Cavitary developmental defects of the optic disc. Visual loss associated with optic pits and colobomas.

Congenital cavitary anomalies of the optic disc (optic pit and coloboma) are uncommon and frequently go unrecognized. Patients with these defects have a high incidence of visual loss and the abnormal appearance of the discs may be mistaken for atrophy due to acquired neurological disease. Occasionally, associated midline neurological developmental malformations will be found associated with pits, colobomas, or other dysplastic optic discs, as in one of our cases. A group of patients with optic pits and colobomas referred for neurological evaluation of visual loss are presented to remind the neurologist of the appearance of these congenital crater-like defects and of the causes of loss of vision.

Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss.

The prognosis for vision in most patients with pseudotumor cerebri is excellent; however, visual loss, which is the only serious complication, may occur either early or late in the course of the disease. A group of 57 patients was followed up five to 41 years with visual fields, visual acuity, and fundus photographs. Blinding visual loss or severe visual impairment in one or both eyes occurred in 14 patients, and in seven patients, this occurred months to years after the initial symptoms. Systemic hypertension was a significant risk factor for visual loss in patients with pseudotumor cerebri, and blindness occurred in eight of 13 patients who were hypertensive. Despite suggestions that blind spot measurement is useful for following up patients with this condition, we believe that sequential quantitative perimetry gives more complete information and is essential to rational decision making in the treatment of pseudotumor cerebri.

Optic tract syndrome. A review of 21 patients.

Twenty-one patients with lesions compromising the optic tract were reviewed. The involvement of the optic tract may be diagnosed in the presence of highly incongruous hemianopia, an afferent pupillary defect, and characteristic atrophy of the optic discs. Behr's pupil, hemianopic pupillary reaction (Wernicke's sign), and associated major neurologic deficits were encountered rarely.

Neuro-ophthalmological complications of enlargement of the third ventricle.

A wide variety of visual sensory and ocular motor problems may occur as a direct result of enlargement of the third ventricle. Four patients are described with optic nerve dysfunction, partial third nerve palsy, proptosis, and Sylvian aqueduct syndrome all resulting from an enlarged third ventricle. The pathogenetic mechanisms are discussed.

The return of stereotaxic surgery.

Stereotaxic surgery, which declined with the advent of dopaminergic treatment of Parkinson's disease, has found new life and new uses with unknown limits.

The syndrome of facial nevi, anomalous cerebral venous return, and hydrocephalus.

A 25-year-old man with facial and nuchal pigmentation and contrast radiographic changes identical to those in the syndrome described by Shapiro and Shulman is reported. Without surgical intervention in infancy, the patient attained normal maturation and development.