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)

Histopathologic findings were examined in skeletal muscle biopsies from 6 patients with myoclonus epilepsy with ragged-red fibers (MERRF) who had an A to G base substitution at mitochondrial DNA (mtDNA) nucleotide pair 8344. In addition to variation in fiber size and ragged-red fibers, all specimens in cross sections showed focal cytochrome c oxidase (CCO) deficiency, suggesting that this finding is crucial in elucidating the role of the mutant mtDNA in the pathogenesis of this disorder. Along the length of single muscle fibers, defects in CCO activity were distributed segmentally with blurred borders in 5 patients which were in contrast with segmental defects with sharply delineated borders seen in chronic progressive external ophthalmoplegia with deleted mtDNA. These morphologically heterogeneous defects in CCO activity may in part be due to differing populations of and distributions of wild and mutants mtDNAs.
J Neurol Sci 1991 Dec
PMID:Muscle histopathology in myoclonus epilepsy with ragged-red fibers (MERRF). 166 7

A patient with superior orbital fissure syndrome caused by metastatic hepatocellular carcinoma is reported. She had painful ophthalmoplegia and decreased sensation along the first branch of the trigeminal nerve. Histological confirmation was done by biopsy of sternal metastases. The awareness of various neurological presentations apart from hepatic failure in this malignancy is emphasized.
J Med Assoc Thai 1991 Dec
PMID:Superior orbital fissure syndrome as a presenting symptom in hepatocellular carcinoma. 166 29

We studied 39 blind painful eyes in 39 patients who were treated with retrobulbar injection of absolute (96%) alcohol for their severe ocular pain at the King Khaled Eye Specialist Hospital from January 1984 to January 1987. There were 21 (54%) male and 18 (46%) female patients; all were followed for at least three months. The protracted ocular pain was mainly due to: end-stage (absolute) glaucoma in 31 (80%) eyes, uveitis or endophthalmitis in four (10%) eyes, or corneal ulcer in two (5%) eyes. One eye had painful phthisis bulbi, and one eye had infraorbital neuralgia. The complications encountered were transient and included blepharoptosis in eight (21%) eyes, external ophthalmoplegia, and corneal epithelial defect. The effective time of the injection to relieve pain ranged from two weeks to two years (mean, 29 weeks). The authors believe that there is still a place for retrobulbar alcohol injection for blind painful eyes when enucleation or evisceration is not possible.
Ann Ophthalmol 1990 Dec
PMID:Retrobulbar alcohol injection in blind painful eyes. 170 80

Mutant mitochondrial DNA with large-scale deletions (delta-mtDNA) has been frequently observed in patients with chronic progressive external ophthalmoplegia (CPEO), a subgroup of the mitochondrial encephalomyopathies. To exclude involvement of the nuclear genome in expression of the mitochondrial dysfunction characteristic of CPEO, we introduced the mtDNA of a CPEO patient into clonal mtDNA-less HeLa cells and isolated cybrid clones. Quantitation of delta-mtDNA in the cybrids revealed that delta-mtDNA was selectively propagated with higher levels of delta-mtDNA correlating with slower cellular growth rate. In these cybrid clones, translational complementation of the missing tRNAs occurred only when delta-mtDNA was less than 60% of the total mtDNA, whereas accumulation of delta-mtDNA to greater than 60% resulted in progressive inhibition of overall mitochondrial translation as well as reduction of cytochrome c oxidase activity throughout the organelle population. Because these cybrids shared the same nuclear background as HeLa cells, these results suggest that large-scale deletion mutations of mtDNA alone are sufficient for the mitochondrial dysfunction characteristic of CPEO.
Proc Natl Acad Sci U S A 1991 Dec 01
PMID:Introduction of disease-related mitochondrial DNA deletions into HeLa cells lacking mitochondrial DNA results in mitochondrial dysfunction. 172 May 44

The signs and symptoms of the Steele-Richardson-Olszewski syndrome (SRO) are described and the therapeutic possibilities are outlined. Two cases are presented. SRO must be suspected when a patient over the age of 40 years presents axial rigidity, dementia, pseudobulbar palsy, and supranuclear ophthalmoplegia.
Ugeskr Laeger 1991 Dec 02
PMID:[Steele-Richardson-Olszewski syndrome. Progressive supranuclear paresis]. 177 79

Migraine is classified into four basic categories: common, classic, cluster, and complicated. Ocular migraine is a periodic loss of vision in one eye typically lasting 30 minutes or less. Ophthalmoplegia is a severe ipsilateral hemicranial headache that is preceded by aura of lightning flashes and expanding circles of light, accompanied by temporary ocular motor nerve palsies.
Insight 1991 Dec
PMID:Vision and migraine. 178 28

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.
Cent Afr J Med 1991 Dec
PMID:Cavernous sinus thrombophlebitis in Zimbabwe. 148 21

Ophthalmoplegia is a rarely observed sign in carbamazepine and other anticonvulsant overdoses. We present a patient who developed transient complete external ophthalmoplegia and asterixis with relative preservation of consciousness, in association with carbamazepine toxicity. Previously reported cases and proposed mechanisms are reviewed.
Aust N Z J Med 1991 Dec
PMID:Complete external ophthalmoplegia and asterixis with carbamazepine toxicity. 181 51

A 42 year old man presented with a slowly progressive gait disturbance, generalised weakness, dysarthria, clumsiness and tremor of his hands, and involuntary jerks. Hexosaminidase A activity in plasma, leucocytes and fibroblasts was considerably reduced, establishing the diagnosis of GM2 gangliosidosis. Clinical examination showed two previously unreported features, a clinically evident sensory neuropathy and internuclear ophthalmoplegia.
J Neurol Neurosurg Psychiatry 1991 Dec
PMID:An adult onset hexosaminidase A deficiency syndrome with sensory neuropathy and internuclear ophthalmoplegia. 183 93

We describe a patient with a dissociated vertical nystagmus and an internuclear ophthalmoplegia. The vertical nystagmus consisted of a left downward nystagmus with a synchronous right intorting nystagmus when the patient looked down and to the left. This rare type of nystagmus has been described both in isolation and in association with an internuclear ophthalmoplegia. Previous authors postulated a lesion in the midbrain in the region of the medial longitudinal fasciculus. In our patient, a discrete midbrain infarction was demonstrated on magnetic resonance imaging in the hypothesized location, thus providing supportive anatomical evidence for a vertical gaze coordination pathway in the region of the medial longitudinal fasciculus.
Arch Neurol 1991 Dec
PMID:Dissociated vertical nystagmus and internuclear ophthalmoplegia from a midbrain infarction. 184 38


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