Rosa A. Tang

Intracarotid infusion of cis‐diamminedichloroplatinum in the treatment of recurrent malignant brain tumors

Thirty‐five patients with malignant brain tumors (23 with primary brain tumors and 12 with brain metastases) progressing after cranial irradiation chemotherapy received cisplatin, 60 to 120 mg/m2, into the internal carotid artery by a transfemoral approach. Courses of therapy were repeated every 4 weeks. Therapeutic evaluation was performed monthly using the CT scan of the brain and clinical neurologic examination. Thirty patients were evaluable for response. Of 20 evaluable patients with primary malignant brain tumors, 6 responded to therapy and 5 had stable disease. The median time to tumor progression for responding patients was 33 weeks, for stable patients 16 weeks, and 13 weeks for all patients. Five of 10 evaluable patients with brain metastases responded to intracarotid cisplatin, and 2 patients had stable disease. The estimated median time to progression for responding patients was 30+ weeks and 12+ weeks for patients with stable disease. Side effects included seizures in 5 courses, mental agitation and motor restlessness in 1, and transient hemiparesis in 7. One patient may have had a drug‐related death, and one patient appeared to develop encephalopathy after treatment. Five patients had clinical deterioration in vision; in two patients it was bilateral. Intracarotid cisplatin has definite activity in patients with malignant primary brain tumors and in patients with brain metastases. The recommended starting dose for intracarotid cisplatin is 60 to 75 mg/m2. At this dose level side effects are uncommon, but includes the risk of neurologic and retinal toxicity.

Reversal of Visual Loss with Hyperbaric Oxygen Treatment in a Patient with Susac Syndrome

PURPOSE The purpose of the study is to report on a 34-year-old woman with Susac syndrome who presented on two separate occasions with visual acuity loss from a recurrent branch retinal artery occlusion and underwent hyperbaric oxygen treatment with favorable outcome. METHODS Visual acuity was 20/400 in the left eye and count fingers in the right eye after separate occlusive events. The patient underwent hyperbaric oxygen treatment on each occasion of visual acuity loss. The patient subsequently was diagnosed with Susac syndrome (microangiopathy of the retina, brain, and middle ear). The authors present visual fields, photographs, angiograms, and ancillary tests documenting diagnosis and course of treatment. RESULTS On each occasion, visual acuity improved to 20/25 during and was maintained after hyperbaric oxygen treatment. Visual fields showed improvement immediately after treatment. CONCLUSION This is the first report of hyperbaric oxygen treatment for Susac syndrome. Dramatic post-treatment visual field and acuity changes suggest benefit of treatment. Hyperbaric oxygen treatment can be considered a treatment option for visual complications of this syndrome. Susac syndrome should be included in the differential diagnosis of recurrent branch retinal artery occlusion.

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

Interferon therapy for orbital infiltration secondary to Erdheim-Chester disease

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)

Bilateral anterior ischemic optic neuropathy following influenza vaccination

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

Optic neuritis is an occasional complication of vaccination. Visual loss can be unilateral or bilateral, and most patients recover substantially without treatment. The presumptive mechanism is an immune-mediated demyelinating injury of the optic nerve. We report two patients who had permanent visual loss following influenza vaccination. Their pattern of visual loss, segmental optic disc changes, and failure of visual recovery were atypical for demyelinating optic neuritis and reminiscent of a primary ischemic injury to the optic nerve. We speculate that an immune complex-mediated vasculopathy following vaccination can cause anterior ischemic optic neuropathy. Clinicians should be aware of this entity because of the less favorable prognosis for visual recovery in these cases.

Orbital apex lesion as the presenting manifestation of sarcoidosis

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.

Cilioretinal circulation and branch arterial occlusion associated with preretinal arterial loops

A 32-year-old black woman presented with progressive proptosis, diplopia, and optic disc edema of the right eye. A computed tomography scan of the orbit showed a right retroorbital mass. A gallium scan showed increased radiotracer activity in the right retroorbital region. Biopsy of the mass showed non-caseating granulomas that were compatible with sarcoidosis. The patient was treated with systemic steroids, and the proptosis and diplopia resolved. Seven months later, the patient presented with contralateral optic neuropathy. Neuroimaging showed enlargement of the left optic nerve. The patient again responded to systemic steroid treatment and experienced complete restoration of vision. An orbital apex lesion may be the presenting manifestation of sarcoidosis.

Third nerve palsy as the presenting manifestation of esthesioneuroblastoma.

Three patients had occlusion of preretinal loops and associated branch arterial occlusion. On fluorescein angiography all three eyes showed extensive cilioretinal circulation and that the occluded preretinal loops were part of the cilioretinal vascular anomaly. This supports the hypothesis that one form of preretinal arterial loop is associated with a malformation of the cilioretinal circulation.

Neuro-ophthalmologic manifestations of adenoid cystic carcinoma

A 52-year-old patient experienced unilateral third nerve palsy because of cavernous sinus involvement of biopsy-proven esthesioneuroblastoma. The patient experienced resolution of diplopia after surgical debulking of the tumor. Clinicians should be aware of the neuro-ophthalmologic manifestations of esthesioneuroblastoma.