Cases reported "Eye Diseases"

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1/8. Cerebro-oculo-facio-skeletal syndrome as a human example for accelerated cochlear nerve degeneration.

    BACKGROUND: Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal-recessive disorder that includes microcephaly, severe mental retardation, and multiple congenital anomalies. Otologic findings are usually limited to descriptions of the auricles. PATIENT AND methods: The authors report inner ear histopathologic findings of a deceased 13-year-old patient with COFS. A histologic study of the inner ear in COFS syndrome has not yet been described. This patient was documented as having a profound bilateral sensorineural hearing loss at the age of 2 years. RESULTS: Histologic evaluation revealed accelerated neural and neuronal degeneration at the cochlear and retrocochlear levels. Remaining myelinated nerve fibers, counted in the spiral lamina, had degenerated by up to 97% when compared with normal innervation densities. Afferent nerve fibers innervating inner hair cells were completely absent, whereas medial efferent fibers to outer hair cells were found. vestibular nerve fibers were less affected. CONCLUSION: The authors report inner ear findings that differ from animal models of primary cochlear neural degeneration and that resemble the pattern of hereditary cochlear nerve degeneration reported in Friedreich's ataxia.
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keywords = animal
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2/8. Occupational exposures to pesticides containing organoarsenicals in california.

    The only organic arsenicals used in agriculture are methanearsonic acid (MSMA) and its sodium and ammonium salts and dimethylarsinic acid (cacodylic acid) and its sodium salt. They have an oral LD50 in the rat of 700-1,000 mg/kg and are classified as toxicity category 3 pesticides. During the three-year period 1975, 1976 and 1977 in california there were 34 reports by physicians of injury due to exposure to pesticides containing organic arsenicals of which nine resulted in systemic symptoms and the remainder being eye and skin irritations. There appeared to be prompt recovery from these exposures. They were caused primarily by use of faulty equipment, not using due care in its operation, poor work practices and improper use of protective equipment. There is no evidence that this group of chemicals is carcinogenic in animals or man.
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keywords = animal
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3/8. N-3-pyridylmethyl-N'-p-nitrophenylurea ocular toxicity in man and rabbits.

    Ingestion of the rat poison N-3-pyridylmethyl-N'-p-nitrophenylurea (PNU) produced ocular toxicity in three humans and in an animal model, the Dutch Belted rabbit. The electroretinogram b wave was especially susceptible to the effects of the rodenticide, and the target tissue appeared to be the retinal pigment epithelium. Injection of PNU itself did not produce ocular toxicity. The poison had to be administered orally. Gentamicin administered orally with PNU prevented the ocular toxicity. Presumably this antibiotic killed those gastrointestinal bacteria responsible for PNU's metabolism into an ocular toxin. L-tryptophan, a known antidote for the lethal effects of PNU, was an antidote for the ocular toxicity when administered orally but not when administered parenterally.
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keywords = animal
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4/8. GM1 gangliosidosis, type 2: ocular clinicopathologic correlation.

    The clinical and pathological manifestations of a case of juvenile GM1 gangliosidosis are presented and the pathological findings compared with those previously reported for GM1 gangliosidosis in man and in animal models. The most striking finding in the present case was the marked degeneration of the retinal ganglion cell and nerve fiber layers. Although such extensive ganglion cell loss was not observed in any of the other cases reviewed, the presence of multimembranous inclusion bodies in retinal ganglion cells strongly suggests that the pathological process was similar in all cases. Much remains to be learned about the function of gangliosides in the healthy retina and about the pathophysiological consequences of deranged ganglioside metabolism. The many parallels, including those observed in pathological studies, between the human and animal forms of GM1 gangliosidosis allow an optimistic appraisal of the value of further research using the animal models.
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keywords = animal
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5/8. Reaction to intraocular penetration of bentonite.

    A 20-year-old dental assistant had a severe case of anterior segment inflammation believed to be caused by bentonite, a component of Prophypaste. The patient's clinical course as well as animal studies, implicated bentonite as the offending agent.
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keywords = animal
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6/8. Complex visual hallucinations in partial blindness due to eye disease.

    Three patients experienced complex formed hallucinations during progressive visual failure from eye disease. The hallucinations began abruptly, were brightly coloured stereotyped figures, animals or objects, and appeared to be provoked by light. As blindness progressed the clarity, frequency and duration of the hallucinations faded. The patients had no abnormalities other than their eye disease, which in two cases was macula degeneration, and choroideraemia in the third.
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keywords = animal
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7/8. Bilateral intraocular pressure elevation and decrease of facility of aqueous humour outflow as a consequence of regional lymphoedema of head and neck.

    Aqueous humour drainage to the deep cervical lymphatics has been proven in animal experiments, but there have been no observations of changes in intraocular pressure (IOP) or aqueous humour drainage as a consequence of lymph drainage dysfunction. The history of a patient with non-Hodgkin's lymphoma, who underwent a left-sided radical neck dissection in January 1991, is reported. From the end of 1991 lymphoedema of the head and neck, predominantly on the right side, developed without venous stasis but with significant bilateral IOP elevation and aqueous humour outflow reduction, refract to conventional treatment. Two days after cytostatic treatment the lymphoedema disappeared, and IOP and aqueous humour outflow became normal. Four days later the patient died because of an acute bronchopneumonia. The pathological examination revealed an intact internal jugular venous system, enlarged lymph nodes and, as a consequence of previous irradiation, cicatrization in the right side of the neck. On the left side, there was absence of the internal jugular vein and jugular lymphatic trunk due to previous radical neck dissection. Our case suggests that regional lymphatic stasis and the absence of the collateral drainage to the contralateral side significantly reduces the aqueous humour outflow and leads to a bilateral, secondary IOP elevation.
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keywords = animal
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8/8. Ocular cryptococcosis - experimental and clinical observations.

    During the previous studies, we could demonstrate that there are strains of cryptococcus neoformans which after intraperitoneal inoculation, are capble of surviving only in the brain of mice without causing any apparent clinical symptom; only in about 4% of the animals the fatal involvement of the CNS occurred. The present investigations suggest that the selective involvement of eye, i.e., formation of intraocular cryptococcoma, with subsequent blindness is also possible under similar experimental conditions. It was observed that a short-lived uremia caused by intramuscular injection of 0.2 ml glycerine could enhance the rate of the selective involvement of the CNS including the eye. The uremia was controlled by the auxanographic method using STAIB's technique of serum-residual-nitrogen-auxanogram and employing the strain used for infecting the mice. These animal experiments are discussed in connection with a clinical case of intra-ocular cryptococcosis in which the only known basic disease was uremia of unknown orgin. The spontaneously healing cryptococcosis detected in this case after the treatment of uremia, has been discussed. Cases of ocular cryptococcosis described in the world literature have been also briefly discussed inconnection with our results. These microbiological observations will be supplemented by the histopathological findings to be published separately.
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ranking = 2
keywords = animal
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