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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An extrinsic total ophthalmoplegia developing two years before radiologic evidence of bronchial carcinoma and onset of Eaton-Lambert myasthenic syndrome is reported. Clinical and ENG data showed the neuromuscular location of the ophthalmoplegia, but repeated Tensilon and Prostigmine tests were negative. CT scan and CSF examinations revealed neither carcinomatous metastases nor inflammatory CNS disease. The case is an exceptional example of a paraneoplastic myasthenic syndrome long confined to the oculomotor muscles.
Ital J Neurol Sci 1985 Dec
PMID:Total extrinsic ophthalmoplegia as only paraneoplastic sign two years before X-ray diagnosis of bronchial carcinoma. 300 7

Lead exposure is an ancient malady. Its history serves as a useful paradigm through which to understand many other pollutants that our technological society has inserted into the human environment and may guide preventive steps for other agents. Lead poisoning was first recognized in workers exposed to high doses. The discovery of childhood toxicity occurred a century ago in Australia, when children with striking symptoms of paralysis, ophthalmoplegia, or meningitis were found to be highly lead exposed. Encephalopathy generally occurs at blood lead levels of 80 micrograms/dL or more, but unequivocal brain damage has been demonstrated at doses well below this level. At lower doses, the neurocognitive effects of lead are expressed as diminished psychometric intelligence, attention deficits, conduct problems, alterations in the electroencephalogram, school failure, and increased referral rates for special needs. Careful epidemiologic studies, which have controlled for the important confounders, have set the effect level at 10-15 micrograms/dL. Elegant animal studies in which confounding is not an issue have confirmed these findings. Although blood lead levels in the population have dropped over time for a number of reasons, there are some 3-4 million American children with blood lead levels of more than 15 micrograms/dL. Biochemical and functional changes have been demonstrated in the heme biosynthetic pathway and in the renal, cardiovascular, endocrine, immune, and nervous systems. The threshold for effect depends on the sensitivity of the methods used. A no-effect level has not been found. Further, this is not a disease of the poor alone. But the poor are exposed to much more lead than are the more economically favored. Deficiencies in body calcium, zinc, iron, and protein stores are associated with increased uptake. Optimizing nutrition enhances the resistance to lead. All children should be screened for lead at regular intervals, especially those with anemia, growth failure, and developmental or behavioral problems. Treatment protocols are well worked out, but chelation is only part of the therapy. Controlling the environment, strengthening the family's supports, enhancing nutrition, and offering remedial education are essential to a successful therapeutic outcome. Lead control has involved a continuing struggle between vested economic interests and regulatory agencies. In one area, the control of airbone lead, science, and public health have prevailed. In the past decade, the amount of alkyl lead consumed in gasoline additives has been reduced by 99%. Body lead burdens have dropped in close correspondence.(ABSTRACT TRUNCATED AT 400 WORDS)
Curr Probl Pediatr 1988 Dec
PMID:The persistent threat of lead: medical and sociological issues. 306 40

A new patient with Leigh's syndrome (subacute necrotizing encephalomyelopathy due to pyruvate dehydrogenase complex deficiency) is presented. A Turkish boy of consanguinously married healthy parents developed progressive muscle weakness since infancy. At the age of 3 years he was unable to sit, stand or walk. Clinical examination showed general muscle weakness, hypotonia, muscle hypotrophy, bilateral ptosis, partial bilateral external ophthalmoplegia, nystagmus, intention tremor and hypoactive tendon reflexes. The EEG showed diffuse slowing, the cerebral CT scan disclosed mild hydrocephalus e vacuo. Motor nerve conduction velocity was slightly decreased, the EMG revealed signs of neuropathy. In the biopsied muscle only a mild hypotrophy of type 2 fibres was found, no abnormal mitochondria could be detected. The sural nerve was slightly abnormal: loss of large myelinated axons, loss of unmyelinated nerves. CSF protein was elevated to 80 mg/dl, protein electrophoresis revealed the pattern of markedly impaired blood-CSF barrier. Serum lactate and pyruvate were permanently elevated. In the urine the excretion of alanine was raised. The clinical state deteriorated during intercurrent infections; somnolence, vomiting and Cheyne-Stoke's respiration occurred. At the age of 3 1/2 years the child died of pneumonia. In the liver tissue a decreased activity of the pyruvate dehydrogenase complex was found. Neuropathological examination of the brain demonstrated wide-spread changes of Leigh's spongiform encephalopathy. Several enzyme deficiencies have hitherto been associated with Leigh's syndrome: This patients confirms earlier findings that a subgroup of Leigh's syndrome is caused by pyruvate dehydrogenase complex deficiency.
Monatsschr Kinderheilkd 1987 Dec
PMID:[Leigh's subacute necrotizing encephalomyelopathy due to decreased activity of the pyruvate dehydrogenase complex]. 312 26

Kearns-Sayre syndrome is clinically defined by progressive external ophthalmoplegia, atypical retinitis pigmentosa and the potential occurrence of complete atrioventricular (AV) block. Right septal endomyocardial biopsy specimens from nine patients (four men and five women with a mean [+/- SD] [corrected] age of 36.3 +/- 14.4 years) with chronic progressive external ophthalmoplegia and mitochondrial skeletal myopathy were studied. Three patients had atypical retinal pigmentation. An atrioventricular or intraventricular conduction defect was observed in five patients. A pacemaker was prophylactically implanted in one patient because of abnormal conduction distal to the His bundle. Ultrastructural investigations revealed mitochondriosis in many heart muscle cells and an increased variability of mitochondrial form and size in all patients. In seven patients, 0.4 to 2.1% of all examined myocytes contained exclusively abnormal mitochondria. Three main types were observed: huge, mainly round mitochondria with concentric cristae; large, round or oval mitochondria with transverse or curved cristae; and small, vacuolated mitochondria. The volume density of myofibrils was reduced (41.9 +/- 11.1 compared with the normal value of 56.5 +/- 2.5 volume density [in percent], p less than 0.01) in these myocytes. Increasing numbers of vacuolated mitochondria correlated significantly with a reduction of myofibrils (r = -0.64, p less than 0.01). The data suggest that the ventricular myocardium of most patients with complete and even incomplete Kearns-Sayre syndrome is affected by disseminated mitochondrial cytopathy.
J Am Coll Cardiol 1988 Dec
PMID:Ultrastructural findings in endomyocardial biopsy of patients with Kearns-Sayre syndrome. 319 50

Vertical smooth eye movements were quantified by magnetic search coil oculography in 7 patients with internuclear ophthalmoplegia (INO), 4 bilateral and 3 unilateral, and compared with age-matched normal subjects. The upward and downward vestibulo-ocular reflex (VOR) had reduced gain and abnormal phase lag in both unilateral and bilateral INO during active head motion at frequencies from 0.25 to 2 Hz. The phase lag was attributed to disruption of vestibular eye velocity signals and relative preservation of eye position signals. Fixation of a stationary target enhanced smooth eye movement gain during vertical head motion but it remained subnormal. Vertical smooth pursuit gain was mildly reduced. All patients had normal gaze (eye plus head) gain when tracking with both the head and eyes despite defective cancellation of the vertical VOR; they employed high amplitude head tracking to compensate for the uncancelled VOR. Cancellation was more impaired than smooth pursuit. Dissociation between cancellation and ocular pursuit was also demonstrated by a model of VOR cancellation that uses measured pursuit gain with the head immobile to predict gaze gain of eye-head tracking. The model predicted eye-head tracking gain in normal subjects but not in patients with INO. The results indicate that the medial longitudinal fasciculi or neighbouring tegmental tracts convey bidirectional signals for vertical pursuit, cancellation, and vestibular smooth eye movements in man, and that smooth pursuit and cancellation of the VOR can be dissociated in INO.
Brain 1988 Dec
PMID:Vertical vestibulo-ocular reflex, smooth pursuit and eye-head tracking dysfunction in internuclear ophthalmoplegia. 320 59

Five children had an acute choreic syndrome that started three to seven days after hypothermic cardiopulmonary bypass surgery. Improvement after an interval was seen in all, with complete resolution in two. In addition to the chorea, all children suffered complete supranuclear ophthalmoplegia, which has also shown subsequent improvement. Bulbar damage caused considerable speech problems. There was no cognitive impairment. Several drugs were tried without effect. Investigations of the cause, including histological examination, have been unrevealing.
Arch Dis Child 1988 Dec
PMID:Choreic syndrome after cardiac surgery. 323 95

Seven cases of compromised pituitary fossa at the conventional skull X-ray, who had the final diagnosis of giant aneurysm of the intracavernous portion of the carotid artery (6 cases) and one of the anterior communicating artery, are reported. The main findings were: headache (7/7), complex ophthalmoplegia involving the III, IV and VI cranial nerves (5/7), compromised V cranial nerve (4/7) and eyeball pain (4/7). Other manifestations were: meningeal signs (2/7), unilateral blindness (1/7), hemiparesis (1/7), cacosmia (1/7) and inferior bitemporal quadrantanopsia (1/7). Five patients with intracavernous carotid artery aneurysm showed benefits with progressive occlusion of the internal carotid artery at the cervical level. One died before surgery. The case with anterior communicating artery aneurysm improved after its surgical clipping. Our data, in accord with the literature, support the conclusion that the differential diagnosis of aneurysms in the parasellar region remains a very difficult task. The accurate final diagnosis requires cerebral angiography and the surgical treatment with progressive occlusion at the cervical portion of the internal carotid artery has a relatively low risk with promising results.
Arq Neuropsiquiatr 1988 Dec
PMID:[Giant parasellar aneurysm simulating pituitary tumor]. 324 75

Orbital myositis implies orbital inflammation confined to one or more of the extraocular muscles. Orbital computerised tomography (CT) demonstrates irregular extraocular muscle enlargement which extends anteriorly to involve the tendon (muscle insertion). Six cases of presumed orbital myositis are reported, in each of whom the diagnosis was suspected clinically and confirmed by the orbital CT scan appearances. The mean age of the patients was 33 years (range 8-45 years). All presented with painful ophthalmoplegia and the majority manifested proptosis (five cases), conjunctival congestion (five cases) and periorbital and eyelid edema (two cases). Systemic corticosteroid therapy was used in two patients initially and also in another patient who relapsed, with rapid and dramatic responses. Extraocular muscle biopsy was performed in one case, disclosing features of non-specific muscle inflammation and no evidence of vasculitis. It is considered that orbital myositis is a discrete, identifiable subgroup within the spectrum of the nonspecific idiopathic orbital inflammatory syndromes; termed previously orbital 'pseudotumours'. Although the clinical features are frequently suggestive, they are nonspecific, and non-invasive investigations such as orbital ultra-sonography and CT scanning are required for precise anatomical tissue localisation and diagnosis. The role of ocular muscle biopsy is probably limited to atypical cases, or those unresponsive to steroid therapy, particularly to exclude neoplasia. Orbital myositis may be acute, subacute or recurrent. The acute form responds well to high doses of oral corticosteroids tapered gradually, but it may recur or become chronic. The subacute form of the disease responds less well.
Aust N Z J Med 1987 Dec
PMID:Orbital myositis: a study of six cases. 332 76

Although metastatic tumors of the pituitary gland and the sellar region are not common, they may radiographically mimic a pituitary tumor. Because the metastasis frequently involves the posterior lobe and because lateral extension to the cavernous sinus is common, patients may present with ptosis, diplopia, or diabetes insipidus. Decrease in anterior pituitary function is less common. Metastatic tumors contain abundant vascular networks, as evidenced by frequent blush on cerebral angiography and increased bleeding at operation. We report the case of a 57-year-old woman who presented with rapid onset of bilateral ptosis and ophthalmoplegia and was found to have a metastatic carcinoma within the pituitary gland.
Neurosurgery 1987 Dec
PMID:Metastatic tumor of the pituitary gland. 343 67

The case of a 25-year-old female with bilateral internuclear ophthalmoplegia as a presenting feature of systemic lupus erythematosus (SLE) is described. It is important to recognize that bilateral internuclear ophthalmoplegia may be an early manifestation of cerebral involvement in SLE in addition to its occurrence in multiple sclerosis.
J Rheumatol 1986 Dec
PMID:Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. 356 Jan 4


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