Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cranial arteritis (CA) presenting as sudden blindness is well documented, and early recognition of this entity in an elderly patient with visual loss or diplopia is of critical importance. This entity presents a difficult diagnostic problem when temporal artery biopsy is negative, as in our case, or when the erythrocyte sedimentation rate is normal. The present report details an unusual patient with "occult temporal arteritis" who sustained abrupt monocular visual loss and subsequent ipsilateral ophthalmoplegia involving all functions of the oculomotor nerve. Despite negative biopsies of each temporal artery, other probable etiologies of the symptom complex were excluded, and the diagnosis of cranial arteritis is warranted. The patient is unique in that the oculomotor palsy is complete and permanent. This report emphasizes cranial arteritis masquerading as an intracranial aneurysm.
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PMID:Biopsy-negative cranial arteritis with complete oculomotor nerve palsy. 43 39

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

Unilateral blindness and total ophthalmoplegia developed on the same side on which transantral ligation of the internal maxillary artery was performed for persistent posterior epistaxis. Evaluation with arteriograms and phlebograms showed no compromise of retinal vessels or cavernous sinus thrombosis. At reexploration of the maxillary antrum, marked swelling of previously placed oxidized cellulose gauze was found. An experimental model, using the rabbit, was designed to determine if implantation of oxidized cellulose gauze adjacent to the optic nerve would produce blindness and ophthalmoplegia.
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PMID:Blindness and total ophthalmoplegia. A complication of transantral ligation of the internal maxillary artery for epistaxis. 94 28

Intraneural and perineural spread of squamous carcinoma from the face to the cranial cavity is an important cause of delayed cranial nerve palsies after local excision of a skin tumour. As exemplified in reports of two cases, signs of this type of centripetal spread of squamous cell tumour along the branches and trunk of the supraorbital nerve are (i) severe unremitting orbital and forehead pain with associated hypoaesthesia, (ii) palpable or radiological evidence of thickening of the nerve at the supraorbital notch and (iii) evolution of ophthalmoplegia, blindness, and sensory loss in the first division of the trigeminal nerve. Appearance of severe supraorbital neuralgia months or years after excision of a skin tumour from the forehead should alert the clinician to extension of tumour cells along the supraorbital nerve. This may enable him to institute timely treatment before a complete orbital apex syndrome has developed.
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PMID:Painful ophthalmoplegia following treated squamous carcinoma of the forehead. Orbital apex involvement from centripetal spread via the supraorbital nerve. 95 Sep 37

Two cases are described presenting a clinical picture of sudden headache, fever and vomiting, accompanied by unilateral ophthalmoplegia and acute deterioration of visual acuity. In one this amounted to monocular blindness with a field defect in the other, indicative of chiasmal compression. Necrotic pituitary adenoma material was removed by the transphenoidal route in both cases. Recovery of eye movements was obtained and considerable improvement of visual acuity, which in one patient, was restored completely.
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PMID:Trans-sphenoidal surgical approach in pituitary apoplexy. 126 21

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

Cavernous sinus thrombophlebitis (CST) was diagnosed in 19 black African patients who presented to two large, general hospitals in Harare, Zimbabwe, over an eight-year period. Diagnosis was based on clinical criteria. The mean age of patients was 22.5 years (range 8 months-70 years). Only three patients (15.5 pc), all of whom were promptly diagnosed and commenced on a regimen including intravenous, high-dose cloxacillin, recovered completely. In ten cases (52.6 pc), initial treatment was penicillin and chloramphenicol. Four of the 19 patients who had serious sequelae including residual blindness, complete ophthalmoplegia or unilateral proptosis, and six patients (31.6 pc) died. The higher than usual mortality rate in this series can be attributed to various factors including late presentation, delay in diagnosis and delay before initiation of effective antibiotic treatment. Whenever CST is suspected, antibiotic treatment should be administered without delay pending further evaluation, and the initial regimen should include high-dose antistaphylococcal antibiotics.
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PMID:Cavernous sinus thrombophlebitis in Zimbabwe. 148 21

The authors report on one case of Wolfram syndrome, a rare condition, which is characterized by juvenile onset diabetes mellitus, diabetes insipidus, optic atrophy and sensorineural deafness. The findings of this 13-year follow-up show that this patient developed typical neurological complications of long-standing diabetes mellitus as in the common type 1 variant. Moreover, some peculiar signs occurred such as anosmia, ophthalmoplegia interna, and central nystagmus. Since Wolfram syndrome is probably part of a more generalized neurodegenerative disorder, long-term prognosis will depend both upon the severity of chronic diabetic complications and upon the rapidity, by which degeneration of cerebellar, pontine and brain stem structures appear. Prognosis of the cardinal clinical signs is such that optic atrophy, though usually quite rapid in the beginning, generally does not lead to complete blindness. Sensorineural hearing loss progresses very slowly so that deafness might be expected exceptionally only. The hearing deficit in classical diabetics, however, is of retrocochlear origin. Therefore, in Wolfram syndrome, a combined inner-ear and retrocochlear hearing loss may occur.
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PMID:Juvenile onset diabetes mellitus, central diabetes insipidus and optic atrophy (Wolfram syndrome)--neurological findings and prognostic implications. 185 94

Maple Syrup Urine Disease is an autosomal recessive disorder of branched chain amino acid metabolism with an incidence in Ireland of one in 140,154 births. Ocular complications in untreated or late diagnosed patients includes optic atrophy, grey optic papilla, nystagmus, ophthalmoplegia, strabismus and cortical blindness. Seven patients with maple syrup urine disease were studied. All were diagnosed with the aid of newborn screening and commenced on early dietary treatment (mean age at diet introduction = 5 days). All remain physically well, with average intellectual performance, three having minor neurological defects and one strabismus. Early diagnosis, proper therapy and subsequent vigilant management may reduce substantially the risks of ophthalmic complications in this rare disease.
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PMID:Ophthalmic findings in maple syrup urine disease. 192 19

When blood flow through the internal and external carotid arteries is completely interrupted by ipsilateral common carotid artery occlusion, the arterial orbital circulation may be more compromised than the brain supply. We studied a pure and extreme example of this situation in a patient who presented with acute orbital infarction, but no cerebral ischemia on clinical, CT and single-photon emission computerized tomography (SPECT) grounds. Ipsilateral blindness corresponded to retinal, choroidal and optic nerve infarction. The pattern of ophthalmoplegia, with relative sparing of adduction, was more compatible with a muscle than a nerve dysfunction, but a reactive dilated pupil, corneal anesthesia, and orbital pain suggested that the intraorbital branches of the ocular motor nerves and ophthalmic division of the trigeminal nerve were not spared. In addition, signs of widespread ocular ischemia were present. Sequential examinations documented the evolution pattern over 1 year. The absence of an orbital collateral supply from the contralateral external carotid and muscular cervical arteries systems, which contrasted with an adequate middle cerebral artery supply via the contralateral internal carotid artery, may explain this isolated and complete form of orbital ischemia due to common carotid artery occlusion.
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PMID:Isolated complete orbital infarction: a common carotid artery occlusion syndrome. 204 19


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