Jade S. Schiffman

Medical and surgical management of idiopathic intracranial hypertension in pregnancy

Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion with elevated cerebrospinal fluid (CSF) pressure but otherwise normal CSF composition. It has been found that pregnancy occurs in IIH patients at about the same rate as in the general population, that IIH can occur in any trimester of pregnancy, that patients have the same spontaneous abortion rate as the general population, and that the visual outcome is the same as for nonpregnant patients with IIH. Although it is also stated that pregnant patients with IIH should be managed and treated the same way as any other patient with IIH, the use of imaging and drug contraindications do make a difference between the two groups. The treatment has two major goals, which are to preserve vision and to improve symptoms. The medical therapy includes weight control, nonketotic diet, serial lumbar punctures, diuretics, steroids, and certain analgesics. When medical therapy fails, surgical procedures should be considered. The two main procedures are optic nerve sheath fenestration and lumboperitoneal shunt. Anesthetic considerations in the pregnant patient are an additional factor when surgeries are contemplated. It is also noted that therapeutic abortion to limit progression of disease is not indicated and that subsequent pregnancies do not increase the risk of recurrence.

Comparison of multifocal visual evoked potential, standard automated perimetry and optical coherence tomography in assessing visual pathway in multiple sclerosis patients

Background: Multifocal visual evoked potentials (mfVEP) measure local response amplitude and latency in the field of vision. Objective: To compare the sensitivity of mfVEP, Humphrey visual field (HVF) and optical coherence tomography (OCT) in detecting visual abnormality in multiple sclerosis (MS) patients. Methods: mfVEP, HVF, and OCT (retinal nerve fiber layer [RNFL]) were performed in 47 MS-ON eyes (last optic neuritis [ON] attack ≥6 months prior) and 65 MS-no-ON eyes without ON history. Criteria to define an eye as abnormal were: (1) mfVEP amplitude/latency — either amplitude or latency probability plots meeting cluster criteria with 95% specificity; (2) mfVEP amplitude or latency alone (specificity: 97% and 98%, respectively); and (3) HVF and OCT, mean deviation and RNFL thickness meeting p < 0.05, respectively. Results: MfVEP (amplitude/latency) identified more abnormality in MS-ON eyes (89%) than HVF (72%), OCT (62%), mfVEP amplitude (66%) or latency (67%) alone. Eighteen percent of MS-no-ON eyes were abnormal for both mfVEP (amplitude/latency) and HVF compared with 8% with OCT. Agreement between tests ranged from 60% to 79%. mfVEP (amplitude/latency) categorized an additional 15% of MS-ON eyes as abnormal compared with HVF and OCT combined. Conclusions: mfVEP, which detects both demyelination (increased latency) and neural degeneration (reduced amplitude), revealed more abnormality than HVF or OCT in MS patients.

Retinal changes associated with tamoxifen treatment for breast cancer

Purpose: This study was undertaken to estimate the incidence of retinal changes and determine the prevalence of ocular toxicity associated with tamoxifen treatment in a breast cancer population.Methods: The study was based on a population cross-sectional survey, including 290 patients taking tamoxifen from 6 months to 12 years; 274 patients were analysed. The main outcome measures were the incidence of retinal changes and visual impairment.Results: The incidence of retinal changes was 0.9% (3 of 274 patients). All 3 patients were asymptomatic. The length of tamoxifen treatment ranged from 39 months to 120 months in the affected patients, with cumulative tamoxifen doses ranging from 23.7 g to 73 g.Conclusions: Retinopathy in patients receiving low doses of tamoxifen is rare and, in our study, did not result in changes in visual acuity. We found no retinopathy in patients receiving tamoxifen within the first 3 years of treatment or in patients receiving a total tamoxifen dosage of less than 23.7 g. Although retinopathy can occur in a tamoxifen-treated population its low incidence and an associated good prognosis for vision does not merit special screening for this problem.

Orbital presentations of giant cell arteritis

Abstract. Background: giant cell arteritis (GCA) is a systemic vasculitis that may affect the optic nerve and cause blindness (e.g. ischemic optic neuropathy). Orbital inflammatory disease, however, is an uncommon presentation of GCA. Purpose: to alert clinicians to the orbital presentations of GCA. Patients and methods: a retrospective case series from tertiary care academic ophthalmic referral centers of four patients with orbital manifestations of giant cell arteritis. Results: presentation of cases and review of the literature. In three cases, a temporal artery biopsy was diagnostic of GCA, but in one case, an orbital biopsy was needed to confirm the diagnosis Conclusion: GCA can have orbital manifestations and clinicians should be aware of this unusual presentation of GCA in cases of presumed orbital inflammatory pseudotumor in the elderly.

A practice pathway for the initial diagnostic evaluation of isolated sixth cranial nerve palsies

Purpose: To define a practice pathway for the evaluation of sixth-nerve palsies (SNPs) and to determine its cost—effectiveness and validity in a retrospective chart review. Methods: A Medline search of the English-language literature from 1966 to 1995 was performed to define the available clinical evidence and develop the practice pathway. The authors retrospectively reviewed 407 charts with the diagnosis of SNP seen at three centers. Information obtained included: etiologic diagnosis if known; development of new neurologic or ophthalmologic findings; and results and costs of neuroimaging studies, if performed. Results: Of the 407 patients, 98 underwent computed tomogra phy scans and 212 underwent magnetic resonance imaging of the head. Eighty cases were non-isolated, 317 were isolated SNP, and ten could not be classified from chart information. Of the 317 cases of isolated SNP, 49 were classified as traumatic; 5, congenital; 158, vasculopathic; 63, nonvasculopathic; and 42, progressive or unre solved. Following the recommendations of the practice pathway, the 158 patients clas sified as having vasculopathic SNP would not have undergone neuroimaging studies, realizing a savings of

Post‐irradiation neuromyotonia affecting trigeminal nerve distribution An unusual presentation

100,000 in this study population of 407 patients. Conclusion: The recommendations of the practice pathway are supported by review of the literature and the retrospective review of these cases. However, a prospective study with a matched control group is needed to demonstrate regional and specialty-specific van ations in care and to strengthen the clinical certainty of the pathway recommendations. Key words: practice pathway; diagnostic evaluation; sixth cranial nerve palsies; oph thalmology. (Med Decis Making 1999;19:42-48)

Interferon therapy for orbital infiltration secondary to Erdheim-Chester disease

We describe two patients who developed neuromyotonia of the floor of the mouth after irradiation of a motor branch (V3) of the trigeininal nerve. The neuromyotonia manifested as sustained muscle contraction due to peripheral nerve dysfunction. The neuromyotonia in both patients was controlled with carbamazepine. Radiation-exposed nerves can become symptomatic months or years after completion of radiation therapy.

Diversity in autoimmunity against retinal, neuronal, and axonal antigens in acquired neuro-retinopathy

PURPOSE To describe a 55-year-old male with Erdheim-Chester disease with bilateral orbital infiltration and visual loss who was successfully treated with interferon-alpha. METHODS Interventional case report. RESULTS The patient was treated with interferon-alpha and had an improvement in his clinical signs, including his visual acuity, after 4 weeks of interferon therapy. CONCLUSION Interferon-alpha can be effective in the treatment of orbital infiltration secondary to Erdheim-Chester disease.

Assessing visual pathway function in multiple sclerosis patients with multifocal visual evoked potentials

PurposeAutoimmune retinopathies and optic neuropathies are complex disorders of the retina and the optic nerve, in which patients develop autoantibodies (AAbs) against retinal and optic nerve proteins. Autoimmunity might significantly influence the outcome of retinal and optic nerve degenerative process but the pathogenic process is not fully elucidated. To better understand the role of AAbs in pathogenicity of these suspected autoimmune visual disorders, we focused on unique AAbs specificities associated with the syndrome to identify their antigenic targets in the optic nerve and retina.MethodsSerum samples were obtained from patients, whose visual disorders were potentially autoimmune in nature, including patients with cancer with possible paraneoplastic syndrome. Autoantibodies were tested against human optic nerve and retinal antigens for specificity by Western blotting and immunofluorescence.ResultsOut of 209 tested for anti-optic nerve autoantibodies, 55% showed specific neuronal autoantibodies. The repertoire of anti-optic nerve autoantibodies often differed from anti-retinal antibodies. The major antigenic targets for these antibodies could be divided into four groups. Autoantibodies specific to classical glycolytic enzymes involved in energy production (α and γ enolases, glyceraldehyde 3-phosphate dehydrogenase) also reacted with retinal antigens. Autoantibodies targeted neuronal-specific myelin proteins (MBP, MOG), aquaporin 4, and collapsing response mediator protein 5 reacted with optic nerve antigens. They showed immunostaining of axons and myelin in the optic nerve as determined by double immunofluorescence.ConclusionWe identified novel neuronal autoantigens not previously known to be associated with acquired autoimmune retinopathy and optic neuropathy. Knowledge of the full autoantibody repertoire perpetuating this syndrome is an important first requirement in increasing our understanding of the autoimmune process to facilitate better diagnosis, prognosis, and treatment.

The significance of white-centered retinal hemorrhages in the shaken baby syndrome

Multifocal visual evoked potentials provide a topographic measure of visual response amplitude and latency. The objective of this study was to evaluate the sensitivity and specificity of the multifocal visual evoked potential technique in detecting visual abnormalities in patients with multiple sclerosis. Multifocal visual evoked potentials were recorded from 74 patients with multiple sclerosis with history of optic neuritis (MS-ON, n = 74 eyes) or without (MS-no-ON, n = 71 eyes), and 50 normal subjects (controls, n = 100 eyes) using a 60-sector pattern reversal dartboard stimulus (VERIS). Amplitude and latency for each sector were compared with normative data and assigned probabilities. Size and location of clusters of adjacent abnormal sectors (p < 0.05) were examined. Mean response amplitudes were (± SE) 0.39 ± 0.02, 0.53 ± 0.02, and 0.60 ± 0.01 for MS-ON, MS-no-ON, and control groups, respectively, with significant differences between all groups (p < 0.0001). Mean latencies (ms; ±SE relative to normative data) were 12.7 ± 1.3 (MS-ON), 4.3 ± 1.1 (MS-no-ON), and 0.3 ± 0.4 (controls); group differences again significant (p < 0.0001). Half the MS-ON eyes had clusters larger than five sectors compared with 13% in MS-no-ON and 2% in controls. Abnormal sectors were distributed diffusely, although the largest cluster was smaller than 15 sectors in two-thirds of MS-ON eyes. Cluster criteria combining amplitude and latency showed an area of 0.96 under the receiver operating characteristic curve, yielding a criterion with 91% sensitivity and 95% specificity. We conclude that the multifocal visual evoked potential provides high sensitivity and specificity in detecting abnormalities in visual function in multiple sclerosis patients.