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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.

Precursor B-cell lymphoblastic lymphoma (PB-LBL) is a rare and aggressive sub-type of non-Hodgkin lymphoma (NHL) that rarely manifests in the orbit, and is exceedingly rare in adults. Here, we repo...

A 37-year-old patient presented with acute visual field loss diagnosed as non-arteritic anterior ischemic optic neuropathy in the setting of optic nerve drusen. Her visual field loss had progressed when compared to the visual field done 2 years previously. Fundus examination showed bilateral optic nerve head drusen and left retinal nerve fiber layer edema consistent with non-arteritic anterior ischemic optic neuropathy.

A 73-year-old woman presented with a 2-day history of decreased vision in here right eye. The patient had a history of giant cell arteritis successfully treated 5 years previous to this presentation. She had been off corticosteroids for 3 years. A repeat temporal artery biopsy was positive.

A 38-year-old woman presented with bilateral optic neuropathy, leg weakness, and urinary symptoms. Despite her spinal MR being normal, her serum was positive for NMO-IgG. This indicated that she likely had Devic disease.

Visual loss after nonocular surgery is a relatively uncommon complication, however reports of postoperative visual loss have been increasing over the last ten to fifteen years. 1, 2, 3, 4, 5, 6 Int...

A case of progressive optic neuropathy in a woman with a history of breast cancer is presented. Differential diagnoses including optic neuritis, infiltrative optic neuropathy, carcinomatous meningitis, and toxic optic neuropathies are discussed. Risk factors for metastatic brain lesions are also discussed.

A 42-year-old woman presents with headache and anisocoria. Gonioscopy suggested narrow angles. Ultrasound biomicroscopy confirmed the diagnosis. The patient underwent bilateral peripheral iridotomies with resolution of her symptoms. The differential diagnosis of headache and anisocoria are reviewed. Plateau iris syndrome and subacute angle closure are discussed.

To review the neuroophthalmic manifestations of cavernous and posterior communicating artery aneurysms as well as the diagnosis and treatment options for patients with these kinds of aneurysms.The natural history of cavernous aneurysms has recently been systematically followed. Comparison of coiling and clipping for posterior communicating artery aneurysms has yielded new suggestions on how to best treat these patients.The review discusses how to follow these cases, diagnose and treat, and assess for complications after treatment decisions have been made.

An 18-year-old girl presented with an acute acquired comitant esotropia. Investigation showed a brainstem glioma. Discussion of types of acute acquired comitant esotropia, differential diagnosis and neurologic work-up is given. Review of the literature involving acute acquired comitant esotropia is provided.

A 22-year-old woman presents with headache and an acquired cranial nerve VI palsy. The differential diagnosis and work-up were negative. The causes of cranial nerve VI palsy in young patients are discussed. Follow-up for patients in this age group is also discussed.

Objective To evaluate the necessity of neuroimaging in patients with acute, isolated ocular motor mononeuropathies. Methods A prospective case series evaluating diagnostic technology results in 93 patients older than 50 years with acute isolated mononeuropathies was performed. Patients were included in the study if they had new-onset diplopia with an isolated cranial neuropathy (cranial nerve III, IV, or VI palsy) and no other signs of neurologic dysfunction. All patients had gadolinium-enhanced magnetic resonance imaging (MRI). The number of patients with lesions noted on MRI and the overall cost of imaging the patients were determined. Cost analysis of the MRI was conducted using Current Procedural Terminology codes and Medicare costs in 2010 dollars. Cost utility was estimated using cost data as well as published utility values for adults with diplopia and sex-specific life tables for life expectancy in the United States. Results Four of 93 patients had lesions on MRI; however, only 1 of the 93 patients had a lesion related to the cranial mononeuropathy. The total modeled cost of imaging for these 93 patients was $131 688 to determine an underlying cause in 1 patient with no change in treatment. The estimated cost utility for the patient with a causative lesion found by MRI was $90.19 for diagnosis alone. Conclusions It may not be medically necessary to perform MRI scanning on every patient with an isolated cranial nerve III, IV, or VI palsy. In adults older than 50 years with an isolated mononeuropathy, physicians should carefully review the patients' history and findings to determine which patients to image at the initial evaluation.

A 64-year-old woman presented with bilateral optic neuropathy leading to a diagnosis of Sjogren syndrome. She improved with high-dose corticosteroids and oral azathioprine and was subsequently found to have asymptomatic bilateral iridocyclitis. Although central nervous system manifestations of Sjogren syndrome are documented in the literature, they are not widely recognized in clinical practice. Associated optic neuritis often mimics demyelinating disease such as multiple sclerosis. Treatment of CNS disease related to Sjogren syndrome is highly controversial. Uveitis is an uncommon finding associated with Sjogren syndrome.

A 46-year-old man presented with acute confusional syndrome, ataxia, dysarthria, and right hemiparesthesia. Brain MRI showed small bilateral infarcts and fluorescein angiography revealed multiple peripheral retinal infarcts bilaterally. No visual loss was present, and no other organs were involved. The diagnosis of Susac syndrome (microangiopathy of the brain, retina and cochlea) was made and immunosuppressive therapy begun.

A 58-year-old woman presented with a problem with her peripheral vision. Computed tomography scan showed an occipital hemorrhagic stroke. She subsequently suffered gastrointestinal bleeding and at surgery biopsy of a portion of the middle colic artery aneurysm revealed changes consistent with polyarteritis nodosa.

We report a patient with a long history of hydroxychloroquine use. Clinical examinations had been performed according to American Academy of Ophthalmology guidelines with no abnormalities. A multifocal electroretinogram (mfERG) was performed to further assess macular function. Multifocal ERG may detect macular dysfunction earlier than the currently recommended screening guidelines in patients with potential for macular toxicity from hydroxychloroquine.

Optic neuritis (ON) is the initial presentation in 15% to 20% of cases of multiple sclerosis (MS). Thirty-eight percent to 50% of patients with MS develop ON at some point during the course of their disease. The Optic Neuritis Treatment Trial (ONTT) provided much prospective data about the clinical presentation, clinical course with respect to treatment, and development of MS in patients with ON. The clinical course of MS initially involves episodes of demyelination followed by full recovery; however, later attacks often leave persistent deficits that lead to secondary progression of the disease. The risk of developing progressive neurologic deficits can be reduced by starting therapy with immunomodulating drugs early in the course of the disease. Optical coherence tomography is a noninvasive way to monitor patients with ON to determine if they are undergoing subclinical axonal loss of ganglion cells. Progression of axonal loss on optical coherence tomography may prompt a change in therapy or further imaging.

A 32-year-old man presented with optic neuritis (papillitis). A discussion of optic neuritis, the role of magnetic resonance imaging, and possible treatment options are presented. The role of optical coherence tomography in following patients with optic neuritis is discussed.

Fragile-X syndrome is a common form of mental retardation resulting from the inability to produce the fragile-X mental retardation protein. The specific function of this protein is unknown; however, it has been proposed to play a role in developmental synaptic plasticity. Examination of human brain autopsy material has shown that fragile-X patients exhibit abnormalities in dendritic spine structure and number, suggesting a failure of normal developmental dendritic spine maturation and pruning in this syndrome. Similar results using a knockout mouse model have previously been described; however, it was noted in retrospect that the mice used in that study may have carried a mutation for retinal degeneration, which may have affected cell morphology in the visual cortex of those animals. In this study, dendritic spines on layer V pyramidal cells of visual cortices, taken from fragile-X knockout and wild-type control mice without the retinal degeneration mutation and stained using the Golgi-Cox method, were investigated for comparison with the human condition. Quantitative analyses of the lengths, morphologies, and numbers of dendritic spines, as well as amount of dendritic arbor and dendritic branching complexity, were conducted. The fragile-X mice exhibited significantly more long dendritic spines and significantly fewer short dendritic spines than control mice. Similarly, fragile-X mice exhibited significantly more dendritic spines with an immature-like morphology and significantly fewer with a more mature type morphology. However, unlike the human condition, fragile-X mice did not exhibit statistically significant dendritic spine density differences from controls. Fragile-X mice also did not demonstrate any significant differences from controls in dendritic tree complexity or dendritic arbor. Long dendritic spines with immature morphologies are characteristic of early development or a lack of sensory experience. These results are similar to those found in the human condition and further support a role for the fragile-X mental retardation protein specifically in normal dendritic spine developmental processes. They also support the validity of these mice as a model of fragile-X syndrome.

Perioperative visual loss has been associated with many types of non-ocular surgery.1 Risk factors include combinations of prolonged surgical times, hypotension, anaemia due to blood loss, or prone positioning.2 We are not aware of another report of perioperative visual loss in which the only risk factor was hypotension. A 55 year old man had left rotator cuff surgery. He was supine, surgical time was 3 hours, and blood loss was minimal. Postoperatively he experienced “kaleidoscopic” vision in the inferior visual field of his left eye which cleared. Four weeks later he had right …

M YASTHENIAGRAVIS (MG) is an autoimmunedisorder that affects the postsynapticneuromuscular junctionmembrane.Acetylcholine receptor (AChR) antibodiesare frequentlypresent, andthe numberoffunctioningpostsynapticreceptors isreduced.Muscle-specifickinaseantibodiesmaybeidentifiedinindividuals with and without AChR antibodies. Ptosis or diplopia due to ocular muscle involvement are frequently the first symptomsof thedisease. Approximately half of the patients are first seen with purely ocularMG(OMG);of these,53%develop generalizedMG(GMG)within2years ( 80% in the first year). Furthermore,aspontaneousremissionrateof 30%hasbeenreportedinpatientswith OMG during a 15-year period. Both AChRandmuscle-specifickinaseantibodies may be present in OMG. The goal of therapy for OMG and GMG is to minimize the patient’s symptoms. Another recently discussed goal is to prevent the progression of OMG to GMG. Symptomatic treatment may be as simple as observation for patients with minimal symptoms, but some patients are uncomfortable owing to diplopia or ptosis and desire a more definitive treatment. The choice of the initial medication in patients with OMG depends on each drug’s efficacy, adverse effects, contraindications, availability, and cost. Classically, the mainstay of pharmacotherapy is acetylcholinesterase inhibitors (eg, pyridostigmine bromide). However, there is evidence that pyridostigmine may not be as effective in treating purely OMG. The clinical response is variable, but approximately 50% of patients with OMG have a good response. Ptosis tends to respond better than diplopia. The most common adverse effect is diarrhea. Very large doses occasionally lead to increased weakness from cholinergic crisis. The drug should be used with caution in patients with asthma, cardiac arrhythmia, or urinary obstruction. If treatment with this agent is unsuccessful, other approaches, such as immunosuppressive agents (typically corticosteroids) or thymectomy, may be used. Prednisone is the immunosuppressive agent most frequently used in the treatment of OMG. Most patients experience relief from ptosis and diplopia within weeks of starting prednisone therapy. The typical approach is to titrate the dose up to 1.0 mg/kg per day over several weeks. Maintenance doses at this level are continued for 6 to 12 weeks and are followed by a slow taper over several months. Treatment with prednisone longer than 2 to 4 weeks can have significant morbidity. Patients require careful monitoring for development of peptic ulcer disease, diabetes mellitus, hypertension, psychosis, insomnia, myopathy, hypokalemia, hypocalcemia, osteoporosis, cataracts, glaucoma, masking of infections, and worsening MG symptoms. The failure of OMG-related ptosis and diplopia to improve after corticosteroid therapy ranges from as low as 8 of 89 patients (9%) in one study to as high as 6 of 18 (33%) in another. Somestudies suggest thatthe useofcorticosteroidsor thymectomy astheprimarytreatmentofOMGmay decrease theriskofdevelopingGMG. The firstof thesestudies summarized 32patientswhoweretreatedforOMG with prednisone for 2 months to 1 year. Only 3 of the 32 patients (9%) went on to develop GMG, a significantlylowerpercentagethanexpected fromthenaturalhistorystudiesof the disease. How long these 3 patients who progressed to GMG had symptomsbeforestartingtreatmentwasnot specified. This is critical information because patients with ocular symptoms for more than a year are less likelytodevelopGMGwhetherornot they are treated. The majority of patients who progress to GMG do so within the first year of OMG symptom onset. Two subsequent studies included patients from a myasthenia database anddidnot specifyhowlongpatients hadsymptomsbeforestartingtherapy. Therefore,patientswhohadbeen followed up for more than 2 years with onlyocularsymptomsmayhavebeen included, skewing the likelihood of progression. Amorerecent study included only patients with ocular symptoms for less than 2 years. Thirteen of 31 patients (42%) did not progress to GMG, and 10 of these patientsunderwentimmunosuppressive therapy. Two of the 10 patients receivedazathioprinesodiuminaddition to prednisone, 3 underwent prednisoneandazathioprinetherapyplusthymectomy,and3receivedimmunosupAuthor Affiliations: Neuroophthalmology Service, Wills Eye Institute (Drs Gilbert and Savino), and Department of Neurology, Thomas Jefferson University (Dr De Sousa), Philadelphia, Pennsylvania. Dr Gilbert is now with the Department of Neuro-Ophthalmology, Illinois Eye and Ear Infirmary, Chicago.