Faculty Bibliography

We report a patient who developed an acute loss of vision in the left eye associated with proptosis, ophthalmoparesis, conjunctival injection, and chemosis. Funduscopy revealed optic disc swelling, and retinal whitening consistent with an ophthalmic artery occlusion. Angiography disclosed bilateral carotid dissections presumably resulting from head trauma 11 days earlier. An orbital ischemic syndrome may be a delayed manifestation of traumatic carotid dissection and precede cerebral hypoperfusion.

We report a patient with meningovascular syphilis who had a dorsal midbrain syndrome, cognitive dysfunction, and a left peripheral seventh nerve palsy. Magnetic resonance imaging (MRI) disclosed a large lesion of the midbrain and thalamus with intense enhancement of the interpeduncular cistern, both of which resolved after treatment with intravenous penicillin. The clinical features, radiographic appearance, and response to therapy suggest that this lesion was a focal syphilitic inflammatory process, or gumma. We conclude that MRI with intravenous contrast may reveal the full spectrum of pathologic involvement in neurosyphilis and, in certain situations, may obviate the need for biopsy of an associated mass lesion.

Four patients with isolated acute ocular ischemic syndromes also had circulating antiphospholipid antibodies. Two patients had vaso-occlusive retinopathy and two, anterior ischemic optic neuropathy (which was successive in one). Extensive clinical laboratory evaluation identified vascular risk factors in two patients. One patient had essential thrombocytosis, confirmed by bone marrow biopsy; the other had stable hypertension and a history of coronary artery disease. These cases suggest that small vessel thrombosis in situ may be a mechanism for antiphospholipid-associated ocular and cerebral ischemia.

Histiocytic infiltration of the optic chiasm is rare. We report a patient with a seizure disorder on anticonvulsant therapy, who developed systemic reactive histiocytosis. Treatment with splenectomy, corticosteroids, and anticonvulsant medication change resulted in clinical remission. The patient later developed recurrent lymphohistiocytosis restricted to the optic chiasm. Extensive re-evaluation yielded no evidence for infectious or malignant etiology. Radiation therapy and withdrawal of anticonvulsant therapy resulted in clinical remission. We conclude that both phenytoin and phenobarbital may trigger a lymphohistiocytic process and that infiltration of the visual pathway is possible.

We report a patient with a pathologically proven pontine cavernous angioma and a horizontal one and a half syndrome. Following recurrent hemorrhages, the vascular malformation was removed from the dorsal pons using microsurgical techniques with partial resolution of the motility disturbance. This case demonstrates that vascular malformations of the brainstem parenchyma may be removed, thereby precluding further hemorrhage and facilitating the return of neurologic function

We report the simultaneous occurrence of biopsy-proven temporal arteritis in husband and wife. Serologic and viral studies were negative, including viral culture of the wife's temporal artery. The concurrent incidence of giant cell arteritis in a married couple would suggest a common exogenous exposure.

Rhinocerebral mucormycosis is a rare but often fatal fungal infection. We present 2 patients with mucormycosis complicated by internal carotid artery thrombosis. Magnetic resonance imaging was superior to computed tomography in localizing the pathological process. Subtotal resection of devitalized tissue and intravenous amphotericin B therapy resulted in a successful outcome for both patients. Prompt recognition of this disorder by using modern diagnostic and therapeutic modalities promises to improve survival rates.