy alcohol consumers (OR = 0.25; 95% CI=0.11 to 0.58; P = .0011) were less likely to be affected than individuals who did not consume alcohol after adjusting for age, sex, and mutation. In a categorical analysis of sibships with the 3460 or 14484 mutation, no relationship of vision loss with tobacco or alcohol consumption was observed. CONCLUSION: Unlike previous studies, the present study calculated exposure based on self-reported consumption of tobacco or alcohol before vision loss. No significant deleterious association between tobacco or alcohol consumption and vision loss among individuals harboring Leber hereditary optic neuropathy mutations was observed. Tobacco and alcohol do not appear to promote vision loss in Leber hereditary optic neuropathy.

Congenital homonymous hemianopsia is an uncommon asymptomatic visual field defect discovered typically in young adult life that is caused by a diverse group of insults to the retrochiasmal afferent visual system occurring prenatally, at birth, or during early childhood. We treated eight patients with congenital homonymous hemianopsia; seven with damage involving the optic radiations and one with an abnormality of the optic tract. We performed positron emission tomography using 18 F-fluoro-2-deoxyglucose on two patients with dense homonymous hemianopsias, lesions of the contralateral optic radiations, and largely intact occipital cortex. These studies showed minimal abnormalities in resting visual cortex glucose metabolism of the affected visual cortex.

Purpose/Methods A patient with osteitis deformans (Paget's disease) and hemifacial spasm underwent magnetic resonance tomographic angiography. Bone-modulating bisphosphonates and botulinum injection were administered to treat the hemifacial spasm. Results/Conclusions Computed tomography showed marked temporal bone overgrowth. Magnetic resonance tomographic angiography showed no vascular compression of the facial nerve root. The hemifacial spasm failed to resolve with intravenous pamidronate. Subsequent botulinum injection rendered the patient spasm free for 22 weeks. Further research on the use of bisphosphonates in the treatment of pagetoid hemifacial spasm is required.

Going from signs and symptoms to diagnosis can be the most difficult step in evaluating a patient, particularly in neuro-ophthalmology. And it is this step that has always been the strength of Clinical Decisions in Neuro-Ophthalmology. The structure of Clinical Decisions has always been problem-based, starting with disease manifestations and then leading the reader through the differential diagnosis. Decision trees are used throughout the book to guide the reader. The first edition of Clinical Decisions was one of the first texts to use this problem-based approach. Textbook authors in many other medical fields have since adopted this approach. Although the text is problembased, each of the disease entities within thedifferentialdiagnosis isdescribed and discussed. These discussionsareappropriatelysupplemented by diagrams of functional anatomy, clinical photographs, neuroimages, anddescriptionsofclinical tests.Sidebars called “Clinical Management Boxes” give practical advice for the management of commonly encountered neuro-ophthalmic diseases, includingopticneuritis, arteritic ischemic optic neuropathy, pseudotumor cerebri, and cranial neuropathies. Although the basic structure of the third edition is the same as the previous edition (published in 1992), there are some important changes. Treatment guidelines have been updated based on new data that have become available in the intervening years. The most obvious example is the treatment of optic neuritis, which has changed significantly based on the results of the Optic Neuritis Treatment Trial and the introduction of interferons. Diseases that we were only beginning to recognize in 1992, such as the paraneoplastic retinopathies, have also been added to the decision trees. The other major change is that the subject of functional problems has its own chapter in the new edition, “Neuro-Ophthalmic Disorders of Psychogenic Origin.” It isdifficult tocoverabroadsubject such as neuro-ophthalmology completely inatextbookthat ismeant to be a practical guide and an overview. Nevertheless, one topic that is lacking is a discussion of traumatic brain injuries, the common ophthalmic manifestations of these injuries, andtheir treatment.Althoughaspects of traumatothevisualsystemarecovered in several different sections throughout the book, the subject of traumatic brain injury is never discussed in a comprehensive fashion. Because we live in a world of motor vehicles, competitive sports, and occupational exposures, the topic of brain injury isbecoming increasingly relevant tomanyaspectsofmedicine, including ophthalmology. The target audience for Clinical Decisions is comprehensive ophthalmologists. This text would also be useful to the on-call ophthalmology resident or neuro-ophthalmology fellow because it teaches one to approach a patient in a systematic, logical fashion. Optometrists, neurologists, and neurosurgeons with an interest in neuro-ophthalmology will also appreciate the problem-based approach of Clinical Decisions.

A 40-year-old white woman had slowly progressive unilateral loss of visual acuity and increasing proptosis during an eight-year period. Ophthalmoscopy and fluorescein angiography revealed chronic disk edema and optociliary shunt vessels in the right eye. Polytomography showed an enlarged right optic canal. Cerebral arteriography demonstrated a dumbbell-shaped tumor blush in the right juxtasellar region and a diffuse tumor blush in the right orbit. Right frontal craniotomy and orbital exploration showed a cranio-orbital junction (spheno-orbital) meningioma that invaded the sclera and peripapillary choroid. Anomalous optociliary veins were demonstrated histologically at the optic disk.

Acute posterior multifocal placoid pigment epithelidpathy ( A P M P P E ) is becoming a frequently described entity." The manifes­ tations of the disease, however, appear to be protean and associated with anterior and pos­ terior uveitis. This has led some authors 6 to suggest the choriocapillaris as the primary site of inflammation. Gass and Ryan and Maumenee, however, favor the retinal pig­ ment epithelium as the primary site of in­ volvement.

A 24-year-old woman developed bilateral blindness after recovery from coma secondary to acute intermittent porphyria. Gradual return of vision in the right eye with a permanent unilateral visual field defect and optic atrophy followed. We believe the pathophysiologic mechanism was spasm of the vessels supplying the optic disk leading to ischemia and infarction of the optic nerve.

We used 18F-2-fluoro-2-deoxyglucose and positron emission tomography to evaluate the effect of visual deprivation on brain glucose metabolism. In experiment 1, we compared local cerebral metabolic rates for glucose in seven normal volunteers studied with eyes closed to 11 age- and sex-matched normal volunteers studied with eyes open. Whole brain metabolism was similar in the two groups, and region/whole brain analysis of metabolic data showed that metabolism in the calcarine posterior cortex was decreased by 14% (P less than .05) with eye closure. Glucose metabolism in other regions was not different between the two groups. In experiment 2, we compared glucose metabolism in six patients with severe bilateral optic neuropathies to 12 age- and sex-matched normal controls. Whole brain glucose metabolism was unchanged in the optic neuropathy group compared to controls. However, statistically significant reductions in glucose metabolism in the optic neuropathy group were found in anterior calcarine cortex (17%), posterior calcarine cortex (27%), peristriate cortex (27%), and lateral occipital cortex (15%). The metabolic effects of damage to the pregeniculate visual system went well beyond those of simple eye closure.

Of 196 patients with anterior ischemic optic neuropathy, 169 had the nonarteritic form and 27 had the arteritic type. Visual acuities were 20/40 or better in 83 of 184 eyes with nonarteritic anterior ischemic optic neuropathy but only eight of 45 eyes with the arteritic type. We found systemic disease associations for hypertension and diabetes mellitus only for patients with nonarteritic anterior ischemic optic neuropathy who were between 45 and 64 years of age. After a mean follow-up period of five years, 92 nonarteritic patients showed no changes in the first affected eye; there was eventual involvement of the second eye in 20 patients.

We performed visual contrast sensitivity testing with Regan's low-contrast letter charts on 30 patients with optic neuropathies and good Snellen visual acuity. Results of this testing were compared with color vision and the presence of an afferent pupillary defect. Regan's low-contrast letter charts were found to be 93% sensitive for detecting subtle optic neuropathies, with the apparent false-negative findings being readily explainable. Color vision testing was abnormal in only 49% of eyes with known optic neuropathies.

Three patients with multiple sclerosis (women 20, 24, and 30 years old) had demyelinative plaques that produced homonymous hemianopsia. These lesions were identified by computed tomography which showed the plaques as hypodense areas. All patients with homonymous hemianopsia should undergo computed tomography; if a hypodense or enhancing lesion is identified, the diagnosis of multiple sclerosis must be considered

To distinguish optic nerve conduction defects from macular disease in patients with otherwise unexplained loss of central vision we first determined the best visual acuity with correction at distance in unilateral defects. The normal eye was tested first and photostressed for ten seconds by looking at an ordinary penlight held 2 to 3 cm from the eye. The time required to read three letters on three Snellen test lines just larger than the best acuity was used as the end point. In 63 eyes with maculopathy the recovery time was prolonged. Prolonged recovery time was not observed in 20 patients who had optic nerve disease.