1/16. The clinical spectrum of Sweet's syndrome (acute febrile neutrophilic dermatosis)-a report of eighteen cases.Eighteen cases of Sweet's syndrome are described. This is the largest series so far reported and the first from the tropics. The essential features are the characteristic morphology, the histological appearances, the dramatic response to corticosteroids and the absence of scarring. attention is drawn to the frequent involvement of the eyes and joints during the course of the illness. fever and neutrophilia were found less frequently than a raised ESR or a preceding upper respiratory tract or skin infection. The term Sweet's syndrome is preferred to acute febrile neutrophilic dermatosis. The frequency of occurrence of different clinical manifestations is discussed.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
2/16. Ocular manifestations of Gaucher's disease.A 27-year-old woman not only had the usual clinical and laboratory features of the adult form of Gaucher's disease but also had typical pingueculae and a peculiar lesion with a pigmented temporal edge in the fundus of her left eye. Bone-marrow aspiration demonstrated infiltration by Gaucher's cells and a biopsy of the conjuctival masses disclosed areas of infiltration by Gaucher's cell beneath normal epithelium.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
3/16. Menkes' kinky hair disease: a light and electron microscopic study of the eye.light and electron microscopic studies of the ocular tissue of a case of Menkes' kinky hair disease are described. The copper deficiency responsible for this systemic and neurologic disease appears to cause a progressive degeneration of retinal ganglion cells, loss of nerve fibers, and optic atrophy. The pigment epithelium is also abnormal with only small and irregular melanin granules present among electron-dense inclusion bodies. Abnormal elastica is present in Bruch's membrane.- - - - - - - - - - ranking = 4keywords = eye (Clic here for more details about this article) |
4/16. cystinosis with crystal-induced synovitis and arthropathy.A clinically and experimentally verified intermediary form of cystinosis is described. A 30-year old patient has been examined initially for eye complications confined to the frontal segment of the eye on the cornea and conjunctiva. The joint disease received no attention despite the fact that the motor apparatus of the patient was involved from her childhood. As regards the arthrology, the disease can be classed among crystal-induced synovitis and arthropathy. The lesion of the tubular apparatus of the kidney, which cannot transform vitamin d into its active form, represents an important factor in the genesis of osteoporosis. A description of the intermediary from of cystinosis with eye, kidney and joint symptoms was not found in the literature.- - - - - - - - - - ranking = 3keywords = eye (Clic here for more details about this article) |
5/16. Ophthalmic manifestations of esthesioneuroblastoma.Esthesioneuroblastoma, a tumor that arises from the olfactory sensory epithelium, often manifests with ocular and orbital signs and symptoms. A review of 38 cases of esthesioneuroblastoma at the Mayo Clinic revealed that ophthalmic signs or symptoms occurred in 28. Twenty patients had ophthalmic signs or symptoms at the time of presentation, and five of these had ocular symptoms as the primary complaint. Five patients presented to an ophthalmologist, but a nasal or paranasal sinus tumor was not suspected. Periorbital pain and excessive tearing were the most commonly reported symptoms. The most common ophthalmic sign was eyelid edema followed by proptosis, globe injection, and ptosis. Recognition of the signs and symptoms of neoplasms of the nose and paranasal sinuses and consideration of esthesioneuroblastoma in the differential diagnosis by clinicians and pathologists allow for prompt management of this tumor.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
6/16. osteoma: an unusual cause of amaurosis fugax.A 16-year-old white boy presented with a history of amaurosis fugax associated with an afferent pupillary defect, proptosis, discomfort of the left eye. Tomograms of the skull and of the orbit revealed a lobulated 4-cm ivory osteoma arising from the left ethmoid sinus and extending into the maxillary sinus and left orbit. After surgical extirpation, visual acuity returned to normal and the papilledema resolved after 4 months. This patient is 1 of 37 patients with osteoma of the paranasal sinuses or of the orbit (or of both) who underwent surgical treatment at the Mayo Clinic between the years 1948 and 1977. Twelve of these patients had orbital involvement, and only these patients had ocular signs and symptoms.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
7/16. Abnormal visual-evoked responses and opsoclonus.A 73-year-old woman presented with classic opsoclonus two weeks following an acute febrile illness. Four weeks later a complete clinical recovery was evident. Six weeks after onset of the problem, or 2 weeks after resolution of the abnormal eye movements, a pattern reversal visual evoked response showed a delayed response in both eyes and had an unusually high amplitude. For the next 2 months the patient complained of occasional blurring of vision. A repeat visual evoked response test after 4 months revealed normal latency and amplitude in both eyes. The possible mechanisms for the visual evoked response abnormality are discussed.- - - - - - - - - - ranking = 3keywords = eye (Clic here for more details about this article) |
8/16. The eyes in hysterical states of unconsciousness.Forced downward deviation of the eyes has been described as a useful sign in patients with hysterical coma and seizures. Two patients are described emphasizing the value of this test in prolonged feigned seizured states as well as in hysterical syncopal episodes.- - - - - - - - - - ranking = 5keywords = eye (Clic here for more details about this article) |
9/16. optic disk neovascularization in hemoglobin sc disease.A 37-year-old black man had a clinically documented case of optic disk neovascularization associated with hemoglobin sc disease. Through medical evaluation, we ruled out other disorders associated with proliferative retinopathy. Extensive areas of retinal nonperfusion were associated with vasoproliferative retinopathy at the junction of the perfused and nonperfused retina; and a delicate network of neovascularization extended from the optic disk into the vitreous gel in one eye.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
10/16. Clinical and genetic aspects of Conradi-Hunermann disease. A report of three familial cases and review of the literature.Most reported cases of Conradi-Hunermann disease are said to be sporadic. The diagnostic radiographic features present in early life tend to disappear with age. Skeletal deformities may persist but are not adequate for diagnosis. It is critical that the examiner look for nonskeletal manifestations of the disease, particularly eye and skin changes, in parents and relatives before assuming that the proband represents a sporadic case. Some of the sporadic cases may also be accounted for by the existence of environmental phenocopies. The proband's mother in the family described in this paper was recognized as a carrier of the gene only after careful eye and skin examination. This observation was particularly important because she was pregnant at the time. Her 16-week-old fetus is the earliest documented example of the disease. Autosomal dominant mode of inheritance is suggested by the familial cases, but the variability of expression could be the result of either genetic or clinical heterogeneity. sex-influenced factors may account for the different severity of the disease in the two sexes.- - - - - - - - - - ranking = 2keywords = eye (Clic here for more details about this article) |
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