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CARDIOPATHY IN FRIEDREICH'S ATAXIA (SPINAL FORM OF HEREDITARY SCLEROSIS)Review of Literature and Analysis of Cases of Five Siblings
THOMAS H. LORENZ, M.D.;
CHESTER M. KURTZ, M.D.;
HERMAN H. SHAPIRO, M.D.
Arch Intern Med. 1950;86(3):412-426.
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IT IS REMARKABLE that up to 1948 there had been no publication by American investigators on any aspect of the cardiopathology of Friedreich's ataxia (spinal form of hereditary sclerosis). At this time a report by Ellwood appeared in which he described 2 patients having Friedreich's disease, one of whom died seemingly of coronary occlusion and the other in congestive failure. Recently, a second report has appeared (Hejtmancik and others) in which descriptions of 2 patients with Friedreich's disease are presented, both having striking electrocardiographic changes and apical diastolic murmurs.
REVIEW OF LITERATURE
In Friedreich's original report (1863 a) of 6 patients, one had experienced palpitation, dyspnea and paroxysmal auricular tachycardia, while 2 others had complained of frequent palpitation. On physical examination 5 were reported as showing no cardiac abnormality, while 1 had cardiomegaly and an apical systolic murmur. Postmortem examination of this patient revealed hypertrophy of the left and right
. . . [Full Text PDF of this Article]
Author Affiliations
MADISON, WIS.
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