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THE PERONEAL TYPE OF MUSCULAR ATROPHY: WITH AN ACCOUNT OF A FAMILY GROUP OF CASES

1909; BMJ; Volume: 2; Issue: 2546 Linguagem: Inglês

10.1136/bmj.2.2546.1114

0959-8138

J. R. Halliday, A. J. Whiting,

Genetic Neurodegenerative Diseases

Aortic disease of the acquired type is not as rare in chil- dren as is commonly supposed, and some of the cases are no doubt, of congenital origin.Another point of clinical interest, a recognition of which will prevent falling into error, is that aortic disease in children is apt to be associated with a bruit which is heard loudest over the pulmonary area, and not over the aortic cartilage.Hypoplasia of the aorta is characterized by diminution in size of the aorta and arteries throughout the body, the walls of which are thin and elastic.A congenital origin has been claimed for this condition, because it is so commonly associated with anomalies iia the generative and circulatory systems.A congenital tendency to the dwarfing of the arterial system has been ascribed to tuberculosis in the parents, and it may be due to other states of bad health.Hypoplasia of the aorta is associated with a dwarfed heart in some instances, and in others with an enlarged heart confined to the left side or involving both ventricles.If the heart be small during childhood it hypertrophies at puberty.The period of danger in these cases appears to belong to adolescence, when heart failure is not unlikely to-occur, and this is more likely to arise under physical strain.Defects in the aurioulo-ventricular valves are very un- common.They are commonly combined with other cardiac abnorma,lities, uncomplicated lesions of the mitral and tricuspid valves being exceedingly rare.Of the two valves the tricuspid is more frequently attacked than the mitral, and of the lesions tricuspid atresia is the commonest.Tricu8tpid etenos8i in association with disease of the pulmonary valves is occasionally seen.Mitral atresia is very rare.Cases have been recordedby Rokitansky, Thergmin, and by Lawrence and Nabarro, and in each instance the condition has been associated with somatic abnormalities.A spscimen of congenital mitral steno8si was exhibited at the society for the Study of Disease in Children by Fisher in 1901.Of mitral insufficiency Cautley has afforded an example in a girl aged 22 months.The physical signs were those of mitral regurgitation, and another case in a boy, aged 10 months, has been quoted by Maude Abbott.Hypertrophy of the heart undoubtedly arises as an idiopathic disease of congenital origin.Simmons was the first to record a case of its existence at birth.He found the heart of a newly born child, who died during the labour, to be enlarged to twice its natural size, all other organs of the body being quite healthy.Prior to that Hauser had recorded a case in a baby, aged 11 months, in which the heart was enormously dilated, with hyper.trophied right and left ventricles.Virchow suggested that the hypertrophy might be congenitaL Cardiac displacements such as dextrocardia and meso- cardia are the only congenital malpositions that are of interest to the physician and therefore that need be reviewed.Dextrocardia, accompanied by transpositions of the viscera, is commonly viewed rather as a rare develop- mental freak than recognized, as should be the case, as a not very out-of-the-way clinical experience.Dextrocardia without associated transposition of the viscera is rare, and not only is it rare, but it must be view.d with suspicion, because the diagnosis of congenital dextrocardia has been made on some few occasions, and this opinion has nAt bEea confirmed by autopsy.Sometimes the position of the fetal heart in the centre of the thorax is maintained, giving rise to the condition known as mesocardia.The apex beat will then be felt.in the epigastrium, and the position of the organ will readily be demonstrated by the x rays.AT the eighty-flrst annual meeting of the West Riding Medical Charitable Society, of which Dr. Gibson, of Harrogate, is now President, it was reported that forty- eight grants, amounting in all to £1,680, had been made during the year, and that since the foundation of the charity the total sum disbursed in grants had been £46,295 lOs.Of those on the society's list of grantees four hxad died during the year, and five new applications had been received.Mr.