Validity of the American College of Rheumatology criteria for the diagnosis of 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.

Bilateral optic disk swelling plus.

A 64-year-old woman presented with bilateral optic neuropathy leading to a diagnosis of Sjögren 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 Sjögren 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 Sjögren syndrome is highly controversial. Uveitis is an uncommon finding associated with Sjögren syndrome.

Neuroimaging and acute ocular motor mononeuropathies: a prospective study.

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.

Retinal infarcts in a patient with an acute confusional 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 woman with unilateral visual loss and bilateral disk edema.

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.

Missing the bull's eye.

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.

New directions in optic neuritis and multiple sclerosis.

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.

Migraine and anisocoria.

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.

Double vision worth a double take.

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.

Intracranial aneurysms.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: The review discusses how to follow these cases, diagnose and treat, and assess for complications after treatment decisions have been made.

Too young to...

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.

New ways to look at an old problem.

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.

Dendritic spine and dendritic field characteristics of layer V pyramidal neurons in the visual cortex of fragile-X knockout mice.

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.