Neuro-ophthalmic Examination Research Articles

Trial End Points and Natural History in Patients With G11778A Leber Hereditary Optic Neuropathy

IMPORTANCE Establishing the natural history of G11778A Leber hereditary optic neuropathy (LHON) is important to determine the optimal end points to assess the safety and efficacy of a planned gene therapy trial. OBJECTIVE To use the results of the present natural history study of patients with G11778A LHON to plan a gene therapy clinical trial that will use allotopic expression by delivering a normal nuclear-encoded ND4 gene into the nuclei of retinal ganglion cells via an adeno-associated virus vector injected into the vitreous. DESIGN, SETTING, AND PARTICIPANTS A prospective observational study initiated in 2008 was conducted in primary and referral institutional practice settings. Participants included 44 individuals with G11778A LHON, recruited between September 2008 and March 2012, who were evaluated every 6 months and returned for 1 or more follow-up visits (6-36 months) as of August 2012. EXPOSURES Complete neuro-ophthalmic examination and main measures. MAIN OUTCOMES AND MEASURES Visual acuity, automated visual field testing, pattern electroretinogram, and spectral-domain optical coherence tomography. RESULTS Clinical measures were stable during the follow-up period, and visual acuity was as good as or better than the other visual factors used for monitoring patients. Based on a criterion of 15 or more letters from the Early Treatment Diabetic Retinopathy Study chart, 13 eyes of 8 patients (18%) improved, but 24 months after the onset of symptoms, any further improvements were to no better than 20/100. Acuity recovery occurred in some patients despite continued marked retinal nerve fiber layer thinning indistinguishable from that in patients who did not recover visual acuity. CONCLUSIONS AND RELEVANCE Spontaneous improvement of visual acuity in patients with G11778A LHON is not common and is partial and limited when it occurs, so improvements in vision with adeno-associated virus-mediated gene therapy of a synthetic wild-type ND4 subunit gene should be possible to detect with a reasonable sample size. Visual acuity appears to be the most suitable primary end point for the planned clinical trial.

Selective retinal ganglion cell loss in familial dysautonomia

To define the retinal phenotype of subjects with familial dysautonomia (FD). A cross-sectional study was carried out in 90 subjects divided in three groups of 30 each (FD subjects, asymptomatic carriers and controls). The study was developed at the Dysautonomia Center, New York University Medical Center. All subjects underwent spectral domain optical coherence tomography (OCT) and full neuro-ophthalmic examinations. In a subset of affected subjects, visual evoked potentials and microperimetry were also obtained. We compared the retinal nerve fiber layer (RNFL) thickness from OCT between the three groups. OCT showed loss of the RNFL in all FD subjects predominantly in the maculopapillary region (63 % temporally, p < 0.0001; and 21 % nasally, p < 0.005). RNFL loss was greatest in older FD subjects and was associated with decreased visual acuity and color vision, central visual field defects, temporal optic nerve pallor, and delayed visual evoked potentials. Asymptomatic carriers of the FD gene mutation all had thinner RNFL (12 % globally, p < 0.005). OCT and clinical neuro-ophthalmological findings suggest that maculopapillary ganglion cells are primarily affected in FD subjects, leading to a specific optic nerve damage that closely resembles mitochondrial optic neuropathies. This raises the possibility that reduced IKAP levels may affect mitochondrial proteins and their function in the nervous system, particularly in the retina.

English

A 50 year old lady with past history of breast carcinoma surgery presented with progressive diplopia of 15 days duration. Examination revealed paresis of right abducens nerve. Though risk factor like Hypertension was present, patient was ordered MRI whic h showed Clivus and vertebral metastatic foci highly suggestive of metastasis from breast carcinoma. The patient was referred for radiation therapy. Hence, meticulous neuroophthalmic examination and management is necessary to rule out localised metastasis causing isolated abducens nerve palsy.

Asymptomatic and Symptomatic Postoperative Visual Dysfunction After Cardiovascular Surgery With Cardiopulmonary Bypass: A Small-Sized Prospective Observational Study

Postoperative visual dysfunction (POVD) after cardiovascular surgery rarely is reported, since it is more likely underdetected and underreported. This study was designed to verify the presence of POVD, including a variety of asymptomatic as well as symptomatic visual dysfunctions after cardiovascular surgery with cardiopulmonary bypass (CPB). A prospective observational study. Cardiothoracic surgery in a medical university hospital. Seventy-one patients undergoing elective cardiovascular surgery with CPB. None. All patients were assessed by a battery of 7 neuro-ophthalmic examinations preoperatively and postoperatively, including fundus, visual field, eye movement, color vision, visual acuity, intraocular pressure, and critical flicker frequency. Patients were considered to have POVD if they had postoperative new abnormal findings of neuro-ophthalmic examinations. One patient was excluded due to a failure of postoperative neuro-ophthalmic examinations. In 16 of 70 patients analyzed in this study, selective cerebral perfusion was required for aortic arch surgery. Of 70 patients, a total of 8 patients (11.4%) had postoperative new abnormal findings in neuro-ophthalmic examinations, including new visual field deficits in 4, reduced visual acuity in 4, and/or increased intraocular pressure in 1 patient. Of these 8 patients, symptomatic POVD was recognized in 1 patient (1.4%) with postoperative visual field deficit and reduced visual acuity. There were no new abnormal findings compared with preoperative results in postoperative funduscopy, eye movement, color vision, and critical flicker frequency. These results indicated that the asymptomatic as well as symptomatic POVD can develop after cardiovascular surgery with CPB, and their incidence may be relatively high.

Magnetic Resonance Imaging in Acute Unilateral Optic Neuropathies in Adults

Non-arteritic ischaemic optic neuropathy (NAION) and optic neuritis may have overlapping presentations, making differentiation between the two difficult. All adults presenting with acute unilateral optic neuropathy (symptoms <3 weeks of onset) between January 2011 and January 2012 underwent complete neuro-ophthalmic examinations and were diagnosed with either NAION or ON. Patients underwent pre- and post-contrast magnetic resonance (MR) with gadolinium, diffusion-weighed imaging (DWI), T2, short tau inversion recovery (STIR), and gadolinium-enhanced fast imaging employing steady state acquisition (FIESTA) sequences of the brain and orbit. A blinded neuroradiologist analysed the images as normal, consistent with NAION, consistent with ON, or unrelated incidental findings and the results were correlated to clinical diagnoses on initial presentation. Sixteen patients were included, 4 of whom had optic neuritis and 12 had NAION. Concordance rate for the blinded neuroradiological diagnosis and the final clinical diagnosis was seen in only 3 of the 16 patients. Nonspecific or unrelated findings were seen in 6 cases. Our study demonstrates a low concordance rate between radiographic and final clinical diagnoses, with only 3/16 patients correctly diagnosed by blinded neuroradiological evaluation. Despite the limitations of our study, we believe that neuroradiologists can assist clinicians in the final diagnosis of in patients in whom the clinical presentation is uncertain. Future work is necessary to determine whether more rapid imaging in these cases might expand the utility of DWI in optic neuropathy.

The Diagnostic and Economic Yield of Neuroimaging in Neuro-ophthalmology

Diagnostic studies such as computed tomography scans (CT) and magnetic resonance imaging (MRI) are ordered frequently in neuro-ophthalmic practice, although the diagnostic yield and cost-effectiveness of these tests have been studied for only a few conditions. We assessed the diagnostic and economic yield of CT and MRI across all patients evaluated in a neuro-ophthalmology practice. This retrospective review included all patients referred by the division of neuro-ophthalmology at the Scheie Eye Institute for CT, CT angiography, MRI, MRA, or magnetic resonance venography over a 12-month period. Abnormal imaging findings were categorized as significant (one that elicited changes in management) and/or relevant (one that related to the patient's neuro-ophthalmic complaint or examination findings). The diagnostic yield of the test ordered was analyzed according to the patient's chief complaint, neuro-ophthalmic examination findings, and indication for imaging. The total costs for each diagnostic group and costs per significant finding were calculated using the global Resource-Based Relative Value Units for each examination from the Centers for Medicare and Medicaid Services Web site. Two hundred eleven imaging studies in 157 patients were evaluated. 28.9% (95% confidence interval, 22.5%-36.2%) of imaging studies had significant abnormalities relevant to the neuro-ophthalmic complaint. Imaging obtained for evaluation of progressive optic nerve dysfunction and cranial nerve palsy had statistically significant higher diagnostic yield than studies performed for other reasons. Total cost of all imaging studies performed was $107,615.72. Cost per clinically significant and relevant finding was $1,764.19. In comparison to the diagnostic yield of neuroimaging studies in other specialties, CT and MRI of the brain requested by neuro-ophthalmologists provide significant and relevant data at a reasonable cost.

Neuroimaging in Patients Referred to a Neuro-ophthalmology Service: The Rates of Appropriateness and Concordance in Interpretation

Neuroimaging studies frequently are ordered to investigate neuro-ophthalmic symptoms. When misused, these studies are expensive and time consuming. This study describes the type and frequency of neuroimaging errors in patients referred to an academic neuro-ophthalmology service and measures how frequently these neuroimaging studies were reinterpreted. Prospective cohort study. Eighty-four consecutive patients referred to an academic neuro-ophthalmology practice. From November 2009 through July 2010, 84 consecutive new patients who had undergone a neuroimaging study in the last 12 months specifically to evaluate their presenting neuro-ophthalmic symptoms were enrolled prospectively. Participants then underwent a complete neuro-ophthalmic evaluation, followed by a review of prior neuroimaging. Questions regarding appropriateness of the most recent imaging, concordance of radiologic interpretation, and re-evaluation of referring diagnoses were answered by the attending physician. The frequency and types of errors committed in the use of neuroimaging and the frequency of reinterpretation of prereferral neuroimaging studies after neuro-ophthalmic history and examination. Most study participants (84.5%; 71/84) underwent magnetic resonance imaging before referral; 15.5% (13/84) underwent only computed tomography. The rate of suboptimal neuroimaging studies was 38.1% (32/84). The 3 most common reasons for suboptimal studies were incomplete area of imaging (34.4%; 11/32), wrong study type (28.1%; 9/32), and poor image quality (21.9%; 7/32). Twenty-four of 84 subjects (28.6%) required additional neuroimaging. The authors agreed with the radiology interpretation of the prior neuroimaging studies in most patients (77.4%; 65/84). The most common anatomic locations for discordance in interpretation were the intraorbital optic nerve (35%; 7/20) and the brainstem (20%; 4/20). There was a high rate of suboptimal neuroimaging studies performed in patients referred for neuro-ophthalmology examination. These findings have significant implications given the increasing attention to resource use currently and in the near future.

Predicting dysthyroid optic neuropathy using computed tomography volumetric analyses of orbital structures

OBJECTIVE:To evaluate the ability of orbital apex crowding volume measurements calculated with multidetector-computed tomography to detect dysthyroid optic neuropathy.METHODS:Ninety-three patients with Graves' orbitopathy were studied prospectively. All of the patients underwent a complete neuro-ophthalmic examination and computed tomography scanning. Volumetric measurements were calculated from axial and coronal contiguous sections using a dedicated workstation. Orbital fat and muscle volume were estimated on the basis of their attenuation values (in Hounsfield units) using measurements from the anterior orbital rim to the optic foramen. Two indexes of orbital muscle crowding were calculated: i) the volumetric crowding index, which is the ratio between soft tissue (mainly extraocular muscles) and orbital fat volume and is based on axial scans of the entire orbit; and ii) the volumetric orbital apex crowding index, which is the ratio between the extraocular muscles and orbital fat volume and is based on coronal scans of the orbital apex. Two groups of orbits (with and without dysthyroid optic neuropathy) were compared.RESULTS:One hundred and two orbits of 61 patients with Graves' orbitopathy met the inclusion criteria and were analyzed. Forty-one orbits were diagnosed with Graves' orbitopathy, and 61 orbits did not have optic neuropathy. The two groups of orbits differed significantly with regard to both of the volumetric indexes (p<0.001). Although both indexes had good discrimination ability, the volumetric orbital apex crowding index yielded the best results with 92% sensitivity, 86% specificity, 81%/94% positive/negative predictive value and 88% accuracy at a cutoff of 4.14.CONCLUSION:This study found that the orbital volumetric crowding index was a more effective predictor of dysthyroid optic neuropathy than previously described computed tomography indexes were.

Pituitary Apoplexy Presenting Atypical Time Course of Ophthalmic Symptoms

Pituitary apoplexy is defined as a sudden loss of blood supply to the pituitary gland, leading to tissue necrosis and hemorrhage. Its clinical symptoms are characterized by sudden onset of headache, nausea, vomiting, ophthalmic symptoms and hormonal dysfunction. A 65-year-old woman presented with left-sided ptosis and blurred vision. These ophthalmic symptoms gradually worsened for one month without headache, visual acuity and field deficit. Neuro-ophthalmic examination revealed left oculomotor nerve palsy. Magnetic resonance imaging (MRI) revealed a round mass lesion in the left cavernous sinus, which was initially suspected as thrombosed cerebral aneurysm or hemorrhagic Rathke's cleft cyst. The mass lesion was finally diagnosed as pituitary apoplexy. The patient underwent trans-sphenoidal surgery and oculomotor nerve palsy improved after the surgery. Early diagnosis and treatment including surgical decompression are crucially important in patients with oculomotor nerve palsy in pituitary apoplexy, but the symptoms of pituitary apoplexy may slowly progress. It should be noted that pituitary apoplexy could be misdiagnosed as cerebral aneurysm or Rathke's cleft cyst.

Recurrent nasal polyp and pansinus mucopyocele associated with bilateral blindness: A case report

A 39 year old female with 24-month history of recurrent mucoid rhinorhea associated with progressive bilateral nasal blockage, headache, anosmia and bilateral impaired vision is reported. There was a past history of sinus surgery. The neuro-ophthalmic examination revealed left non-axial proptosis and visual acuity of nil light perception in both eyes at 3 meters. CT scan of the paranasal sinuses and brain showed markedly expanded left frontal sinus with sclerotic walls and expanded mixed density lesion in all the sinuses. She had surgery via trans-nasal, external and skull base approaches for effective clearance of the polypoidal masses and drainage of the sinus mucopyocele. Histological examination confirmed chronic inflammatory polyp. She remains stable to date although visual impairment and anosmia remained persistent. Patients with nasal polyposis require frequent follow-up even after surgical treatment for the early detection and management of complications to avoid consequences such as visual loss.

Pseudotumor cerebri and ciprofloxacin: a case report

IntroductionWe present a case of ciprofloxacin-associated pseudotumor cerebri in a 22-year-old African American woman. Withdrawal of ciprofloxacin in our patient resulted in complete resolution of ciprofloxacin-associated pseudotumor, as evidenced by a normal neuro-ophthalmic examination and a cerebrospinal fluid opening pressure of 140 mmH20.Case presentationA 22-year-old African American woman presented with a headache of two weeks duration, visual blurring and horizontal diplopia after starting ciprofloxacin for pyelonephritis. An ophthalmic examination revealed that she had left eye esotropia, and a picture of the fundus demonstrated bilateral disc swelling without spontaneous venous pulsations. Magnetic resonance imaging of the brain and a magnetic resonance venogram were normal. A diagnostic lumbar puncture demonstrated an elevated opening pressure of 380mmH2O in a supine position. Laboratory examinations, including a cerebrospinal fluid exam, were unremarkable.Conclusionciprofloxacin-associated pseudotumor can cause chronic disabling headache and visual complications. Therapy is sub-optimal, often symptomatic, insufficient and complicated by side effects. When ciprofloxacin-associated pseudotumor presents in an atypical population, an inciting agent must be suspected because prompt withdrawal of the agent may lead to complete resolution of symptoms and prevent recurrence of similar episodes.

Long-term visual outcome in idiopathic intracranial hypertension

Objective:To characterize the course, outcome, and risk of relapse or late worsening in a clearly defined cohort of idiopathic intracranial hypertension (IIH) over a long period of follow-up.Materials and Methods:Retrospective chart review of patients with definite IIH was evaluated at the Sree Chitra Tirunal Institute for Medical Sciences and Technology between 1998 and 2006. Patients’ demographic data, clinical, neuro-ophthalmic examinations, and treatment details were abstracted. Patients were further categorized into three groups based on whether they improved, worsened, or relapsed on follow-up. Final visual outcome of each patient was defined according to grading of the worse eye at the last visit. Statistical analysis included t test to compare group means and chi-square test to compare proportions.Results:Of the 43 women included, visual impairment was observed in 80 eyes (93%) at presentation and it was moderate to severe in 14%. The mean CSF opening pressure at presentation did not differ significantly in those with visual impairment compared to those with normal vision. Those having early severe visual impairment had significantly higher (P = 0.015) likelihood of severe visual impairment on last follow-up. Of the total, 34 patients (79%) improved, 4 (9.3%) relapsed on follow-up after period of stability, and 5 (11.6%) worsened over 56 months follow-up (range, 26-132 months). The groups were comparable, except those who improved were younger (P<0.05). At last examination, 9% had significant vision loss.Conclusion:IIH patients can have delayed worsening or relapses and about tenth of patients can have permanent visual loss early or late in the course of the disease. All patients with IIH need to be kept under long-term follow-up, with regular monitoring of visual functions.

Unilateral Facial Paresis Secondary to a Suspected Brainstem Arachnoid Cyst in a Maltese Dog

An 8-year-old, intact female Maltese dog was presented with decreased tear production and unilateral loss of eye blinking. Neuro-ophthalmic examination and brain magnetic resonance imaging were performed to determine the origin of facial paresis. A cystic lesion in the left pontomedullary region which displayed equal intensity to cerebrospinal fluid was revealed. Hyposignality was noted on fluid attenuated inversion recovery sequences, and the lesion was suggestive of an arachnoid cyst. This report described unilateral facial nerve dysfunction that resulted from a suspected brainstem arachnoid cyst in an unusual anatomic location.

Leber Hereditary Optic Neuropathy Gene Therapy Clinical Trial Recruitment

To describe the patient profiles of the Leber hereditary optic neuropathy (LHON) Gene Therapy Clinical Trial, year 1. This study aims to identify and characterize affected patients and carriers with the G11778A mutation in mitochondrial DNA for planned gene therapy that will use "allotopic expression" by delivering a normal nuclear-encoded ND4 gene into the nuclei of retinal ganglion cells via an adeno-associated virus vector injected into the vitreous. Patients with LHON with visual loss as well as asymptomatic maternally related family members were molecularly screened for ND1, ND4, and ND6 mutations in mitochondrial DNA commonly associated with LHON. All patients and maternal relatives also underwent complete neuro-ophthalmic examination, automated visual field testing, pattern electroretinogram (PERG), and OCT3. Twenty-five subjects with LHON and 21 carriers positive for the G11778A mitochondrial DNA mutation were recruited. Three additional mutations in the ND4 gene, G11719A, G11947A, or G11914A, were detected. Mean retinal nerve fiber layer (RNFL) thickness was 78.3 μm up to 32 months after visual loss. It was 63.5 μm for all affected patients and 100.7 μm for carriers (P < .01). Mean PERG amplitude was lower in affected patients (40% of normal) than in carriers (94% of normal) (P < .01). Four carriers with PERG amplitudes less than 75% of normal had Early Treatment Diabetic Retinopathy Study acuity more than 20/25, mean defect more than -2 dB, and average RNFL thickness more than 80 μm. Potential candidates for future gene therapy may include affected patients, as late as 32 months after loss of vision, with mildly reduced RNFL thickness or carriers with low PERG amplitudes and normal RNFL thickness, if the PERG amplitude is a predictor of conversion to LHON in these carriers.

Predictive factors for the development of visual loss in patients with pituitary macroadenomas and for visual recovery after optic pathway decompression

Objective: To investigate clinical and MRI findings that are predictive of both visual loss in patients with pituitary adenomas and visual recovery after treatment. Design: Cohort study. Participants: Thirty patients (60 eyes) with pituitary adenoma. Methods: Patients underwent neuro-ophthalmic examination and MRI before and after optic chiasm decompression. Visual field (VF) was assessed using the mean deviation in standard automated perimetry (SAP) and temporal mean defect, the average of 22 temporal values of the total deviation plot. Tumour size was measured on sagittal and coronal cuts. Results: Visual loss was found in 47 eyes; 35 had optic atrophy (subtle in 9, moderate in 14, and severe in 12). Before treatment, the average SAP mean deviation and temporal mean defect were − 11.78 (SD 8.56) dB and − 18.66 (SD 11.20) dB, respectively. The chiasm was 17.3 (SD 6.2, range 10–34) mm above the reference line on the sagittal and 21.8 (SD 8.3, range 12–39) mm on the coronal images. Tumour size correlated with the severity of VF defect. VF improvement occurred in 80% of eyes after treatment. The degree of optic atrophy, visual loss, and tumour size were significantly associated with improvement after treatment. Conclusions: The best predictive factor for visual loss was tumour size, and factors related to visual recovery were the degree of optic atrophy, the severity of VF defect, and the tumour size. Diagnosing pituitary adenomas before optic atrophy becomes severe may be related to a better prognosis in such patients.

Neuro-ophthalmology: A review

Evaluation of horses for eye problems and for suspected impairment of visual acuity often requires a thorough neuroophthalmic examination to be conducted. Along with a full ophthalmic evaluation, the results of such an examination should indicate the likely site of any lesion(s) accounting for blindness, miosis, mydriasis, anisocoria, Horner's syndrome, strabismus, facial paralysis and facial spasm, nystagmus and other signs of vestibular disease, and cerebellar disorders, that may be present. Such a thorough examination with guidance for correct interpretation of findings is given here.

Evaluation of retinal nerve fiber layer thickness measurements using optical coherence tomography in patients with tobacco-alcohol-induced toxic optic neuropathy

Three patients with progressive visual loss, chronic alcoholism and tabagism were submitted to a complete neuro-ophthalmic examination and to retinal nerve fiber layer (RNFL) measurements using optical coherence tomography (OCT) scanning. Two patients showed marked RNFL loss in the temporal sector of the optic disc. However, a third patient presented RNFL measurements within or above normal limits, based on the Stratus-OCT normative database. Such findings may be due to possible RNFL edema similar to the one that may occur in the acute phase of toxic optic neuropathies. Stratus-OCT was able to detect RNFL loss in the papillomacular bundle of patients with tobacco-alcohol-induced toxic optic neuropathy. However, interpretation must be careful when OCT does not show abnormality in order to prevent diagnostic confusion, since overestimation of RNFL thickness measurements is possible in such cases.

Altered vision during motion: an unusual symptom of cerebellar dysfunction, quantifiable by a simple clinical test

To report a series of patients with cerebellar dysfunction and altered vision during motion, and to quantify their visual impairment in motion with a simple clinical test. Twenty consecutive patients suffering from cerebellar dysfunction and altered vision during motion were examined between 1994 and 2007. A control group consisted of 20 age- and sex-matched healthy people. All patients had a full neuro-ophthalmic examination. Near visual acuity (NVA) was measured at rest (static NVA) and during chair rotation (dynamic NVA). Distance visual acuity (DVA) was measured at rest (static DVA) and during rotation of the patient's head (dynamic DVA). Only four of the 20 patients reported altered vision during motion spontaneously. The remaining 16 patients admitted this unusual visual disturbance only when asked specifically. All patients exhibited abnormal eye movements, including saccadic smooth pursuit (20/20), dysmetric saccades (15/20), nystagmus (19/20) and impaired suppression of vestibulo-ocular reflex (VOR) (20/20). During rotation of the examination chair (dynamic NVA), the drop in NVA averaged 5.6 lines (range 1-10 lines). During rotation of the patient's head (dynamic DVA), the drop in DVA averaged only 2.5 lines (range 0-10 lines). For the control group, there was no significant drop in NVA under dynamic conditions. Patients with cerebellar dysfunction rarely complain spontaneously of altered vision during motion. However, specific questioning may bring up this unusual symptom. The use of a simple clinical test, consisting of NVA measurement during rotation of the examination chair (dynamic NVA), allows practitioners to quantify the level of visual impairment in patients presenting altered VOR modulation.

The Role of Spontaneous Venous Pulsations in the Diagnosis of Adult Chiari Malformation

Objective: To perform neuro-ophthalmologic evaluations in patients with symptomatic Adult Chiari Malformations (ACM).Methods: Observational case series of 56 patients with symptoms and neuro-imaging consistent with ACM. Comprehensive histories and complete neuro-ophthalmic examinations were performed. Findings: Sixteen men and forty women were evaluated. The mean age was 41 years old (range 8 - 73 years old). The most common visual complaint was transient visual obscurations lasting seconds (n= 34). Pain or fullness behind the eyes was present in 32 patients. Forty-eight patients had headaches. Other frequent symptoms included neck pain, dizziness, facial pain, and numbness. Fundus examination was remarkable for absent spontaneous venous pulsations in one or both eyes in 28 patients. Interpretation: Adult Chiari Malformation (ACM) presents with characteristic symptoms that may be overlooked or misdiagnosed. Neuro-ophthalmic evaluation is helpful in ruling out other entities and identifying characteristic absent venous pulsations.

Characteristics of patients presenting with ocular pain

Background: The purpose of this study was to assess the utility of neuroimaging in patients with normal neuro-ophthalmic examinations referred to a neuro-ophthalmology clinic for complaints of chronic isolated eye pain. We were also interested in the prevalence of primary headache syndromes in these patients, particularly medication overuse headache (MOH). Methods: Charts of 125 consecutive patients referred to a neuro-ophthalmology clinic with some form of head or facial pain were reviewed to find patients meeting the criteria of having complaints of chronic eye with normal neuro-ophthalmic examinations. Excluded were patients with primary complaint other than eye pain. A single examiner performed all examinations in a standardized fashion. Each patient was queried about the use of prescription and over-the-counter analgesics. Results: Seventy-three percent of patients had some form of primary headache syndrome, and 21% of patients had some component of probable MOH. Another 4.5% had a presumed component of medication overuse. None of those who received imaging had any results explaining the ocular pain. Interpretation: Primary headache is present in most patients with chronic, isolated ocular pain. MOH is common and probably underrecognized in this population. It needs to be addressed if the patient is ever to be pain free. Neuroimaging has very low yield in this group of subspecialty referral patients.