EPILEPSY AND THE DRIVER

103

In

England

Now

Letters

to

the Editor

Running Commentary by Peripatetic Correspondents I FIND doing a locum is the best kind of refresher course, for you can pick up so many useful tips. No doctor’s surgery is quite the same as any other’s but they are almost all alike in their untidiness. The first surgery in strange surroundings is for all the world like a game of hunt the slipper " with astonished patients watching spellbound as the new doctor crawls about on hands and knees looking for E.c.l0s, maternity records, or private certificates. At last, after a cup of extra strong coffee provided by a sympathetic receptionist and armed with your absent colleague’s medical bag, you set off, map in hand, to find Dr. X’s patients. It is then that the real difficulties begin. Whole new housing estates have sprung up since the doctor’s map was made. Access by the obvious route is impossible now because of a new motorway across the town. Or you arrive at length at the patient’s house, this time after a long walk across fields, to be greeted by " It’s his ear, Doctor," and you find that the worthy Dr. X for some unaccountable There are, reason never includes an auriscope in his bag. however, compensations. I never emerge from a locum without an enhanced affection for that amalgam of science, human kindness, and intuition called general practice. Furthermore, A

SPONTANEOUS DESCENT OF THE TESTICLE SIR,-The valuable article by Mr. Cour-Palais1 raises the important question of how to get anatomy of the age of Henry Gray altered in the formal textbooks-whence the alteration might gradually spread to those of surgical anatomy, and " to finally clinics and operating-theatres. This process is proceeding in regard to my work on the tonsil,2 but I managed to start it only through a personal connection with an eminent and generous professor of anatomy. What may be called " the testicular constant " of 70% has remained unaltered for over quarter of a century. Mr. CourPalais agrees with me in finding this the proportion of normal retractile testicles among those diagnosed as undescended by the profession at large-it is the rate of cure announced by enthusiasts for hormone treatment, and also the rate of spontaneous descent without any treatment at all. My various publications3 on the subject have encountered the abuse and disbelief normal for new ideas in our country and profession; but neither anatomist nor endocrinologist has ever accepted my invitation to see in the operating-theatre what the situation really is. Is it not possible to get the question considered by anatomists when you return home you are more than ever convinced of as a body even if they have to announce: " Nous avons change the superiority of your own methods and grateful for once that tout cela " ? DENIS BROWNE. you know your own district like the palm of your hand. Even London W.1. your chronic neurotics seem remarkably easy to deal with now that you have seen Dr. X’s, and it’s a positive delight to know where everything is, even the death certificates, so unaccountably absent from Dr. X’s drawers. When challenged all COMFORTING CHILDREN BY TELEPHONE Dr. X could say about this was, " Oh, I really didn’t think SIR,-Dr. Rosen’s letter4 rightly draws attention to the you’d need those while I was away "-a subtle compliment need for continued communication between mother and child perhaps. when mother is in hospital; I am sure that Dr. Rosen, however, * * * would not disagree with the substitution of the word parent The morning started for us at 10 o’clock with liqueur for mother in this context. I fully agree with Dr. Rosen about brandy. Black coffee followed with little sweet and savoury the ill-effects that can follow on the insecurity felt by a child biscuits, while the deputy Minister of Health explained about when a parent is in hospital, but the reverse process is of his country’s Health Service. He was determined to exercise course equally true and probably better publicised-i.e., his English, and only when he repeated that " the infant separation of child from mother when the child goes into mortality is sufficiently high " (Darwinian cheers ?) did our hospital. interpreter try to interpose. In fact, throughout our trip she For the past five years, since this unit was opened, we have was almost superfluous, and it was I who had to explain to her encouraged parents to telephone children in the ward whensome of the scientific terms being bandied about. (I also had ever for one reason or another they are unable to visit regularly, to compile a list of her country’s rare birds which I am sure and this is usually done early in the evening before the children will rebound at future visitors when monuments, battles, folkgo to bed. This scheme has proved most successful, and lore, and economic development have been exhausted.) while it is relatively easy to accomplish in a ward environment In the end it was the mice which drove her from the where patients are normally up and about all the time I feel laboratories to the sunshine outside. " Ours is only smell that an extension of the system to paediatric wards would not laboratory but we have many enthusiasm ", I was told as we prove too difficult. balanced a plate of cheese-straws on a cage of mice. Department of Child and Family Psychiatry, Enthusiasm, indeed, was the keynote. In one town of not much G. L. DAVIES. St. Luke’s Hospital, Middlesbrough. more than 100,000 inhabitants, whose steeples and minarets rise from a hillside of peach trees and pines, the first assistant had 600 splenic punctures. " Do the patients ever just completed " object ? we wondered. " The question has not arisen." This EPILEPSY AND THE DRIVER same small town has a ballet company which tours other parts SIR,-Your annotationdraws attention to a world-wide of Europe with its modern repertoire. In one typical producproblem. You point out that even among sympathetically tion we saw a series of miscellaneous characters who threw disposed doctors there is no unanimity on this difficult problem. a symbolic trumpet to each other around the stage, but what To my mind the crux of the matter is that the medical prothis signified not even our interpreter could say. However, at fession should strive to have epileptics regarded as individuals,6 least it gave us the feeling that we had somehow Exchanged each considered on his merits. some Culture. A * * 1(0 parallel may be drawn with the consumption of alcohol and driving. It is not an offence to drive after consuming The old chap in room twelve was not too well; he was paying alcohol, but it becomes an offence if there is interference with the

price of the usual 20 a day for 40 years. It had been a longish day and I just popped in to see him after I had finished my clinic and letters. It was the visiting hour, and his wife was sitting beside him. His voice was weak and husky, and I had tc over him to hear his answer when I asked him how he His wife looked up and said He says his chest’s sore: Doctor but he didn’t, he said "What’s the Test score

stoop was.

Doctor

one’s

driving ability. By

the

same

token,

no

epileptic

whose

Cour-Palais, I. J. Lancet, 1966, i, 1403. Browne, D. J. Anat. 1928, 63, 82. Browne, D. Br. med. J. 1938, ii, 168; ibid. 1957, i, 1163; Lancet, 1933, i, 460; in British Encyclopædia of Medical Practice: vol. XII; p. 34. London, 1952; Proc. R. Soc. Med. 1936, 29, 1409. 4. Rosen, I. Lancet, 1965, i, 1426. 5. ibid. 1966, i, 1143. 6. Bird, A. V. Med. Proc. 1966, 12, 248.

1. 2. 3.

104 fits would make driving dangerous should be permitted to obtain a driving licence. Although rationally this would apply to every epileptic, in practice a significant proportion would be permitted to obtain a driving licence. The onus on the doctor would be not only that of establishing the diagnosis of epilepsy, but also of assessing which cases could be reasonably expected to drive with safety. The final decision whether or not a licence could be issued should rest with the licensing

authority. Department of Neurology, University of Witwatersrand, Johannesburg General Hospital, South Africa.

A. V. BIRD.

CHROMOSOMES IN KOSTMANN’S DISEASE

SIR,-We report here a 37-day-old female with infantile genetic agranulocytosis (Kostmann’s disease 1) who showed chromosomal abnormalities which to the best of our knowledge have

not

Our patient had all the features of fatal infantile genetic agranulocytosis. Moreover, the familial character of the disease is corroborated by the death of three siblings in infancy. The chromosomal aberrations were similar to those in megaloblastic ana:mia.56 Indeed, the appearances of the bone-marrow with bizarre giant cells (some of which showed megaloblastoid changes) were reminiscent of the features of megaloblastic marrows. Perhaps infantile genetic agranulocytosis reflects a deficiency of an element essential to growth. In this connection Bjure et al.,7 in a study of familial neutropenia, found that transfusion of fresh plasma was followed by a significant rise in circulating neutrophils, suggesting that in this condition some extracellular factor which is indispensable for normal myelopoiesis may be deficient. N. MATSANIOTIS Department of Pædiatrics, K. A. KIOSSOGLOU University of Athens, J. KARPOUZAS St. Sophie’s Children’s Hospital, K. ANASTASEA-VLACHOU. Goudi, Athens 609, Greece.

been described before.

The baby was admitted to this department on Sept. 11, 1965, with manifestations of sepsis, temperature of 38°C, and cough. She was the product of a normal full-term gestation. Three other siblings, two females and one male, had died at 7 months, and 17 and 55 days, respectively, with clinical pictures similar to that of the propositus. Her father’s age was 39 and her mother’s 38 years; there was no

consanguinity. The patient was pale and had ulcers on the tongue, oral moniliasis, and ecchymotic areas over the abdomen. The liver was palpable 4 cm. below the right costal margin. The spleen was just palpable. There was no lymphadenopathy. The blood findings were: haemoglobin 5-7 g. per 100 ml., haematocrit 16-5%, platelets 10,000 per c.mm., white blood-cells 6700 per c.mm. (neutrophils 2%, eosinophils 3%, and lymphocytes 95%), and erythrocyte-sedimentation rate 100 mm. in the lst hour. The bone-marrow was moderately hypercellular with a shift to the left of the granulocytes and maturation arrest of the granulocytic elements at the stage of promyelocytes and myelocytes, and showed giant, bizarre cells, exhibiting abnormal lobulation of the nucleus and cytoplasmic vacuolisation. There was some degree of eosinophilia and monocytosis, but mature forms such as band or segmented cells were conspicuously absent. A direct bone-marrow chromosomal study was carried out according to the method jointly described by one ofus,2 and using the combined Denver3 and Patau 4 system of nomenclature, on a preparation made on Sept. 25. Twenty-five metaphase plates were counted and

KARYOTYPE OF TREATED THROMBOCYTHÆMIA SIR,-We here present apparently the first case of reversion to normal of an abnormal marrow karyotype, in an adult with a

progressive myeloproliferative disorder other than leukaemia, following treatment, and coincident with clinical improvement. A 57-year-old Negro housewife was first seen in February, 1964, because of a peptic ulcer.8 Physical examination was negative. Hxmoglobin 10-4 g.; haematocrit 34%; total-leucocyte count 10,100, with varied from 667,000

phosphatase Sternal

a

normal differential. Platelet-counts

1,375,000. Leucocyte-alkalineranged from 39 to 104 (normal, 15-50). was of normal cellularity, but with abundant to

scores

marrow

SUMMARY OF CHROMOSOME STUDIES

analysed on photomicrographs. The number of chromosomes ranged

Bone-marrow metaphase plate containing 47 chromosomes, showing " giant" chromosomes, and numerous structural aberrations (arrowed); the supernumerary chromosome is an acentric fragment

(curved arrow). (Reduced by half from x 5000.)

from 38 to 47. The predominant cell line (in eighteen cells) had 46 chromosomes. A second hypodiploid line with 45 chromosomes was present in three cells. Occasional cells with 38, 42, and 47 chromosomes were found. One cell was totally disintegrated. " " Giant chromosomes were observed in one half of the metaphase plates. Seven cells were karyotyped: three with 46, one with 47, and three with 45 chromosomes. All karyotyped cells with 46 chromosomes had a normal female pattern. In the hypodiploid cells containing 45 chromosomes the loss was random, affecting the C(6-12 in-

cluding X), D(13-15) and F(1920) series. The extra chromosome in the hyperdiploid cell was an acentric fragment (see accompanying figure). Numerous structural aberrations,

such as chromatid breaks and gaps, and dicentric chromosomes were demonstrated in many of the analysed cells. Blurred and hazy chromosomes were seen occasionally. Details on the clinical picture, laboratory data, and treatment will be presented elsewhere. 1. 2. 3. 4.

Kostmann, R. Acta pædiat., Stockh. 1956, 45, suppl. 105. Kiossoglou, K. A., Mitus, W. J., Dameshek, W. Am. J. clin Path. 1964, 41, 183. See Lancet, 1960, i, 1063. Patau, K. ibid. 1961, i, 933.

number of metaphases photographed and analysed in detail shown in parentheses. t A portion of this sample was incubated with 3H-thymidine for 6 hours. t Before this, patient received busulphan, and 32P therapy.

are

megakaryocytes and platelets. Busulphan (’ Myleran’)’) 2-16 mg. daily was started in April, 1965, but led to only a temporary fall in the platelet-count, and therefore on June 7, the patient was given 32P, 3-0 mC intravenously, which has resulted in a more prolonged fall in the count. The results of chromosome studies using direct marrowe and 72-hour peripheral-blood-cultures are summarised in the accompanying table. Most of the metaphases in the marrow specimens before therapy contained one cell-line with a normal karyotype, and 25%of the metaphases in the first sample had a second cell-line with 48 chromosomes including 2 extra C-group chromosomes (see accompanying figure). Metaphases with 47 chromosomes appeared to be broken hyperdiploid cells. No hyperdiploid cells were found in the first sample after therapy. One metaphase containing 47 chromosomes, including an extra C chromosome, was present in the most 5. 6. 7.

Kiossoglou, K. A., Mitus, W. J. Clin. Res. 1964, 12, 217. Kiossoglou, K. A., Mitus, W. J., Dameshek, W. Blood, 1965, 25, 662. Bjure, J., Nilsson, D. R., Plum, C. M. Acta pædiat., Stockh. 1962, 51,

8.

Rowley, J. D., Blaisdell, R. K., Jacobson, L. O. Blood (in the press). This patient is case three.

497.