Magnetic resonance imaging signs of optic nerve gliomas in neurofibromatosis 1.

We reviewed the magnetic resonance images of four children with neurofibromatosis 1 and orbital optic gliomas. The images showed double-intensity tubular thickening characteristic of perineural arachnoidal gliomatosis, elongation of the nerves, and downward kinking of the nerves in the midorbit. We believe these findings typify the orbital gliomas in patients with this disease.

Exercise-induced transient visual events in young healthy adults.

Six patients with a variety of exercise-induced transient visual events are described. Each patient was in excellent health and most exercised regularly. None of the patients had a history of migraine, and only one gave a history of migraine in the family. One patient reported a single event, and the others reported recurrent events. These recurrent visual events were stereotyped except in one patient who experienced two distinctly different visual phenomena. Two patients described classic scintillating scotomas. Two described flickering phenomena in the central vision. Three described monocular events: one reported central scotomas, another blurring in the temporal field, and the third reported amaurosis. In no case could it be established that these events were associated with underlying disease. We believe that exercise-induced migraine equivalents are the most plausible explanation for these transient visual symptoms.

Systemic sarcoidosis presenting initially with bilateral orbital and upper lid masses.

A patient with systemic sarcoidosis presented initially with bilateral orbital and upper lid masses. Computed tomographic scan showed bilateral anterior orbital and upper lid masses, diffuse irregular thickening of the rectus muscles, circumferential cuffing of both globes, and irregular thickening of the distal portion of the right optic nerve sheath. A biopsy specimen of the right upper lid mass showed fibroadipose tissue with multiple noncaseating granulomas. Bilateral pulmonary hilar lymphadenopathy, a positive gallium scan, anergy by skin testing, and the presence of erythema nodosum confirmed the diagnosis of systemic sarcoidosis.

Acute idiopathic blind spot enlargement. A big blind spot syndrome without optic disc edema.

We examined seven patients who had a syndrome of symptomatic monocular blind spot enlargement without optic disc edema. Two patients had previous blind spot enlargement that resolved over several months. The scotoma in each patients was absolute, measured 15 degrees to 20 degrees in diameter, had steep geographic margins, and extended to within 5 degrees to 10 degrees of fixation. Typically, patients had normal visual acuity, color vision, pupillary responses, and ophthalmoscopic findings. Photostress recovery, tested in two patients, was prolonged in the affected eye. Fluorescein angiography showed no abnormalities corresponding to the scotoma. Orbital computed tomographic scans in three patients and visual evoked responses in one patient were normal. Multifocal electroretinography, performed in two patients, showed loss of retinal waveforms in a large region surrounding the optic disc. Our findings suggest that retinal dysfunction produces this big blind spot syndrome, but we do not know its cause.

Chiasmal gliomas: appearance and long-term changes demonstrated by computerized tomography.

A survey of 22 cases showed the broad spectrum of lesions collectively termed "chiasmal gliomas." Three computerized tomography (CT) patterns were diagnostic: a tubular thickening of the optic nerve and chiasm, a suprasellar tumor with contiguous optic nerve expansion, and a suprasellar tumor with optic tract involvement. Globular suprasellar tumors lacking these features required a histological examination for diagnosis. Tumor growth was documented by CT in only three chiasmal gliomas; all were the globular type. Failing visual function did not reflect chiasmal tumor growth, and stable vision did not exclude it. Both patients with tubular optic nerve thickening and two of three patients with unilateral optic nerve expansion had neurofibromatosis. Five tumors became smaller after irradiation. Complications of radiation therapy included calcification of lenticular nuclei and remote infarcts. In patients who underwent biopsy, the CT appearance did not differentiate juvenile pilocytic astrocytoma from anaplastic astrocytoma. Thus, CT guides the diagnosis and neurosurgical treatment of chiasmal gliomas, establishing the need for biopsy or ventricular shunting.

Childhood chiasmal gliomas: update on the fate of patients in the 1969 San Francisco Study.

We review the current status of 28 patients with chiasmal glioma who were originally reported on by Hoyt and Baghdassarian in 1969. The median period of follow-up was 20 years. Sixteen patients are now dead, five from chiasmal glioma and the rest from other causes. Four of the five deaths from chiasmal glioma occurred before 1969. The mortality rate in patients with neurofibromatosis and those without neurofibromatosis was essentially the same. Nine of 16 patients with neurofibromatosis are dead, two from chiasmal glioma, one from aspiration, and six from another tumour. Seven of 12 patients without neurofibromatosis are dead, three from chiasmal glioma, two from an operation on their tumour, and two from a medical illness. In general the quality of life of the surviving patients is good; none reported having a noticeable decrease in vision since 1969.

Bilateral congenital restriction of upward eye movement.

Neurologic evaluation of a child with a severe headache revealed a bilateral inability to move the eyes above the horizontal plane. Computed tomographic and magnetic resonance scans of the brain obtained for a suspected midbrain lesion were normal. Photographs from early childhood confirmed that the deficit was congenital.

[Unilateral papilledema in pseudotumor cerebri]. / Papiledema unilateral na sindrome do pseudotumor cerebral.

The appearance of unilateral papilledema in the fundus photos of 6 women with pseudotumor cerebri is presented. Associated findings included obesity in 5 and a partially empty sella in 2. The possible explanations for the development of unilateral papilledema in pseudotumor cerebri are reviewed and favor a mechanism that is present at the distal end of the optic nerves.

Evolution of optociliary veins in optic nerve sheath meningioma. Evolution.

Sequential fundus photographs, obtained during an eight-year period, documented the development of optociliary veins. Disc edema preceded their appearance by three years, and as optic atrophy became profound, the veins changed in number and in size.

Ophthalmoplegic migraine with proximal posterior cerebral artery vascular anomaly.

We report herein the unique finding of a small perimesencephalic vascular anomaly in a patient with a partial oculomotor nerve palsy and a 25-year history of recurrent ophthalmoplegic migraine.