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Biomedical subjects

Robert C Sergott

Publications and source records attributed to Robert C Sergott.

At least 19 recordsLinked to original sources

Optic-nerve gliomas, chiasmal gliomas and neurofibromatosis type 1.

PURPOSE OF REVIEW: To review the most recent literature describing the natural history and disease progression patterns of optic-pathway gliomas in neurofibromatosis type 1 (NF1) patients. To aid in clarifying the current treatment patterns and follow-up recommendations. RECENT FINDINGS: Contrary to prior documentation, current literature reveals that optic-pathway gliomas in NF1 can be diagnosed after the age of 6, and may progress until the age of 12. The disease progression occurs most frequently in the first two years following diagnosis. SUMMARY: Optic-pathway gliomas in NF1 can display a variety of manifestations and exhibit an unpredictable disease course. No specific characteristics have been found thus far to predict an aggressive compared with indolent disease course. Recently primary diagnoses of optic-pathway gliomas have been made in children aged six or older, and have been shown to progress until the age of 12. Although large-scale studies are required to change current follow-up recommendations, the data suggest that NF1 patients should be vigilantly evaluated for optic-pathway gliomas past the age of 12.

Humans↗

Pathological optic-disc cupping.

PURPOSE OF REVIEW: Pathological optic-disc cupping is most often caused by glaucoma, but may be seen in many less-common neuro-ophthalmic conditions. The goal of this article is to examine a host of entities causing optic-disc cupping, present key differentiating characteristics and pathophysiologies, and outline diagnostic approaches. RECENT FINDINGS: Multiple entities not associated with elevated intraocular pressure or glaucomatous optic-nerve disease may result in pathologic optic-nerve excavation. Even with the photography and imaging of today, it is still difficult for the clinician to accurately diagnose other causes of optic-disc cupping. Up to 20% of patients may be misdiagnosed and treated for glaucoma due to misinterpretation of the optic-disc cupping. Newer forms of imaging including optical coherence tomography may assist the clinician in decision making. A scrutinizing history, close observation of disc appearance, and the vasculature will aid in the diagnosis of glaucoma or other entity of optic-disc cupping. SUMMARY: Optic-disc cupping is a consequence of myriad disorders. Knowledge of the anatomy and vasculature of the disc is quintessential to the understanding of how, why, when, and what type of optic-disc cupping occurs in various conditions. Cupping can be seen with neurological processes, including benign tumors, which are treatable. Patient history, visual fields assessment, and funduscopic findings are the key to unlocking the diagnosis of glaucomatous versus nonglaucomatous optic-disc cupping. As clinicians, we must remain vigilant and receptive to the findings of potentially ominous forms of nonglaucomatous optic-disc cupping.

Diagnosis, Differential↗

Nonarteritic anterior ischemic optic neuropathy in patients with sleep apnea while being treated with continuous positive airway pressure.

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.

Continuous Positive Airway Pressure↗

Isolated optic nerve lymphoma diagnosed by optic nerve biopsy.

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.

Aged↗

Optical coherence tomography: measuring in-vivo axonal survival and neuroprotection in multiple sclerosis and optic neuritis.

PURPOSE OF REVIEW: This review considers the latest developments in the use of optical coherence tomography in neuro-ophthalmology. RECENT FINDINGS: Optical coherence tomography can accurately and reproducibly quantitate the micron thickness of the peripapillary retinal nerve fiber layer, as well as the thickness and volume of the macula. It is able to perform both cross-sectional and longitudinal studies in patients with multiple sclerosis and optic neuritis. It is able to measure and assess axonal preservation and protection when used in clinical trials. SUMMARY: Specific guidelines when undertaking optical coherence tomography analyses for patients with multiple sclerosis and optic neuritis are needed to ensure uniformity among clinical trials; this development would be similar to the parameters devised when magnetic resonance imaging emerged as an important technology.

Axons↗

Surgical treatment of trauma-induced periodic alternating nystagmus.

PURPOSE: To report a new treatment for acquired periodic alternating nystagmus in which medical therapy with baclofen was not tolerated. DESIGN: Interventional case report and literature review. INTERVENTION: Simultaneous adjustable bilateral retroequatorial horizontal rectus muscle recessions. MAIN OUTCOME MEASURES: Ocular alignment and ocular motility recordings. RESULTS: A 30-year-old man had blurred vision after an emergency left temporal craniotomy for gunshot wounds. Ophthalmologic examination revealed periodic alternating nystagmus that was documented by an infrared electronystagmogram. Baclofen was started but was not tolerated. Bilateral lateral and medial retroequatorial rectus muscle recessions were performed and were successful in the treatment of this patient. CONCLUSION: Simultaneous bilateral retroequatorial horizontal rectus recessions may be an effective treatment for intractable acquired periodic alternating nystagmus.

Adult↗

Orbital radiotherapy for thyroid-related orbitopathy.

PURPOSE OF REVIEW: Orbital radiotherapy has been one of the primary treatment modalities for moderate to severe thyroid-related orbitopathy. In this review the authors discuss the role of orbital radiotherapy in this disease with particular emphasis on questions raised by recent studies. RECENT FINDINGS: Despite its widespread use, until recently there have been few well-designed, randomized clinical trials on the role of orbital radiotherapy for thyroid-related orbitopathy. Although most the literature points to a favorable effect of orbital radiotherapy, recent studies have provided us with conflicting results. SUMMARY: Orbital radiotherapy remains a safe and a widely used treatment option for severe and progressive thyroid-related orbitopathy. More randomized clinical trials are needed, especially to examine the role of orbital radiotherapy for severe thyroid-related orbitopathy characterized by severe congestion and/or compressive optic neuropathy with and without the use of corticosteroids. There is a wide consensus among clinicians that this subgroup of patients is the most frequent one to which orbital radiotherapy is offered.

Chemotherapy, Adjuvant↗

The antiphospholipid antibody syndrome: diagnostic aspects.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.

Antiphospholipid Syndrome↗

Visual loss despite anticoagulation in radiation-induced optic neuropathy.

Radiation therapy to the brain may produce necrosis and loss of function months after completion of the treatment. The case is presented of a 62-year-old man who developed radiation-induced optic neuropathy 2 years after treatment for a glioma of the left temporal lobe, despite being on anticoagulation therapy. Although anticoagulation appears to be beneficial in cerebral radiation necrosis, its usefulness in the treatment of radiation-induced optic neuropathy is unclear.

Anticoagulants↗

Recovery of visual function in patients with biopsy-proven giant cell arteritis.

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.

Aged↗

Use of methotrexate in sarcoid-associated optic neuropathy.

OBJECTIVE: To demonstrate the possible beneficial effects of methotrexate (MTX) therapy for patients with sarcoid-associated optic neuropathy (SAON). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Three patients diagnosed with SAON who received MTX. Two patients had tissue biopsies consistent with sarcoidosis, and 1 patient had clinically diagnosed sarcoidosis based on laboratory and radiographic studies. All 3 patients developed side effects with corticosteroid treatment of their optic neuropathy. INTERVENTION: Patients were treated with weekly doses of oral MTX and monitored with neuro-ophthalmic, medical, and laboratory examinations. MAIN OUTCOME MEASURES: Visual acuity, automated perimetry, and reduction of oral prednisone therapy. RESULTS: After initiation of MTX, all 3 patients showed an improvement or stabilization of visual acuity. All patients had a decrease in their corticosteroid requirements, and all had improved or stabilized visual field deficits. One of the 3 patients developed leukopenia that necessitated a reduction of the methotrexate dose. CONCLUSION: Methotrexate may be effective for SAON as an adjunct to corticosteroid therapy or as an alternative for corticosteroid-intolerant patients. Oral MTX reduced the corticosteroid requirements of 3 patients with SAON, and all 3 demonstrated stable or improved visual function.

Adult↗

Scanning laser polarimetry of the retinal nerve fiber layer in central retinal artery occlusion.

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.

Adult↗

Ischemic optic neuropathy as the first manifestation of elevated cholesterol levels in young patients.

PURPOSE: To investigate the relationship between idiopathic nonarteritic ischemic optic neuropathy (NAION) and serum lipid levels in patients </= 50 years of age. DESIGN: Case-control study. PARTICIPANTS: Thirty-seven consecutive patients with NAION and 74 age- and gender-matched comparison patients. METHODS AND MAIN OUTCOME MEASURES: Serum lipid levels and the presence of several historical diseases and exposures were abstracted from the medical records in cases and controls. RESULTS: The mean total cholesterol level was significantly increased in patients with NAION compared with controls (235.4 vs. 204.0 mg/dl, P < 0.001). 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. CONCLUSIONS: 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.

Adult↗

Pseudotumor cerebri.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.

Acetazolamide↗

Embolic central retinal artery occlusion detected by orbital color Doppler imaging.

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.

Aged↗