John A. McCrary

A Randomized, Controlled Trial of Corticosteroids in the Treatment of Acute Optic Neuritis

Background and Methods. The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. Results. Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did ...

Nonarteritic Ischemic Optic Neuropathy: The Impact of Tobacco Use

BACKGROUND Numerous associations to anatomic variation and systemic vascular disease have been made to anterior ischemic optic neuropathy (AION) but exogenous agents have not been emphasized. The authors studied the effect that smoking had in the development of AION. The relevance of other intraocular and systemic vascular disease to AION also is discussed. METHODS Over a 10-year period (January 1980-May 1990), nonarteritic AION was diagnosed in 148 patients, 137 of whom were included in this analysis. FINDINGS Of the 137 patients identified with nonarteritic AION, 28 smokers were statistically younger, at 51 years of age, compared with 83 nonsmokers whose mean age was 64 years (P = 0.005). CONCLUSIONS Cigarette smoking is an important risk factor in the development of AION. Cessation of smoking appears to reduce the risk of AION to that of the nonsmoking population.

Intraosseous Capillary Hemangioma of the Frontal Bone

A 31-year-old woman with a history of slowly progressive proptosis associated with blepharoptosis was found radiographically to have a lytic lesion of the frontal bone. Complete surgical excision of the bony mass was performed. Histopathologically the tumor was a capillary hemangioma. Follow-up information two years later showed that the patient was alive and well, without clinical evidence of recurrence. Plain x-ray films appear to be more reliable for diagnosis than computed tomography exhibiting the characteristic sunburst pattern of intraosseous hemangiomas.

Visual function at baseline and 1 month in acute optic neuritis: Predictors of visual outcome

Objective: To identify cutpoints for visual measures at baseline and 1 month predictive of abnormal 6-month vision that could be used as eligibility criteria in a clinical trial to test potential neuroprotection or myelin repair agents in patients with optic neuritis. To determine whether moderate-to-severe dysfunction in one or more visual measures at baseline or 1 month correlates with having major vision loss at 6 months. Methods: We used the Optic Neuritis Treatment Trial database to evaluate various cutpoints for baseline and 1-month vision levels that predicted abnormal 6-month vision. For selected cutpoints, we computed a 95% CI for positive predictive value and the required sample size if the cutpoint was to be used for clinical trial eligibility. We evaluated whether the degree of visual loss at baseline, 1 month, or change in visual function from baseline to 1 month correlated with 6-month visual acuity, contrast sensitivity, or threshold visual field. Results: The best cutpoints for baseline and 1 month were visual acuity ≤ 20/50, contrast sensitivity < 1.0 log units, and visual field mean deviation ≤ −15 dB. The same levels of visual dysfunction at 1 month, but not at baseline, correlated with having 6-month moderate-to-severe loss for each of these measures (p = 0.01). A trial could require as few as 100 subjects for an outcome variable of one or more abnormal measures. Cutpoints at 1 month were highly predictive of abnormal 6-month vision, but the proportion of patients who would be eligible for a trial would be small. Conclusion: Provided data can be used either for the clinician to counsel patients on expected visual outcome or for designing studies to test therapies that might reduce the amount of permanent optic nerve damage due to optic neuritis in high-risk patients.

Epithelioid Angiosarcoma of the Orbit Presenting as Tolosa-Hunt Syndrome: A Clinicopathologic Case Report with Review of the Literature

The clinical diagnosis of Tolosa-Hunt syndrome was first considered in a 66-year-old man with facial pain and diplopia. A complete neuroradiologic evaluation as well as an oncologic work-up yielded normal results. Several courses of oral prednisone provided no significant benefit. Within a year the patient became clinically worse and a CT scan disclosed an abnormal area of enhancement at the left orbital apex. An orbital exploration was performed elsewhere and a histologic diagnosis of myositis was obtained. Because of further worsening the patient was re-evaluated 3 months later and a CT scan showed a mass in the left orbital apex and superior orbital fissure. A second orbital exploration was performed and a sausage-shaped mass encompassing the optic nerve was excised. By light microscopy a poorly differentiated malignant tumor was infiltrating the orbital tissues with areas of intra- and perineural invasion. The tumor cells were arranged in strands and tubules with a definite tendency to form lumens that often contained red blood cells. Electron microscopic studies disclosed features consistent with a neoplasm of endothelial cell origin displaying a polarized basal lamina and rare micropinocytotic vesicles on the luminal side. The presence of multiple, slender microvilli and sometimes tonofilaments as well as desmosomes were interpreted as epithelioid metaplasia of an angiosarcoma.

Opsoclonus-myoclonus with Beckwith-Wiedemann syndrome and hepatoblastoma.

The association between Beckwith-Wiedemann syndrome and hepatoblastoma is well established and relatively commonplace. The occurrence of opsoclonus-myoclonus syndrome in individuals with occult neoplasia is also well documented. However, the development of opsoclonus-myoclonus syndrome in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma has not been reported previously. The list of underlying causes of opsoclonus-myoclonus syndrome should be expanded to include hepatoblastoma, particularly in any child with features suggestive of Beckwith-Wiedemann syndrome.

Computed tomography and magnetic resonance imaging in the diagnosis of inflammatory disease of the optic nerve

Enlargement of the optic nerve and chiasm as demonstrated by high-resolution CT scanning or magnetic resonance imaging is often assumed to indicate tumor. However, this is not always true. The mode of clinical presentation of the disease process must be correctly applied to the interpretation of the radiographic studies. In the case illustrated here, neuro-ophthalmologic evaluation prevented an unnecessary biopsy in a patient whose presentation was atypical for anterior visual pathway glioma and whose clinical course was otherwise compatible with optic neuritis.

Multiple Cranial Nerve Palsies in Late Metastasis of Midline Malignant Reticulosis

A 12-year-old man had a sudden onset of multiple cranial nerve palsies after treatment for a necrotizing lesion of the soft palate two years previously. It was thought that neurologic signs were secondary to extension of the local disease and radiation therapy to the base of the brain was begun. The patient died shortly thereafter. A diffuse atypical histiocytic lymphoma involving multiple cranial nerves, lumbosacral nerves, orbital muscles, and other organs was found on autopsy. Initial nasopharyngeal biopsy and autopsy findings were compatible with midline malignant reticulosis, a malignant lesion of the upper airway sometimes associated with metastasis. Our case is the first reported autopsy-documented case, to the best of our knowledge, of metastatic involvement of the cranial nerves in midline malignant reticulosis.

Nuclear magnetic resonance diagnosis of an anaplastic astrocytoma

A patient presented with an 8-month history of a progressive left homonymous visual field deficit, left hemiparesis, and a left thalamocortical sensory deficit that was not detectable by repeated conventional neurodiagnostic evaluations. Proton nuclear magnetic resonance (NMR) imaging revealed a right parietal lesion characterized by a prolonged T2 (spin-spin relaxation time). At surgery, the mass proved to be an anaplastic astrocytoma. NMR appears to be more sensitive than x-ray computerized tomography scanning in some patients with malignant gliomas and offers the clinician an additional probe with which to evaluate these patients.