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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To our knowledge, the juvenile form of spongy degeneration of the CNS (SD-CNS); van Bogaert-Bertrand disease) has been described previously only three times. We report the case of 21 1/4-year-old Japanese woman who was first seen at the age of 11 with growth retardation, ptosis, and ophthalmoplegia. Her progressive neurodegenerative disease included retinitis pigmentosa, blindness, partial deafness, cerebellar dysfunction, hyporeflexia, and muscle wasting. Simultaneous endocrine defects were diabetes mellitus and probable hyperaldosteronism. Heart block developed later. She died of bronchopneumonia. Autopsy showed CNS stigmas typical of spongy degeneration. Additional findings included peripheral nerve demyelination, neurogenic muscle atrophy, pituitary and pancreatic atrophy, right adrenal agenesis, and a left adrenal coritcal lipid-cell adenoma. To our knowledge, our patient was the oldest survivor, the first patient of Japanese ancestry, and had a unique concurrence of certain oculoendocrine defects.
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PMID:Spongy degeneration of the CNS: an instance of the rare juvenile form. 50 59

In 34 patients with hypertelorism aged on the average 12.6 years, 24 underwent intracranial surgery (combined intra- and extracranial approach), 2 (moderate) U-osteotomy (subcranial approach), and 8 (mild) canthoplasties. Improved results were obtained in the patients with various types of hypertelorism. The complications were reviewed. Of the 24 patients undergone intracranial surgery, one died, 4 had cerebrospinal fluid leakage, and 4 had keratitis. No seizure, cerebral edema, meningitis, blindness, and ptosis were observed in these patients. Average blood loss was 72% of blood volume. Average duration of surgery was 7.5 hours. The causes of hypertelorism such as craniofacial cleft, craniosynostosis, frontoethmoidal meningoencephalocele, frontonasal fibrous dysplasia, and trauma were also discussed. Satisfactory appearance was seen in most of the patients.
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PMID:[Surgical correction of 34 patients with hypertelorism]. 147 4

A 10-year-old schoolboy was referred to the Ophthalmic Unit of Ahmadu Bello University Teaching Hospital because of sudden loss of sight following 5 days of severe frontal headache. The child had bilateral ptosis with internal and external ophthalmoplegia and fixed and dilated pupils. There was no papilloedema. Eight days later, a jaw tumour and a rapidly enlarging abdominal tumour appeared. A fine needle aspiration biopsy of the jaw tumour confirmed Burkitt's lymphoma. Combination chemotherapy with cyclophosphamide, vincristine and methotrexate (COM) led to a rapid resolution of the jaw and abdominal tumour but the child never regained his sight. Cerebrospinal fluid examination was not helpful in reaching a diagnosis.
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PMID:Burkitt's lymphoma presenting with blindness: a case report. 170 53

With the possible exception of aesthetic considerations, the visual system is more involved in craniosynostosis than any other. Changes may be an inherent feature of the pathologic process or occur as a secondary complication. Several of these are potentially devastating, resulting in substantial permanent impairment in visual function. Because these are avoidable if recognized and managed early, prompt involvement of an ophthalmologist in the care of children with craniosynostosis is essential. Improvement in the appearance of these cases cannot obscure the importance of functional problems. In particular, disc edema, optic atrophy, and progressive optic nerve dysfunction may accompany increased intracranial pressure even without evidence of hydrocephalus and even with apparently open fontanelles. Uncorrected refractive error (particularly anisometropia), strabismus, ptosis, and corneal exposure problems are an invitation to the development of amblyopia. If not reversed, this can lead to permanent visual disability. Proptosis and corneal exposure problems are a third potentially treatable cause of functional blindness. Early diagnosis and prompt care will hopefully continue to improve the prognosis for these increasingly treatable children.
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PMID:Ophthalmic features of craniosynostosis. 182 6

Blepharospasm is a relatively frequent cranial dystonia which may be seen either alone or related to orofacial-mandibular dystonia (Meige's syndrome). In its maximum degree it can cause functional blindness.Twelve patients with blepharospasm (4 essential and 8 Meige's syndrome) who had been previously treated unsuccessfully with drugs (trihexyphenidyl, biperiden, carbamazepine, lithium, baclofen, lisuride, imipramine, clonazepam and butyrophenones) were treated for 12 months with periocular injections of botulinum toxin (BOTOX). A "low" dose of 12,5 U per eye was employed. With this dose, eleven out of twelve patients experienced significant improvement which lasted from five to fifteen weeks. The only nonresponder obtained complete relief upon duplicating the dose. The only side effect was uni or bilateral ptosis in six patients which improved completely in seven to twenty one days. One patient developed a peripheral facial palsy with complete remission in nineteen days. No systemic side effects were noted. There was only one desertion from this study due to depression enhanced by prolonged (21 days) ptosis. All patients (including the deserter) agreed that treatment with BOTOX provided more relief than any other previous therapeutic method. Our results confirm those obtained by others but a more prolonged study is needed to better evaluate long term effects.
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PMID:[Treatment of blepharospasm with botulinum toxin]. 210 46

Clinical signs in dogs with pseudorabies (Aujeszky's disease) were tabulated from 25 confirmed cases. The duration of disease was short, ranging from 6 to 96 hours. Eight dogs were euthanatized. Of those not euthanatized, 12 (71%) died within 24 hours of onset, 16 (94%) died within 48 hours, and only 1 (6%) lived longer than 48 hours (96 hours) after the onset of clinical signs. All of the dogs had ptyalism, 84% were restless, 84% were anorectic, 76% were atactic, and 64% wandered aimlessly. Sixty-four percent of the dogs had tachypnea, 60% had dyspnea, 56% vocalized, 52% were pruritic, 48% held their necks rigidly, 36% vomited, 36% had muscle spasms, 36% were aggressive, 28% had trismus, and 24% had dysphagia. Five of 25 dogs (20%) had abnormal pupillary light responses. Two of the 25 dogs circled and 2 walked backwards. Each of the following were detected once: blindness, ptosis, facial paresis, excessive lacrimation, head-tilt, head-pressing, signs of abdominal pain, and photophobia. All dogs had been exposed to swine, although in some instances the farmer was unaware pseudorabies existed in the herd or believed it was not in the herd on the basis of negative results on serologic testing.
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PMID:Clinical signs associated with pseudorabies in dogs. 277 5

The orbital apex syndrome is a very rare complication of fractures of the facial skeleton, as well as other conditions and is characterized by blindness, fixed dilated pupils, proptosis, ptosis of the eye and ophthalmoplegia. We are reporting such a case we had the opportunity of treating.
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PMID:Orbital apex syndrome. 311 66

A case of Stickler's syndrome is reported. This syndrome associates eye defects, craniofacial and musculo-skeletal abnormalities, sensori-neural hearing loss and mitral-valve prolapse. It is an autosomal dominant disorder probably resulting from a connective tissue dysplasia. There is a major risk for the occurrence of serious ocular problems (blindness), and ophthalmologic follow-up has to be performed on affected persons and relatives in order to improve the long term prognosis.
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PMID:[Stickler's syndrome or hereditary progressive arthro-ophthalmopathy]. 341 72

Four cases of orbital cellulitis following the extraction of maxillary molars are presented. The time interval between dental extraction and development of orbital symptoms ranged from two hours to 13 days. All patients presented with fever, elevated leukocyte counts, and radiologic evidence of acute ipsilateral paranasal sinus infection. In addition, one patient presented with meningitis. Predisposing factors in three patients included nephrotic syndrome with chronic antral inflammation, pregnancy with upper respiratory tract infection, and heroin addiction. Sequelae included empyema and death, severe loss of vision, and blindness with ptosis and exotropia. One patient recovered completely. The anatomic pathways by which dental infection can spread to the orbit are discussed, and general therapeutic considerations are emphasized.
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PMID:The spread of odontogenic infections to the orbit: diagnosis and management. 386 71

An epidemic of Neisseria gonorrheae keratoconjunctivitis in African adults occurred in Malawi in 1983. Sixteen patients, seven females and nine males, aged 18 to 60 years, were admitted to the inpatient ocular services at Queen Elizabeth Central Hospital in Blantyre from 1 February to 28 May 1983, all with severe bilateral purulent keratoconjunctivitis and concomitant venereal infection secondary to N. gonorrheae. Corneal melting, corneal perforation with iris prolapse, and endophthalmitis occurred in 10 eyes, of which five required enucleation. Thirteen additional eyes sustained severe visual loss secondary to corneal ulceration, leucomata, and healed corneal perforation. The route of transmission and factors of epidemicity are speculative. Because of worldwide epidemic venereal infection ophthalmologists and epidemiologists should be alert to probable sporadic N. gonorrheae epidemics in adults. Such outbreaks could occur elsewhere, especially in the developing world, and ocular gonococcal infection might become a significant cause of irreversible blindness.
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PMID:Destructive epidemic Neisseria gonorrheae keratoconjunctivitis in African adults. 401 48


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