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)

The authors report two cases of complete ophthalmoplegia occurring during surgical section of the homolateral vidian nerve for chronic nasal obstruction due in one case to hypertrophic rhinitis and in the other to nasal polyposis. In both cases, whilst there was more or less complete regression of the oculomotor paralyses, unilateral amaurosis persisted as a permanent sequela. This paper is published with the aim of drawing attention to the possibility of complications which are out of proportion with the initial symptomatic discomfort and which may occur whatever the surgical approach used since one of the operations was performed via a pure endonasal approach whilst the other was transmaxillary. In both cases there was no damage to the orbit during surgery and the most likely pathogenesis would appear to be a reflex aciton.
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PMID:[Two cases of total ophthalmoplegia after section of the vidian nerve (author's transl)]. 50 43

This is the third reported case of a neonate with ophthalmoplegia in whom the diagnosis of maple syrup urine disease (MSUD) was delayed into infancy. In this case, the demonstration of significant residual branched-chain ketoacid decarboxylase activities suggests that this atypical presentation may be characteristic of the MSUD variant form.
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PMID:Ophthalmoplegia and bulbar palsy in variant form of maple syrup urine disease. 50 61

The Kearns-Sayre syndrome (KSS) appears to be a distinctive disorder characterized by progressive external ophthalmoplegia, pigmentary degeneration of the retina, heart block, and elevated CSF protein levels. Recent reports have suggested that abnormalities of muscle mitochondria may also be a consistent finding in KSS. We recently examined a patient with KSS whose skeletal muscle contained abnormal mitochondria. In addition, a computerized tomographic scan of the head showed cerebellar and brain stem atrophy, as well as calcification in the region of the basal ganglia.
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PMID:Basal ganglia calcification in Kearns-Sayre syndrome. 50 31

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

A 70-year-old woman with a long history of nasal and sinus polyps developed bilateral proptosis and left total external ophthalmoplegia. She was known to have hypercalcemia, which was later discovered to be caused by a parathyroid adenoma. X-ray studies, including computerized tomography, revealed increased radiodensity in the ethmoid sinuses (due to dystrophic calcification from the hypercalcemia), partial absence of the orbital walls from earlier surgical procedures, and bilateral rounded, retrobulbar tumors. At surgery, glistening, yellow, transparent, and encapsulated-lobulated masses were removed from the left orbit; they appeared to have prolapsed through a postsurgical dehiscence in the superomedial orbital wall. Light and electron microscopy confirmed that the lesional tissue represented polyps covered by respiratory epithelium; the yellow color was the consequence of secondary lipidization of the stromal fibroblasts.
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PMID:Sino-orbital polyposis. 51 88

On the basis of two cases of progressive external ophthalmoplegia the authors discuss the clinical manifestations of this disease and call attention to diagnostic difficulties.
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PMID:[2 cases of ocular myopathy]. 52 39

In the reported case unilateral external ophthalmoplegia developed in a young woman, with concomitant pain and anaesthesia in the area innervated by the 1st branch of the trigeminal nerve. After ruling out an expanding lesion of the central nervous system, aneurysm, diabetes, myasthenia, multiple sclerosis and other diseases dexamethasone was administered in a total dose of 30 mg. Complete clinical remission was achieved. In the light of observations and a survey of the peritinent literature it has been accumed that the disease was caused by a non-specific process in the small vessels in the vicinity of the cavernous sinus.
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PMID:[Tolosa-Hunt syndrome (ophthalmoplegia dolorosa)]. 52 41

Fusiform Aneurysms are encountered in elderly persons with advanced arteriosclerosis. When they are enlarged to compress the neighboring structures, they may resemble tumors symptomatically. Especially when situated in close relation to the optic nerve or chiasmatic region, pituitary tumor is likely to be suspected. This is a case of 62 year old female with a giant fusiform aneurysm of the right internal carotid artery extending to the middle cerebral artery which caused left homonymous hemianopsia and a large aneurysm in the cavernous portion of the left internal carotid artery with fusiform extension of the distal portion of the carotid artery which presented left blepharoptosis, dilated pupil and total ophthalmoplegia. The basilar artery and the right meningohypophyseal artery showed fusiform dilatation as well. Systemic atherosclerotic change, abdominal aortic aneurysm, nephrosclerosis were also accompanied. The computed tomography demonstrated the aneurysms distinctly. Left oculomotor palsy disappeared after six months with residue of left homonymous hemianopsia. Surgical intervention was not attempted, because of the widely distributed constitutional aneurysms in the whole body.
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PMID:[A case of multiple fusiform aneurysms presenting homonymous hemianopsia (author's transl)]. 52 70

The anterior internuclear ophthalmoplegia, a dissociated ophthalmoplegia of adduction secondary to an ipsilateral supranuclear paresis of the internal rectus, represents a well-know clinical picture. The dissociated ophthalmoplegia of abduction, however, sometimes referred to as posterior internuclear ophthalmoplegia, is debated. Such an oculomotor disturbance emerging from an ophthalmoplegic polyneuropathy (Fisher syndrome) is presented and illustrated by oculographic tracings. A secondary finding was a limited range of accommodation. Oculographically, the dissociated ophthalmoplegia of abduction reflects a true mirror image of the anterior internuclear ophthalmoplegia. Because of anatomical considerations the dissociated ophthalmoplegia of abduction cannot be regarded as internuclear ophthalmoplegia, and the terminology of posterior internuclear ophthalmoplegia is not tenable. Discussing the pathophysiology of the Fisher Syndrome, it is reflected upon an infranuclear lesion causing an ocular motility disorder featuring supranuclear aspects, and avoiding limitations of peripheral nerve damage.
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PMID:[Dissociated ophthalmoplegia of abduction: so-called posterior internuclear ophthalmoplegia in Fisher's syndrome]. 53 May 53

Three patients are reported in whom paralysis of accommodation and mydriasis (internal ophthalmoplegia) developed following inferior oblique myectomy. In all three patients, accommodation eventually returned to normal; in two, mild anisocoria persisted; in one patient, pupillotonia was noted after 2 1/2 years. The mechanism of this previously unreported surgical complication is thought to be excessive stretching of the nerve to the inferior oblique muscle with secondary trauma to the ciliary ganglion.
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PMID:Internal ophthalmoplegia following inferior oblique myectomy: a report of three cases. 54 43


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