Ophthalmic presentation of giant cell arteritis in African-Americans.

PurposeTo determine the differences in the presentation of ophthalmic giant cell arteritis between African-Americans and Caucasians.MethodsThis was a multicenter retrospective case series comparing African-American patients with ophthalmic GCA to a previously published Caucasian cohort. Neuro-ophthalmic centers across the United States were contacted to provide data on African-American patients with biopsy-proven ophthalmic giant cell arteritis. The differences between African-American and Caucasian patients with respect to multiple variables, including age, sex, systemic and ophthalmic signs and symptoms, ocular ischemic lesions, and laboratory results were studied.ResultsThe Caucasian cohort was slightly older (mean=76.1 years) than the African-American cohort (mean=72.6 years, P=0.03), and there was no difference in sex distribution between the two cohorts. Headache, neck pain, and anemia were more frequent, while jaw claudication was less frequent in African-Americans (P<0.01, <0.001, 0.02, and 0.03 respectively). Acute vision loss was the most common presentation of giant cell arteritis in both groups, though it was less common in African-Americans (78 vs 98% of Caucasians, P<0.001). Eye pain was more common in African-Americans (28 vs 8% of Caucasians, P<0.01).ConclusionsThe presenting features of ophthalmic giant cell arteritis in African-Americans and Caucasians are not markedly different, although a few significant differences exist, including higher rates of headache, neck pain, anemia, and eye pain, and lower rates of jaw claudication and acute vision loss in African-Americans. Persons presenting with suspicious signs and symptoms should undergo evaluation for giant cell arteritis regardless of race.

The role of orbital ultrasonography in distinguishing papilledema from pseudopapilledema.

PURPOSE: To determine the sensitivity and specificity of orbital ultrasonography in distinguishing papilledema from pseudopapilledema in adult patients. METHODS: The records of all adult patients referred to the neuro-ophthalmology service who underwent orbital ultrasonography for the evaluation of suspected papilledema were reviewed. The details of history, ophthalmologic examination, and results of ancillary testing including orbital ultrasonography, MRI, and lumbar puncture were recorded. Results of orbital ultrasonography were correlated with the final diagnosis of papilledema or pseudopapilledema on the basis of the clinical impression of the neuro-ophthalmologist. Ultrasound was considered positive when the optic nerve sheath diameter was ≥3.3 mm along with a positive 30° test. RESULTS: The sensitivity of orbital ultrasonography for detection of papilledema was 90% (CI: 80.2-99.3%) and the specificity in detecting pseudopapilledema was 79% (CI: 67.7-90.7%). CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive, but less specific in differentiating papilledema from pseudopapilledema in adult patients, and can be useful in guiding further management of patients in whom the diagnosis is initially uncertain.

Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study.

OBJECTIVE: To determine whether weight gain is associated with recurrence in idiopathic intracranial hypertension (IIH). METHODS: Medical records of adult patients with IIH seen between 1993 and 2009 at 2 university hospitals were reviewed to identify those with and without recurrence. Patients with documented height and weight at presentation and at subsequent visits were studied. The Wilcoxon rank sum test was used to compare mean body mass index (BMI) and percent weight change between the groups of patients with recurrence and without recurrence. The signed-rank test was used for comparing BMI within groups at the various time points. RESULTS: Fifty women with IIH were included in the analyses: 26 had IIH recurrence and 24 did not. Patients with recurrence had greater BMI at the time of recurrence compared to BMI at diagnosis (p = 0.02, signed-rank test). They also demonstrated a greater degree of weight gain between initial resolution and recurrence (BMI change +2.0 kg/m(2) [-1.5 to 10.8]) compared to patients without recurrence (-0.75 kg/m(2) [-35 to 3.6], p = 0.0009, Wilcoxon rank sum test). Patients without recurrence demonstrated stable weights (0%[95% CI -9.6 to 10.1%]), while patients with recurrence demonstrated a 6% weight gain ([-3.5 to 40.2%], p = 0.005), with an average rate of BMI gain of 1.3 kg/m(2)/year vs -0.96 kg/m(2)/year in those without recurrence. CONCLUSION: Patients with IIH recurrence had significant increases in BMI compared to patients without recurrence in this cohort. Patients with resolved IIH should be advised that weight gain may be a risk factor for IIH recurrence.

Adult hypertropia: a guide to diagnostic evaluation based on review of 300 patients.

PURPOSE: To highlight the key clinical features of various aetiologies of adult hypertropia and to discuss the diagnostic approach towards evaluation of vertical double vision. METHODS: This is a retrospective cross-sectional study. A total of 300 consecutive patients with vertical diplopia were evaluated by a single neuro-ophthalmologist and strabismologist in a tertiary care setting from 2005-2008. The medical records of all patients with vertical diplopia coded with one of the following diagnoses; hypertropia, diplopia, thyroid eye disease, fourth nerve palsy, ocular myasthenia, congenital strabismus, and third nerve palsy were reviewed. The main outcome measures were determination of aetiologies of hypertropia. RESULTS: Fourth nerve palsy and thyroid eye disease were the most common causes of vertical diplopia in our series and comprised more than 50% of patients. The other causes of vertical diplopia were ocular surgery, orbital fracture, neurosurgery, childhood strabismus, skew deviation, third nerve palsy, myasthenia gravis, and decompensated hyperphorias. Ocular motility deficits were seen in 33% of the cohort of whom thyroid eye disease comprised the largest group. Orbital ultrasonography was sensitive in detecting thyroid orbitopathy. CONCLUSION: In the majority of patients, the aetiologies of hypertropias can be ascertained by history and careful ophthalmic examination alone. Fourth nerve palsy and thyroid eye disease were the most common causes of vertical diplopia in this series.

Microsurgical lab testing is a reliable method for assessing ophthalmology residents' surgical skills.

BACKGROUND: Formal assessment of clinical competencies is necessary to ensure that all residents are acquiring important skills and, in the United States, will soon become a requirement for residency programme accreditation by the Accreditation Council for Graduate Medical Education (ACGME). The Eye Surgical Skills Assessment Test (ESSAT), a laboratory-based surgical skills obstacle course, was developed in response to the need for improved tools for the assessment of surgical skills during residency. The ESSAT has previously been shown to have face and content validity, and in this study we sought to determine its inter-rater reliability and, to some extent, its construct validity. METHODS: Twenty-seven content experts (residency programme directors and faculty members involved with resident surgical training) watched videos of a junior resident and senior resident completing the three ESSAT stations (skin suturing, muscle recession, and phacoemulsification: wound construction & suturing technique) and completed assessment forms, both task-specific checklists and a global rating scale of performance. RESULTS: The ESSAT showed strong inter-rater reliability for determining whether a resident "passed" a threshold of competency at each station for both the checklists and global rating scale. In addition, for each station, the senior resident was consistently rated above a "passing" threshold using either assessment form, whereas the junior resident was more often rated below (94% vs 30% passing on completed forms). CONCLUSION: These results, along with the findings of our face and content validity analysis, support the reliability and validity of the ESSAT, and indicate that it could be a useful tool for improving the assessment of surgical skill during residency. The ESSAT is a tool that all residency programmes could implement as a part of their ophthalmic surgical curriculum and competency assessment, and may be useful to set a threshold of competence that all residents would need to achieve prior to entering the operating room.

Oculomotor synkinesis following a midbrain stroke.

A 33-year-old man developed a complete third nerve palsy in the setting of acute bacterial endocarditis. MRI revealed an ischemic stroke in the cerebral peduncle involving the third nerve fascicle. Subsequently, he was observed to have paradoxic elevation of the eyelid on adduction and downgaze. To the authors' knowledge, this is the first demonstration of oculomotor synkinesis after an acquired, ischemic CNS lesion.

Posterior ischemic optic neuropathy after uncomplicated cataract extraction.

PURPOSE: We report a case of posterior ischemic optic neuropathy after uncomplicated cataract extraction. DESIGN: Observational case report. METHODS: Retrospective description of the natural course of posterior ischemic optic neuropathy in one patient seen at an academic institution. RESULTS: The patient had sudden vision loss after uncomplicated cataract surgery with the features of an optic neuropathy, no acute disk swelling, and delayed optic nerve head perfusion on fluorescein angiography. CONCLUSIONS: Posterior ischemic optic neuropathy rarely occurs after uncomplicated cataract surgery, and should be considered in the setting of postoperative vision loss.

Current neuro-ophthalmic therapies.

The clinical characteristics, differential diagnosis, and treatment options are presented for five different categories of neuro-ophthalmic disease. Nystagmus, optic neuritis, diplopia, pseudotumor cerebri, and temporal arteritis, are frequently encountered in neuro-ophthalmic practice. This article focuses on current therapies for these neuro-ophthalmic disorders. Potential differences in approach to pediatric versus adult patients are emphasized.

Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas: relation to tumor location by magnetic resonance imaging.

PURPOSE: To examine the potential for visual acuity loss, and its relation to extent and location of optic pathway gliomas in a cohort of children with neurofibromatosis type 1 studied with magnetic resonance imaging. METHODS: We reviewed the neuro-ophthalmologic records and brain/orbital magnetic resonance imaging scans for 43 consecutive pediatric patients with neurofibromatosis type 1 and optic pathway gliomas who were followed at the Children's Hospital of Philadelphia. The presence of visual loss, defined as abnormal visual acuity for age in one or both eyes, was determined. Optic pathway gliomas were classified by tumor extent and location according to involvement of the optic nerves, chiasm, and postchiasmal structures by magnetic resonance imaging. RESULTS: Involvement of the optic tracts and other postchiasmal structures at tumor diagnosis was associated with a significantly higher probability of visual acuity loss (P =.048, chi-square test). Visual loss was noted in 20 of 43 patients (47%) at a median age of 4 years; however, three patients developed visual acuity loss for the first time during adolescence. CONCLUSIONS: In pediatric patients with neurofibromatosis type 1 and optic pathway gliomas, the likelihood of visual loss is dependent on the extent and location of the tumor by magnetic resonance imaging and is particularly associated with involvement of postchiasmal structures. Furthermore, older age during childhood (adolescence) does not preclude the occurrence of visual loss. Close follow-up beyond the early childhood years, particularly for those with postchiasmal tumor, is recommended.

Acute idiopathic blind spot enlargement syndrome: a review of 27 new cases.

OBJECTIVE: To describe the clinical findings in patients with acute idiopathic blind spot enlargement (AIBSE). METHODS: Medical record review of 27 patients with AIBSE (without sufficient optic nerve head swelling to cause blind spot enlargement) seen in 2 academic neuro-ophthalmology units. RESULTS: All patients were women aged between 19 and 53 years. Twenty-three patients reported positive visual phenomena. Visual acuity was normal in 16 patients. All patients had enlarged blind spots of variable size and density. Dyschromatopsia and afferent pupil defects were prevalent. Ophthalmoscopic features included uveitis, mild optic nerve swelling, granularity of macular pigment, subretinal white dots, and peripapillary pigment disturbances. Twelve of the 13 patients who underwent fluorescein angiography had optic disc staining and 5 had retinal pigment epithelial lesions with late staining. Full-field electroretinogram results were normal in 8 of 9 patients, although focal electroretinogram results were abnormal in 8 of 9 patients. Photopsia always decreased but visual fields did not improve. Six patients experienced recurrence. CONCLUSIONS: The clinical features of AIBSE include photopsia, visual field defects, abnormal findings from fundoscopic and fluorescein angiography, and abnormal results of focal electroretinography. The disease affects the peripapillary retina and may cause an afferent pupillary defect. The striking predilection for the peripapillary retina suggests a local etiologic factor and distinguishes AIBSE from the multiple evanescent white dot syndrome. Unlike patients with multiple evanescent white dot syndrome, recovery of visual field did not occur in patients with AIBSE.

Optic neuritis: historical aspects.

Optic nerve disorders were not reliably diagnosed until the late nineteenth century when ophthalmoscopy became part of the ophthalmic examination. By the early 1900's, all of the salient clinical features of optic neuritis and its relationship to "systemic sclerosis" were recognized, but there was much controversy and misunderstanding about its differential diagnosis, pathogenesis, and possible treatment. During the twentieth century, physicians began to distinguish optic neuritis from infectious, hereditary, toxic, nutritional, and ischemic optic neuropathies. The development of magnetic resonance imaging and the results from recent clinical trials have enhanced our understanding of the relationship between optic neuritis and multiple sclerosis. The next decade holds the promise of further elaborating the pathogenesis and treatment of optic neuritis.

Portable pupillography of the swinging flashlight test to detect afferent pupillary defects.

OBJECTIVE: To investigate the ability of a portable, personal computer-driven, pupillometer to record the pupillary response curve during the swinging flashlight test. Also, to determine whether these response curves can be used to identify and quantify relative asymmetry in the pupillary light reflex between eyes in healthy volunteers with simulated afferent pupil defects (APDs) and patients with optic neuropathies. DESIGN: Comparative, observational case series and instrument validation. PARTICIPANTS: Healthy volunteers with no known ocular disease and patients (n = 20) with various optic neuropathies noted to have relative APDs on examination. METHODS: Pupillary response curves of the right eye were recorded with a portable, electronic, infrared pupillometer from healthy volunteers (with and without simulated APDs) and patients with APDs while the light stimulus alternated between eyes, simulating the swinging flashlight test. Simulated APDs in healthy volunteers were created with increasingly dense neutral density filters in front of the left eye. MAIN OUTCOME MEASURES: Differences in constriction amplitude, latency, and constriction velocity of the pupillary response with right eye stimulation versus left eye stimulation in both groups of subjects. RESULTS: A significant correlation between neutral density filter strength and intereye differences was seen for all measurement parameters in volunteers with simulated APDs. Depending on the measurement parameter and stimulus intensity, simulated APDs of 0.6 log units or more could be distinguished from normal responses. Clinically graded true APDs had intereye differences similar to simulated APDs of the same density. Those with real and simulated APDs of 0.9 log units or more could be distinguished from healthy volunteers with 80% sensitivity and 92% specificity. Responses from those with real and simulated small APDs of 0.3 to 0.6 log units could not be distinguished reliably. CONCLUSIONS: Portable, personal-computer driven, electronic, infrared pupillography can record the swinging flashlight test accurately and identify large afferent pupillary defects. An affordable, portable, reliable device for identifying relative APDs would be useful in the identification and follow-up of patients with neurogenic vision loss.

Use of a portable head mounted perimetry system to assess bedside visual fields.

AIM: This study was designed to test the ability of a portable computer driven, head mounted visual field testing system to perform automated perimetry on patients at their bedside and to compare these results with the "gold standard" for bedside examinations, confrontation visual fields. METHODS: The Kasha visual field system is a portable automated perimeter which utilises a virtual reality headset. 37 neurosurgery patients were examined at their bedside with a central 24 degree suprathreshold testing strategy after confrontation visual field testing. The patterns of visual field defects were categorised and compared with the results of confrontation testing. RESULTS: A total of 42 field examinations were completed on 37 patients, and the average testing time for both eyes was 4.8 minutes with the perimetry system. Each of the 11 fields (100%) classified with defects on confrontation testing was similarly categorised on head mounted perimetry. 26 out of 31 (84%) visual fields were normal on both confrontation and perimetry testing, while five out of the 31 fields (16%) which were full on confrontation had visual field defects identified by head mounted perimetry. CONCLUSION: The head mounted, automated perimetry system proved easily portable and convenient for examining neurosurgical patients at their bedside in the perioperative period. The device demonstrated equal sensitivity to confrontation visual field testing methods in detecting field defects and offers the advantage of standardised, quantifiable testing with graphic results for follow up examinations.

Orbital drainage from cerebral arteriovenous malformations.

OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). METHODS: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). Magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. Magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.

Optic nerve and orbital tumors.

The close proximity of the orbit to the brain and the high frequency of ocular symptoms in patients with neurosurgical disease make it mandatory for neurosurgeons to be familiar with manifestations of orbital disease. Tumors of the optic nerve and orbit are important causes of vision loss and eye movement abnormalities. Similarly, intracranial tumors frequently present with eye movement abnormalities, vision loss, and optic nerve swelling. This overlap in the clinical characteristics of patients with orbital tumors and patients with neurosurgical problems makes familiarity with the types of clinical presentation of various orbital tumors important to the neurosurgeon. The history and examination of patients with orbital tumors are discussed and the clinical presentation of various orbital tumors is presented.

Evidence for preganglionic pupillary involvement in superficial siderosis.

A 36-year-old man presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation. MRI demonstrated hemosiderin deposition along the superficial surfaces of the brain, brainstem, cerebellum, and spine. The pupillary changes were localized to the preganglionic oculomotor nerve. In contrast to vasculopathic oculomotor nerve palsies, superficial siderosis may cause selective involvement of the superficially located pupillary fibers.