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

Three cases of Kearns syndrome are reported. The neuro-ophthalmological signs were comparable with ophthalmoplegia, pigmentary retinal degeneration and polymorphic neuro-muscular and sensory deficits. The electrocardiological signs were observed 2-4 years after the onset of the condition; the cause of death in each case was related to complete heart block. The post-mortem findings in one of the cases were spongial degeneration of the central nervous system and a seemingly primitive degeneration of the Bundle of His and its branches.
Arch Mal Coeur Vaiss 1979 May
PMID:[Cardiological aspect of Kearns' syndrome. Apropos of 3 cases, with histopathological study of the conduction tissue in one of them]. 11 5

The authors report 3 cases with an association of progressive external ophthalmoplegia (OEP) and disordered intracardiac conduction. These cases, and the twenty or so similar ones reported in the literature, show that this association is important for two reasons:--there is a therapeutic importance in that the condition affects young patients, who are at risk from sudden death due to the conduction defect; for this reason electrocardiographic follow-up must be regular, and an intracavitary pacemaker must be introduced definitively at the least indication;--there is a physiopathological importance in that the effect of the myopathies on the myocardium is well known, but most information relates to the diffuse cardiomyopathies, and in only 10% of cases are there conduction defects. By contrast, the conductive tissue appears to be involved in all cases of OEP, while cardiac failure is rare. It seems likely, therefore, that cases of OEP have a pathogenesis different from that of the diffuse myopathies, whether or not these involve the external occular muscles.
Arch Mal Coeur Vaiss 1977 Aug
PMID:[Progressive external ophthalmoplegia and disorders of ventricular conduction. Apropos of 3 recent cases]. 40 68

A 21 year old patient was operated for bilateral ptosis and external ophthalmoplegia at 13 years of age. At this time there were no signs of retinitis pigmentosa or atrioventricular block, features of the Kearns and Sayre Syndrome (1958) which were detected five years later. His bundle recording showed an intrahisian block (1 degree proximal and a complete distal block) with a trifascicular block, the latter persisting alone during a brief return to sinus rhythm. This is one of the rare cases of the Kearns and Sayre Syndrome with documented His bundle recordings and the only reported case with intrahisian block. The patient also suffered from bilateral neural deafness. The patient's condition remains stable after implantation of an isotopic cardiac pacemaker and he now leads a normal life. A review of 52 previously published cases shows that this rare condition appears to be caused by a mitochondrial abnormality, which, for an unknown reason, affects only the neuromuscular and cardiac conduction systems. The prognosis is poor when swallowing and respiration are affected, but this does not occur in all cases. As cardiac conduction abnormalities are the other life-threatening complication, cardiac pacing has greatly improved the prognosis of these patients.
Arch Mal Coeur Vaiss 1983 Mar
PMID:[Auriculo-ventricular block in the Kearns-Sayre syndrome. Apropos of a case]. 640 30

Bickerstaff's brainstem encephalitis (BBE) is a rare diagnosis different from meningoencephalitis with ophthalmoplegia, ataxia and confusion. Less than 100 cases have been described in the literature. We described the fatal case of a 69-year-old man who presented a severe consciousness disorders, distal weakness in the four limbs, and the BBE triad a few days after his admission for pneumonia.
Med Mal Infect 2007 Apr
PMID:Bickerstaff's brainstem encephalitis: a case report. 1733 76