Magnetic resonance imaging of leukemic invasion of the optic nerve.
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Biomedical subjects
Publications and source records attributed to J C Horton.
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Wolfram syndrome is an autosomal recessive disorder beginning in childhood that consists of four cardinal features: optic atrophy, diabetes mellitus, diabetes insipidus, and neurosensory hearing loss. Aside from these features, the clinical picture is highly variable and may include other neurologic abnormalities such as ataxia, nystagmus, mental retardation, and seizures. We present two unrelated patients with Wolfram syndrome, both of whom had the four cardinal features and several other neurologic abnormalities. MRIs showed widespread atrophic changes throughout the brain, some of which correlated with the major neurologic features of the syndrome.
Obstruction of the dural sinuses produces a clinical syndrome that resembles pseudotumor cerebri. In these patients, unremitting papilledema can cause blindness. We performed decompression of the optic nerve sheath in four patients who had occlusion of the dural sinuses. The operation achieved rapid relief of papilledema and recovery of vision. In three patients, fenestration of the nerve sheath of only one eye resulted in resolution of papilledema in both eyes. The procedure appears to relieve papilledema by filtering small quantities of cerebrospinal fluid into the orbit. It did not lower cerebrospinal fluid pressure in three patients who underwent lumbar puncture after surgery. Optic nerve sheath decompression is an effective operation for salvage of vision in patients with obstruction of the dural sinuses.
We treated four patients who developed a homonymous hemianopsia from a bacterial abscess in the occipital lobe of the brain. All four patients were treated successfully by surgical drainage of the abscess and administration of parenteral antibiotics for at least six weeks. Despite cure of the brain abscess, each patient was left with a permanent residual homonymous visual field defect. Cultures from the abscess fluid in three of the four patients grew oral flora. Moreover, each patient had a history of dental care two to four weeks before the onset of visual symptoms. A history of recent dental treatment in a patient with a new hemianoptic field defect should alert the ophthalmologist to the possibility of a bacterial abscess in the occipital lobe.
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We have tested the accuracy of Gordon Holmes' retinotopic map of human striate cortex by correlating magnetic resonance scans with homonymous field defects in patients with clearly defined occipital lobe lesions. Our findings indicate that Holmes underestimated the cortical magnification of central vision. In a revised map of the human striate cortex, we expand the area subserving central vision and reduce the area devoted to peripheral vision. These changes bring the map of human striate cortex into agreement with data reported for closely related nonhuman primate species.
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We report 2 patients with homonymous quadrantic visual field defects. The first patient experienced scintillations in the left lower quadrant, leading to the discovery of an astrocytoma in the cuneus of the right occipital lobe. Postoperatively she had a left lower quadrantanopia that precisely respected the horizontal meridian. The second patient presented with a left lower quadrantanopia, sparing the central 10 degrees of vision that also respected the horizontal meridian. An astrocytoma was resected from the right upper peristriate cortex. We must explain how a lesion in extrastriate cortex produced a homonymous field defect with a sharp horizontal edge in these 2 patients. Areas V2 and V3 are each divided along the horizontal meridian into separate halves flanking striate cortex. Consequently, the upper and lower quadrants in extrastriate cortex are physically isolated on opposite sides of striate cortex. We propose that a lesion involving V2/V3 may be sufficient to create a visual field defect. Although the lesion may have irregular margins, if it crosses the representation of the horizontal meridian in extrastriate cortex, it will produce a quadrantic visual field defect with a sharp horizontal border because of the split layout of the upper and lower quadrants in V2/V3.
A 59-year-old woman with von Hippel-Lindau disease developed erythrocytosis and a recurrent intracranial hemangioblastoma. Radioimmunoassay showed an elevated level of erythropoietin in her serum. Cyst fluid from the tumor also contained erythropoietin, concentrated a thousandfold relative to the serum level. Production of erythropoietin by hemangioblastomas may explain the erythrocytosis present in some patients with von Hippel-Lindau disease.
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We used magnetic resonance imaging to map the human lateral geniculate body. The optimal imaging plane was determined by obtaining axial and coronal scans in two normal brains obtained at autopsy. The brain specimens were then sectioned and individual slices were compared with matching magnetic resonance images. After the lateral geniculate body was identified using this correlative anatomic approach, the nucleus was imaged in four normal subjects.
The arrangement of the ocular dominance columns in the human primary visual cortex was studied by examining cytochrome oxidase activity in autopsy specimens of occipital lobes obtained from two patients who became blind in one eye before death. By artificially flattening the cortex before processing, it was possible to reconstruct the pattern formed by the ocular dominance columns throughout most of the primary visual cortex. The columns form a mosaic of irregular parallel stripes about 500 microns to 1000 microns wide (right eye column plus left eye column measures 1 to 2 mm), oriented at right angles to the boundary of the primary visual cortex. The columns are wider near the boundary of the primary visual cortex and within the representation of the peripheral visual field, the ocular dominance columns of the ipsilateral eye become fragmented until they disappear altogether at the border of the monocular crescent representation. The arrangement of ocular dominance columns in the human visual cortex is very similar to the pattern reported in the macaque monkey, although the columns in humans are wider.
We conducted a retrospective analysis of 451 women with an arteriovenous malformation (AVM) of the brain to determine whether pregnancy is a risk factor for cerebral hemorrhages. A total of 540 pregnancies occurred among our patient population, resulting in 438 live births and 102 abortions. There were 17 pregnancies complicated by a cerebral hemorrhage. The hemorrhage rate during pregnancy for women with an unruptured AVM was 0.035 +/- 0.005 per person-year. The hemorrhage rate for nonpregnant women of childbearing age with an unruptured AVM was 0.031 +/- 0.002 per person-year. Pregnancy did not increase significantly the rate of first cerebral hemorrhage from an AVM (P = 0.35). We found that women with an AVM face a 3.5% risk of hemorrhage during pregnancy. Pregnancy is not a risk factor for hemorrhage in women without a previous hemorrhage. This conclusion assumes no selection bias exists in our study population; a bias would be introduced if the risk of fatal outcome after a hemorrhage were greater in pregnant women than in nonpregnant women.
We report a pyogenic granuloma of the upper palpebral conjunctiva in a soft contact lens user. Pathological examination revealed numerous foreign bodies embedded within the specimen. The contact lens may have contributed to development of the lesion.
A patient with a 30-year history of blindness in the right eye developed progressive temporal visual loss in the left eye. Examination showed right optic atrophy with optociliary shunts and left band atrophy. These clinical findings suggested that the visual deficit was caused by a right optic nerve sheath meningioma that had grown intracranially to involve the chiasm. Magnetic resonance imaging and surgical exploration revealed a perioptic meningioma extending from the orbit through the optic canal and over the tuberculum sellae. The tumor did not impinge on the optic chiasm or the left optic nerve. The chiasm was compressed by a thrombosed giant right internal carotid artery aneurysm.
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Fibres in the mammalian optic nerve are generally thought to be organised retinotopically. Recording electrophysiologically from the cat optic nerve, we found little evidence to support this notion, which led us to investigate the problem by anatomical methods. We made a localised injection of horseradish peroxidase into the lateral geniculate body of the cat, labelling a small clump of retinal ganglion cells and their axons in the optic nerve. These fibres, emanating from neighbouring cells in the retina, became widely scattered through the optic nerve, indicating that retinotopic order is essentially lacking.