the Ætiology of mongolism
TRANSCRIPT
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THE ÆTIOLOGY OF MONGOLISM.
ABOUT a year ago the Section of Psychiatry of theRoyal Society of Medicine met to discuss the problemof the Mongol,1 but their combined efforts threwno light whatever on the cause of the phenomenonnor even. on its true nature. The picture is much toocommon and well-defined to be the accidental resultof a number of separate causes, and it is a reproachto pathological science that the explanation shouldstill- be unknown. Dr. A. B. Marfan, in a compre-hensive summary published in the Presge Médicaleof Nov. 3rd, deals with the three theories whichcommand the most attention. The first is that of W. J.Langdon-Down, who has suggested that Mongolismis an anomaly of reversion to a remote ancestral type.The standing argument against this view is that, if:Mongols are really throw-backs to Mongolian ancestors,they ought to be physically and mentally as fit asnormal Chinese or Japanese, whereas, in fact, everyone of their characteristics marks them as " half-baked." F. G. Crookshank points out a number offeatures which he says are characteristic of theMongolian race and the Mongolian imbecile alike-the short, thick-set limbs; the tendency to a relativediminution in the size of the arms and thighs, as inachondroplasia ; the tendency of the two lowercutaneous folds of the palms to join into one, as incertain monkeys ; the fissured tongue ; and manyothers. These may be characteristic of the’ Mon-golian race, says Dr. Marfan, but they are certainlynot uniformly characteristic of the Mongolian imbecile ;.some of them, moreover, are not racial characteristicsbut pathological stigmata. The second theory,expounded in this country by G. E. Shuttleworth.and in France by Jules Comby, sees the cause in a kindof accidental impairment of the reproductive cells,a result of debility in either parent, but especiallyin the mother. Thus the possible causes of thecondition are legion ; any intoxication, infection, orinjury suffered during gestation may be responsiblefor the change. The exponents of the theory, however,lay particular stress on the importance of unregulatedemotion and repeated pregnancy in the mother.Mongols are generally, they say, the children ofwomen who have experienced severe mental pain,fear, or disappointment during pregnancy, or whohave been exhausted by many births. The chiefobjection to this opinion is, again, that it does notaltogether fit the facts. Such causes may oftenpredispose to abnormality, but it is difficult to regardthem as essentials, for innumerable women who arethe slaves of their emotions do not produce Mongols,and not a few Mongols are the offspring of motherswhose habits and environment are irreproachable.The third theory is that Mongolism is due to congenitalsyphilis. Those who support it advance the argu-ments that careful scrutiny of the family history andexamination of the patient will show the existence ofthe infection or its stigmata in half the cases. Inmost of the other half they find good presumptiveevidence that a syphilitic taint has been inheritedfrom an ancestor two generations back.
Protagonists of the various theories of Mongolismmay take courage from the undoubted fact that allthe predisposing causes have been met with in some,cases. It is, indeed, very probable that in manyinstances more than one predisposing condition ispresent. The last two children of a syphilitic motherknown to us are Mongols, though the other childrenare normal, one being sufficiently bright to hold ascholarship. All are Wassermann-negative. Themother’s infection is of long duration, and before thelast two births she was worn out by poverty, worry,and repeated childbirth, while during the earlierpregnancy she underwent an emergency operation fortwisted ovarian cvst. Which of the three factors-endocrine disturbance, exhaustion, or syphilitic taint-is to be held responsible in this case ? Dr. Marfan,who himself inclines to the syphilis theory, points out- that a number of signs exhibited by many Mongols in
1 THE LANCET, 1926, i., 190.
the first six months of life, the so-called scrotal tongueand malformations of the heart and adenoids for
example, are strongly indicative of hereditary syphilis.This view is supported by the work of Babonneix, whostates that post-mortem examination of a Mongol’sbrain shows lesions of the meninges and vessels thatstrongly suggest syphilis. As these lesions occur
before birth and are responsible for the corticalhypoplasia that is an invariable feature of Mongolism,it is not to be expected that specific treatment wouldhave much effect on them. The hypothesis thatMongolism can be due to syphilis in a more or lessremote ancestor, as well as in a parent, is certainlyingenious. Taking into account the high incidenceof the disease in the eighteenth century, when sexualmorals were at a low ebb, it is argued that few familiesnowadays, however respectable, can claim that theirline of descent is absolutely free from it. The taintis doubtless modified and attenuated, says Dr. Marfan,but is nevertheless still capable of causing harm.Attacking the endocrine glands at the outset of theirgrowth, it inhibits their effleiency according to a
more or less regular plan-and the result is theMongol, whose all-round shortcomings are fairlyattributable, considering the pathological evidence,to this cause. M. Jansen’s theory of abnormal amniotictension is consistent with this supposition, but unfor-tunately, although autopsies generally show a defi-ciency in one or more glands, not only are differentglands affected in different cases but there is noregularity in the nature of the lesions.
OXYGEN ADMINISTRATION.
IN a paper read before the Therapeutic Sectionof the Royal Society of Medicine on Dec. 14thDr. R. Hilton compared the oxygen in the alveolarair after its administration by the following methods :1. Large glass funnel held 10 cm. away from the mouth.2. Large glass funnel held in close contact with thebridge of the nose and under the chin. 3. Largeglass funnel, the funnel being shaped to the face.4. Nasal catheter, with its tip in the nasopharynx,and with one hole near the end. 5. Face-mask pro-vided with inspiratory and expiratory valves, con-nected so as to supply oxygen only. Oxygen wasgiven at rates varying from one to nine litres a minuteby funnel or catheter. The figures were obtainedfrom a normal subject, for the measure of successof any method of giving oxygen for the relief ofanoxaemia must depend on estimations of the oxygensaturation of the arterial blood, and repeated arterialpunctures, although safe, are not desirable in a sickman. The first essential step in the carriage of
oxygen from the exterior to the blood can be examinedeasily by analysis of the alveolar air; the secondstep-namely, its further transference to the blood-cannot succeed if the first step fails. Dr. Hilton’sfindings appear to be definite and consistent. In thefirst place, they show convincingly that a glass funnelheld 10 cm. from the mouth has no effect whateveron the oxygen in the lungs, even wth high ratesof flow. This is a conclusion, as one of the speakersremarked in discussing the paper, at which manyclinicians have already arrived by their own obser-vations. When the funnel is held in close contactwith the face, however, even the lower rates of flowcause a definite rise to alveolar oxygen. Thus, theoriginal pressure while breathing air (100 mm.,i.e., 14 per cent.) was doubled with an oxygen flowof three litres per minute. With a flow of seven
and a half litres per minute 350 mm.-i.e., 50 percent.-was reached. The nasal catheter gave similarfigures at low rates, but even at three litres perminute caused discomfort, and above four was nottolerated. Evidence was given to show that even thelower rates of flow had a definite effect in anoxaemia.The Haldane mask and bag, and its modification byGilchrist and Davies, was found to be considerablymore efficient than the funnel method, while thehighest figures of all (over 90 per cent. oxygen illalveolar air) were obtained with the two-valved face-