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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)
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)
Strabismus presenting after cataract surgery is etiologically related to a heterogenous group of disorders. Clinical data from 63 patients so affected revealed four broad etiologic categories: 1) pre-existing disorders that preceded the cataract surgery, but were rendered asymptomatic by the occluding cataract (e.g., thyroid eye disease, cranial nerve(More)
We reviewed the records of 38 consecutive patients who had persistent binocular diplopia after cataract surgery. The patients were divided into ten categories based on the suspected conditions underlying the diplopia. Of 16 patients who underwent strabismus surgery, five achieved the therapeutic goal of single binocular vision in the primary and reading(More)
There has been a steady increase in the number of cases of syphilis in the United States since the middle 1980s, with a dramatic rise in incidence among heterosexual men and women and of congenital syphilis. There also have been changes in geographic distribution of cases and an association with cocaine use. The ophthalmologic manifestations of syphilis are(More)
Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study(More)
Thallium-201 chloride single photon emission computed tomography (201Tl SPECT) has been proposed as a diagnostic tool in the assessment of patients with brain tumors. We performed SPECT scans coupled with magnetic resonance imaging (MRI) in children with brain tumors to determine the sensitivity and potential value of SPECT in neuro-oncology. Each patient(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)
Ipsilateral hypotropia with restricted elevation is an increasingly recognized strabismic entity resulting from injury to the inferior rectus muscle after local retrobulbar anesthesia. Eight patients with this disorder are described. Computed tomography of the orbit in three patients demonstrated isolated segmental enlargement of the retrobulbar portion of(More)
Wegener's granulomatosis causes a variety of ophthalmologic disorders, some of which occasionally constitute the initial presentation of the disease. We describe a patient who presented with bilateral, isolated, consecutive, posterior optic neuropathy with light perception and no light perception vision. The patient had no other symptoms or signs of orbital(More)