SEVERE CHOREA GRAVIDARUM TREATED WITH CORTICOTROPHIN

874 A

four-day

fat-balance showed that, of

a daily intake of (13%) was excreted. Vitaiinin-Dl2 absorpstudied by Dr. C. C. Booth, using a preparation

82 g. of fat, 10-5

g.

tion was labelled with radioactive cobalt. The

absorption was initially improved by giving the vitamin with intrinsic factor (as it is in addisonian pernicious ansemia). After a four-day course of aureomycin (chlortetracycline) 250 mg. six-hourly, however, absorption increased to normal. During the initial period of observation the bowels were opened on an average 3 or 4 times daily. Initially the motions

slight

and was not

a light diet little fat they became semi-formed. When the special diet for the fat-balance was given, the frequency increased to 7 a day. During the four-day course of aureomycin it decreased to 1 or 2 a day, and the patient complained of constipation. Just before discharge a seven-day course of aureomycin was given, the bowels being opened once daily during this time. .F’ollozv-2cp.-After her discharge the folic acid, which she had had regularly while in hospital, was stopped. She continued to have injections of vitamin B12 1 mg. at monthly intervals, together with symptomatic treatment for her gastrointestinal symptoms. Ten weeks later (March 5, 1956) the blood picture was normal (see table). She was well and leading a normal life. She was still having occasional abdominal pain and flatulence and passing one or two formed stools daily. In May, 1956, the vitamin-B12 injections were stopped.

were

watery, but after the administration of

containing

the diverticula, and that, when these are numerous, the combined effect is equivalent to a " blind-loop " of intestine, causing steatorrhcea, with consequent impairment of vitamin-B12 absorption and, hence, megaloblastic anaemia. Though this explanation is probably correct in its essentials, one may question the importance of the steatorrhcea in these cases, since it is apparently never more than slight. An alternative explanation is that the abnormal bacterial flora of the intestine destroys, or competes for, the antimegaloblastic substances (Davis and Brown 1953, occur

in

sufficiently

Girdwood 1956). The beneficial effects of broad-spectrum antibiotics. suggest that infection plays an important part in the pathogenesis of the anaemia. That they do not act merely by controlling the diarrhoea was shown by Halsted et al. (1956), who found (in a similar case) that administration of a sulphonamide-sulphafurazole (’ Gantrisin’)— stopped the diarrhoea but did not improve the vitamin-B12 absorption. It would have been of great interest to observe, in the present patient, whether

fat-absorption did in fact improve pari passu with absorption of the vitamin, but unfortunately it was impossible to make this observation. Summary

Discussion

Patients with jejunal diverticulosis often present with abdominal pain, vomiting, and a distended abdomen ; and Dick’s (1955) patient, like the one reported here, also had visible peristalsis. These findings suggest subacute intestinal obstruction, which diagnosis may appear to be supported by the finding of multiple fluid levels on barium-meal examination (Badenoch et al. 1955). It is important to recognise that these levels are due to stasis in the diverticula and do not represent obstruction in the lumen of the small intestine. On the other hand, the diverticula may not fill with barium (Taylor 1930), and then a radiological diagnosis is

A woman, aged 60, with multiple diverticula of the small intestine, developed steatorrhcea and megaloblastic anaemia. Vitamin-Bi2 absorption was impaired but became normal after a course of aureomycin.

impossible.

Badenoch, J., Bedford, P. D. (1954) Quart. J. Med. 23, 462. Evans, J. R. (1955) Ibid, 24, 321. Blachford, R. D., Dawson, D. W. (1956) Brit. med. J. i, 1407. Davis, L. J., Brown, A. (1953) Megaloblastic Anæmias. Oxford. Dick, A. P. (1955) Brit. med. J. i, 145. Friedlander, P. H., Gorvy, V. (1955) Ibid, ii, 809. Girdwood, R. H. (1956) Quart. J. Med. 25, 87. Halsted, J. A., Lewis, P. M., Gasster, M. (1956) Amer. J. Med. 20, 42. Krevans, J. R., Conley, C. L., Sachs, M. (1954) J. clin. Invest. 33, 949. Taylor, G. W. (1930) New Engl. J. Med. 202, 269.

colicky

The defect of

In the in the present case 87% of the intake was other published cases 82-88% was absorbed. This degree of malabsorption can only be detected by careful fat-balance studies. The motions are often watery and do not suggest steatorrhoea. It may well be that in some other published cases of diverticulosis and megaloblastic anæmia—e.g., Krevans et al. (1954) and Taylor (1930)-a defect of fat-absorption was also present but was not detected by examination of single specimens of faeces. Dick (1955) noted cessation of diarrhcea after a course of aureomycin in his patient, and this, of course, is quite the reverse of the effect on normal people. He suggested that there may also have been remission of the anaemia, but the evidence was inconclusive.* In the present case the absorption of vitamin B12 became normal A similar after the administration of aureomycin. observation was made by Badenoch et al. (1955) in two of their four cases and by Krevans et al. (1954) and Halsted et al. (1956) in patients who had diverticulosis and megaloblastic anaemia without proved steatorrhoea. It will be of interest to see whether the anaemia can be kept permanently in remission merely by controlling intestinal function by intermittent courses of broad-spectrum " antibiotics, without the need for parenteral administration of vitamin B12. Badenoch et al. (1955) discuss the reasons for believing that the association of jejunal diverticulosis, steatorrhoea, and megaloblastic anaemia is not fortuitous. They suggest that stagnation and bacterial growth

fat-absorption

is not

great. absorbed ;

"

* Since this was written a case has been published (Blachford and Dawson 1956) in which a definite haemopoietie response was obtained after the administration of aureomycin and before the exhibition of any haematinic.

I wish to thank Dr. F. H. Scadding for permission to report this case ; Dr. C. C. Booth and Dr. D. L. Mollin, of the Postgraduate Medical School, for studying the vitamin-B12 absorption ; Dr. J. W. Stewart for help with the investigations and in the preparation of this paper ; and Sister M. Hammond and her staff for their cooperation in carrying out balancestudies for several weeks. REFERENCES -

-

SEVERE CHOREA GRAVIDARUM TREATED WITH CORTICOTROPHIN

A. D. FORREST M.B. St. And., M.R.C.P., D.P.M. LATE MEDICAL REGISTRAR GENERAL

I. B. HALES Sydney, M.R.C.P.,

M.B.

M.R.C.P.E. MEDICAL REGISTRAR HOSPITAL,

LONDON

CHOREA gravidarum, which is generally considered to a rheumatic manifestation identical with Sydenham’g chorea, is a rare complication of pregnancy, with a reported incidence of from 1 : 2200 to 1 : 3500 (Willson and Preece 1932). Although plenty of cases of chorea. gravidarum have been published; we found only on6 report of its treatment with cortisone (Schwarz and de Saint-Victor 1952) and none with corticotrophin.

be

Case-report A married primigravida, aged 25, had had no previous rheumatic manifestations. On Aug. 24, 1955, she noted restlessness followed by twitching of her left arm. During the next two days these movements spread to her other limbs. On Aug. 27, the fourth day of her illness, she developed abdominal pain and was admitted to hospital. On examination she was well nourished but showed generalised choreiform movements and grimacing ; her

875

temperature was 99°F, and pulse-rate 90. Her uterus was just and she had some tenderness palpable through her abdomen, in her right iliac fossa. There were no signs of involvement of the cardiovascular system or of arthritis. Her erythrocytesedimentation rate was 40 mm. in the first hour (Westergren). Progress.—During the thirty-six hours after admission the chorea became more severe and she became maniacal, threshing continuously about in a padded cot, incontinent, screaming intermittently, quite out of touch with her surroundings, and unable to swallow. This deterioration had taken place in spite of heavy sedation with phenobarbitone, paraldehyde, and carbromal together with salicylates and nursing in a single darkened room. In view of her inability to swallow she was fed by intravenous drip and sedation was increased, hyoscine and ‘ Omnopon ’ being the most effective drugs. However, it was only a dosage that depressed respiration to a dangerous degree that adequately controlled the movements. Corticotrophin Therapy.-On Aug. 30, the seventh day of her illness, the patient’s condition had become so critical that it was thought that the pregnancy should be ended ; but this was put off while treatment with corticotrophin 120 units daily was started, sedation being continued. Next day there was considerable improvement in spite of reduced sedation, and by Sept. 3, 1955, the patient was eating normally and intravenous fluids were discontinued. Corticotrophin dosage was now reduced to 80 units daily. Meanwhile the choreiform movements were diminishing in force and amplitude and finally disappeared on Oct. 7, 1955. By this time the dosage of corticotrophin had been reduced to 20 units daily, and corticotrophin therapy was discontinued on Oct. 21, 1955.

trial, particularly in the severe proin which the pregnancy might otherwise The evidence whether these have to be terminated. drugs may prevent permanent cardiac involvement is inconclusive. In the case of Schwarz and de Saint-Victor (1952) it seems probable that permanent damage occurred. In our case there is no evidence of such involvement, but admittedly it is too early to rule out this possibility. In neither case was any ill effect of either corticotrophin or cortisone noted on the foetus. to deserve further

gressive

cases

Summary A

severe case

the patient was delivered of a healthy male child weighing 7 lb. 10 oz. Re-examination on June 5 revealed no evidence of involvement of the cardiovascular system, and there were no persisting signs in the The baby was continuing to thrive. nervous system.

Follow-up.-On April 3, 1956,

Discussion

According to Willson and Preece (1932) there is a high maternal mortality, 33% of cases in which delivery The foetal mortality was artificial and 18% of all cases. was 50% ; but, if cases terminating before viability were excluded, the mortality was only 20%. Beresford and Graham (1950) disagree with these figures and report a maternal mortality as low as 1-4% with a foetal mortality of 33%. It seems that either the condition has become less severe over the intervening years or that treatment has improved. Pregnancy was terminated artificially in 189% of the earlier series but in less than 5% of the later series. These figures may merely indicate that the severity of the condition has diminished over the years, but they also suggest that there is an increased maternal mortality when pregnancy is artificially terminated. It therefore seems advisable to avoid this procedure whenever possible in the presence of chorea. Is there reason to believe that either corticotrophin or cortisone can further decrease the mortality-rate ?’? t These drugs are known to be of value in the treatment of rheumatic fever. The results in Sydenham’s chorea are not very satisfactory (Dixon and Bywaters 1952), and there is no real reason to think that they would be more effective in chorea gravidarum. However, chorea in pregnancy is much more severe than the chorea of childhood; consequently improvement may be more dramatic and more easily assessed. In the case reported by Schwarz and de Saint-Victor (1952) improvement began between twenty-four and forty-eight hours after the start of cortisone therapy, and the choreiform movements had ceased five days after treatment had begun. The verity of their case seems very similar to that of the present case with improvement occurring at about the same time. We feel that the dramatic improvement in the present case was probably attributable to the torticotrophin, but a spontaneous remission cannot be ;

r

:

t

excluded. Nevertheless this method of treatment

seems

gravidarum

was

treated with

corticotrophin. The successful outcome was probably due to the corticotrophin. Corticotrophin therapy is worthy of further trial, particularly when termination of pregnancy may be the alternative. Our thanks are due to Dr. T. A. Kemp and Mr. J. P. Erskine for their advice and permission to publish. REFERENCES

Beresford, O. D., Graham, A. M. (1950) J. Obstet. Gynœc., Brit. Emp. 57, 616. Dixon, A. St.J., Bywaters, E. G. L. (1952) Arch. Dis. Childh. 27, 161. Schwarz, H., do Saint-Victor, H. (1952) Canad. med. Ass. J. 66, 583. Willson, P., Preece, A. A. (1932) Arch. intern. Med. 49, 471, 671.

There was no recurrence of the choreiform movements, and the patient was discharged from hospital on Nov. 7, 1955. The only complication of the treatment was a moderate degree

of "moon-face."

of chorea

Reviews of Books Pott’s Paraplegia D. LL. GRIFFITHS, F.R.C.S., Manchester ; H. J. SEDDON, D.M., F.R.C.S., London; R. ROAF, M.CH:. ORTH., F.R.C.S., Liverpool. London : Oxford University Press. 1956. Pp. 129. 50s. No comparable work on this subject has appeared since the classical papers by Butler and Seddon in 1935. Here is an enemy with which surgeons have been in intimate and largely impotent contact for many decades. Only recently have methods of attack been developed which are commonly successful. This book crystallises what the authors have been teaching for several years past : here they give us the fruit of their combined experience, which far exceeds that of any individual in this country. They have shown that in all but exceptional cases it is compression alone that matters ; they are impressed by the high proportion of cases in which this compression was due to solid matter, and it was natural that their thoughts and efforts should be directed towards the best means of removing the compressing element. They review in detail the clinical features and pathology of Pott’s paraplegia, the indications for operation, and the results of the various procedures that have been tried. They do not decry conservative treatment but lay down tentative rules for when such treatment should be abandoned. Operatively their work is a development of the lead given by Capener, Dott, and Alexander. They are concerned to place the procedure of anterolateral decompression in perspective. This is a serious operation which should be confined to the hands of experienced spinal surgeons ; but the results warrant the risks. While there remain indications for costotransversectomy, and occasionally for laminectomy, it is evident that only anterolateral decompression can possibly relieve many of the patients, and this book gives expert guidance on whether, when, and how to do it. As an aid to that most difficult art-surgical judgmentit could scarcely be bettered.

For a short book this is worth the money.

highly priced,

of the

Alimentary

AsTLEY,

M.B., D.M.R.,

Radiology

but it is well

Tract in

Infancy radiologist, Children’s Hospital, Birmingham ; clinical lecturer in radiology, University of Birmingham. London : Edward Arnold. 1956. Pp. 287. 50s. Roy

IN recent years the value of radiological examination of the alimentary tract in infancy has been fully appreciated, owing largely to the work of Dr. Astley, who in this admirable book fills a gap in the radiological

_