Faculty Bibliography

This article outlines neuro-ophthalmic findings and diseases which may present in an emergency setting. The abnormal optic disc, visual loss, double-vision and disorders of gaze, skew deviation, and the neuro-ophthalmology of vascular lesions, intracerebral hemorrhage, increased intracranial pressure, neuromuscular emergencies, metabolic disturbances, and trauma are all reviewed.

We report two patients with unique homonymous hemianopias from occipital lobe infarctions. One patient had vertical meridian sparing, and the other displayed horizontal meridian sparing. MRI correlation with the visual field defects confirmed that the vertical hemianopic meridian was represented along the border of the calcarine lips, and the horizontal meridian lies at the base of the calcarine banks deep within the calcarine fissure.

OBJECTIVE: To report four children with visual impairment associated with mutism after posterior fossa surgery. Mutism after posterior fossa surgery is a well-described phenomena, but to our knowledge, visual impairment has not been reported in association with it. METHODS: Record review of four children (age range, 3-7 yr) who underwent posterior fossa surgery (via suboccipital craniotomies) for removal of a medulloblastoma (three patients) or ependymoma (one patient). Each presented with headache, ataxia, or nausea and vomiting, but none had preoperative visual complaints other than diplopia. Postoperatively, all patients were mute, and because of apparent visual loss, neuro-ophthalmic consultation was requested. Postoperative scans and examinations were also reviewed. RESULTS: Each child was awake but appeared withdrawn without verbal output. No child blinked to threat or fixed or followed. In each case, pupillary reactivity was normal, and funduscopic examinations revealed only papilledema. One child reached for money. Within weeks or months postoperatively, the mutism spontaneously resolved, and visual behavior in general improved, roughly in parallel. During the follow-up period, papilledema resolved and the disc color was normal in each case. Magnetic resonance images obtained postoperatively revealed nothing remarkable, except surgical defects, without lesions in the retrogeniculate pathway. CONCLUSION: Impaired visual behavior, mimicking cortical visual loss, may be associated with mutism after posterior fossa surgery in children. The prognosis for recovery is excellent and parallels the return of normal speech. The mechanism is unclear.

In 1904 and 1905, respectively, William Campbell Posey and William Spiller both described the case of a patient with progressive ophthalmoparesis and imbalance that has come to be regarded as the earliest report of progressive supranuclear palsy. No autopsy was thought to have been performed on this patient. In this report, we review the clinical history provided by Posey and Spiller. We also report on the subsequent autopsy of their patient, which was performed by Spiller in 1906. The chief finding was a tumor involving the right cerebral peduncle and periaqueductal area. The autopsy findings prove conclusively that the patient described by Spiller and Posey had a midbrain neoplasm and not progressive supranuclear palsy.

We report an elderly patient with aggressive steroid-refractory giant cell arteritis manifesting as myelopathy and bilateral visual loss while on treatment. Pathologically, spinal cord infarction was observed and was due to extensive necrotizing granulomatous arteritis of spinal arteries. Spinal cord damage in giant cell arteritis is rare. One prior autopsy report of spinal cord infarction in giant cell arteritis did not identify vasculitic changes in the spinal arteries.

We report two patients with giant cell arteritis and unusual neuro-ophthalmic findings. One patient developed a horizontal one and a half syndrome associated with upright posture. The responsible lesion was dorsal pontine infarction. The other patient had bright light-induced amaurosis fugax in the absence of extracranial carotid occlusive disease. Both patients continued to have symptoms despite the use of high-dose intravenous corticosteroids. The manifestations of both patients occurred early in the course of giant cell arteritis and were flow related.