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Significant restoration of visual function can occur following pituitary tumor resection, although the time course of visual recovery remains poorly understood. This single-centre, two-year, prospective cohort study investigated the temporal patterns of visual recovery in consecutive patients undergoing pituitary tumor resection, between 2009 and 2018. Eyes were stratified based on pre-operative optical coherence tomography (OCT) retinal nerve fibre layer (RNFL) thickness measurements, with thin RNFL being defined as those within the fifth-percentile of age-matched normative values, and normal RNFL as those above the fifth-percentile. Visual function and OCT parameters were assessed pre-operatively, and at 6 weeks, 6 months, and 2 years post-operatively. 456 eyes of 228 patients (mean ± SD age, 53 ± 15 years) were included, of which 114 (25%) eyes had thin RNFL pre-operatively. Visual field recovery was observed in both groups during the first 6 weeks post-operatively (all Q ≤ 0.02), although improvements in visual field parameters between 6 weeks to 6 months were limited to eyes with thin RNFL (both Q 0.05). No further improvements in visual function were detected beyond 6 months in both groups (both Q 0.50). Similar trends were observed in linear regression analysis according to baseline visual function in both groups. In summary, eyes with normal RNFL thickness at baseline experienced most of their recovery within the first six weeks following surgery, while eyes with thin RNFL exhibited gradual improvements during the first six months. These findings have important implications when providing patient counselling and prognostication in the pre-operative setting.

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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 report a case of septic thrombophlebitis producing bilateral abducens nerve palsy. Observation: A 65 year-old woman with recent sinus surgery experienced the onset of horizontal diplopia during treatment for bacteremia. Computer tomography of head and a neck ultrasonography showed right internal jugular vein occlusion. Ophthalmology examination was consistent with bilateral abducens nerve palsy. She was treated with systemic antibiotics and antiplatelet therapy with resolution of the internal jugular vein occlusion. The diplopia improved over a six-months. Conclusion and importance: Our patient had Lemierre syndrome with an unusual presentation. The patient was treated for septic thrombophlebitis with a resolution of her ocular symptoms. Keywords: Lemierre syndrome, Septic thrombophlebitis, Abducens nerve, Sixth cranial nerve

To evaluate the diagnostic yield and concordance of color duplex ultrasound (CDU) of the superficial temporal artery (STA), temporal artery biopsy (TAB), and American College of Rheumatology (ACR) criteria in the diagnosis of giant cell arteritis (GCA).Prospective, masked study of all patients evaluated in one institution suspected of having GCA. All patients with a suspected diagnosis of GCA were admitted for pulsed intravenous corticosteroids. Patients underwent serologic work-up and ACR criteria were documented. All patients had a CDU and TAB performed within 3 days of initiation of systemic corticosteroid therapy. Main outcome measure: Concordance of CDU and TAB. Secondary outcome measures: Concordance between unilateral and bilateral CDU and TAB by side and segment, concordance between TAB and ACR criteria, and statistical analysis of serologic markers for GCA.The diagnosis of biopsy-proven GCA was found in 14 of 71 (19.7%) patients. The sensitivity of CDU compared to the reference standard of TAB ranged between 5.1% and 30.8% depending on the signs studied on CDU and correlation of specific TAB parameters. Of the serologic studies, a platelet count threshold of 400,000μL had the highest positive (18.32) and lowest negative (0.37) likelihood ratios for a diagnosis of GCA.In this study, CDU showed minimal value in diagnosing GCA compared to TAB. There was poor correlation between CDU results and ACR criteria for GCA. The threshold platelet count had higher positive and negative predictive values for GCA than CDU and is a useful serologic marker for GCA.

Our purpose was to analyse the demographics, prevalence and pattern of visual field defects in patients with pituitary adenoma. We prospectively recruited 103 consecutive patients (206 eyes) presenting to a neurosurgical unit with pituitary adenoma. Ophthalmological examination and standard automated perimetry (Humphrey, 24-2 threshold) was performed. Severity of visual field defects was also assessed. The mean population age was 53.9 years (standard deviation=15). Visual loss was the most common reason for presentation (39%) followed by endocrine abnormality (21%) and headache (15%). Patients with endocrine abnormality on presentation were 10.9 years younger than those presenting with visual loss (p=0.001). Bitemporal defects were the most prevalent pattern (n=22, 41%) followed by homonymous defects (n=7, 13%). Of the patients with visual field loss, 33% had unilateral visual field defects. The mean visual acuity in those with bitemporal defects was 6/7.5 with half of these patients having 6/6 vision in both eyes. In conclusion, the majority of patients with pituitary adenoma have visual acuity better than 6/7.5 despite having visual field defects. While a bitemporal pattern of visual field loss is the most common, a significant proportion of patients had unilateral and altitudinal defects. Assessment of the visual field is essential to rule out chiasmal compression.

Compression of the optic chiasm causes an optic neuropathy that may be associated with reversible visual loss often immediately following surgical decompression. While the precise pathogenesis of retinal ganglion cell impairment and eventual death remains poorly understood, a number of putative mechanisms may play a role. In this article we review the evidence supporting various stages of visual loss and recovery in chiasmal compression. These include conduction block, demyelination, ischemic insult, and retrograde and anterograde degeneration. We also describe novel advances in magnetic resonance imaging with specialized modalities such as diffusion tensor imaging have provided further information to explain the underlying mechanism of visual loss. Functional measures including electrophysiology are time-consuming but have shown moderate prognostic ability. Optical coherence tomography has provided novel new biomarkers for predicting outcome following surgical decompression. Both retinal nerve fiber layer thickness and ganglion cell complex thicknesses have shown to have excellent predictive power. Such advances serve to inform patients and clinicians of pre-operative factors that predict the extent of visual recovery following medical or surgical treatment of para-chiasmal lesions.

Background Evaluate the incidence of biopsy-proven giant cell arteritis for seasonal or annual variability in the Mid-Atlantic United States. Design Retrospective chart review of all patients undergoing temporal artery biopsy from 1994 to 2011. Participants The charts of 744 patients were reviewed, and 215 patients were diagnosed with giant cell arteritis based on positive temporal artery biopsy results. Methods All results between 1994 and 2011 were reviewed. Giant cell arteritis incidence data were evaluated by year, season and month for any trends or cyclic patterns. Main Outcome Measure Incidence of biopsy-proven giant cell arteritis. Results The majority of patients were female (74%) and over the age of 60 (98.6%). The diagnosis of biopsy-proven giant cell arteritis was found in 215 of 744 (28.9%) patients. The incidence of biopsy-proven giant cell arteritis by year varied, with the peak incidence in 1996. The monthly incidence peaked in July and had a trough in October. However, Poisson regression analysis did not show any statistically significant trend over time or cyclic pattern to the incidence by year, season or month. Conclusions The incidence of biopsy-proven giant cell arteritis in the population studied did not have any significant cyclic pattern over the last 17 years. The highest incidence by month was noted in July with a trough in October. However, this was not a significant pattern by month or season to support infectious or periodic environmental factors inciting giant cell arteritis.

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 investigate the ability of optical coherence tomography (OCT) parameters of macular thickness (MT) and peripapillary retinal nerve fibre layer (RNFL) thickness to differentiate eyes with nonarteritic anterior ischaemic optic neuropathy (NAION) from uninvolved eyes and to identify the relationship between macular and RNFL parameters and visual field sensitivity (VFS). Methods: Thirty patients with unilateral NAION participated in a prospective observational cross-sectional study. Patients underwent Humphrey visual field (SITA Standard 24-2, HVF) testing and OCT to measure MT and RNFL. The contralateral uninvolved eye was used as controls. Areas under the receiver operating characteristic curves (AUROCs) of MT and RNFL for discriminating NAION from control eyes were also determined. The prespecified outcome measure was the correlation between RNFL, MT and mean deviation (MD). Results: Average RNFL and MT were thinner in NAION eyes: 72.8 μm versus 98.9 μm (p

One of the most common causes of enophthalmos is previous orbital fracture. In a patient with a known history of breast carcinoma, enophthalmos is concerning for metastatic disease. This report presents a patient with a history of scirrhous breast carcinoma and enophthalmos who was found to have orbital amyloidosis. This is the first report of enophthalmos as the presenting sign of orbital amyloidosis.

For nearly a century, ophthalmologists have recognized that thinning of the retinal nerve fiber layer (rNFL) could be observed ophthalmoscopically in diseases of the optic nerve. Using high-resolution red-free fundus photography, Hoyt found slit-like rNFL defects that corresponded to visual field defects in glaucoma. Frisén extended these observations to multiple sclerosis, predicting the later discovery that axonal loss occurs in the retina without clinical bouts of optic neuritis. In measurement of the rNFL, red-free fundus photography has been superseded by more widely available, robust, and quantitative retinal imaging techniques, including Heidelberg retinal tomography, scanning laser polarimetry, and optical coherence tomography (OCT). Having emerged as the technique of choice in measuring the rNFL, OCT has shown that the degree of preoperative rNFL thinning reliably predicts whether vision will recover after surgery for pituitary adenoma. Such quantitative studies of the rNFL have the potential, therefore, of providing descriptive and predictive information that will be valuable in clinical care.

Objective Giant cell arteritis (GCA) is the most common type of primary vasculitis. Matrix metalloproteinase 9 (MMP-9) is present in arterial lesions of GCA and may be involved in its pathogenesis. We investigated whether certain genotypes of 4 single-nucleotide polymorphisms (SNPs) of MMP-9 are overrepresented in patients with histologically confirmed GCA. Methods Four SNPs of MMP-9, rs3918242 in the promoter region and 3 nonsynonymous coding SNPs (rs3918252, rs17576, and rs2250889) were genotyped by polymerase chain reaction–restriction fragment length polymorphism analysis in 58 white patients for whom there was a clinical suspicion of GCA. Thirty of these patients had histologically confirmed GCA (group 1), and 28 patients had negative results of a temporal artery biopsy for GCA (group 2). Estimates of the genotype distributions of each of these SNPs in a white population were determined using publicly available genotype data for a panel of 23 individuals (group 3). Results Although 1 SNP was monomorphic in all 3 groups, we observed statistically significant differences in the genotype distributions for rs2250889 between group 1 and group 2 (P = 0.005) and between group 1 and group 3 (P = 0.009), but not between groups 2 and 3 (P = 0.965). Conclusion These data derived from a sample of patients with GCA suggest that the G allele of MMP-9 polymorphism rs2250889 is overrepresented in patients with histologically confirmed GCA. Clearly, larger sample sizes will be necessary to confirm this suggestive association and better characterize a possible linkage disequilibrium structure among polymorphisms.

To identify cutpoints for visual measures at baseline and 1 month predictive of abnormal 6-month vision that could be used as eligibility criteria in a clinical trial to test potential neuroprotection or myelin repair agents in patients with optic neuritis. To determine whether moderate-to-severe dysfunction in one or more visual measures at baseline or 1 month correlates with having major vision loss at 6 months.We used the Optic Neuritis Treatment Trial database to evaluate various cutpoints for baseline and 1-month vision levels that predicted abnormal 6-month vision. For selected cutpoints, we computed a 95% CI for positive predictive value and the required sample size if the cutpoint was to be used for clinical trial eligibility. We evaluated whether the degree of visual loss at baseline, 1 month, or change in visual function from baseline to 1 month correlated with 6-month visual acuity, contrast sensitivity, or threshold visual field.The best cutpoints for baseline and 1 month were visual acuityor= 20/50, contrast sensitivity1.0 log units, and visual field mean deviationor= -15 dB. The same levels of visual dysfunction at 1 month, but not at baseline, correlated with having 6-month moderate-to-severe loss for each of these measures (p = 0.01). A trial could require as few as 100 subjects for an outcome variable of one or more abnormal measures. Cutpoints at 1 month were highly predictive of abnormal 6-month vision, but the proportion of patients who would be eligible for a trial would be small.Provided data can be used either for the clinician to counsel patients on expected visual outcome or for designing studies to test therapies that might reduce the amount of permanent optic nerve damage due to optic neuritis in high-risk patients.

We evaluate if the relationship between optical coherence tomography (OCT) of the retinal nerve fibre layer (RNFL) and visual outcome continued over long-term visual recovery in 107 patients undergoing pituitary decompression. Recently, it has been recognized that OCT of the RNFL has prognostic value in predicting visual outcomes after surgery for chiasmal compression caused by pituitary tumours. Patients were followed up at three time points: pre-operative (visit 1), 6-10 weeks post-operative (visit 2) and 9-15 months follow-up (visit 3). We found that patients with thin pre-operative RNFL had more severe visual field defects (mean deviation [MD] -9.22 versus -3.96 decibels [dB]; p = 0.001), but pre-operative visual acuity (VA) was good in both normal and thin RNFL groups (Snellen VA 6/5 and 6/4; p = 0.039). For those with thin RNFL the greatest improvement was between visit 2 and 3 (MD -7.1 dB versus -3.4 dB, respectively; p < 0.001) compared with pre-operative -9.8 dB. Normal RNFL patients showed greatest improvement between visits 1 and 2 (pre-operative -4.8 dB, visit 2 -2.0 dB, visit 3 -0.9 dB; p = 0.001). For long-term follow-up, 81.0% of eyes with normal RNFL, compared to 37.1% with thin RNFL (p < 0.001), achieved an MD of -2.00 dB (final visit). At final follow-up, 97.5% of normal RNFL eyes achieved VA of 6/12 or better compared with 88.2% with thin RNFL (p = 0.034). Our results indicate that long-term visual recovery after surgical decompression of pituitary lesions is predicted by pre-operative OCT RNFL. Patients with normal RNFL thickness show an increased propensity for visual recovery. This effect continues after long-term follow-up, however, most visual recovery occurs within the first 6-10 weeks.

Purpose To report the rate of occurrence of cerebral venous sinus thrombosis (CVST) in patients with presumed idiopathic intracranial hypertension (IIH). Design Retrospective chart review. Participants All patients diagnosed with papilledema from November 1, 2002, through October 31, 2003, at 3 tertiary care neuro-ophthalmology centers. Methods Consecutive patients with a diagnosis of papilledema from 3 tertiary care neuro-ophthalmology centers were identified. Patients with space-occupying lesions, hydrocephalus, or meningitis were excluded. The remaining patients were evaluated with lumbar puncture, magnetic resonance imaging (MRI), and magnetic resonance venography (MRV). Main Outcome Measures The rate of occurrence of CVST in patients with presumed IIH. Results One hundred thirty-one patients with papilledema were identified. Excluding patients with mass lesions, meningitis, or hydrocephalus, the occurrence of CVST was 10 (9.4%) of 106 patients with presumed IIH. Two additional patients had a diagnosis of suspected CVST. Cerebral venous sinus thrombosis was diagnosed in 1 of the 10 patients with MRI alone, whereas it was evident in all 10 patients with MRV. Underlying risk factors for CVST were identified in 9 of 10 patients. Conclusions Cerebral venous sinus thrombosis accounts for 9.4% of patients with presumed IIH in 3 tertiary care neuro-ophthalmology services. Magnetic resonance venography in combination with MRI is recommended to identify this subgroup of patients.

Objective The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are laboratory tests that have been said to have a strong correlation with a positive temporal artery biopsy in patients with suspected giant cell arteritis (GCA). Published reports suggest that the CRP is a more sensitive diagnostic indicator of GCA and can be elevated when the ESR is normal. It is also clear that the CRP and ESR can both be normal or both be elevated in patients with biopsy-proven GCA and that the CRP can be elevated when the ESR is normal. The purpose of this study was to ascertain if the CRP can be normal when the ESR is elevated in biopsy-proven GCA. Design Retrospective, longitudinal, comparative study. Participants One hundred nineteen patients from 6 major tertiary-care university-affiliated medical centers. Methods The charts from 119 patients with temporal artery biopsies positive for GCA were reviewed for age, gender, pretreatment ESR, and pretreatment CRP. Main Outcome Measures The ESR in millimeters per hour Westergren was graded as normal or abnormal based on 2 validated formulas. The CRP was graded as normal or abnormal based on established criteria set forth in the literature as well as at The Johns Hopkins Hematology laboratory. Results In this study, the ESR had a sensitivity of 76% to 86%, depending on which of 2 formulas were used, whereas an elevated CRP had a sensitivity of 97.5%. The sensitivity of the ESR and CRP together was 99%. Only 1 of the 119 patients (0.8%) presented with a normal ESR and normal CRP (double false negative); 2 patients (1.7%) had a normal CRP despite an elevated ESR according to both formulas. Conclusion Although most patients with GCA have both an elevated ESR and CRP, there can be nonconcordance of the 2 blood tests. Although such nonconcordance is most often a normal ESR but an elevated CRP, the finding of an elevated ESR and a normal CRP also is consistent with GCA. The use of both tests provides a slightly greater sensitivity for the diagnosis of GCA than the use of either test alone.

Objective Quantitative data are not available regarding the morphological appearance of optic nerve morphology after arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION). The purpose of this study is to evaluate the appearance of the optic nerve head in patients after AAION and NAION with the Heidelberg Retina Tomograph (HRT). Design Prospective comparative observational study. Participants Thirty-eight patients with either unilateral AAION (15) or NAION (23). Methods Heidelberg Retina Tomograph images were taken of both affected and unaffected eyes. The quantitative changes in the optic nerve head were morphologically evaluated and compared with those of the normal contralateral eyes. Main outcome measures Heidelberg Retina Tomograph parameters were analyzed using the uninvolved contralateral eye as a control. Results The ratio between cup area and disc area was 42% larger in the affected eyes in the NAION group than in the fellow eyes ( P = 0.03, t test). In the AAION patients, the ratio between cup area and disc area was 116% larger in involved eyes than in their fellow eyes ( P = 0.0003, t test). In addition, in the AAION group, relative to their fellow eyes, the rim area was 27% smaller ( P = 0.0006), cup volume was 160% larger ( P = 0.021, t test), mean cup depth was 29% larger, cup shape measure differed by 41% (suggesting more excavation), and mean retinal nerve fiber layer thickness was 30% less. Relative to the fellow eyes in the NAION group, the rim area was 6% smaller ( P = 0.13, Student's t test), and the cup shape measure was 13% different, although these were not statistically significant. When the uninvolved eyes of the NAION and AAION groups were compared directly, there was no statistical difference between any of the HRT parameters, except mean cup depth (0.09 vs. 0.14 mm, respectively; P = 0.02). Conclusions This study is the first to demonstrate quantitatively an enlargement and excavation of the optic cup after an event of AAION, but not NAION. The eyes affected with AAION showed significant excavation and enlargement of the optic cup when compared with contralateral uninvolved eyes. All HRT-generated parameters showed changes consistent with increased enlargement and excavation of AAION-involved eyes. These changes were not observed in eyes that experienced an event of NAION.

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.

Background Participants enrolled in the Optic Neuritis Treatment Trial have been observed for more than a decade to assess the relationship between optic neuritis and the development of clinically definite multiple sclerosis. Objective To assess neurologic disability 10 to 12 years after an initial episode of optic neuritis. Design Longitudinal follow-up of a clinical trial. Setting Fourteen Optic Neuritis Treatment Trial clinical centers performed standardized neurologic examinations, including an assessment of neurologic disability. Participants One hundred twenty-seven patients who had developed clinically definite multiple sclerosis. Main outcome measures Functional Systems Scale and Expanded Disability Status Scale. Results The disability of most patients was mild, with 65% of patients having an Expanded Disability Status Scale score lower than 3.0. The degree of disability appeared to be unrelated to whether the baseline magnetic resonance imaging scan was lesion-free or showed lesions (P =.51). Among patients with baseline lesions, the degree of disability was unrelated to the number of lesions that were present on the scan (P =.14). Two patients died owing to severe multiple sclerosis, one of whom had no lesions revealed on the baseline scan. Conclusion Most patients who develop clinically definite multiple sclerosis following an initial episode of optic neuritis will have a relatively benign course for at least 10 years.

Giant cell arteritis (GCA) is a systemic vasculitis that affects large and medium sized arteries. Visual loss is one of the most devastating complications of GCA and usually occurs from occlusion of the posterior ciliary arteries (PCA) leading to anterior ischaemic optic neuropathy (AION). Visual loss can also occur from occlusion of other arteries that supply the visual pathway, such as the central retinal and ophthalmic arteries. Corticosteroid therapy, given orally or intravenously, is the standard treatment for GCA associated visual loss.1 The optimal route of administration and dosage to prevent further visual loss are not known; however, most clinicians advocate higher doses in patients who already have experienced visual loss. Treatment with corticosteroids usually results in stabilisation of visual loss and some patients may have some degree of visual recovery.2,3 However, despite treatment with high dose intravenous corticosteroids, visual loss may progress.1 The reported use of adjunctive agents under these circumstances has been limited. We report a patient who had progressive visual loss while on high dose intravenous corticosteroids and who markedly improved after treatment with heparin. An 85 year old man presented to his optometrist for a routine eye examination. His visual acuity was 20/40 both eyes and his optic discs were normal. Three weeks later (day 1), he lost vision in his right eye. His visual acuity was now 20/100 right eye and 20/40 left eye. On visual field testing with static perimetry he had an inferior altitudinal defect in the right eye and his visual field was normal in the left. His right optic disc was pale and oedematous and his left optic disc was normal. He was suspected of having GCA and was admitted for treatment with intravenous methylprednisolone (IVMP) 250 mg every 6 hours. On day 2, the visual field …

Objective To describe multifocal electroretinogram (mfERG) responses in 2 patients with nonorganic visual loss and in 11 eyes of 6 healthy persons who suppressed their mfERG responses. Design Observational case series. Methods The mfERG results were recorded in all individuals using the Veris Science 4.2 instrument. All subjects were instructed to adjust the hexagonal test pattern so that it was in best focus. A second mfERG was recorded subsequently in volunteers who attempted suppression with inattention and poor fixation and by adjusting the focus to greatest blur. Main outcome measures Amplitude and latency of mfERG responses. Results Suppressed mfERGs in patients with nonorganic visual loss and healthy volunteers demonstrated reduced amplitude, especially centrally. Amplitude reduction was statistically significant in the postsuppression as compared with the presuppression recordings in wave forms N1 and N2. Statistically significant shortening of postsuppression implicit times of P1 and N2 waveforms also was demonstrated. Conclusions The mfERG responses may be suppressed voluntarily. Amplitude may be reduced. In contrast to most reported pathologic conditions, the implicit time is shortened.

To discuss the current standard in diagnosis and treatment of pseudotumor cerebri (PTC), the syndrome of increased intracranial pressure without a brain tumor. Since PTC is a diagnosis of exclusion, the differential diagnostic work-up is of special importance.Modern imaging techniques have facilitated the differential diagnosis of PTC. Various therapeutic options are available to prevent irreversible visual loss. New treatment modalities are being explored, but the standard therapy has undergone little change in the past years.PTC, a potentially blinding disease, most commonly manifests with headache and slightly blurred vision due to papilledema. This review seeks to present a methodical approach to its diagnosis and treatment.

To investigate the relationship between idiopathic nonarteritic ischemic optic neuropathy (NAION) and serum lipid levels in patients/= 50 years of age.Case-control study.Thirty-seven consecutive patients with NAION and 74 age- and gender-matched comparison patients.Serum lipid levels and the presence of several historical diseases and exposures were abstracted from the medical records in cases and controls.The mean total cholesterol level was significantly increased in patients with NAION compared with controls (235.4 vs. 204.0 mg/dl, P0.001). The odds ratio of having high blood cholesterol (/= 240 mg/dl) with NAION was 3.3 (95% confidence interval, 1.4-7.8), and the likelihood increased when the comparison was restricted to nondiabetic patients. Diabetes mellitus was more common in cases than controls (P = 0.027), but systemic hypertension was not significantly different (P = 0.63). No patient (0 of 24) had a magnetic resonance imaging study consistent with optic neuritis or central nervous system demyelination. Visual improvement was uncommon.This study demonstrates that hypercholesterolemia is associated with NAION in younger patients. NAION may be the first manifestation of a lipid disorder, a previously unrecognized syndrome. These patients have experienced a focal, microvascular central nervous system ischemic event at a relatively young age. Aggressive treatment of lipid abnormalities in these patients may be warranted.

Purpose To report the results of scanning laser polarimetry (NFA/GDx; Laser Diagnostic Technologies, San Diego, CA). Design Prospective, consecutive observational case series. Participants Ten consecutive patients with central retinal artery occlusion (CRAO). Methods Neuro-ophthalmic examination and scanning laser polarimetry of the retinal nerve fiber layer (RNFL) of 10 patients with CRAO. Main outcome measures Duration of visual loss, visual acuity, funduscopy, and scanning laser polarimetry of the RNFL in 10 eyes of 10 patients with CRAO. Results The duration of visual loss before examination and scanning laser polarimetry ranged from 1 to 7 days. Visual acuity was counting fingers at 1 foot or worse in all 10 eyes with CRAO, and funduscopy revealed pallid retinal edema accompanied by a cherry red spot in all affected eyes. Funduscopy of the fellow eyes in all but one patient, who had anterior ischemic optic neuropathy and was subsequently diagnosed with giant cell arteritis, revealed no acute changes. Scanning laser polarimetry of all eyes with CRAO revealed diffuse attenuation of the retardation of the RNFL. Scanning laser polarimetry of the fellow eye revealed a normal bimodal distribution in the eight patients in whom it could be measured. In one patient who was examined 1 day after the onset of visual loss, repeat nerve fiber analysis 6 weeks later revealed further depression of the RNFL compared with the initial scan. Repeat analysis of the RNFL in four other patients showed persistence of the diffuse depression noted during their initial examinations. Conclusions Scanning laser polarimetry in CRAO reveals diffuse depression of the retardation of the RNFL, which occurs acutely after the onset of visual loss. To our knowledge these patients represent the first reports of scanning laser polarimetry of the RNFL in acute CRAO.

Objective To assess the visual function of patients with giant cell arteritis (GCA) who had visual loss from either anterior ischemic optic neuropathy (AION) or central retinal artery occlusion and had a subsequent improvement in visual acuity after treatment with corticosteroids. Design Retrospective, observational case series. Participants Thirty-two consecutive patients with biopsy-proven GCA treated at one institution between January 1992 and December 1997. Intervention Treatment with intravenous methylprednisolone 250 mg every 6 hours for 3 days, followed by oral prednisone 1 mg/kg daily for at least 4 weeks duration. Main outcome measures The number of patients with an improvement in visual acuity after treatment with intravenous methylprednisolone; neuro-ophthalmic evaluation, including visual acuity, funduscopy, and visual field examination of these patients. Results Improvement in visual acuity occurred in 5 of 39 eyes (13%) with visual loss from biopsy-proven GCA, and all 5 patients had AION. Despite the improvement of visual acuity in these 5 patients, perimetry revealed marked constriction of the visual field in each affected eye. Conclusions The prognosis for visual improvement in GCA is poor. Although an improvement in visual acuity occurred in 5 of our patients, marked constriction of the visual field was present in all of them.

Nonarteritic anterior ischemic optic neuropathy refers to an idiopathic ischemic process of the anterior portion of the optic nerve. The typical presentation is sudden and painless visual loss with examination features of an optic neuropathy. Among the various associated risk factors are optic disc morphology, advanced age, systemic arterial hypertension, diabetes mellitus, and nocturnal hypotension. Currently, there is no proven effective long-term treatment for this disorder.

Acute demyelinating optic neuritis associated with multiple sclerosis (MS) is the most common cause of inflammation of the optic nerve. The Optic Neuritis Treatment Trial (ONTT) has provided important clinical data on the use of corticosteroids, and demonstrated that patients with characteristic inflammatory lesions within the brain on magnetic resonance imaging had a greater chance of developing clinically definite MS (CDMS). The current approach to patients with optic neuritis has been modified by the results of the Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS). Patients with an initial clinical episode of demyelination (optic neuritis, incomplete transverse myelitis, or brain-stem/cerebellar syndrome) and at least two characteristic demyelinating lesions within the brain were randomized to receive interferon beta-1a or placebo after initial treatment with intravenous corticosteroids. At the 3-year point patients treated with interferon beta-1a showed a 50% less risk of CDMS. The results of this study have set the standard for patients with a first bout of demyelinating optic neuritis.

OBJECTIVE We investigated the safety and efficacy of stereotactic radiotherapy as an alternative therapy to surgical resection for optic nerve sheath meningiomas (ONSMs). METHODS Thirty patients and 33 optic nerves with ONSMs were treated with conventional fractionated stereotactic radiotherapy treatment (CF-SRT) between July 1996 and May 2001 with the use of a 6-MeV LINAC designed for and dedicated to radiosurgery. The LINAC technique involved daily CF-SRT involving a relocatable frame, an average of three isocenters, and high-radiation dose conformality established by noncoplanar arc beam shaping and differential beam weighting. The patients who were treated with CF-SRT were followed clinically with serial visual fields and radiographically with both magnetic resonance imaging and functional 111In-octreotide single-photon emission computed tomography. The results of treatment were compared with a historical control group of ONSM patients who were either observed or treated surgically and then observed. RESULTS Our study population comprised 18 women and 12 men with a median age of 44 years (age range, 20–76 yr). The median isosurface radiation dose was 51 Gy (dose range, 50–54.0 Gy), and the median clinical follow-up time was 89 weeks (range, 9–284 wk). Of 22 optic nerves with vision before CF-SRT, 20 nerves (92%) demonstrated preserved vision, and 42% manifested improvement in visual acuity and/or visual field at follow-up. Comparison of our patients with a historical control group revealed preserved vision in only 16% of patients in a comparable period of observation, along with a 150% greater probability of visual improvement. Four patients (13%) had posttreatment morbidities, including visual loss (two patients), optic neuritis (one patient), and transient orbital pain (one patient). On magnetic resonance imaging studies, there was no evidence of tumor progression or recurrence in all patients, including tumor volume reductions noted in four patients. All six patients monitored with 111In-octreotide scintigraphy demonstrated significant decreases in tumor activity after CF-SRT. CONCLUSION To date, this article describes the largest reported series of ONSMs. Although longer follow-up is necessary, we think that CF-SRT represents a safe alternative to surgery and offers a higher likelihood of preserved or improved vision in patients with ONSM. Our analysis suggests that CF-SRT is also preferable to observation. Functional 111In-octreotide single-photon emission computed tomographic scintigraphy provides a useful technique for the assessment of tumor control that complements serial posttreatment magnetic resonance imaging in patients with ONSMs.

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.

To compare the platelet counts, complete blood counts, and Westergren sedimentation rates (WESR) of patients with a biopsy positive for giant cell arteritis (GCA) with those of patients with negative biopsies.Retrospective, case-control series.The medical records of 91 consecutive patients who underwent temporal artery biopsy for possible GCA, over an 8-year period, were reviewed.Values obtained for the complete blood counts, including platelet counts, WESR, and biopsy results of 91 consecutive patients undergoing temporal artery biopsy were analyzed.The mean platelet count of 47 patients with positive temporal artery biopsies (433 x 10(3)/microl) was significantly higher than that of 44 patients with negative temporal artery biopsies (277 x 10(3)/microl), P0.0001. There was no statistically significant difference in the mean WESR between the biopsy-positive (82 mm/hour) and biopsy-negative (70 mm/hour) groups, P = 0.12. The sensitivity of an elevated WESR for biopsy-positive patients was greater (79%; 95% confidence interval [CI], 64%-89%) than that of an elevated platelet count (57%; 95% CI, 42%-72%). However, the specificity (91% [95% CI, 78%-97%] versus 27% [95% CI, 15%-43%]), positive predictive value (87% [95% CI, 70%-96%] versus 54% [95% CI, 41%-66%]), and negative predictive value (67% [95% CI, 53%-78%] versus 55% [95% CI, 32%-76%]) favored an elevated platelet count compared with WESR, or to the combination of platelets and WESR, as a better test for diagnosing GCA in the 91 patients studied. The area under the receiver operating characteristic function for platelets (0.72) was greater than that for WESR (0.59) or the combination of platelets and WESR (0.65).In patients suspected of having GCA, an elevated platelet count greater than 400 x 10(3)/microl is a useful marker of a positive temporal artery biopsy.

Objective To describe the detection of emboli to the retrobulbar circulation using orbital color Doppler imaging in patients with central retinal artery occlusions (CRAO) without visible retinal emboli. Design Noncomparative, retrospective, observational case series. Patients and methods Twenty-nine patients with CRAO, without funduscopic evidence of embolic material within the retinal arterioles, underwent neuroophthalmic examination and evaluation with orbital color Doppler imaging. Main outcome measures The detection of retrobulbar plaques using orbital color Doppler imaging. Results Nine of 29 (31%) patients with CRAO demonstrated hyperechoic retrobulbar plaques. All nine patients had visual acuity of counting fingers or worse at presentation. Six of the nine patients (67%) had a history of hypertension, and seven (78%) had coronary artery disease as vascular risk factors for arterial occlusive disease. Two of the nine patients (22%) subsequently died from cardiac-related events. Conclusions This study demonstrates that orbital color Doppler imaging is an important diagnostic procedure for establishing embolism as the cause of CRAO when no emboli are visible in the retinal circulation. This noninvasive technology enables prompt differentiation of embolic disease from arterial occlusion caused by intrinsic atherosclerosis, vasospasm, or vasculitis from giant cell arteritis. Recognition of emboli has important management implications for these patients.

A 39-year-old healthy woman presented for decreased vision at distance and near for 4 years. She also noted a decrease in her color vision. Her best-corrected visual acuities were 20/70 in each eye. Her visual fields were abnormal, and she had bilateral sluggish pupils, impaired color vision, and optic disc pallor. The magnetic resonance imaging of the brain, heavy metal screen, autoimmune work-up, B12, B6, folate, erythrocyte sedimentation rate, rapid plasma reagin, and Lyme titer were all normal. Optical coherence tomography of the macula and electroretinogram were normal; the visual evoked potential was unrecordable in both eyes. She denied a family history of similar ocular issues, and genotyping of the OPA1 gene revealed a novel previously unreported mutation at IVS12+10T >C.

We report a case of non-arteritic anterior ischemic optic neuropathy (NAION) associated with retinal emboli in a 68-year-old man with decreased vision. Carotid Doppler imaging revealed critical stenosis (80–99%) of the ipsilateral internal carotid artery. Carotid endarterectomy was performed and two weeks postoperatively a partial improvement in visual acuity was noted with resolution of the disc swelling.

To evaluate the change in intraocular pressure (IOP) in subjects with Graves' orbitopathy (GO) after orbital decompression, strabismus surgery, and orbital radiation.Retrospective case review.The charts of 172 consecutive subjects from the Neuro-ophthalmology Service at Wills Eye Hospital (Philadelphia, PA) with GO who underwent either orbital decompression, strabismus surgery, or orbital radiation between 1994 and 1999 were analyzed. Subject age, gender, diagnosis of glaucoma in either eye, use of systemic steroids or topical glaucoma medications, procedure performed, and the preoperative and postoperative IOP (in primary position and upgaze) were evaluated.Of 116 eyes that underwent orbital decompression, the mean preoperative IOP was 21.6+/-4.6 mmHg (standard deviation) in primary position and 27.9+/-6.8 mmHg in upgaze. The postoperative IOP was 17.5 mmHg +/- 3.0 mmHg in primary position and 20.1+/-4.7 mmHg in upgaze, a decrease in IOP of 18.9% in primary position and 27.9% in upgaze (P0.001). Subjects taking glaucoma medication or who had IOP greater than 21 mmHg demonstrated a significantly (P0.001) greater reduction in IOP postoperatively. The mean preoperative IOP in the 32 subjects who had strabismus surgery was 18.5+/-2.8 mmHg (primary position), and 24.7+/-4.3 mmHg (upgaze). Postoperative IOP was 16.1 mmHg (primary position) and 16.9 mmHg (upgaze), a decrease of 2.4 mmHg (13.3%, P0.01 in primary position) and 7.8 mmHg (31.2%, P0.01 in upgaze). There was no statistically significant reduction in IOP after orbital radiation.In the selected subgroup of subjects with GO who required intervention, orbital decompression and strabismus surgery resulted in a significant reduction in IOP in the early postoperative period, especially in subjects with preoperative IOP greater than 21 mmHg.

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 To compare optic disc topography in eyes with compressive optic neuropathy (CON) and open-angle glaucoma (OAG) using spectral-domain (SD) optical coherence tomography (OCT) and Heidelberg retinal tomograph (HRT) (Heidelberg Engineering GmbH, Heidelberg, Germany). Design Cross-sectional, observational study. Participants A total of 200 eyes from 123 patients with CON (69 eyes) or OAG (58 eyes) and controls (73 eyes). Methods Univariate and multivariate analyses of HRT parameters, SD-OCT circumpapillary retinal nerve fiber layer (RNFL) thickness, and optic nerve head (ONH) parameters. Main Outcome Measures Circumpapillary RNFL, OCT ONH parameters, and HRT parameters. Results The univariate analysis of OCT parameters demonstrated significant differences between the temporal and nasal quadrants; clock hours 3 (55 vs. 73 μm), 4, 8 (93.9 vs. 70.7 μm), 9, and 10; vertical cup-to-disc ratio (C:D) (0.6 vs. 0.8) and cup volume (0.2 vs. 0.5) ( P 0.001) between patients with CON and OAG, respectively. The CON discs were significantly different from normal discs for all OCT parameters except cup volume. The CON discs were not significantly different from normal discs for HRT parameters, except for mean RNFL thickness and cup shape measure. The OAG discs were significantly different from normal discs in all HRT and OCT parameters ( P 0.001). Multivariate analysis demonstrated that the OCT 3 o'clock temporal sector, average C:D ratio, vertical C:D ratio, and cup volume measurements were able to differentiate OAG from CON. Conclusions Compressive optic neuropathy is associated with significantly thinner nasal and temporal sectors compared with OAG, whereas OAG results in larger cups and cup volume with OCT measurements. The Heidelberg retinal tomograph is not able to differentiate CON from normal discs.

Dominant cystoid macular dystrophy (DCMD) is characterized by a prolonged course of bilateral macular cystoid change associated with leakage on fluorescein angiography, progressive loss of central vision, peripheral retinal pigmentary disturbances and subnormal electro-oculogram. The pigmentary changes and the electro-oculographic findings suggest dysfunction of the retinal pigment epithelium. We report the case of a woman affected by this dystrophy with its characteristic features.

The antiphospholipid antibody syndrome is an important cause of arterial and/or venous thrombosis. In this review the authors discuss recent trends in the clinical and laboratory diagnosis of this syndrome that are of relevance to ophthalmologists.The diagnostic criteria of antiphospholipid antibody syndrome have been reviewed and revalidated recently. Few prospective studies have been conducted to determine the prevalence of antiphospholipid antibodies in patients with retinal vascular occlusion and the spectrum of ophthalmologic findings in patients with established antiphospholipid syndrome. New antibody tests for antiphospholipid antibody syndrome are available with increased sensitivity and specificity, but lack of standardization, and interassay and interlaboratory variation makes it difficult to determine their clinical value.Ophthalmologists should be familiar with the spectrum of clinical manifestation of antiphospholipid syndrome, the diagnostic criteria of this disease, and be able to carry out a basic laboratory workup for this entity. A high index of suspicion for this disorder is also important in making the diagnosis.

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.

Background. Gliomas of the hypothalamus and optic pathways (H/OPG) comprise 5% of pediatric intracranial tumors, present most frequently in patients younger than age 5 years, and may have a more aggressive course in younger children. This study examined clinical characteristics and consequences of treatment of young children diagnosed with H/OPG. Methods. The authors reviewed the course, treatment, and outcomes of 46 children diagnosed with H/OPG younger than age 5 years; the median follow-up was 72 months. The median age at diagnosis was 27 months. Results. Fifteen (33%) of 46 patients had neurofibromatosis-1 (NF-1).Forty children (87%) had tumor progression in the follow-up period, and tumor growth was less common in children with NF-1. Initial therapy was limited to surgical resection in three and radiation in five children. To postpone radiation until after the age of 5 years, initial therapy was limited to chemotherapy in 32 patients. Radiation was not required in 9 of these patients and was postponed for 40 months (mean) in 17. Of the 46 children, 5 died of tumor progression, 4 became blind, and 20 of 34 evaluable patients had endocrine abnormalities. Endocrinopathy did not correlate with therapy. Ten of 17 children evaluated by questionnaire required special education. There was a trend for educational problems to occur in children who were irradiated before the age of 5 years. Conclusions. Gliomas of the hypothalamus and optic pathways and their treatment cause long term morbidity in young children. Chemotherapy postpones radiation effectively, and this delay may reduce neurologic morbidity; however, 60% of children eventually relapse. By contrast, patients with NF-1 have indolent disease. Cancer 1995;75:1051-9.

A 65-year-old white woman developed progressive visuospatial abnormalities over an eight-year course, secondary to Alzheimer's disease with amyloid angiopathy. Imaging studies demonstrated focal atrophy of the parietal and occipital lobes without hemorrhage. This patient manifested simultagnosia without ocular dysmetria or optic ataxia; hence, a true Balint's syndrome was not present. Her visual acuities and fields have remained stable status post-occipital lobe biopsy.

Intraocular pressure (IOP) is dependent on the rate of aqueous production, facility of outflow, and episcleral venous pressure. Increased IOP and visual field loss may result from an elevation of episcleral venous pressure, the causes of which include large vessel venous obstruction (venous sinus thrombosis and superior vena cava syndrome), arteriovenous shunts or fistulas, Sturge-Weber syndrome, scleritis, thyroid related orbitopathy, and orbital tumours.1 Increased episcleral venous pressure may result in elevated IOP and optic neuropathy, even in the absence of these entities. We report a 34 year old man with idiopathic dilated episcleral veins (IDEV) and increased IOP, with visual field loss. A 34 year old man presented for neuro-ophthalmic evaluation because of decreased vision in the right eye and injection of both eyes. His intraocular pressure (IOP) had been elevated in the right eye more than the left for the past 13 years, reaching 32 mm Hg in the right eye. His eyes appeared red for as long as he could remember, but details of his previous ophthalmic examinations were unavailable. Because of the bilateral injection he saw two other neuro-ophthalmologists, who suspected an arteriovenous fistula. One year before presentation he underwent magnetic resonance imaging (MRI) of the head and orbits and catheter arteriography on two occasions, which were both normal. He had no other previous medical problems, was taking …

Evaluate the incidence of biopsy-proven giant cell arteritis for seasonal or annual variability in the Mid-Atlantic United States.Retrospective chart review of all patients undergoing temporal artery biopsy from 1994 to 2011.The charts of 744 patients were reviewed, and 215 patients were diagnosed with giant cell arteritis based on positive temporal artery biopsy results.All results between 1994 and 2011 were reviewed. Giant cell arteritis incidence data were evaluated by year, season and month for any trends or cyclic patterns.Incidence of biopsy-proven giant cell arteritis.The majority of patients were female (74%) and over the age of 60 (98.6%). The diagnosis of biopsy-proven giant cell arteritis was found in 215 of 744 (28.9%) patients. The incidence of biopsy-proven giant cell arteritis by year varied, with the peak incidence in 1996. The monthly incidence peaked in July and had a trough in October. However, Poisson regression analysis did not show any statistically significant trend over time or cyclic pattern to the incidence by year, season or month.The incidence of biopsy-proven giant cell arteritis in the population studied did not have any significant cyclic pattern over the last 17 years. The highest incidence by month was noted in July with a trough in October. However, this was not a significant pattern by month or season to support infectious or periodic environmental factors inciting giant cell arteritis.

Purpose Temporal artery biopsy (TAB) is considered the gold standard in the diagnosis of suspected giant cell arteritis. The most commonly accepted length for an adequate postfixation TAB specimen is 20 mm. There is a reported 2.4-mm mean shrinkage with the fixation process, but to date, there is no data correlating shrinkage after specimen fixation with the biopsy results. Methods A prospective, Institutional Review Board-approved study of all patients undergoing TAB over 1 year was performed. The pre- and postfixation measurements were recorded. Comparison of the pre- and postfixation lengths was performed, and potential correlation was sought with biopsy results and patient gender, age, and race/ethnicity. Results Sixty-two TABs were performed over a 1 year period with 53 (85.5%) negative for giant cell arteritis. The mean shrinkage length was 4.61 mm ± 2.97 overall, and the amount of shrinkage between positive and negative TAB specimens was not significant (p = 0.43). Linear regression analysis did not show any correlation between the amount of shrinkage and the length of the specimen or duration in fixative. There was no significant difference between the amount of shrinkage by surgeon (p = 0.82), patient gender (p = 1.00), or race/ethnicity (p = 0.695). Conclusions Surgeons should be aware of the amount of shrinkage of TAB specimens to meet the commonly accepted goal of 20 mm postfixation length. Based on the 4.61-mm mean shrinkage with 2.97-mm standard deviation, a 27.58-mm specimen would have to be obtained to reach the 20-mm goal. Surgeon, patient age, gender, race/ethnicity, and biopsy results did not have a significant impact on the amount of TAB specimen shrinkage.

Purpose: To determine whether the "gatekeeper physician system" for evaluating neuro-ophthalmologic problems is cost effective. Methods: The authors retrospectively reviewed the records of 588 patients referred for neuro-ophthalmologic evaluation between July and December 1989 to determine the frequency and cost of unnecessary diagnostic testing ordered by "gatekeeper physicians". Pre-referral diagnostic testing costs were compared with the cost of neuroophthalmologic consultation for four common problems: (1) optic neuropathy; (2) diplopia; (3) ptosis; and (4) proptosis. Results: Between 16% and 26% of patients in the first three diagnostic categories were subjected to overtesting, resulting in $57,900 of excessive costs, a 724% overcharge. Although the evaluation of proptosis was performed correctly, the quality of 10 of the 18 neuro-imaging procedures was substandard. Conclusions: The gatekeeper system managed by primary care physicians for these four neuro-ophthalmologic problems not only did not conserve healthcare dollars but also had a negative impact on cost control. For neuro-ophthalmologic disorders, prompt subspecialty evaluation and examination appear to be a cost-effective strategy.

Leber's hereditary optic neuropathy has been considered a bilateral, sequential hereditary optic neuropathy occurring overwhelmingly in young men. Until recently the diagnosis has been based on clinical criteria: severe loss of vision associated with central scotomas and classic ophthalmoscopic findings (circumpapillary telangiectatic microangiopathy, pseudoedema of the disk with absence of staining on fluorescein angiography, tortuous vessels in the early stages and eventually optic disk pallor). In 1988 a genetic mutation associated with Leber's hereditary optic neuropathy affecting mitochondrial DNA was recognized. Subsequently other mutations have been discovered as well. The ability to identify these patients technically has allowed us to recognize cases that do not fit our previous clinical criteria. One such case is presented and the question asked is whether the findings are related to the clinical course.

Objective To describe three patients with giant cell arteritis (GCA) who suffered profound, irreversible visual loss due to delayed initiation or discontinuation of corticosteroid therapy pending results of histopathologic examination of temporal artery biopsy specimens and to review the appropriate management of GCA once the clinical diagnosis is made. Study design Case series. Setting University-affiliated eye hospital. Patients Three women with biopsy-proven GCA and bilateral visual loss due to delayed institution or discontinuation of corticosteroid therapy. Main outcome measures Visual acuity and onset, and time course of corticosteroid therapy. Results Permanent visual loss resulted from delayed initiation of corticosteroid therapy in two patients and discontinuation of therapy in one patient with clinically suspected diagnosis of GCA. In two of these patients, visual loss was preceded by amaurosis fugax and other visual disturbances, although one patient did not volunteer this information to her physician. Conclusions Giant cell arteritis may cause rapid and profound loss of vision bilaterally, often without warning. It is essential that systemic corticosteroid therapy be initiated as soon as the diagnosis of GCA is made clinically. Corticosteroid therapy should not be delayed until results of examination of the temporal artery biopsy specimen are obtained. Biopsy specimen interpretation will not be affected if the biopsy is performed within 7 to 10 days of starting therapy.