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)

Whipple's disease presenting as a neurological disease without gastrointestinal symptoms is an unusual occurrence. A 40 year old man suffered hypersomnia, memory loss and progressive ophthalmoplegia for 6 months prior to death. The nature of this disease was not established during life. Extensive granulomatous inflammation affecting the hypothalamus, hippocampus and periaqueductal gray matter of the brain was found to represent Whipple's disease by electron microscopy. Characteristic lesions were also present in spleen, mesenteric lymph nodes, small intestine and myocardium. Bacillary bodies and membranous inclusions similar to those seen in visceral lesions of Whipple's disease were present in macrophages. The findings supported the theory of direct involvement of the central nervous system by bacilli rather than a metabolic origin for the lesions.
Acta Neuropathol 1976 Sep 15
PMID:Whipple's disease of the central nervous system. 6 98

A family was found to exhibit progressive external ophthalmoplegia, congenital cataracts, variable somatic weakness, gonadal dysgenesis, and, in one member, an abnormal chromosomal pattern. Muscle biopsy specimens showed "ragged-red" fibers; electron microscopy showed widespread paracrystalline mitochondrial inclusions. Orbicularis oculi reflex testing in the propositus showed bilateral absence of the late response. The family was evaluated in relation to other familial mitochondrial myopathies; a central defect in brain stem neural transmission was suggested as a mechanism for the progressive external ophthalmoplegia.
Arch Neurol 1979 Sep
PMID:A familial mitochondrial myopathy with central defect in neural transmission. 22 46

Two elderly patients had unilateral ophthalmoparesis and retrobulbar pain. Both had subtle lid signs of aberrant regeneration of the third nerve without proceding acute oculomotor paralysis, and both were found to have intracranial aneurysms. Although primary aberrant oculomotor regeneration has been reported previously in patients with aneurysms and meningiomas, the diagnostic importance of this sign in the elderly has not been emphasized. The presence of lid elevation on downgaze associated with slowly progressive ophthalmoplegia in patients aged 65 or older suggests aneurysm in or near the cavernous sinus.
Arch Neurol 1979 Sep
PMID:Primary aberrant oculomotor regeneration due to intracranial aneurysm. 22 47

A left infraclinoid carotid aneurysm (3 x 2-5 x 2 cm) in a 60-year old woman presented with total ophthalmoplegia and trigeminal symptoms. A staged internal carotied occlusion in the neck was performed using a SALIBI-clamp. A chronic infectious fistula developed (Klebsiella aerogenes). When superficial debridement failed, operative removal of the SALIBI-clamp was planned seven months after its insertion. The clamp was found subcutaneously without any relation to the internal carotid artery and was easily removed. Subsequent catheter angiography proved the left internal carotied to be completely occluded in the neck. No cross filling of the aneurysm could be demonstrated from the right carotied nor the vertebro-basilar arteries. The neurological deficit remained unchanged. The wound closed promptly without further signs of infection.
Neurochirurgia (Stuttg) 1977 Sep
PMID:Explusion of SALIBI-clamp from the internal carotied artery in the neck by infection. 33 3

Four term infants were found by computerized cerebral tomography (CT) to have intraventricular hemorrhage. All were male infants who had experienced substantial intrapartum complications. One infant had subtle clinical signs at 2 days of age; the others had major neurologic findings including seizures and ophthalmoplegia that occurred at 1, 4, and 6 weeks of age, respectively. Three infants in whom the hemorrhages were indistinguishable by CT from those described in preterm infants now have arrested hydrocephalus and normal development. The fourth infant was found to have a large glioblastoma at the site of origin of his initial hemorrhage. These cases emphasize the need to consider this diagnosis in neonates born at term who have abnormal neurologic signs.
Am J Dis Child 1979 Sep
PMID:Intraventricular hemorrhage in the neonate born at term. 47 46

A 70-year-old man with known vascular disease, who had bilateral internuclear ophthalmoplegia, and an 18-month-old girl with a long-term variable esotropia culminating in a postinfectious oculopharyngeal neuropathy both responded with a positive edrophonium chloride (Tensilon) test, strongly suggesting the diagnosis of myasthenia gravis. Both subsequently received maintenance doses of neostigmine methylsulfate (Prostigmin).
Am J Ophthalmol 1979 Sep
PMID:Ocular myasthenia gravis mimicking pseudointernuclear ophthalmoplegia and variable esotropia. 48 61

Sixty-five cases of berry aneurysm on the supraclinoid portion of internal carotid artery were reviewed on the viewpoint of oculomotor palsy. 27 of 65 patients (42%) showed oculomotor palsy. Most cases of complete oculomotor paralysis developed immediately after having subarachnoid hemorrhage, whereas the case of gradually developing oculomotor palsy tended to manifest diplopia first, and followed with blephaloptosis. The recovery rate from oculomotor palsy seemed to be correlated with the duration from the onset of symptom to surgery. The recovery from the complete oculomotor paralysis lasting for a long time seemed to be unsatisfactory unless the surgical direct procedure to the aneurysm was successful in early stage.
No Shinkei Geka 1979 Sep
PMID:[Oculomotor palsy due to aneurysm of internal carotid artery (author's transl)]. 49 66

The case of a 16-year-old boy with progressive external ophthalmoplegia ("Ophthalmoplegia plus") is reported. Clinical symptoms consisted of complete external ophthalmoplegia, ptosis, short stature, atrophy and weakness of skeletal muscles, and elevated csf protein. Abnormal mitochondria were found by electron microscopy in scattered atrophic fibres of the muscle biopsy. The syndrome and it's relevance for paediatric differential diagnosis is discussed.
Klin Padiatr 1978 Sep
PMID:[Progressive External Ophthalmoplegia (authors transl)]. 56 96

Ten cases of painful ophthalmoplegia are reported. In all cases pain marked the onset of the disease; the condition was mostly unilateral. Recurrence and dramatic response to steroid therapy were more constant features than angiographic findings which may be completely normal. CAT permitted us to eliminate a tumor of the cavernous sinus in 7 cases, but in one case, which was operated, an aggravation followed surgery. Despite complete investigations of all patients, some questions remain unanswered. Is it an inflammatory or an allergic inflammatory process? (And the presence of antinuclear factor in an only case does not permit it to respond.) Why is the process confined to the superior orbital fissure? Mathew and Chandy tried to find a similarity between this syndrome and Bell's palsy although the 2 syndromes have not been shown to coexist. Is the Tolosa-Hunt syndrome comparable to the pseudotumor of the orbit? Although the dura mater is inserted on the border of the sphenoidal cleft, some patients may altern the 2 syndromes.
Ann Ophthalmol 1978 Sep
PMID:Tolosa-Hunt syndrome. 73 3

Two hundred and twenty-seven cases of carcinoma metastatic to the eye and orbit have been reviewed previously. The orbit alone was involved in 28 cases: theses lesions constitute the basis of this clinicopathologic study. The most common signs and symptoms produced by orbital metastasis included exophthalmos (75%1, pain (29%), decreased vision (29%), periorbital swelling (25%), a visible mass (21%), ophthalmoplegia, and diplopia (18%). The ophthalmologists' preoperative (or premortem) clinical diagnoses were: orbital mass, 36%; metastatic carcinoma, 29%; leukemia, 7%; melanosarcoma, 4%; mixed tumor of lacrimal gland, 4%; and meningioma, 4%. The sites of the primary tumors in the 28 patients with orbital metastasis were as follows: breast, eight; lung, four; genitourinary tract, four; pancreas, one; and ileum, one. In 10 patients the site of the primary carcinoma was not determined. In 17 of the 28 patients, symptoms of orbital metastasis preceded detection of a primary tumor elsewhere in the body. In 10 of the remaining 11 patients, detection of the primary tumor had preceded the onset of orbital symptoms. In one patient, symptoms of the primary tumor and of orbital metastasis appeared at about the same time. The median survival of patients with carcinoma metastatic to the orbit was 15.6 months from the time of orbital surgery. This was much better than the median survival of the 227 patients in the overall study (7.4 months) and far better than the median survival of the patients with metastasis to the anterior segment of the eye (only 5.4 months).
Cancer 1976 Sep
PMID:Carcinoma metastatic to the eye and orbit III. A clinicopathologic study of 28 cases metastatic to the orbit. 95 71


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