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We describe two patients with complete homonymous hemianopia who were unaware of their visual field defects. The clinical impression of an optic tract syndrome was confirmed by magnetic resonance imaging, which showed absence of the corresponding tract in each case. The pathogenesis of congenital absence of the optic tract is unclear but it could represent(More)
A 10-year-old boy had a slowly enlarging left limbal mass, extending into the corneal stroma, but not into the anterior chamber. The lesion was excised by lamellar sclerokeratectomy and subsequently confirmed histopathologically as a juvenile xanthogranuloma. Though rare, juvenile xanthogranuloma of the corneoscleral limbus should be considered in the(More)
Joubert syndrome is characterized by episodic hyperpnea and apnea, developmental delay, hypotonia, truncal ataxia, ophthalmologic abnormalities, and vermian dysgenesis. We studied 15 patients with the diagnosis of Joubert syndrome to (1) more fully define the syndrome's clinical features, and (2) correlate the clinical features with magnetic resonance(More)
Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and(More)
BACKGROUND Upper eyelid retraction in thyroid eye disease may be caused by proptosis, levator and Müller's muscle infiltration with fibrosis or individual fiber enlargement, excessive sympathetic innervation, abnormal adhesions between levator palpebrae muscle and surrounding tissues, or fixation duress. Fixation duress refers to upper eyelid retraction(More)
Synergistic divergence is a congenital syndrome of monocular adduction deficit, with simultaneous abduction of both eyes on attempted gaze into the field of action of the paretic medial rectus muscle. Based on electromyographic data, it has been speculated that it is a neural miswiring disorder related to Duane's retraction syndrome. The association between(More)
Magnetic resonance angiography (MRA) is a noninvasive, rapidly evolving technique for imaging the intra- and extracranial carotid and vertebrobasilar circulations. It may in some circumstances obviate conventional angiography and the accompanying risks associated with catheterization and contrast injection. MRA exploits the different physical properties(More)
BACKGROUND The tonic ocular tilt reaction (OTR) consists of vertical divergence of the eyes (skew deviation), bilateral conjugate ocular torsion, and paradoxical head tilt. The head and superior pole of both eyes are rotated toward the hypotropic eye. OBJECTIVE To describe ocular motility and torsion findings in 5 patients with OTRs that mimicked superior(More)
Unlike acquired oculomotor nerve palsy in childhood, the congenital form is usually considered to be an isolated disorder, not associated with other neurologic or systemic disease. In a series of 14 patients with this diagnosis, 10 had associated neurologic disorders. Clinical-neuroradiologic correlation identified the brainstem as the site of the lesion in(More)
Gliomas that arise in the brain stem have been associated with a poor prognosis. Diagnostic neuroimaging readily identifies the tumor as it extends between normal brainstem structures. Histologic sampling of tumor with stereotactic methods is notoriously unreliable in establishing a definitive prognosis. Clinical trials that incorporate high-dose(More)