Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Reading-evoked visual dimming.

PURPOSE: To carry out a neuroradiologic investigation in a monocular 49-year-old patient who during the past five years described symptoms of dimming of central vision in his left eye, which was provoked only by reading. METHODS: Computed tomography and magnetic resonance imaging were performed. RESULTS: An orbital apex intraconal tumor situated laterally to and above the optic nerve was found. CONCLUSIONS: Reading-evoked visual dimming can be a variant of gaze-evoked amaurosis. The optic nerve displaced laterally and superiorly, and stretched by the act of reading, may be compressed between the tumor above and the contracted inferior rectus muscle inferiorly.

Orbital porencephalic cyst following penetrating orbitocranial trauma.

In a 10-year-old boy an orbitocranial penetrating wound produced by an umbrella tip caused an orbital roof bone fragment to penetrate up to the anterior part of the third ventricle behind the left foramen of Monro. Hemorrhages and encephalomalacia developed along the trajectory of the fragment and subsequently a porencephalic cyst was formed at this site. Six months after the trauma, increased pressure developed in the left ventricular system due to obstructive hydrocephalus and consequently the porencephalic cyst herniated into the orbit through the orbital roof fracture, producing intermittent diplopia, left exophthalmos, and palpebral swelling. A ventriculo-peritoneal shunt led to shrinkage of the orbital cyst content and resolution of the symptoms.

Relationship between visual acuity and nasal field thresholds in patients with temporal hemianopia.

The area of nasal field found with Goldmann static perimetry and the sum of decibels by Humphrey threshold 30/2 was calculated in normal subjects and in subjects with chiasmatic lesions, temporal field loss, and normal or abnormal visual acuity. There was a significant reduction of the mean of the area of the nasal field by static Goldmann perimetry and of the mean of decibels in the nasal field on Humphrey perimetry in patients with temporal field loss and chiasmatic lesions, as compared with normal controls. There were significant correlations of nasal field depression (Goldmann) and visual acuity and for sums of nasal field decibels (Humphrey) and visual acuity. Thus, a generally depressed nasal field was found in patients with chiasmatic lesions and temporal field loss when accompanied by lowering of visual acuity. This would appear to be the earliest stage of nasal field involvement.

Pineal germinoma with unilateral blindness. Seeding of germinoma cells in optic nerve sheath.

A 13-year-old boy presented with blindness and loss of the papillomacular bundle in the left eye, bilateral papilledema sparing the area of axonal loss, and Parinaud's syndrome. Computerized tomography (CT) revealed an enlarged kinked left optic nerve and enlarged optic canal as well as a tumor in the pineal area producing hydrocephalus. Magnetic resonance (MR) scans showed multiple spinal metastases. The histological diagnosis was germinoma. On completion of four courses of chemotherapy with cis-platin, vinblastine, and bleomycin, repeated CT of orbita and MR imaging of the spine demonstrated the disappearance of the tumor surrounding the left optic nerve and of the spinal metastases. This is believed to be the first case report of seeding of germinoma into the perioptic arachnoid space.

Entoptic [corrected] phenomena in pregeniculate and postgeniculate hemianopsia with splitting of macula by perimetry.

With splitting of macula by perimetry, the entopic phenomena (the perception of one's own foveal xanthophilic pigment and macular vessel leukocytes) were utilized to study the character of macular sparing or splitting in patients with pregeniculate or postgeniculate hemianopsia. In the pregeniculate group, 11 of 14 eyes perceived the Haidinger brushes figure as a half circle corresponding to the perimetric macular splitting, whereas flying corpuscles were not perceived at all or were less numerous on the hemianoptic side than on the normal field side. In contrast, six of seven patients with postgeniculate lesions perceived the Haidinger brushes figure as a complete circle, four of six perceived fewer flying corpuscles on the hemianoptic side than on the normal field side, and two patients reported perceiving an equal number in all quadrants. This study indicated that in pregeniculate hemianopsia there is usually a true splitting of the macula, whereas in postgeniculate hemianopsia, there is some macular sparing even when perimetry shows macular splitting.

Loss of accommodation produced by peristriate lesion in man?

We observed a bilateral accommodative paresis associated with a spontaneous parieto-occipital hematoma in a 37-year old patient. There was no clinical or computerized tomography (CT) evidence of transtentorial herniation or upper brainstem pathology. With resolution of the hematoma, accommodation returned to normal.

Amaurosis fugax at downward gaze.

A 54-year-old man with a past history of hypertension, diabetes mellitus, hypercholesterolemia and two myocardial infarctions presented with repeated attacks of amaurosis fugax in the right eye. The fact that the amaurosis occurred only on downward gaze is sufficient evidence to exclude carotid atheromatous disease and to imply an orbital etiology.

Papilledema in traumatic lesion of optic nerve with amaurosis.

A patient with subdural hematoma and right amaurosis resulting from traumatic injury to the optic nerve developed papilledema in both eyes a few days after the trauma. This was interpreted as supplementary proof of the existence of axonal flow in the first weeks after occurrence of the optic axon lesion. In the amaurotic eye, the papilledema was less pronounced and disappeared more rapidly (after 3 weeks, as opposed to 6 weeks for the other eye). The nerve fiber layer disappeared between 4 and 6 weeks after the trauma.